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Neurology Resident Handbook

2018-2019

Property of: _________________________________________

If found, please return to Department of Neurology

245 North 15th Street, NCB, 7th Floor, Suite 7102

Drexel/TennetGUH/WHC Passwords:
CernerAmalga/Medconnect Username: _________________________

Cerner Amalga/Medconnect Password: _________________________

Allscripts Centricity Username: _________________________

AllscriptsCentricity Password: _________________________

Email: http://owa.medstar.net username/password same as medconnect

VA Passwords
Windows Username: _________________________

Windows Password: _________________________

Cprs Access code: _________________________

Cprs Verify code: _________________________

Cprs Signature code: _________________________

PIN number ________________

CNMC Password
Windows Username: _________________________

Windows Password: _________________________

Tracks Username: _________________________

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 Tracks Password: _________________________

New-innov login: www.new-innov.com/login/login.aspx

Institution Name: Drexelmedstar

Username: _________________________

Password: _________________________

Sharepoint: sharepoint.drexelmed.edu/clinical/departments/dni/SitePages/Homepage.aspx

Username: __________________________

Password: __________________________

Medlink Neurology: www.medlink.com

Username: DrexelNeuro

Password: Neuro123

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 TABLE OF CONTENTS
GENERAL
Page
1. Resident/Fellow/Attending contact information
2. Staff/Drexel/Hospital Contact Information
3. Survival Guideon…………………………………………………………………………3
Georgetown Phone Numbers………………………………………………………………….…………………4
VA and WHC Phone Numbers……………..………………………………………................................5
Georgetown Hospital Survival Tips………………………………………………………………….……......6
VA Hospital Survival Guide…………………………………………………………………………………………7
Washington Hospital Center Survival Tips..……………………………………………………………...11
Children’s National Medical Center Guide……………………………..…………………………........12
TOPICS
10. WHEN TO CALL ATTENDINGS……………………………………………………….…6
11. Encephalopathy…………………………………………………….
……………………………………………….….714
12. Neuroradiology Quick Guide…………………………………………………815
13. Stroke evaluation, management, syndromes…………….…106
14. Diagnosis of
Aphasias…………………………………………………………………………………….….149
15. Antiepileptic Chart………………………………………………….1620
16. Management Status Epilepticus ……….……………..…….1822
17. Assessment and Management of Seizures………………253
18. Spinal Cord Compression………………………….…………....264
19. Guillain-Barre Syndrome/AICIDP/CICP…………..……….…………….275
20. Myasthenic crisis .…………………………….……….……………29
21. Mitochondrial Management……………………………………316
22. ALS………………………………………………………………………….39
23. Lumbar Puncture…………………………………………………….4128
24. CSF Interpretation.……………………………….….……………..4329
25. Elevated Intracranial Pressure………………………….………4430
26. Acute Headache Syndromes……………………….…..……..4531
27. Migraine Exacerbation in ED………………………………….4632
28. Headache Treatment Charts. ………………………………..4733
29. Headache Treatment in Pregnancy…………………………4934

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 30. Vertigo………………………………………………………..…........5135
31. Coma/Brain Death…………………………………………….......5336
32. Peripheral Neuroanatomy ……………………….…............5437
33. Peripheral Differentials………………………………………….5740
34. Neuro-Oncology/Paraneoplastic Syndromes………… ..6041
35. Parkinson’s Disease and Parkinson’s Plus Syndromes6242
36. EEG Rooms with Remote Access…………………………………115
37. Morning Report and Sign out...………………………………………..116

Physicians
Binning, Mandy M.D. 844-464-6387 609-306-3941 mbinning@gnineuro.org
Dougherty, Michelle MD 762-6915 401-741-7046 mld25@drexel.edu
Farmer, Jill D.O. 762-6915 908-208-1354 jmg477@drexel.edu
Gallo, Jennifer PhD 762-1869 jlg37@drexel.edu
Gliebus, Peter M.D. 762-8210 267-325-1145 gg65@drexel.edu
Hakma, Zakaria MD 844-464-6387 267-972-1174 zhakma@gnineuro.org
Hedayat, Hirad, MD 626-757-4757 hhedayat@gnineuro.org
Lester, Kathryn PsyD 267-971-3151 kvl26@drexel.edu
Liebman, Kenneth M.D. 844-464-6387 609-571-0869 kliebman@gnineuro.org
Lo Pinto-Khoury, Carla, M.D. 762-4513 267-886-3048 cfl35@drexel.edu
Pillai, Jyoti, M.D. 762-7925 484-410-1035 jap48@drexel.edu
Sirken, David H. DO dsirken@gnineuro.org
Shneidman, Paul S. MD pshneidman@gnineuro.org
Strenger, Scott MD 844-464-6387 sstrenger@gnineuro.org
Tufenkjian, Krikor MD 762-3434 216-645-0912 kt572@drexel.edu
Veznedaroglu, Erol MD 844-464-6387 609-571-0858 evez@gnineuro.org

Patient Office 762-6915

Bologa, Dolores (Dee) 762-5033 dab34@drexel.edu
Dolan, Marianna 762-5507 md45@drexel.edu
Hurley, Meghan (MA: Farmer, Gliebus) 762-5565 mch338@drexel.edu
Iannuzzi, Kathy 762-5449 kiannuzzi@gnineuro.org
Landolf, Kimberly (MA: Neurosurgery) 762-5428 Klandolf@gnineuro.org
Jackson, Dawn 762-5562 dkj22@drexel.edu
Johnson, Valerie (MA: Romano) 762-5568 vdl27@drexel.edu
Nast, Christine 762-5576 cmn24@drexel.edu

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Roubert, Lillian (MA: Dougherty, Pillai, 762-5536 lr566@drexel.edu
Lopinto)

Infusion Suite/Medicine Room

Meyer, Kristin RN 762-5594 kmm383@drexel.edu
Ruiz, Maria 762-5594 mr447@drexel.edu

Department of Neurology Drexel

Phone # Cell # E-mail
University College of Medicine

Administrative Office
Brown, Kristen (Admin: Farmer, 762-1949 kbb54@drexel.edu
Dougherty, Lester & Gallo)
Damico, Donald 215-962- ddamico@gnineuro.org
9600
Erwin, Kirsten 762-7684 kle27@drexel.edu
Myers, Nicole 762-4592 856-498- nm542@drexel.edyu
2898
Gilbert née Negron, Jennifer (Admin: 762-7783 jnn35@drexel.edu
Gliebus)
Norbutas, Kim (Admin: LoPinto, 762-1267 knorbutas@drexelmed.edu
Pillai, Tufenkjian)

Research
Maxwell, Christina PhD 762-1348 crm327@drexel.edu
Barr, Christine RN 762-3941 cpb25@drexel.edu
Mazurek, Monica RN 762-7420 mtm47@drexel.edu
Rife, Katherine (Katie) 762-7436 215-630- klr98@drexel.edu
9672
Webster, Melinda 762-7437 443-614- mjw377@drexel.edu
3972

Fellows

Daniel “Rocky” Felbaum, MD- 609-608- rfelbaum@gnineuro.org

Neurosurgery 8288

1427 Vine Street

Adorna, Theresa (Terri) 267-507- ta469@drexel.edu
6606
Cosico, Genoveva (Juvie) 762-5032 gac23@drexel.edu

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Foner, Rebecca 762-5175 rmf85@drexel.edu
Giron, Patricia 762-5413 prg42@drexel.edu
Smith, Theresa (Reese) 762-5593 tms388@drexel.edu
ers389@drexel.edu
EEG Scheduling -Debbie Dumas 762-7035 Deborah.dumas@tenethealth.net
EMG Appts 762-7035
Hahnemann Univ. Hosp. 762-7000 762-Page
EEG Reading Room 762-3440

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 Georgetown Neurology Residents/Fellows
Resident Pager Email Phone
PGY-4
Brian Ahuja 405-1961 brian.ahuja@gmail.com (419) 356-645
brian.x.ahuja@gunet.georgetown.edu
Scott Dellorso 405-5437 smdellorso@gmail.com (781) 738-000
scott.m.dellorso@gunet.georgetown.edu
Elana Neches 405-3414 elananeches@gmail.com (714) 345-222
elana.x.neches@gunet.georgetown.edu
Justin Salerian 405-5256 justinsalerian@gmail.com (240) 602-171
justin.a.salerian@gunet.georgetown.edu
Sara Siavoshi 405-1989 ssiavosh@gmail.com (714) 326-929
sara.s.siavoshi@gunet.georgetown.edu
Nathan Starr 405-2145 jnathanstarr@gmail.com (706) 346-221
jame.n.starr@gunet.georgetown.edu
Jaimee Ahmed sabrina820@hotmail.com (615) 397-204
PGY-3
Pearla Baten 405-1961 pearla.baten@gmail.com (570) 244-766
Amit Shah 405-3995 adshah3@gmail.com (678) 524-738
Elizabeth Mundel 405-3948 ere7@georgetown.edu (954) 610-154
Nadia Yusuf 405-4004 nadia87yusuf@gmail.com (240) 505-687
Tracy Fulton 405-2151 tracy.vu@gmail.com (703) 400-013
Nancy Hu 405-5260 nancynhu@gmail.com (301) 836-102
Alejandra Garland 405-5072 alejandra.garland@gmail.com (646) 527-564
PGY-2
Brian Barry bdbarry22@gmail.com
Glenn Burkett jburkett573@gmail.com
Louis Goodrich LJGoodrich@gmail.com
Pooja Rao pampspooja@yahoo.co.in (240) 630-956
Jerdan Ruff jerdanruff@gmail.com
Jessica Stevens JMS6388@gmail.com
Ryan Williamson raw10@med.fsu.edu
Peds Neuro Fellows
Sarah Schlatterer SSchlatt@childrensnational.org
Jeffrey Strelzik JStrelzi@childrensnational.org
EEG Fellow

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 EMG Fellow

MS Fellow

Movement Fellows

Abigail Keys 405-5264 abbey31@hotmail.com (419) 654-458

abigail.c.keys@gunet.georgetown.edu
Yasar Yaghi 405-5274 yasaryaghi@gmail.com (202) 415-769
yasar.torres-yaghi@gunet.georgetown.edu

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 Georgetown University Hospital (202) 444-2000
Number People Numbers Codes/Floors
m x4-3036 Ward pager 405-1110 Long distance code 1
om x4-1282 Stroke pager 405-3957 Bles 7 call rm code 4
x4-8525 Consult pager 405-1875 PHC 7 res rm code 1
Fax x4-4115 PHC 7 copier code 1
Neurorads reading x4-3358
x4-3356 Rads on call x4-6440 C61 x
x4-3383 EEG tech x4-9712 C62 x
x4-3444 EMU tech x4-0951 C63 x
x4-3652 EMG x4-1748 C41 x
x4-3466 C42 x
CM Debbie 40520 / 405-3096 C43 x
X4-3500 CM Vickie 43134 / 405-1603 Bles 2 x
x4-3770 SW Julia 405-1632 Bles 3 x
x4-3664 On-call SW pg 405-0405 Bles 4 x
x4-0643 Rehab NRH 668-2111 Bles 5 x
Main 43 x
x4-2600 Neurosurgery c/s 405-1414 ER x
x4-2111 C63 PA 405-2111
x4-7770 Medicine c/s 405-3955
Psychiatry c/s 405-1217

Please do not share attendings’ contact information with patients.

GUH Attending Pager Cell Home
Dr. Ailani 405-2758 631-807-8194
Dr. Amjad 405-1683 301-675-0014
Dr. Bell 405-0166 202-657-9386
Dr. Bahroo 405-1693 571-245-5999
Dr. Dougherty 405-5459 703-966-7619
Dr. Dromerick Cell: 202-536-6272 202-536-6272 202-333-1436
Dr. Gambhir 405-0895 703-862-4182
Dr. Healton 866-950-0431 202-441-2568 202-364-0378
Dr. Jaitly 405-2711 301-744-9985
Dr. Lo 405-2335 571-345-4101
Dr. Mandir 405-2581 410-419-3370 301-767-0015
Dr. Mayson 405-5721 617-999-7524

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 Dr. Minahan 405-2340 202-487-7723 301-570-3849
Dr. Mora 405-2571 240-888-4509 301-986-0801
Dr. Motamedi 405-2464 301-963-9526
Dr. Nayar 405-5051 301-351-5292
Dr. Osborne 405-2586 202-821-8890 240-450-0394
Dr. Pagan 405-2439 703-909-1929 703-723-3158
Dr. Sirdofsky 405-2341 703-569-8716
Dr. Stemer 405-5305 312-545-1129
Dr. Tornatore 405-2565 301-728-1990 301-864-6623
Dr. Turkeltaub 405-5454 703-609-7383

VA MEDICAL CENTER 202-745-8000

How to send pages VA pager: dial 48, wait for beep then dial four-
within the VA digit #
GW pager: dial 362, wait for beep then dial 7
digit #
If paging a VA 202-516-xxxx
pager from outside
VA

Dept Number Floors Number

uro Clinic x5-8535 / x5-7445 Res & Conf Rooms 3-2-1
Code
uro Clinic Fax 745-8231 2D supply outside 4-(13)
sident Rooms x5-5138/ x5-7119 Pyxis md101
ard pager 516-3172
nsult pager 516-0293 Nurses Station x5-6091
2D Neuro x5-7115/x5-7114
RI techs x5-8361/ x5-6424/x5-5818 2D Surgery x5-7306/x5-8182
Scan x5-6938/ x5-6940 2EN SICU x5-8291
ds Reading Room x5-5499 (H) / x5-7598 (K) 3D x5-7305/x5-7303
diology Dictations x5-8888/x5-8678 3E x5-7136/x5-7142
4BE MICU x5-8112/ x5-6018
cera x5-8899 4BW Telemetry x5-6984
arm inpatient x5-7385/ x5-7386 4N x5-7032/x5-7033
arm outpatient x5-8235/ x5-6431/ x5-5192 ER x5-8360/x5-8357

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boratory x5-7505/ x5-7493
T (stat labs) x5-5820/x5-5821 Echo x5-7030
Ultrasound x5-7591/x5-8361
ry Vrana x5-5809, p3570, or vocera Vascular Lab x5-8168
cial worker x5-6990 or vocera EEG x5-4146
D x5-8236
missions x5-7134 Baltimore NSGY Pager 410-389-0292
vel x5-8252 Walter Reed NSGY 8662954913/106015
NIH Stroke Team 202-801-7616

VA Attending Pager Home/Cell

Dr. Balish 516-3421 301-468-3431 (H)
301-704-1422 (C)
Dr. Kataria 516-3698 202-297-8685
Dr. Li 516-3938 301-518-3576 (C)
Dr. Murinson 516-3890 301-338-3301 (C)
Dr. Pfeiffer 516-0214 202-427-4009
‍
Dr. Sandbrink 516-3825 301-951-0307 (H)
x5-6528 240-838-0914 (C)
Dr. Wallin 516-3433 703-963-1103
Dr. Yaseen 516-3084 301-232-7619 (C)
301-493-7850 (H)
Dr. Zhai 516-3430 301-424-0558 (C)
301-825-5085 (H)
Dr. Zhao 516-3890 x5-8489
301-338-3301
WASHINGTON HOSPITAL CENTER
Main Number (202) 877-7000
Neuro pager 202-801-9697
Dr. Laureno 801-2785 (P)
202-669-8801 (C)
Dr. Lin 801-5097 (P)
202-744-7708 (C)
301-365-0710 (H)
Dr. Choi 801-0269 (P)
202-262-3952 (C)
Neuro Office x7-6435

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Neuro Clinic x7-0333
Neurorads reading x7-3139/x7-2893
ER/CT Rads Reading x7-3604
MRI office x7-5903
MRI technologist x7-3142
Neurosurgery pager 801-0009
Interventional Rad x7-6495, 801-6660
USA Mobility (888) 413-7243

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 Georgetown Survival Tips

Discharges
In MedConnect fill out “Discharge Order Set” and reconcile meds for discharge.
Fill out Amalga “Input” -> “Hospital Summary”, which must be printed for patients going
to a rehab facility but can otherwise be done after patient leaves.
Medical Records
Amalga deficiencies show up emedstar.net
Medconnect deficiencies show up as soon as you log in
You will get GUH emails when your deficiencies are pending suspension of parking
Home services:
Home health nurse/PT form needs to be signed by attending so do it during rounds
Lovenox requires insurance pre-authorization so let CM know asap
On call SW pager: 202-405-0405
Short Call:
Be sure to sign out to the on call person by 4PM, even if you still have a little work.
If you get a “non-urgent” consult and the consulter says “it can wait until AM” you should
still see the patient as a courtesy to your team. If you have a ton of other
admissions/consults, ask the consulter if they can call back in the AM to the consult
resident, and give the consult resident a heads up as a courtesy.
Weekend Call:
On Friday you should clarify with the on call attending when he/she will come in, (usually
8 or 8:30) and if they prefer pre-written notes as templates.
Come in around 7:30-7:45 to prepare notes with vitals and labs. You don’t have to see any
patients before rounds.
The attending will come into the conference room to hear about new patients from the
night float resident. Rounds will follow with the on call resident.
Attending will write all progress notes during rounds. Just have vital signs and pertinent
labs ready for attending.
Food options close at 8pm in the Leavey and at 10pm in Epicurean.
Parking: You can park for free in the Leavey center garage. Just take a ticket when you go
in and on your way out write your name and dept and hand it to the cashier.
Lumbar Puncture (See LP section)
Consent forms are found on Amalga under Forms -> Consents

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 Order all the tests you want in advance so the nurse can print out the test labels.
Ask the nurse for an LP kit and sterile gloves.
Do a time out before the procedure and fill out the time out form with the nurse.
Put patient labels on all 4 tubes (with initials and date/time) and put them in specimen
bag with the test labels.
If you are doing cytology, the sample should preferably be obtained before 4pm, and a
separate cytology form has to be filled out.
Take the tubes down to the Lab yourself—Main 1st floor, through Lab double doors
Write a procedure note and document opening pressure whenever possible.
Lync Lectures
In Firefox or Chrome go to http://sched.lync.com
Username: neurogeorgetown@neurogeorgetown.onmicrosoft.com
Password: neuron4!
Transfering a Patient to the ICU
Contact the NSICU PA and discuss the case with them
Also can contact CCAR (critical care admitting resident) for transfer
write the order “transfer level of care” in medconnect

Specific Events That Mandate A Call From The House Officer To The
Attending:
o When there is a discharge from EDithin one hour of admission (you may send a
text page to the attending)
o Unexpected Death
o Code
o Upgrade in the level of care (ie floor to ICU)
o Significant hemodynamic instability requiring intervention
o Unplanned invasive procedure, surgery or intubation
o
o New onset neurologic finding
o Medication or treatment errors that result in increased patient monitoring,
intervention or patient harm
o Patient contemplating leaving AMA or Dispo problems that are escalating
o Change in discharge planning
o Change in code status

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o Anytime a trainee is unsure of the next step or disagreement about management
among residents
o

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 Resident-to-Resident VA Survival Guide

GENERAL INFO:
● Signouts: “NEURO” drive -> “RESIDENT FILES”
● Didactics:
o Grand Rounds: Wednesdays at noon, conference room
o Brain cutting: Select Wednesdays at 1:30pm in the Path lab
o Neuroradiology conference: Wednesdays at 3pm in the Conference room
o Every Tuesday morning at 8am we have multidisciplinary rounds with PT, OT,
speech therapy, and social work in the conference room by the resident
office.
● Calling other services: VA home page -> on-call schedule

VA CONTINUITY CLINIC:
● Basic Duties:
o After patients check in, a nurse will put the vitals sheet in your door.
o Greet your patient in the waiting area and walk them to your room.
o Once done seeing patient, staff with your designated attending.
o Follow ups get 30 min for a visit, new patients get 1 hour visits.
o A few patients are still assigned to the “Brown” clinic so we are their primary
care physician. They frequently get reassigned to Internists if your attending is
ok with it.
o If you have to change your clinic dates for schedule/vacation changes, put the
requests in as early as possible. Fill out a vacation request form and give it to
Debbie. Also alert VA and Schedule chiefs.

HOW TO PUT IN AN ADMISSION:

● Consult notes from ED:
o New encounter: File -> Update Provider/Location -> New Visit -> Neuro/AEC
Consults-X
o Answer consult by starting new consult note and clicking Neurology, or:
Consult tab -> Action -> Consult Results -> Complete/Update Results
o If the pt gets admitted, you have to write both an H&P and a consult note.
You can just write “Please see H&P for x date for this consult note” or vice
versa.

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● Admission orders:
o “Orders” tab -> Add new orders (neuro)” -> “Admission orders”
o Include diet, activity level, and neurochecks q4hrs x 48hrs
o 2D Neuro doesn’t have tele but our pts can board on Medicine or PCU
o If pt isn’t assigned to bed location yet, write “delayed release orders.”
After the pt ‘s admission status is updated in the computer, go to the
“delayed release orders,” highlight them all, right click, and click on
release delayed orders.

HOW TO DIRECT ADMIT A PT

● Place consult as “Admission Referral Consult” and fill out the pertinent information
● Discuss with Mary Vrana or charge nurse on 2D. Ask about bed availability and tell
them about any contact isolation precautions or warnings (such as violent behavior).
● Let the ward team and chief know.

ENCOUNTERS:
● You must enter “visit type” and “diagnosis” for each completed visit.
● For daily inpatient notes you should create a new encounter:
File -> Update Provider/Location -> New Visit -> Neuro/Inpt Follow Up-X
● Do not fill out visit info for no shows or telephone calls, as they are for billing

MEDS AND FLUIDS:

● Outpatient narcotics: Fill out a green ‘controlled substances’ prescription slip. Include
the patient’s name, home address, and full SSN. Give to our Pharmacist Wendy or
ward nurse.
● Non-formulary meds: Put in a consult to pharmacy for nonformulary med inpt or
outpt.
● Insulin: Under “Sound Alike/Look Alike Drugs” with accuchecks and sliding scales.

LABS/TESTS:
● “Lab collect” is drawn by patient care techs (PCTs) at scheduled times from 4:30a to
5:30p.
● “Ward collect” is stat, drawn by you or by the on call PCT if alerted (Vocera “PCT”).
Orders should be printed to “BB94”.
● Daily labs must be ordered individually.

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● View raw images in Tools -> CPRS IMAGING (Windows 7).
● Echos and Dopplers (Vascular Lab) are ordered as consults and result as consult notes.

LPs:
● Consent can be done in CPRS if your computer has an electronic signature pad (see
below).
● Bring tubes down to the basement lab and fill out a CSF request form. If ordering
cytology, you must check off CJD box or they’ll never run it.
● If you want OCB, make sure you get a blood draw that day for serum OCBs.

DISCHARGES:
1. Transfer new/changed meds to outpatient, so pharmacy can have them ready
(“window”).
2. Write the “Neuro Discharge Instructions” under “Notes”.
3. Under “Orders” add a “Discharge pt” order.
4. Pts going to a facility will need a discharge summary printed (which can only be done
after it’s signed by the attending, unless you copy and paste to a Word doc). All pts
will need a discharge summary even if they leave AMA.
5. You can start discharge summaries during a patient’s admission and even transfer it
to another resident by changing its author.

SPECIAL DISCHARGES:
● Any pt that needs long term placement must get a GEC consult, which Mary can help
with.
● Soldier’s Home: A facility associated with the VA (and located across the street) that
has several levels of care: independent living, assisted living, nursing home, etc.
Patient’s must be transferred back on a weekday before 2PM.
● VA SAR 2H: If PT/OT recommends SAR and there is a bed available, Mary or Pam
Harmon (lady in charge of 2H) will let you know. The patient must be physically
transferred to 2H by noon or they won’t accept till the day after.

TRAVEL ARRANGEMENTS:
● Put in a travel consult under “Consults”. Call Travel x5-8252 to confirm ambulances.
● If a pt has to be transferred to Neurosurgery emergently, the AOD in front of the ER
will give you travel paperwork to fill out, and you have to put in a Travel Note.

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NEUROSURGERY TRANSFERS:
● Baltimore VA: resident pager is 410-389-0292. If before 4pm on M, W, Th, call Silvia
Ayers, neurosx PA at 410-605-7000 x5673. If they take more than 10 minutes to get
back to you or if taking more than 30 mins to let you know if they can accept, call
WRMC.
● Walter Reed Medical Center: 866-295-4913 is paging number, then enter the pin
number of whoever is on call (main one is 1060157 but you can check the call
schedule on the wall of the resident’s room to confirm). If you are getting resistance
from a resident, ask your chief resident or attending from the VA to page Dr Jonathan
Gilhooly 866-295-4913 then 1044932 or cell 202-782-4779.
● Georgetown: Neurosurgery pager is 202-405-1414
● University of Maryland Express Care: 410-328-1234
● WHC: Last resort and usually because a patient is actively herniating. ED usually
arranges this if patient is there.

HOW TO TRANSFER TO NEUROSURGERY:

1. Page neurosurgery resident and leave your cell number as a callback.
2. Start copying the relevant imaging onto Powerpoint to email to the neurosurgery
resident. The Baltimore VA can access the images before 4pm, but not the on call
residents.
3. Sometimes you can get a CD of the images send with patient if you call x5-8461.
4. Get the accepting attending physician and the floor/bed they’re assigned.
5. If before 4pm
a. Vocera Derwin Bryant or call DJ Richardon-Robins at x5-5902 to arrange.
b. Place “Travel-Day Only” consult
c. Give them the accepting attending physician and assigned floor/bed.
d. Dr. Pincus and Dr. Balish should sign the form.
e. The patient will also need to sign the form if he/she is able to, and if not the
next of kin or two physicians.
6. If after 4pm
a. Call the AOD at x5-8236 who acts as the transfer coordinator and give the
name of the accepting attending physician and assigned floor/bed.
b. Go to the front desk by ER and fill out the transfer paperwork from the AOD.

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 c. The patient will also need to sign the form if he/she is able to, and if not the
next of kin or two physicians.
7. If you think a new patient in the ED has a big bleed or SAH, keep them in the ED
because it is easier to transfer to outside facility from the ED.

HOW TO COPY IMAGES ONTO POWERPOINT:

1. Select the study in VistA and change the preset view to Head or Bone etc.
2. Go to a level on the image a little above or below the area of interest
3. Go to “File” -> “Copy” and follow the prompts, that you’re providing clinical care to
this pt.
4. Paste each image into a slide in Powerpoint. Keyboard shortcuts make this faster.

GETTING IMAGES COPIED TO A CD:

1. Try to do it before 4pm. During daytime hours you can go to the Medical Records
office near 4D or to a Rads reading room. After hours you’ll have to get a Rads tech to
make one.
2. You will need a “Release of Medical Information” form either way.
3. Often Powerpoints you already emailed will suffice.

ORDERING OPEN MRI:

● We have no open MRI here at VA, so a Consult must be placed for “Non VA Care
Radiology”
● You have to list the exact study, whether or not contrast should be given, and why it
cannot be done at the VA (such as claustrophobia failed MRI with sedation).
● CD will be given to patient but report is often lost in faxing. You can always call the
facility when the pt comes back to clinic and have the secretary read or fax the report.

PLACING CONSULTS:
● The VA home page has a link to the on-call schedule.
● Eye clinic consults: the patient has to go down to Ophtho clinic unless they are
intubated.

ACUTE STROKES:
1. 3-hr window at VA, get head CT (or if possible MRI) STAT, do NIH stroke scale note,
etc.

20
 2. Call attending/chief resident
3. Notify ICU because pt needs ICU bed
4. You don’t have to get consent because it’s standard of care, but you still should.
5. Get a repeat head CT within 8 hours.

OBTAINING CONSENT:
● For electronic consent (ie. For LPs) you have to use a laptop with an electronic pad.
Under “Tools” go to “IMED consent”. Alternatively you can use a paper form to get
scanned.
● For MRIs with gadolinum, the MRI techs usually fill out the consent from down at
MRI, but they will contact residents if the patient cannot consent for themselves.
● CT with IV contrast, residents get consent, and paper consents are at the nurses
stations.

CPRS TIPS:
1. Checking for consults on Vista: “ser” → “neu” → “2” (then “3” and “5”) → dates “t-
1” to “t” (or “t” and “t” for today’s list only). Consults from “DC/…” are usually
outpatient.
2. Creating your own templates: “Notes” tab → “Options” → “Create New Template”.
Autopopulate pmhx, vitals, meds, or labs by going to “Edit”→“Insert Patient Data
Object” → object you want to insert → “Insert Object” → “Done”
3. Highlight only Neuro notes: “View” → “Custom view” → Check the “Title” and
“Subject” boxes → in the “Contains” box type in “neuro” → “Save as Default View”
4. Changing alerts you get: “Tools” → “Options” → “Notifications”
5. Accessing data from other VA or DOD: Vistaweb or Remote Data button in upper
right. Check other VAs or DOD then → “Reports” → “Health Summary” for VA or
“Depart of Defense reports” for DOD

RTANT POINTS:
1. Check your clinic schedules often as mistakes can sometimes happen, which is bad
when you’re post call etc.
2. Work on discharge summaries as you go; they can be switched to a different author.
3. If you want to schedule an LP in urgent clinic, give the chief and PGY3s on service a
heads up. Make sure to order CBC and coags in advance.

21
 Resident-to-Resident Washington Hospital Center Survival Guide

● General Points:
o Fill out the consult template in its entirety.
o A fundoscopic exam is expected on all new consults.
o We usually consult only. Admits from ER go to IM A (resident) or B
(hospitalist).
o You are expected to take consults from 8am to 6pm during the week. The on
call resident will take pages from home between 6pm and 8am.
● Clinic:
o Two mornings a week (Monday and Thursday) the consult team sees patients
in the 1st floor in the Ambulatory Care Center, in addition to inpatient
consults.
o Your elective time at WHC will likely involve seeing clinic patients.
o If you have VA clinic on Mon or Thurs it will likely be moved to Tues morning.
● Didactics:
o Tuesdays: Radiology conference (1pm in the MRI reading room). The senior
resident should call Neurorads on Tuesday mornings to confirm at x7-3139.
o Wednesdays
▪ 8am: Journal conference (we present articles) in 6 th floor GME. The 4th
Wed of the month is a Stroke conference usually in the POB
▪ 9am: Brain cutting in autopsy suite
▪ 10am-12pm: Lumbar puncture clinic (sometimes)
o Fridays: 8am Case conference on 6th floor GME conference room
● EEGs: If you know an EEG has been done, there may be an official read present on the
blue/yellow folders on the desk in the office, or the secretary may be transcribing it.
Otherwise, look in Amalga under the “Dictations” tab and then “EEG.”
● Scheduling outpatient follow-up: Call 877-0333. The secretary will need patient’s
name, DOB, SSN, address, and type of insurance. This is all on the face sheet or in
Amalga’s “patient info” tab. Appointments can be double booked if cleared by the
attending.

WHC NEUROLOGY RESIDENT’S RESPONSIBILITIES

● Daily:

22
 o If you are presenting for conference, handouts must be ready at 8am
sharp.
o Print out a sign out for attending in AM, and update at the end of every
day.
o See any new consults called in by 6PM, as well as any active follow ups.
o Page on service attending as soon as each consult is completed.
● Weekend on call from home:
o Carry the main pager (9697) from Friday 6PM to Monday 8AM.
o You are expected to see any active/sick follow-ups and any new consults
o Arrange a time with attending to round, and take him to see all the
patients.
o After you leave, return for late consults unless it is for a chronic, stable
problem. Call attending about late consults that you have seen to discuss
each case.
o Try not to carry consults over to Mon as everyone will be in clinic in the
am.
o Dress like a professional. If you wear scrubs, don’t wear t-shirts. No jeans.

23
 Children’s Hospital on Call Survival Guide

Fellow Phones:
Ward x8260
ICU x8261

To Page: 202-259-XXXX
Or in house: 6600 --- XXXX # --- call back #
ASCOM phones: 202-476-XXXX
Long Distance Code - 9-1-xxx-xxxx then 7880 #

Hospital Operator: 202-476-5000

- physician access to operator: 202-476-4880

Inpatient Service Impt #s

- Neurology Resident p1363
- Resident phone x8265 Attending Pagers:
- Neurology Floor x5150 Acosta 0458
- Charge Nurse x8276 Avery 5686
- Derrick(mri/f/up appts) p1044 Carpenter 7017
- Emily G (case management) x8231, p4678 Chang 0170
Civitello 8268
ER: x5203 Conry 8269
Lifeline (transport team) x5433 (L-I-F-E) Depositario-Cabacar 0249
DiSabella 0855
PICU (3 east): Freilich 1233
- Main number x 2010 Gaillard 8272
- Fellow On Call x8038 Gropman 1612
- PICU residents – x8040, x8042, x8045, x8048 Kao 2188
Lateef 1354
NICU (6 east): Lavenstein 8417
- Main number x5040 McClintock 8418
- Fellow On Call x8744 Packer 8274
Pearl 8275
Admissions: x4068 (call in any direct admissions) Scafidi 1217
Tsuchida 0136
Vanderver 8878
Zelleke 4309 24
EEG:
- Downstairs (techs): x5651
- Downstairs Reading Room: x5455
- 5 east Reading Room: x7105
- Tech ASCOM phone: x8263
- Nakeeta (Video EEG scheduling) x5645

MRI:
- Nurses (for scheduling, sedation ?s) x 2927
- Reading Room: x2988, 2989
- Outpatient Scheduling: x3666, x4700

Clinic: x2673
- Audrey (urgent appts) x2666
- Keisha (appointments) x2828
- Fairfax – Nancy Elling 571-226-8343 or 571-226-8316
Appointment Line (for parents) x2610

Office Staff: x2120, x2165

- Jessica x2265
- Domonique x6206
- Fax Number: x2864

IT Help Desk: x4357 (H-E-L-P)

Main Lab x 5355
Pathology Lab: x2051 on call pathologist p0784
Radiology: x8643

Other important pagers:

Michiko p4696
Laura (Dietician) p0335
Language Services: p0370 (x5444)

Stroke
● Come in house, get stat imaging

25
● Consider Tpa if > 18 yrs old , d/w attending
● If it is <11:00 pm try to call radiology resident on call and found out if techs still in
house to get MRI.
● If patient is a SCD, also ask ED top contact hematology
Spinal Cord injury
● Come in, get stat imaging
● For compression, start high dose steroids. Trauma: Methylprednisolone 30mg/kg IV
over 15min follow by 5.4mg/kg over the next 23hrs. If Trauma happened 3-8 hrs ago,
you can continue steroids for another 48 hrs.
● Tumor: use Dexamethasone
● Call neurosurgery. PAs are in house all the time.
GBS or Myasthenic crisis
● Come in house (only if suspected- no need to come if patient has a confirm diagnosis)
● It is safe to observe in ICU in first 24 hrs and easier to get NIF done specially if admit
overnight
● IVIG dosing is 2gr/kg divided in 3-5 days. Pre-medicate with Tylenol and Benadryl
Parent calls:
● For prescription, remember to include patient’s weight. Include your DEA and NPI
number. Prescriptions for Diastat, Concerta or other CNS stimulant for ADHD, cannot
be faxed or call in. You should mail prescription to family or ask them to pick it up at
the hospital.
● If patient has breakthrough seizures you can adjust meds on the phone and ask them
to contact primary neurologist office next day. If you have made a big change in
management, stop AED or started AED; please send an email to primary neurologist
as a courtesy next day.
● If patient was worked up for new onset seizures after first event and was not started
on meds, you can start one the phone if patient has second episode. For focal
seizures, we tend to start with Trileptal or Keppra. Topiramate if it does not respond
to first ones. Primary Generalized: Depakote, Lamictal, also Keppra. Absence:
Zarontin, Lamictal, Depakote. Myoclonic: Depakote (unless suspect metabolic
disease- risk for Alpers), Keppra or Topiramate.

26
 ENCEPHALOPATHY

● Ranges from increased sedation and decreased response to stimulation (lethargy,

stupor, obtundation, coma) to hyper vigilance (delirium)
● History:
o Check medication list
o Clarify baseline with family
o Review lab results
o Establish trend (improving, worsening, wax -wane)
● Physical exam:
o Check for asterixis! Indication of organ dysfunction (liver, kidney, lung) or
toxins (opiates, etoh, bzd)
o Mental status: status @ rest, response to stimulus, attention, language,
higher cortical function
o CN: visual fields, EOMI, facial symmetry, tongue deviation
o Motor: symmetry, drift, tone
o Sensory: response to noxious stimulation
o Reflexes: symmetry, clonus
o ETC: tremor, myoclonus, asterixis, chorea, or dystonia
● CNS is victim
o Organ dysfunction, Cardiovascular, Medications / iatrogenic, Toxins,
Electrolytes, Peripheral infection, Post surgical, Nutritional deficiency, and
others
● CNS is the cause
o Infection (meningitis / encephalitis); Vascular (Stroke / ICH / SAH/ SDH);
Seizures ( non convulsive status, postictal, complex partial seizure)

Evaluation:
CBC w diff CNS work up
Complete metabolic profile Lumbar puncture
UA, CXR, blood dx Head CT scan
Arterial Blood Gas MRI
Urine drug screen EEG
B12 Secondary Testing
RPR Paraneoplastic testing

27
HIV Anti-TPO and anti-
TSH, free T4 thyroglobulin
NH3 14-3-3
Enolase

28
 DELERIUM

Criteria:
- Disturbance in attention and awareness,
- Disturbance develops over short period of time, tends to fluctuate in
severity,
- Is not explained by pre-existing neurocognitive disorder, or impairment in
arousal (coma),
- Disturbance is a direct physiological consequence of another medical
condition, substance intoxication or withdrawal, or exposure to a toxin.

Types: Hypoactive and hyperactive.

Treatment: A. Hypoactive – treat the cause

B. Hyperactive - calming, dark environment,
- diminish stimulation, minimize interaction with non-
essential staff, - very restrictive use of physical
restraints,
- minimize night time awakenings for vital sign check
etc.
- if needed: psychopharmacology – remember that
these medications on their own can impair mental
status.
a. Antipsychotic medications are preferred
first line therapy, choose newer ones over
haloperidol due to lower risk of side effects:
- Quetiapine (Seroquel) – 25 mg bid PO
- Olanzapine (Zyprexa) – 2.5 mg - 5 mg
daily PO or IM; if symptoms are severe – 5 –
10 mg IM x 1 acutely.
- Risperidone (Risperdal) – 0.5 mg bid PO.

29
 - Ziprasidone (Geodon) – 10 mg IM q 2 hrs PRN
- Haloperidol – 0.5 – 1 mg PO or IM; PO can repeat q
4 hrs, IM – q 1 hr. B! watch for QT prolongation and
extrapyramidal symptoms.
b. Benzodiazepines should be used:
1) second line treatment if antipsychotics
failed,
2) as first line therapy in - DLB
- NMS
- for delirium caused by drug withdrawal:
- lorazepam 0.5 – 1 mg PO or IV; watch for
paradoxical excitation and respiratory
depression.

NEURORADIOLOGY QUICK GUIDE

Sequence T1 T2 CT Gad
Bone Black Black Hyperdense
Air Black Black Hypodense
Fat Hyperintense Hyperintense Hypodense
Water (CSF) Black Hyperintense Hypodense
Brain Anatomic Anatomic Anatomic
Infarct Hypointense Hyperintense Hypodense If subacute
Bleed Variable Variable Hyperintense Yes
Tumor Hypointense Hyperintense Hypodense Yes
Demyelinating Hypointense Hyperintense Hypodense Yes acute

30
 Blood Staging per MRI and CT (Iddy-biddy-baby-daddy)
Phase CT T1 T2 Hemoglobin
<6h Hyperacute Hyperdense Isointense Hyperintens Intracellular
e Oxyhemoglobin
8- Acute Hyperdense Isointense Hypointense Intracellular
72h Deoxyhemoglobin
3d- Subacute Hyperdense Hyperintense Hypointense Extracellular
1wk early deoxyhemoglobin
1wk- Subacute Isodense Hyperintense Hyperintens Extracellular
1m late e methemoglobin
1m Chronic Hypodense Hypointense Hypointense Hemosidrin/ferritin

31
 GUH STROKE
PROTOCOL: EMERGENCY EVALUATION

A. History and Examination

1. CONFIRM LAST NORMAL. If patient woke up with symptoms at 8AM, went to bed
normal at 10PM the night before, and you evaluate at 9AM, their last normal was
11 hours ago and they are NOT eligible for TPA.
2. Some patients may be eligible for interventional treatment such as clot retrieval
up to 24 hours after symptom onset especially posterior circulation strokes. If
symptoms out of tPA window but but patient does not have completed infarct on
CT, please discuss case with stroke attending.

32
 3. Perform NIHSS and initially only pertinent parts of the neurological assessment to
determine if stroke code should proceed to MRI or CT. You could always do
reflexes, vibratory sensation, etc later.
4. Check vital signs (if SBP > 220, administer labetalol, hydralazine or nicardipine gtt
for refractory hypertension; if satting <92%, place on nasal cannula)
B. Laboratory (automatically done by stroke nurses)
accucheck, CBC with platelets, chem 7, coags, cardiac enzymes, type and screen It
is not necessary to wait for labwork if considering tpa unless the patient is anticoagulated
or has a reason to have severe thrombocytopenia.
C. Brain Imaging
1. If patient is a candidate for tPa and you suspect stroke, proceed to Head CT,
administer tPA bolus, then obtain CTA/CTP while tPA gtt is running, to see if
patient is also a candidate for intervention.
2. If patient is a candidate for intervention but not tPA, proceed to CT and consider
CTA/CTP
3. If suspicion for stroke is low, proceed to MRI
D. Nursing Care
1. Start 0.9% NS @ 75-100 cc/ hr unless contraindicated; start second IV line
2. Obtain 12 lead EKG
3. If within 4.5 hours, obtain weight and prepare to administer tPA, using the ECASS
3 criteria.
4. Head of bed flat x 24hrs from symptom onset if infarct, even while you are
evaluating them in ED
5. Permissive HTN 24-48hrs (refer to stroke order sets)
6. When admitting to stroke service, please use stroke specific order sets and not
the regular order sets
E. tPA Contraindications
1. Intracranial surgery or intraspinal surgery, serious head trauma, or previous
stroke within the past three months
2. History of intracranial hemorrhage
3. Major surgery within the past 14 days
4. Suspicion of subarachnoid hemorrhage on pretreatment evaluation
5. Uncontrolled hypertension (>185 mmHg systolic or 110 mmHg diastolic) requiring
aggressive treatment. Control blood pressure, then administer tPA if still in
window.

33
 6. Arterial puncture at a non compressible site
7. Active internal bleeding
8. Intracranial neoplasm or arteriovenous malformation
9. Subacute bacterial endocarditis
10. Known bleeding diathesis, platelets <100,000 mm3 or INR >1.7, elevated PTT,
novel anticoagulant use
11. Dabigatran in the last 24 hours
F. tPA Relative Contraindications-MUST discuss on case-by-case basis with vascular
attending
1. Rapidly resolving neurologic deficit
2. Myocardial infarction in previous 6 weeks
3. GI or Urinary tract hemorrhage within past 21 days
4. Seizure on onset of symptoms
5. 5 or more microhemmorhages (best seen on susceptibility weighted sequences)
use caution, particularly if lobar/CAA pattern (see Brazil for ref)
6. Glucose < 50 mg/dl or >400 mg/dl unless stroke confirmed by imaging studies
7. Frank hypodensity on CT or frank FLAIR or T2 shine through suggestive of
subacute stroke (>6 hours)
8. Diabetic hemorrhagic retinopathy or other hemorrhagic ophthalmic conditions
9. Pregnancy
G. tPA administration
1. Verbal consent patient and family (6% risk of intracranial hemorrhage, half of
which are fatal)
2. Administer tPA dose, total is 0.9 mg/kg, max dose is 90 mg, give 10% as bolus and
remaining 90% over as a continuous infusion over one hour
3. Do not shake tPA, roll it in your hand
H. Post IV tPA management
1. No anticoagulation or antiplatelet agents for 24 hours post tPA administration,
and will get 24 hour follow up scan regardless before starting agent
2. Admit to intensive care unit
3. Vitals: Neurochecks, BP, HR, RR will be q15 minutes for 2 hours, then 30 minutes
for 6 hours, then q1 hour for 16 hours then q 4 hours
4. Evaluate for angioedema q 20 minutes X4 times upon starting IV tPA
administration
5. Maintain SBP <180 and DBP <105

34
 6. May place central line at compressible site
7. Non-contrast head CT 24 hours post tPA administration. If no ICH can start
antiplatelets/anticoagulation as appropriate and transfer to step-down as
appropriate (based on level of alertness, stroke volume, etc)
I. BP management post-TPA
1. May give labetalol 10 mg IV over1-2 minutes, may repeated every 10-20 minutes
up to 150 mg
2. Nicardipine drip: starting dose is 5mg/hour and observe (50% effect at 45
minutes); can titrate up to max of 15 mg/h.
3. If above measures fail then use sodium nitroprusside; starting dose of 0.25-0.3
mcg/kg/min, max of 10 mcg/kg/min.
4. Monitor BP every 15 minutes and avoid hypotension!
J. Anticoagulation after stroke
1. Therapeutic heparin is not a standard treatment for acute stroke.
2. Heparin is used include: atrial thrombus, mechanical heart valve, tight carotid
stenosis, basilar disease, venous sinus thrombosis, TIA or minor stroke with
cardioembolic source
TIA risk stratification with ABCD criteria
Admission is recommended for ABCD score of ≥ 2
Criteria Score
Age >60 1
Blood Pressure >140/90 1
Clinical Focal Weakness 2
Speech Impairment without focal weakness 1
Time > 60 minutes 2
Time 10-59 minutes 1
Diabetes Mellitus 1
Johnston et al. Validation and refinement of scores to predict very early stroke after transient ischaemic
attack. Lancet 2007; 369:283- 92.

35
 Common Lacunar Syndromes
Eponym/Artery
Ataxic Hemiparesis
(lenticulostriates)
Pure Motor Hemiparesis or
Dysarthric Hemiparesis
Pure Sensory stroke
Sensory Motor stroke
Clumsy hand syndrome
Anatomy
Posterior limb of the
internal capsule, basis
pontis
Posterior limb of the
internal capsule, ventral
pons, cerebral peduncle,
hemispheric white matter
Ventral Posterior Thalamus
Thalamus and Internal
Capsule
Genu of the Internal
Capsule
Symptoms
Contralateral arm and leg
weakness and ataxia
Contralateral face arm and
leg weakness with
dysarthria
Contralateral sensory loss
Contralateral sensory loss
and weakness
Contralateral loss of hand
fine motor use and
dysarthria

Stroke Syndromes
Eponym/Artery Anatomy
ACA Medial frontal and parietal
Recurrent Artery of Anterior inferior head of the
Heubner (from A1) Caudate, Putamen and
Anterior limb of the internal
capsule
MCA- Superior M2 Frontal/Parietal/Temporal
MCA- Inferior M2 Frontal/Parietal/Temporal
Gerstmann syndrome Dominant Parietal Lobe
(partial MCA)
Symptoms and Signs
Contralateral Leg weakness >arm
weakness, Abulia
Contralateral face weakness
(Heubner) and Contralateral leg
weakness (ACA) sparing the arm
Contralateral Face and Arm
weakness >leg, hemisensory loss,
hemineglect (non-dominant,
expressive aphasia (dominant)
Contralateral homonymous
hemianopsia or superior
quadrantanopia, constructional
apraxia (non-dominant), receptive
aphasia (dominant)
Agraphia, Acalculia, Right Left
confusion, finger Agnosia and

36
 possible ideomotor apraxia
Unilateral PCA Occipital/Inferomedial Contralateral homonymous
temporal lobes and posterior hemianopsia with macular sparing
thalamus or inferior quadrantanopia, anomies
and +/- alexia without Agraphia
Alexia without Agraphia Dominant Occipital lobe and Alexia (splenium of the corpus
(PCA usually left) splenium of the corpus callosum) and contralateral
callosum homonymous hemianopsia
(occipital region)
Balint syndrome (B/L Bilateral parietal-occipital Optic ataxia, Ocular apraxia (loss of
PCA) lobes voluntary eye movements but not
reflex eye movements),
asimultagnosia (inability to
understand visual objects)
Anton Syndrome (B/L Bilateral occipital lobes Cortical blindness, with denial and
PCA and Distal basilar) possible hallucinations
Dejerine-Roussy Thalamus Contralateral hemisensory loss to all
“thalamic pain modalities and hemi-body pain
syndrome” (PC
penetrators to
thalamus)
Weber (PCA Midbrain Contralateral Weakness,
penetrators to Ipsilateral III palsy
midbrain)
Claude (PCA Midbrain, Tegmentum Contralateral arm and leg ataxia,
penetrators to possible III palsy
midbrain)
Benedikt (PCA
Penetrators to
midbrain)
Raymond (Basilar Ventral medial pons Ipsilateral VI palsy and
paramedian branches) Contralateral weakness, spaces
face (VII)
Millard-Gubler (basilar Basis Pontis, fascicles of VI, Contralateral weakness, Ipsilateral
short and paramedian and VII VI palsy and Ipsilateral VII palsy
branches)
Marie-Foix Lateral Pons, Middle Ipsilateral ataxia and contralateral
(Basilar/AICA) Cerebellar peduncle arm and leg weakness and

37
 hemisensory loss (Pain and
Temperature)
Locked-in Syndrome Pons Quadraparesis
(basilar)
Wallenberg, lateral Lateral Medulla Ipsilateral hemisensory loss of
Medullary syndrome face (nucleus V), Facial pain (n. V),
(vert > PICA or both) gait ataxia (vestibular nucleus),
Nystagmus (vestibular nucleus)m
Vertigo (vestibular nucleus),
Hoarseness and Dysphagia
(Nucleus ambiguus) Horner
syndrome (descending
sympathetic chain), Contralateral
hemisensory loss (pain and temp)
spinal thalamic tract
Medial Medullary Medial Medulla Contralateral weakness upper and
Syndrome or Dejerine lower extremities, contralateral
syndrome (Veterbral hemisensory loss to vibration and
artery, anterior spinal propioception and Ipsilateral
artery) tongue weakness (devation) XII.
Aphasia
Type of Auditory
Repetition Naming Fluency Presentation
aphasia comprehension
Fluent and non-sensical
speech, patient will have
Receptive Mild deficit Severe naming fluent paraphrasic errors and use
defective
Aphasia in repetition dysfunction paraphasic neologisms. Lesion is
Dominant parietal lobe near
angular gyrus
Transcortic
Repetition Severe Severe Receptive aphasia with
al sensory fluent
intact dysfunction dysfunction intact repetition
aphasia
Conduction poor Poor relatively good fluent Arises from lesion to the
aphasia arcuate fasciculus, the
circuit that transmits
information between
Wernicke's area and Broca's
area. Similar symptoms,
however, can be present
after damage to the insular
cortex or to the auditory
cortex. Auditory

38
 comprehension is near
normal, and oral expression
is fluent with occasional
paraphasic errors. Repetition
ability is poor.
Lesion to the Broca’s area or
dominant frontal lobe.
Patient understands the
situation and knows what he
Moderate to Moderate – non-fluent,
Expressive No or mild wants to say but cannot
severe Severe effortful,
aphasia dysfunction express himself. It is
dysfunction dysfunction slow
associated with right
hemiparesis, meaning that
there can be paralysis of the
patient's right face and arm.
Occurs to a lesion of
dominant frontal lobe, often
superior but not including
the Broca’s area or
improvement of a Broca’s
aphasia. Similar deficits as
Broca’s aphasia, except
repetition ability remain
Transcortic
No or mild intact. Auditory
al motor mild–severe Mild non-fluent
dysfunction comprehension is generally
aphasia
fine for simple
conversations, but declines
rapidly for more complex
conversations. It is
associated with right
hemiparesis, meaning that
there can be paralysis of the
patient's right face and arm.
Patient are have severe
language deficits or are
mute, cannot follow
Global Severe Severe Severe
non-fluent command, repeat or name,
aphasia dysfunction dysfunction dysfunction
with large Dominant
hemispheric strokes (large
MCA)
Transcortic A similar deficit as in global
Moderate Severe Severe
al mixed non-fluent aphasia, but repetition
dysfunction dysfunction dysfunction
aphasia ability remains intact.

Fluent, non-fluent and "pure" aphasias

The different types of aphasia can be divided into three categories: fluent, non-fluent and
"pure" aphasias.[7]

39
● Fluent aphasias, also called receptive aphasias, are impairments related mostly to
the input or reception of language, with difficulties either in auditory verbal
comprehension or in the repetition of words, phrases, or sentences spoken by others.
Speech is easy and fluent, but there are difficulties related to the output of language
as well, such as paraphasia. Examples of fluent aphasias are: Wernicke's aphasia,
Transcortical sensory aphasia, Conduction aphasia, Anomic aphasia
● Nonfluent aphasias, also called expressive aphasias are difficulties in articulating, but
in most cases there is relatively good auditory verbal comprehension. Examples of
nonfluent aphasias are: Broca's aphasia, Transcortical motor aphasia, Global aphasia
●
● "Pure" aphasias are selective impairments in reading, writing, or the recognition of
words. These disorders may be quite selective. For example, a person is able to read
but not write, or is able to write but not read. Examples of pure aphasias are: Alexia,
Agraphia, Pure word deafness

40
41
42
43
 MANAGEMENT OF STATUS EPILEPTICUS

Defintion: A seizure that lasting 30 minutes or longer (but treat as status if

lasting over 5 mins) or is repeated frequently enough that the individual does
not regain consciousness between seizures

PURPOSE: To ensure the correct management of adult patients presenting with

status epilepticus (SE)

SAFETY STATEMENT:
al emergency with substantial potential for morbidity and mortality. There is
evidence that irreversible brain injury begins at 30 minutes. Because physiological
changes occur making seizures more difficulty to abolish the longer they persist,
every effort should be made to abolish seizures as soon as possible.

A. Definition of SE
1. Continuous generalized tonic-clonic (grand mal) seizures or
generalized tonic-clonic seizures alternating with reduced level of
consciousness (GCS < 8) lasting at least 5 minutes
2. Note: if a patient presents actively seizing to the Emergency
Department or upon discovery by staff at bedside without
knowledge of “last seen normal”, the patient may be considered in
SE

A. Initial Treatment
1. The principal goal of treatment is to emergently stop both clinical
and electrographic seizure activity
2. Notify neurology on call for ongoing seizure activity
3. Obtain IV access, administer O2, and secure airway as needed,
correct any immediately reversible precipitants such as
hypoglycemia

44
 i. If alcohol abuse or poor nutrition is suspected, thiamine 100
mg IV should be administered prior to the administration of
glucose
4. Obtain a urine drug screen, complete blood count (CBC), and
complete metabolic panel (CMP)
5. Administer lorazepam (Ativan®) 2 mg IV push x1 no faster than
2mg/min
i. A second dose may be considered if status persists
ii. Total doses 0.1 mg/kg are sometimes required
iii. It is likely, but not mandatory, that a patient will require
intubation at this time
iv. Where IV access cannot be established, 10 mg diazepam
(2.5 mLs) solution (DiaStat®) can be given rectally x1
1. A second dose may be repeated after 5 minutes, if
necessary
B. Second line anticonvulsants
1. A second anticonvulsant should be ordered to the bedside at the
time of administration of the second dose of lorazepam
i. Fosphenytoin should be administered as the second line
agent following lorazepam
1. Dosing: 20 mg PE/kg IV no faster than 150 mg/min
Wt of patient 40 50 60 70 80 90 100 110
(kg)
Dose of 800 1000 1200 1400 1600 1800 2000 2200
Fosphenytoin mg mg mg mg mg mg mg mg
Time of 16 20 24 28 32 36 40 44
infusion
(mins)

ii. In the event that fosphenytoin is not available, phenytoin

can be used at a dose of 20 mg/kg IV no faster than 50
mg/min

45
 1. It is preferred the phenytoin be given via a large
bore peripheral line or a central line to avoid purple
glove syndrome
2. Use caution in patients with known hepatic disease
iii. If seizures persist, the loading dose should not be repeated
unless serum level results are available and are below the
desired goal
iv. Draw serum phenytoin level approximately 10 mins after
phenytoin is completed
C. Prior to initiating third line anticonvulsants, consider obtaining continuous
EEG monitoring
D. Prior to initiating third line anticonvulsants, a decision needs to be made
regarding intubation, if not already performed, based on patient state and
duration of seizure activity
E. Third line anticonvulsants
1. Use of valproic acid, levetiracetam, or lacosamide, may be
considered in patients that are hemodynamically stable and have
not required intubation
i. Valproic acid can be administered at a dose of 25 mg/kg at a
rate of up to 20 mg/min with appropriate monitoring
1. Use with caution in patients at an increased risk of
bleeding or those with thrombocytopenia and those
in liver failure
ii. Levetiracetam (Keppra®) can be administered at 1000-2000
mg IV over 15 minutes
1. The efficacy of levetiracetam in SE has not been fully
established and many neurologists suspect that it is
inferior to phenytoin in this condition; however, it
may be beneficial for patients with severe hepatic
disease
2. Use caution in patients with renal disease. If used in
renal disease leveteracetam dose must be adjusted.

46
 iii. Lacosamide (Vimpat®) can be administered at a dose of 200-
400 mg IV over 15 minutes
1. There is limited experience in using lacosamide for
SE and its use should not delay the implementation
of an anesthetic agent if the patient is still seizing
after one hour
2. A higher incidence of bradycardia has been seen in
patients receiving lacosamide, especially in the
pediatric population
2. If seizures continue after one hour and with two agents fully
loaded, then the patient should be intubated and ventilated and
continuous infusion therapy with midazolam, pentobarbital, or
propofol should be initiated.
i. For all continuous infusions, titrate until cessation of
seizures or hypotension which cannot be controlled with a
single pressor
ii. Midazolam should be started with an initial bolus of 2-5 mg
IVP followed by a continuous infusion of 2.5 mg/hr and
titrated to a maximum rate of 10 mg/hr
1. Patients needing greater than 10 mg/hr of
midazolam should be dosed at the discretion of the
treating medical team
2. Caution should be used in renally impaired patients.
Midazolam is renally eliminated and has active
metabolites in renal failure.
3. Midazolam has a rapid redistribution and therefore
short duration of action unless a patient has renal or
liver impairment
iii. Propofol should be started with an initial bolus of 1-2 mg/kg
followed by a continuous infusion of 5 mcg/kg/min and
titrated to a maximum rate of 50 mcg/kg/min
1. Hypotension is very common with propofol,
especially with loading doses

47
 2. For prolonged infusion of propofol, triglycerides
need to be monitored every 3 days due to propofol
containing 1.1 kcal/mL of lipid
iv. Pentobarbital is the only agent which may be considered an
absolutely effective agent since it can be used to produce an
isolelectric EEG. It should be initiated with a 10-15 mg/kg IV
bolus over 1 hour followed by a continuous infusion of 1
mg/kg/hour titrated to 3 mg/kg/hour
1. Lacrilube or artificial tears should be ordered for
patients initiated on pentobarbital infusion
2. Pentobarbital can produce severe hypotension,
paralytic ileus, and a chemical pancreatitis
v. An EEG should be implemented as soon as practical in order
to confirm the absence of ongoing subclinical electrographic
seizures and to facilitate titration of the anesthetic agent
vi. Neurology should provide recommendations regarding
which anti-epileptic drugs to use prior to removal of the
anesthetic agent
F. Refractory/Malignant status epilepticus
1. Defined as status epilepticus that continues or recurs 24 hours or
more after the onset of anesthetic therapy, including cases that
recur on the reduction or withdrawal of anesthesia
2. A realistic goal should be set among healthcare providers and the
patients family
3. Ketamine has antagonistic action at the NMDA receptor potentially
have neuroprotective action
i. Bolus: 0.5-4.5 mg/kg
ii. Infusion up to 5 mg/kg/hr
1. Start at 0.5 mg/kg/hr and titrate every 15 mins by
0.5 mg/kg/hr
4. Magnesium could be used to control seizures in eclampsia,
magnesium deficiencies, and porphyria.
i. Bolus: 4 gm

48
 ii. Infusion: 2-6 gm/hour
iii. Magnesium levels should be monitored for a goal level 5-8
mg/dL
iv. Calcium gluconate 10% solution should be administered by
a prescriber only if a patient becomes magnesium toxic
5. Pyridoxine
i. 500 mg IV BID x 3 days
6. Steroids can have effects if the cause is immunologic and in reversal
of BBB opening
i. Methylprednisolone 1gm IV for 3 or 5 days
ii. Dexamethasone 4 mg IV every 6 hours for vasogenic edema
7. IVIG
i. 0.4 gms/kg/day for 5 days
8. Plasmaphoresis
i. If plasma exchange and IVIG are being considered, plasma
exchange should performed prior to IVIG therapy
9. Therapeutic hypothermia
i. Hypothermia has been shown to exert anti-epileptic action
and to be neuroprotective in experimental status
epilepticus. It has also been shown to reduce brain edema.
10. As a general rule, alkalosis and hyperthermia should be avoided
G. Etiologies to consider
1. Metabolic disturbances: electrolyte abnormalities, hypoglycemia,
renal failure
2. Infection: sepsis, meningitis, encephalitis, abscess
3. Stroke: ischemic, intracerebral hemorrhagic, subarachnoid
hemorrhagic, cerebral sinus thrombosis
4. Head trauma with or without epidural or subdural hematoma
5. Drug issues
i. Drug toxicity
ii. Withdrawal from opioid, benzodiazepine, barbiturate, or
alcohol
iii. Non-compliance with antiepileptic regimen

49
 6. Hypoxia, cardiac arrest
7. Hypertensive encephalopathy, posterior reversible encephalopathy
syndrome
8. Autoimmune encephalitis, paraneoplastic syndromes
i. Anti-NMDA receptor antibodies, anti VGKC complex
antibodies, Anti TPO antibodies
9. CNS tumors
10. Mitochondrial syndromes
H. Diagnostic tests to consider
1. All patients
i. POC glucose, vital signs, CT head, CBC, CMP, AED levels,
cEEG monitoring
2. Based on clinical presentation
i. MRI Brain
ii. LP
iii. Comprehensive toxicology
1. Isoniazide, tricyclic antidepressants, throphylline,
cocaine, sympathomimetics, alcohol,
organophosphates, cyclosporine
iv. LFTs, troponins, coagulation studies, ABG
v. Testing for infectious etiologies such as: HSV 1 and 2, HHV6,
parvovirus, CMV, EBV, arboviruses, enterovirus or other
bacterial, fungal or parasitic agents as indicated by clinical
presentation

REFERENCES:
1. Shorvon S, Ferlisi, M. The treatment of super-refractory status epilepticus:
a critical review of available therapies and clinical treatment protocol.
Brain. 2011;134:2802-18.
2. Brophy GM, Bell R, Claassen J, et al. Guideline for the evaluation and
management of status epilepticus. Neurocrit Care. 2012; 17(1):3-23.

50
3. Costello DJ, Cole AJ. Treatment of acute seizures and status epilepticus. J
Intensive Care Med. 2007;22:319-47.
4. Lowenstein DH. The management of refractory status epilepticus: an
update. Epilepsia. 2006; 47:35-40
5. Treiman DM, Meyers PD, Walton NY, et al. A comparison of four
treatments for generalized convulsive status epilepticus. N Engl J Med.
1998; 339:792-98.

51
 Suggested Management of Status Epilepticus

Time Intervention
0-30 0 min Recognition of SE (clinical or EEG)
minutes 0-1 min Airway/breathing/circulation
Suctioning, supplemental oxygen, pulse oximetry
Baseline vital signs, capillary glucose level
1-2 min ECG/cardiac telemetry
Large bore proximal IV access, baseline labs, IV fluids
CBC, Electrolytes, BUN/SCr, Glucose, Ca, Mg,
PO4, LFTs, AED levels, toxicology screen, ABG, urine
pregnancy test
1-3 min Administer 100 mg IV thiamine and/or 50 mL D50W (if
needed)
3-10 Administer lorazepam
min 0.1 mg/kg IV Age 1-50
0.05 mg/kg IV Age 50-75
0.025 mg/kg IV Age > 75
Repeat in 5 minutes if seizure persists
Prepare fosphenytoin or phenytoin loading dose of 20
mg/kg
5-30 Begin fosphenytoin or phenytoin infusion 1 min after end
min of lorazepam bolus if seizure persists
Administer fosphenytoin at 150 mg/min or
Administer phenytoin at 50 mg/min
Monitor ECG, BP every 2 mins
Draw serum phenytoin level approximately 10 min after
fosphenytoin/phenytoin load is complete
30-45 30 min Contact EEG lab to initiate cEEG monitoring
minutes Transfer to ICU (intubate if necessary)
> 45 30-60 3rd line IV Valproic Acid 25 mg/kg IV
minutes min agent Levetiracetam 1000-2000 mg IV

52
 Lacosamide 200-400 mg IV
3 line infusion Midazolam 2 mg IVP followed by 2.5
rd

mg/hr titrated up to max 10 mg/hr

Propofol 1-2 mg/kg IVP followed by 5
mcg/kg/min titrated up to 50
mcg/kg/min
Pentobarbital 10-15 mg/kg IV bolus
over 1 hour followed by 1 mg/kg/hour
titrated up to 3 mg/kg/hour
Titrate infusions to abolish all clinical
and electrographic seizure activity
Etiologies to consider: Metabolic disturbances, infection, stroke, head trauma,
drug issues (toxicity, noncompliance, withdrawal), hypoxia, hypertensive
encephalopathy, posterior reversible encephalopathy syndrome, autoimmune
encephalitis, paraneoplastic syndromes, CNS tumors
A realistic goal should be set among healthcare providers and the patient’s
family
>24 Ketamine 0.5-4.5 mg/kg bolus with infusion up to 5
hours mg/kg/hr
Magnesium for eclampsia, magnesium deficiencies,
porphyria
Bolus: 4 gm, Infusion 2-6 gm/hour
Goal level 5-8 mg/dL
Calcium gluconate 10% for magnesium toxicity
Pyridoxine 100 mg IM daily
Steroids:
Methylprednisolone 1gm IV for 3-5 days
Dexamethasone 4 mg IV every 6 hours
IVIG 0.4 gm/kg/day for 5 days
Plasmaphoresis
Therapeutic hypothermia

53
 Defintion: A seizure that lasting 30 minutes or longer (but treat as status if lasting
over 5 mins) or is repeated frequently enough that the individual does not regain
consciousness between seizures

Diagnose Status Epilepticus

Airway Management
0-5 02 via nasal cannula
min Check ABG or follow O2 sat
Consider intubation if airway is compromised
Vitals, EKG, IV access, finger stick
Rapid physical and neuro exam
Blood for glucose, lytes, ETOH, AEDs, ABG, tox screen, CBC
If hypoglycemic or no blood glucose available, administer glucose
6-9 Adults: 100mg thiamine Children: 2ml/kg of 25%dextrose
min 50mg of D50
If seizures continue, administer benzodiazepine.
Lorazepam 0.1 mg/kg IV up
to 4 mg per dose, may repeat
in 5-10min. Diazepam 0.15
(Recommend not exceeding mg/kg IV up to Diastat PR (if no IV)
10-15 total of 8 mg) 10 mg per dose, 1-5 yo: 0.5mg/kg dose
min may repeat in 5
min
Repeat once if seizures do not stop after 3-5 minutes
15-30 Fosphenytoin: 20mgPE*/kg IV Phenytoin 20mg/kg IV
min (rate 100-150mg PE/min)** (Adult rate: up to 50mg/min)
(Child rate: 1mg/kg/min
(*fosphenytoin is dosed as phenytoin
equivalents)

(**NOTE: fosphenytoin is water soluble, so

can be loaded faster and can be given IM if
needed)

54
 Monitor EKG (for bradycardia) and BP (for acute hypotension)
If seizures continue 10 min after loading infusion, give fosphenytoin 5 mg PE/Kg
or phenytoin 5-10mg/kg
Call Anasthesia or code blue to intubate
Propofol: start 20
mcg/kg/min, with 1-2
mg/kg loading dose.
Maintenance: 30-200
Midazolam: 0.2 mg/kg; mcg/kg/min CI*
administer at an infusion Breakthrough SE*:
rate of 2 mg/min increase CI rate by 5-10
Maintenance: 0.05-2 mcg/kg/min every 5
mg/kg/hr CI min or 1 mg/kg bolus Phenobarbital 20mg/kg IV
Breakthrough SE: 0.1-0.2 plus CI titration (Adult rate: 50mg/min)
mg/kg bolus, increase CI (DO NOT use in (Child rate: 3mg/kg/min)
rate by 0.05-0.1 mg/kg/hr children)
30-60 every 3-4 hr
Min Monitor BP and consider starting EEG
Use caution when administering propofol >80 mcg/kg/min for > 48 hr
*CI – Continuous Infusion, SE-status epilepticus
If still seizing, start Pentobarbital 5-20mg/kg load (rate of 25mg/min).
60-80 Maintenance 0.5-1.0mg/kg/hr
min Transfer to ICU
EEG—monitor for burst suppression
Neuro exam, LP, CT, repeat labs, make decision on chronic management.

Alternative AED options for loading: Valproic Acid 20-40 mg/kg IV,
may give an additional 20 mg/kg (rate 3-6 mg/kg/min, may give
additional dose 10 min after loading infusion); Levetiracetam 1,000-
3,000 mg IV (rate 2-5 mg/kg/min); Lacosamide 200-400 mg IV (200 mg
over 15 min)

55
 Seizure/Epilepsy Assessment and Management
Seizure Provoking factors Epilepsy Risk Factors
● High Fever (febrile seizures up to ● Mother’s Pregnancy History
6yrs) ● Birth/Neonatal History
● Sleep Deprivation ● Retardation/Developmental Delay
● Excessive use of stimulants ● CNS infection
(cocaine) ● Head Trauma (mod or severe)
● Withdraw from sedatives or ● CNS malformation
alcohol ● CNS neoplasm
● Hypoglycemia ● Stroke (hemorrhagic> ischemic)
● Electrolytes (Mag) ● Alcohol use
● Hypoxia ● Drug use (stimulants)
● Acute medical or neurological ● Family History of Seizures
illness (stroke, trauma, CNS
infection)
● Antihistamines
Seizure History
● Precipitants
● Prodrome/Aura
● Seizure manifestations: LOC, fall, breathing, movements of head, face and limbs
(stiffening, jerking, posturing), automatisms (oral and limb), eye movements,
drooling, frothing, clenching, tongue biting, bowel or urinary incontinence, and
HR/blood pressure changes
● Duration
● Postictal Period: Todd’s paralysis, lethargy, confusion, headache, aphasia
● Seizure frequency
● Current antiepileptics (and compliance) and previous antiepileptics failed (and
why)
Evaluation
● EEG
● MRI for structural lesion, obtain seizure protocol to look at mesial temporal
lobes/hippocampus if there is suspect for a primary seizure syndrome.
● Routine labs: Chem 10 (Mg is important), glucose, ABG (if in Status), AED levels,
EtOH level

56
 ● Toxicology screen
Seizure Recurrence Risk
● Single Seizure- 71% risk over 2 years and 30% in children
● No risk factors- i.e. Normal EEG and MRI: about 20%
● Abnormal EEG (epileptiform activity)- 60-70%
● Abnormal imaging (structural lesion)- 60-70%
● Two seizures- 95%
Initial AED of choice
● Partial Seizures: Carbamazepine, Phenytoin, and most new agents
● Primary Generalized Seizures: Valproate, Lamotrigine, and Topirimate
● Absence Seizures: Ethosuximide, Valproate, Lamotrigine and topirimate
● Tonic, atonic, or atypical absence seizures
o Tonic Seizures- Phenobarbital or Phenytoin
o Atonic Seizures- Valproate or Clonazepam
o Atypical Absence Seizures- Clonazepam

57
 ACUTE SPINAL CORD COMPRESSION

Presentation:
1. Assume cord compression in any cancer pt with back pain.
2. Consider if the pt has progressive neurological symptoms, including
weakness/numbness (especially symmetric and without facial involvement) and
bladder/bowel symptoms.
3. Take note of general condition, back pain, history of trauma, known cancer or
infection, duration of symptoms, recreational drug use, or history of chronic steroid
use.
Exam Points:
1. Note the vital signs (especially for respiratory distress/autonomic instability)
2. Check for a sensory level
3. Check rectal exam (for saddle anesthesia, diminished rectal tone)
4. DTR's may be diminished (ie. Spinal shock) or hyperreflexic
5. Percuss the spine for tenderness
Workup and Treatment
1. If tumor suspected, give IV Dexamethasone 20mg bolus, then 10mg Q6hrs.
2. If trauma suspected, immobilize the neck (usually done in the ED) and start IV
Solumedrol 30mg/kg bolus over 15 minutes, then 5.4mg/kg/hr over 23 hours.
3. STAT MRI spine. Consider starting with plain films if there is a delay in getting an MRI,
but the on-call Rads resident should be made aware of the emergent need.
4. STAT neurosurgical consult.
5. Possibly hematology-oncology consult and XRT within 12 hrs if appropriate.
6. Not usually admitted to the neurology service – think neurosurgery, ICU, or oncology.

58
59
GUILLIAN-BARRE SYNDROME (AIDP)

Presentation: ascending motor/sensory loss with areflexia, often with pain, after GI
(campylobacter) or URI symptoms

Workup + Admission:
1. Assess respiratory status with vital capacity (VC) and negative inspiratory force
(NIF), follow q2 hours if worrisome, intubate if VC < 15cc/kg and trending
downward. Pulse ox and ABG will lag hypoventilation! Estimated VC = counting
aloud in one breath x 100cc (ex. Counts to 25 = 2.5L). If facial weakness, VC may
be lower due to poor seal on apparatus. If pt vented for >7 days, consider trach
and PEG.
2. Admit to MICU vs. general neuro based on respiratory status and bulbar
dysfunction.
3. Q4 hour neuro checks, q4 hour vitals, telemetry – watch for dysautonomia
(tachy/bradycardia, hyper/hypotension)- treat with low dose beta-blockers and
IVF boluses as needed.
4. Check HIV (think seroconversion), consider HSV, CMV, Hep titers, Lyme, urine
porphyrin levels, urine heavy metal screen, stool culture for campylobacter, ESR,
SPEP/immunofixation, ANCAs, Na (watch for SIADH)
5. Consider MRI with contrast to rule out cord compression/transverse myelitis. Can
also assess for nerve root involvement.
6. Lumbar Puncture- expect albumino-cytologic dissociation (high protein with low
cells), may be normal in first 48 hrs, may have up to 20 WBC post infection.
7. EMG/NCS (order w/ f-waves) – confirming studies as well as prognostic value.
8. Acute treatment with IVIG or plasma exchange.
9. Pain management – can be intense back and leg pain during acute phase (likely
radicular irritation) or burning paresthesias during recovery. Consider: NSAIDS +/-
opiates early, gabapentin, TCA’s, AEDs late.
10. GI and DVT prophylaxis (lovenox preferable to SCDs to avoid compression of
nerves)
11. Order PT/OT/Rehab consults – may need SNF.
12. Consider nerve specific antibody testing (Serum Anti-GM1, GQ1b, GD1a antibody
– Athena lab send-out requisition)

60
 13. Be aware of variants – Miller Fisher Syndrome, Acute Motor Axonal Neuropathy,
Pharyngo-Cervical-Brachial

Treatment: IVIG or plasma exchange – the effects are equivalent. Steroids are not
effective.
1. IVIG: order 0.4grams/kg/d x 5 doses (2 grams/kg total). Fill out IVIG order form.
Premeds: Tylenol 650mg, Benadryl 25-50mg. Risks: stroke/thrombotic events,
volume overload, aseptic meningitis/HA, infection, expensive, contraindicated in
IgA deficiency.
2. Plasma exchange: 5 treatments over 7 or so days (not more than two days in a
row, usually). need pheresis catheter placed (call surgery early for this) - Risks:
Hypotension, watershed infarction, line sepsis, hypocoagulability (follow fibrin,
fibrinogen per donor center recommendations), electrolyte disturbance (Ca). Will
need to hold some medications prior to exchanges such as ACE inhibitors,
mestinon. Check chart as pathology resident will usually write recommendations.

CHRONIC INFLAMMATORY DEMYELINATING POLYNEUROPATHY (CIDP)

● CIDP patients can relapse as part of their disease process or due to other triggers.
● Initially expect response to previous treatments (steroids, IVIG, or plasma exchange)
● Some CIPD patients come in q 4-6 weeks and know their IVIG dosing and med
regimen well; ask them before entering orders.
● May try IVIG 2grams/kg over 1-2 days, plus pre-meds (Tylenol 650mg PO, Benadryl
25mg PO)
● Pts may require chronic prednisone or other immunosuppressive for stabilization.

61
62
 MYASTHENIC CRISIS

Overview
● Respiratory failure in patients with autoimmune form of myasthenia gravis is caused
by weakness of respiratory muscles, upper airway muscles (bulbar myasthenia) or
both
● Two most common forms of acquired, autoimmune myasthenia gravis:
o anti-AChR antibodies (AChR-MG) (85%): acquired antibodies against
postsynaptic acetylcholine receptors (AChR) in the motor end plate of skeletal
muscles
o anti-MuSK antibodies muscle specific tyrosine kinase (15%)
o for comparison: remember Lambert Eaton myasthenic syndrome (LEMS)
have antibodies directed against voltage-gated calcium channels in the pre-
synaptic side of motor end plate
● Symptoms
o MG with AChR antibodies: muscle weakness tends to initially affect the
intercostal and accessory muscles and then the diaphragm.
o MG with MuSK antibodies: preferentially bulbar weakness before respiratory
muscle weakness. Signs of bulbar weakness: dysphagia, nasal regurgitation,
nasal quality to speech, staccato speech, jaw weakness (jaw closure weaker
than jaw opening), bifacial paresis, and tongue weakness.
● Differentiate myasthenic symptoms from symptoms of cholinergic crisis (rare):
weakness, fasciculations, increased sweating, salivation, and miosis
● Upper airway weakness can lead to respiratory failure by oropharyngeal collapse or
tongue obstruction and by increasing the work of already fatigued respiratory
muscles against a closed airway
● Thymoma is also associated with a worse prognosis in myasthenic crisis

Diagnosis
● Usually clinical at bedside
● EMG: repetitive motor nerve stimulation shows decrement
● Labs: Infectious w/u, CBC, UA and C&S, blood cultures x 2, CK, CMP, K +, Mg2+, and PO43

Respiratory function

63
● At the bedside: counting numbers in a single breath or weak cough, labored SOB,
bulbar weakness
● Respiratory therapy: NIFs and VCs
● When to intubate or send to ICU (any one of the following):
o 20/20/40: NIF < -20 cm H2O, VC < 1.5 L (or <20 mL/kg), PIF < 40
o difficulty counting to 20 in a single breath
o use of accessory muscles
o inability to raise the head due to neck weakness and paradoxical breathing

Acute Treatment
● Pyridostigmine or other anticholinesterase inhibitors:
o Stop these if there is concern for excessive secretions in the patient who is
experiencing respiratory failure. These do not alter the course of the crisis
and are used only for symptomatic relief of MG. They also can have cardiac
complications (arrhythmia and myocardial infarction).
o Stop in event of cholinergic crisis (rare): weakness, fasciculations, increase
sweating, salivation, and miosis
o After patients show definitive improvement in muscle strength (usually
several days after the initiation of IVIg or PE), these, typically oral
pyridostigmine, may be reinitiated after or just prior to extubation. Start on
low dose and titrate to symptomatic relief. 1mg IV = 30mg oral.
● Plasma Exchange or Intravenous Immunoglobulins
● Corticosteroids:
o High-dose prednisone (1-1.5 mg/kg/d) (give via NG tube if intubated)
o May also use methylprednisolone 1mg/kg/d while intubated
o Initiate concurrently with IVIg or PE, since prednisone begins to work after 2
weeks when effects of plasma exchange and IVIg are waning.
o Any worsening from high-dose prednisone is treated with continued
ventilator support.
o Once the patient has begun to show improvement, the prednisone dose can
be decreased slowly and gradually converted to alternate-day dosing. See
maintenance treatment.

Maintenance Treatment (General principles)

64
Symptomatic/Nonimmune treatment: Doesn’t affect the antibodies themselves or
remodeling of NMJ
● Acetylcholinesterase inhibitors: pyridostigmine (Mestinon) increases
concentration of acetylcholine by blocking acetylcholinesterase thus allowing
remaining AChRs to be maximally activated.
Immune-directed treatment: modifies AChR Ab production or the damage to the NMJ by
these antibodies
● Corticosteroids:
o prednisone 1 mg/ kg/d (usually 60 – 80 mg/day)in 3 divided doses until
symptom free as an outpatient. (Typically improvement is noted by
approximately 6 weeks and remission by 3 months.)
o gradually increasing the dose of steroids over 1-2 months reduces the risk
of the early worsening
o taper the dose slowly enough that an exacerbation does not occur (5-10%
or every other dayinitially 10 mg every 2-3 weeks after clinical
stabilization ) and then more slowly at doses below 20 mg/day.
● Steroid-sparing immunodirected treatment:
o azathioprine (Imuran)
o mycophenolate mofetil (CellCept)
o Cyclosporine may be considered after IVIg or PE in patients who cannot
receive or failed steroids
Thymectomy
● If it is to be done, it should be done within the first 2 years of symptom onset for
AchR ab MG (probably not indicated for MUSK MG)
● Usually carried out after symptom stabilization
● Optimize strength first – may need pre-op plasmapheresis to reduce risk of post-
op exacerbation

Precipitants of Myasthenic Crisis or Worsening

Stressors Medications Medications (continued)
Physical stressors a-Interferon Gabapentin
Aspiration pneumonitis Antibiotics Phenytoin
Infection *Aminoglycosides b-Adrenergic
Perimenstrual state (gentamycin/streptomycin) antagonists/B-blockers
Pregnancy Gentamicin Calcium channel
Sleep deprivation Streptomycin antagonists

65
 Surgery Ampicillin Iodinated Contrast media
Environmental Stressors Macrolides Magnesium
Emotional stress Erythromycin Methimazole
Pain *Quinolones Prednisone
Temperature extremes (levofloxacin, Ciprofloxacin) Procainamide
Tapering of immune- *Telithromycin Quinidine
modulating Tetracyclines Neuromuscular Jxn
medications Polymyxin Blockers (vecuronium,
Antiepileptics pancuronium, others)
Thyroid hormones *Botulinum toxin
Lithium
D-penicillamine

*blackbox warning or relatively contraindicated unless absolutely necessary

OVERVIEW OF MITOCHONDRIAL DISEASE

Mitochondrial disease patients are among the most complex and challenging of patients. 
The disease is progressive in its course, although the rate of progression is completely
unpredictable.  The clinical presentation of the disease is heterogeneous.  The symptoms
may vary in their severity due to any number of genetic, physiologic and environmental
factors.  The disease is multi-systemic; there may be many potential causes for a
particular symptom. Mitochondrial disease is not one disease but actually constitutes a
large group of disorders.  All are defects in energy metabolism, i.e., the synthesis and/or
utilization of energy generated as ATP through oxidative phosphorylation or aerobic
metabolism.

The Biochemistry, in brief

Oxidative phosphorylation (OXPHOS) includes the breakdown of pyruvate through the
pyruvate dehydrogenase complex supplying acetyl CoA to the Krebs/tricarboxylic acid
cycle.  High-energy electrons are generated and carried by NADH and FADH 2 to the
electron transport or respiratory chain.  A complex series of reactions produces ATP and
water in the presence of oxygen though energy gradients generated at complexes I, III,
and IV across complex V. A defect at any point in energy synthesis can theoretically
impact the production of adequate energy for a cell's needs and can be considered a
"mitochondrial disorder."  There is no consistent correlation between the severity of a
particular biochemical defect and the severity of that patient's phenotype.

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The mitochondrion not only houses the biochemical pathways that comprise oxidative
phosphorylation but also other metabolic systems, including the proximal half of the urea
cycle and fatty acid β-oxidation which results in ketone production.  This can result in
"secondary" hyperammonemia and/or "secondary" fatty acid oxidation dysfunction with
disturbed ketone production .

The Physiology, in brief

All organ systems are dependent on OXPHOS energy production.  The central nervous
system has a lower threshold (relative to other organs) at which it will start to show
evidence of functional impairment.  Furthermore, energy production over the years of a
lifetime becomes less efficient contributing to why middle-aged adults haven't the same
stamina as they did during the teenage years.  It is therefore possible that an affected
organ's function might not be compromised early in life but as more of its cells show
reduced energy production efficiency over time, evidence of dysfunction will become
apparent.

The majority of cases result from abnormalities affecting nuclear-encoded proteins and
processes.  These may impact mitochondrial structure and assembly, cofactor transport,
the membranes onto which the respiratory components are located, or regulation of
mitochondrial DNA (replication, translation).  When mtDNA replication is defective,
mtDNA deletions and mtDNA depletion can occur . In a minority of cases of
mitochondrial disease, the phenotype results from a defect in the maternally-inherited
mitochondrial DNA.  In many of these cases, there exist two populations of DNA within
the mitochondria of the cell - normal (or wild-type) DNA and mutated (dysfunctional)
DNA, a situation termed "heteroplasmy."  Whether or not an organ shows evidence of
dysfunction depends in part upon the ratio of normal-to-mutated mtDNA in any given
organ (the latter being less effective in generating cellular energy). 
Clinical Phenotypes

Since all organ systems are energy-dependent, any one is at risk for becoming
dysfunctional in the setting of reduced energy synthesis. Theoretically then any organ
system might become involved. This presents a challenge to providers who care for
mitochondrial patients - the need to monitor closely an individual over the long term and
screen for treatable complications. On the other hand, it is easy to understand how any
new symptom could be mistakenly attributed to the underlying energy disorder if one

67
always evaluates such a patient through a "mitochondrial disease lens." Intercurrent
illnesses and unrelated medical issues in these patients can be overlooked or
misdiagnosed.
The following is a list of symptoms and complications noted in or associated with
mitochondrial disease by organ system (some symptoms have their source in more than
one organ system):

Central nervous system: seizures, myoclonus,ataxia,hypotonia, spasticity, dystonia,

tremor, movement disorder,"stroke-like" events, hemiparesis, headaches, migraine,
central apnea, developmental delays, developmental regression, dementia, learning
disabilities, autism or autistic-like features, behavioral issues, psychiatric conditions,
coma,sudden death

Peripheral nervous system: pain, numbness

Autonomic nervous system: heat and cold intolerance, temperature dysregulation, with
often abnormal (usually low) baseline temperatures, abnormal sweating, pallor,
blotchiness, mottling of the skin with or without provocation, dizziness, fainting

Muscle: fatigue, exercise intolerance, pain, spasms, tenderness, myopathy, weakness,

myoglobinuria

Eyes: Ptosis, opthalmoplegia, blurry vision, diplopia, retinitis pigmentosa, diminished

vision, optic atrophy

Hearing: hearing loss

Pulmonary: dyspnea, obstructive sleep apnea

Heart: cardiomyopathy, arrhythmia or heart block

GI: anorexia, easy satiety, failure to thrive, abdominal pain, gastroesophageal reflux,
bloating, abdominal distention, pseudo-obstruction, constipation

Liver and Pancreas: hepatomegaly, dysfunction, fatty liver, cirrhosis, coagulopathy

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Kidney and Bladder: renal tubular dysfunction, acidosis (renal Fanconi syndrome), renal
failure, urinary retention, incomplete emptying, urinary tract infections

Endocrine: short stature, diabetes mellitus, hypothyroidism, hypoparathyroidism,adrenal

insufficiency
Skin: pallor, blotchiness, mottling of the skin with or without provocation,
erythromelalgia, easy bruising

Blood: anemia, sideroblastic anemia, neutropenia, thrombocytopenia

Metabolic: metabolic acidosis, lactic acidemia or acidosis, hyperammonemia,

hypoglycemia, low carnitine, fatty acid ß-oxidation dysfunction,postprandial (paradoxical)
ketosis, secondary neurotransmitter abnormalities

The Genetics of Mitochondrial Disease

Mitochondrial disease is actually a group of disorders that vary in their inheritance

pattern.  The majority are caused by defects in nuclear-encoded genes, and follow an
autosomal recessive pattern in which there may be history of affected siblings but
(unrelated) parents are asymptomatic carriers.  Carrier parents have a 1 in 4 or 25%
chance in every pregnancy of having a child with mitochondrial disease.  It should be
noted that some carriers display mild symptoms (Haas, 2007).
A minority of mitochondrial disease cases are associated with mitochondrial or maternal
inheritance, in which an abnormal lesion in the mitochondrial DNA is potentially passed
on from mothers to all offspring.  Affected males do not pass on their genetic
abnormality.  The phenotypic severity in affected individuals can be very broad, even
within the same family.  Upon questioning, there may be a history of family members
with suspicious symptoms (when the diagnosis has not yet been made), but there are
many cases of patients with documented mtDNA mutations who have no notable family
history.

A smaller subset of disorders are associated with x-linked or autosomal dominant

inheritance.  There are also a group of disorders in which the mitochondrial disease has
occurred sporadically with a negligible risk of recurrence.

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 Diagnosis

The classification of mitochondrial diseases is lacking and remains under development. 

Available classifications (e.g., based on molecular etiology or to functional deficit) do not
necessarily have a meaningful clinical application where the patient is concerned. 
Disorders are called by their enzymatic defect, or by the DNA lesion, or by an acronym
(usually describing certain significant symptoms), or by eponyms based on the name of a
clinician or scientist who played a major role in identifying one or more patients with a
particular phenotype.  However, as a rule, there is broad genetic and phenotypic
heterogeneity within this nomenclature system so that the name is not only of limited
meaning to the clinician and patient but it can actually be confusing and even misleading. 
To complicate matters further, an abnormal biochemical or enzymatic finding does not
necessarily imply a primary finding, i.e., a definite diagnosis.  For example, secondary
defects in complex I are well described in patients with primary genetic diagnoses proven
by molecular testing, including Prader-Willi syndrome, velocardiofacial syndrome, Rett
syndrome, Alstrom syndrome, among others.  Current laboratory testing cannot
necessarily differentiate whether or not the biochemical finding is a primary defect or
secondary phenomenon.
Discussions with patients or families must include a comment about this lack of certainty,
and support provided for the anxiety and confusion that arise as a result.  However, it
should also be pointed out that even if the mitochondrial dysfunction is secondary, it
does not mean it is insignificant.  Furthermore, the management approach developed for
primary mitochondrial disease can also be of benefit for those with secondary
dysfunction.

MANAGEMENT OF MITOCHONDRIAL DISEASE

There is no simple way of simply increasing the capacity of the cell to generate energy.
Treatment then demands attention to aspects of care that can increase or promote
energy production and mechanisms for reducing energy losses.

PROMOTING ENERGY PRODUCTION:

1.      Adequate nutrition. Patients with mitochondrial disease who are not well-nourished
may experience significant fatigue and weakness, and may appear as if their underlying
disease is accelerating in its progression. 

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2.      Adequate sleep. During sleep, energy production continues and consumption is
reduced.  Therefore, it is essential that the quality of a person's sleep be maximized. 
When a sleep disturbance is present, improving sleep is a correctible component of
fatigue and can occur in mitochondrial disease, especially obstructive sleep apnea.
3.      Promoting activity. People who exercise and are active show an improvement in
stamina.  Mitochondrial function improves with regular exercise; even mitochondrial
disease patients can gain benefit from regular exercise.  To maximize energy production,
mitochondrial patients must remain active, though exhaustion should be avoided.  This
generally means regular activity with resting periods.  Goals for physical or occupational
therapy should emphasize maintaining muscle tone, mobility and flexibility, and a slow
increase in exercise tolerance; achieving a "normal" level of endurance is unrealistic;
4.      Supplementation with certain vitamins and cofactors (see below) . The use of
vitamins and cofactors is considered standard of care for mitochondrial disease. 
Coenzyme Q10 participates in the normal functioning of the respiratory chain,
transferring electrons from complexes I and II to complex III.  It may also stabilize the
complexes and serves as an antioxidant to clear free radical accumulation, believed to
contribute to the pathogenesis in mitochondrial disease.  By report, coenzyme Q10
appears to improve stamina and reduces fatigue.  Doses up to 10 mg/kg.
L-carnitine (1000 mg TID to QID) may improve stamina and reduce weakness, and reduce
muscle cramping and headaches.  Its side effects include a fishy odor and loose stools (at
high doses) although this can be a beneficial effect in patients who tend toward
constipation; both effects can be reduced by cutting back on the dose.  Creatine may be
beneficial to mitochondrial function; supplementation can help with stamina and improve
muscle pain.
Vitamins E and C are anti-oxidants (like coenzyme Q10).  Vitamin B2 or riboflavin may be
helpful in certain mitochondrial diseases, and can be an effective anti-migraine agent in
some .  Other potential helpful vitamins include thiamin, vitamin K1 (phylloquinone), and
alpha-lipoic acid, depending on the particular defect.
 

REDUCING ENERGY LOSSES:

1.      Prevention of infections. Patients with mitochondrial disease frequently do not
tolerate infections well; these may cause prolonged and often debilitating fatigue and
weakness.  As a result, these patients should be kept up-to-date as far as their

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vaccinations are concerned, and are good candidates for receiving seasonal vaccinations
(e.g., influenza). 
2.      Avoiding excessive physical activity. While physical activity is an important
component of therapy for mitochondrial disease, "overdoing it" produces no benefit and
can leave a patient exhausted, in pain, perhaps nauseous, and feeling miserable.  There
may also be a prolonged recovery period lasting the rest of the day or even several days
following more intensive exertion.
3.      Treating emotional distress.  What is often overlooked is that frequent or persistent
anxiety, depression, or obsessive-compulsive behaviors are also very energy-demanding. 
These should be recognized and treated aggressively.  In the same way, hyperactive
patients may show an overall improvement with medications that improve attention.
4.      Maintaining a suitable ambient temperature. Patients with mitochondrial disease
often don't tolerate extremes of temperature very well, likely due to secondary
autonomic dysregulation. Because patients cannot always regulate well to the
temperature in their environment, it is important that that the ambient temperature be
monitored and controlled whenever possible.  

MONITORING FOR ORGAN DYSFUNCTION:

Mitochondrial diseases are typically multi-systemic since all organs are dependent upon
energy for their function.  Organ dysfunction should be monitored from time to time since
complications are often identifiable and treatable, thereby preventing a patient from
developing symptoms.  Unless a patient has a very specific disease with a predictable
phenotype, monitoring of the following organs is generally routine on a 1-2 year
schedule:

Blood and Urine Testing

1.      Bone marrow involvement - CBC, WBC differential, platelets;
2.      Liver involvement - AST, ALT, bilirubin;
3.      Kidney involvement - BUN, creatinine (blood); urinalysis, urine amino
acids (quantitative);
4.      Muscle involvement - CPK;
5.      Endocrine involvement - thyroid functions; calcium, phosphorus.  Adrenal
insufficiency is a possibility;

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6.      Metabolic status - lactate and pyruvate; carnitine and acylcarnitines; leukocyte
coenzyme Q10; urine organic acid analysis.

Other Testing
1.      Ophthalmological evaluation.  Screen for visual function if concerned;
2.      Audiology testing.
3.      Cardiac evaluation, including EKG, echocardiogram.

MEDICATIONS CONTRAINDICATED IN MITOCHONDRIAL DISEASE

Treatment includes avoidance wherever feasible of drugs and procedures known to have
a detrimental effect. It is not always possible to avoid a certain drug. But when deciding
between one drug possibility and another, one can factor into the decision the
heightened risks associated with a contraindicated or higher risk medication. If the
medication is still essential, the forewarning at least increases alertness to its potential for
creating further problems, allowing for quicker modifications and/or interventions as
needed. 

Sodium Valproate
Statins
Cox-inhibitors
Tetracyclines
Chloramphenicol
Metformin - can interfere with complex I activity
Barbiturates - Inhibits complex I activity at high levels
Benzodiazepines - Inhibits adenosine nucleotide translocase
Lactated Ringer's solution - contains lactic acid
Aminoglycosides are ototoxic for some forms of mitochondrial cytopathies
Ergotamines - impact on hepatic function
Neuroleptic drugs such as Haloperidol, Chlorpromazine, and Thiothixene
Steroids- preferably use only when absolutely necessary, at low dosage and short time
span
Certain types of anesthesia are also contraindicated.

73
Heightened caution should be used in medications that include liver warnings, and an
alternative used when feasible.

Be alert to increased potential for symptom aggravation i.e. medications prone to GI

problems, that depress appetite, that cause added fatigue, etc.

ACUTE MITOCONDRIAL CARE PROTOCOL

Acute State/Illness Examples:

● illness/infection associated with fever
● vomiting and/or extended diarrhea
● unusual lethargy
● lack of improvement using interventions outlined in "minor acute state" i.e.
further signs of: GI dysfunction, dysautonomia, vacillating alertness; surgery;
dehydration; fasting/compromised nutrition etc.

NEVER use lactated ringers.

1) Regardless of the blood glucose value, start IV D10 0.45NS at 1.5 times
maintenance as soon as possible to minimize risk of further metabolic
decompensation and sequelae. (may alter the concentration of sodium based on
sodium concentration in blood). Blood glucose concentration should be kept
above 80 mg/dl. IV infiltrations or other IV interruptions must be remedied
promptly. Metabolic acidosis may require treatment with boluses of sodium
bicarbonate at 1 - 2 mEq/kg IV.
2) IV rate should not be lowered because of mild to moderate hyperglycemia. If
there is a need to control hyperglycemia, insulin should be started and adjusted
to maintain blood glucose between 100 and 150 mg/dl.
3) ***Do not administer IV lipids. Intralipids are CONTRAINDICATED in patients
with Fatty Acid Oxidation Disorders
4) Labs Blood should be sent for glucose, electrolytes, blood ammonia, CBC, BUN,
creatinine, uric acid, LFTs, CPK, blood gases, and urine for myoglobin and ketones-
get a reading for ketones (beta-hydroxybutyrate). Also take samples for the
following: Acylcarnitines,Quantitative Organic Acids - urine sample

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5) Metabolic acidosis
a. Special attention should be paid to this tendency in mitochondrial
diseases
b. ***There is heightened potential for renal tubular dysfunction, more
usually proximal, and associated acidosis with mitochondrial diseases.
c. Anion gap allows for the differentiation of 2 groups of metabolic
acidosis. a) Metabolic acidosis with a high AG - associated with the
addition of endogenously (i.e. lactate, ketones) or exogenously
generated acids. b) Metabolic acidosis with a normal AG - associated
with the loss of HCO3 (ie. GI, proximal RTA) or the failure to excrete H+
from the body.
d. The HCO3 level that is calculated by arterial blood gas represents a more
accurate measure of plasma HCO3 than the serum chemistries
measurement.
e. Measurement of pH and PCO2 by ABG in a patient with low HCO3 allows
one to differentiate metabolic compensation of a respiratory alkalosis
from a primary metabolic acidosis.

6) Ketones- Pitfalls & Solutions:

i. Some metabolic presentations show a tendency for lactic acidosis
as a red flag for altered redox status. Another reflection of redox
status is ketone body molar ratios. Monitoring ketones has
sometimes been more useful in this family's profile
ii. In healthy people the rate of ketogenesis, and therefore the
concentration of acetoacetate (AA) and beta-hydroxybutyrate
(BOHB) in the blood, will decrease after meals. But in some
mitochondrial disease presentations, a paradoxical postprandial
increase can occur
iii. Most urine testing, like Ketostix and Acetest, detect acetoacetate,
which is insufficient. They do not test for the predominant
ketone- beta-hydroxybutyrate. It is possible for the result to be
negative or trace when in fact there are notable levels of BOHB
This can be especially problematic even in non-metabolic patients
such as those with ketoacidosis who cannot convert BOHB to AA
due to shock or liver failure.

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 MORE SPECIALIZED TESTING:
1) Quantitative urine organic and amino acids
2) Free and acyl carnitine in plasma
3) Anaylsis of plasma and urine acylcarnitine
4) Quantitative plasma amino acids
5) Lactate and pyruvate levels.
   
The above 5 tests are in approximate order of priority, although it may depend on the
circumstances. Note for example that in acute protocol, the most important tests to get
immediate samples for would be urinary organic acids and plasma acylcarnitines,
assuming one has to choose only two.

7) Any precipitating factors should be treated aggressively to help minimize further

catabolism. Treatment for any triggering infection should be started as
appropriate. Avoid Aminoglycosides (higher ototoxicity risk) and tetracyclines if at
all possible

8) Because of potential metabolic stress caused by fever, treat with acetaminophen,

and if necessary, ibuprofen. Avoid aspirin if feasible. Use a cooling blanket if
necessary. Due to dysautonomic tendencies with this profile, patient's
temperature regulation may be problematic.

9) ***Carnitor (100 mg l-carnitine/ml) oral solution give 75-100 mg/kg/day,

divided in 3-4 doses. If oral version is not tolerated and/or in the event of GI
dysfunction, more acute states or potentially life-threatening crisis, switch to IV
version of Carnitor Dosage range- 50-100mg/kg/day. Depending on the
circumstances, can administer as 1/2 of daily dose in bolus, followed by
remainder spread over 24 hrs, or daily dose divided into 4 doses or spread over
24 hr. period. Dosage may require some adjustment according to response.
***Note that IV carnitine may burn if infused too quickly and may cause
reversible pain and irritation if it gets under the skin (interstitial). 

10) ***If patient can handle anything orally at all, despite being otherwise npo, it is
also important to continue taking at least TAU, Riboflavin, and Co-Q-10.

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 a. DW-10 and iv carnitor should continue until the patient demonstrates
with some fair reliability that she can handle adequate PO nutrition

Avoidance of fasting when stopping IV. High carbohydrate/low fat diet. Smaller, more
frequent meals plus high complex carb or ‘slow carb’ pre-bedtime snack (occasionally may
include complex carbohydrate in the form of uncooked edible cornstarch
supplementation- ie. added to pudding or yogurt).

Optimize the Supplements :

1) -Carnitor® (100 mg/ml) oral solution (preferable to pills)- start at 3 gm/day,
divided into 3-4 doses. May raise to 4 gm. if needed. ***May increase up to 75-
100 mg/kg during intercurrent illness, increased physiologic stresses, increased
dysautonomic signs, periods of increased GI dysfunction, etc.***
2) Riboflavin- start at 400 mg./day, divided into 3-4 doses. Urine may turn yellow to
orange. If color of urine does not change to a notable degree, increase total daily
dose to 600 mg.
3) "Q-gel" Co-Q-10 (Tishcon manufacturer, pharmaceutical grade) - start at 200 mg
daily, divided in 2-3 doses. Probable target is 300 mg/day. Under chronic care
management conditions, dosage may be raised to this target after 5-7 days at 200
mg. Under acute care conditions, may go directly to 300 mg. (see
assessment/monitoring guidelines).
4) Tri-acetyl-uridine (TAU) - start at 1.5 gm. Daily if available. At this low amount,
can be taken as one dose in am but preferably divided into 2-3 doses. Final target
dosage may range from 1.5- 5 gm. daily, depending on responsivity, first signs of
which would typically take approximately 3-5 days.
Additional "mito-cocktail” recommendations:
1) B-50-complex - 1 capsule bid.
2) d-alpha-tocopheryl - 400 IU/day.
3) l-acetyl-carnitine (aka ALCAR; Biosint manufacturer) - start at 500 mg daily. Can
increase to 500 mg bid.
4) alpha-lipoic acid- 200 mg.daily, (preferably divided into 2 doses).
5) DHA (Martek, Neuromins) - 200-400 mg. daily.

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78
 ALS PEG Tube Placement Admissions

Prior to any PEG placement, respiratory status needs to be addressed. FVC should be at
least 25% expected. If it has been borderline prior to admission, pulmonary should
evaluate the patient pre-PEG. If pulmonary status is stable then patients may be
scheduled for the PEG followed by admission.
In this case, patients will come to floor after having procedure performed by GI. If not
already placed, consult GI unless there is a reason to consult surgery or IR for alternative
method of feeding tube placement. This occurs due to anatomical issues.
Post-Peg admissions are usually a one or two night admission. The justification for an
admission for PEG tube is based on the refeed phenomenon in which ALS patients are
refed calories which are metabolized to CO2 producing a burden on already compromised
respiratory systems.

NPO until cleared by GI for any po intake. Make sure fluids are ordered. Check chart or
call GI fellow so diet and then tube flushes and then tube feeds can be started asap.
Typically the tube is not used for 12-24 hours followed by only hydration and then
increasing percentages of formula.

May need a speech and swallow evaluation – although most of these patients have had
this and are already on modified diets.
Ascertain the caloric needs and nutritional state with an albumin, prealbumin, 24 hour
Urine Urea Nitrogen and a metabolic cart, the metabolic cart is ordered as a pulmonary
function

Consult nutrition and check chart for their tube feed recommendations.
Pain control with Tylenol, NSAIDs, or narcotics as needed. Be aware of respiratory
depression with narcotics.
Most of these patients will be on NIPPV – check with patient for their NIPPVsetting and if
they have not brought their own machine this will need to be ordered. Consult
respiratory therapy to set up NIPPV per patients home protocol.
May need in-exufflator, suction, and nebulizers ordered (See section on Respiratory Care
in ALS).
Routine CBC, CMP, albumen and prealbumin in morning post op. DVT/GI prophylaxis

79
 ALS Respiratory Care
Remember that in ALS there is progressive weakness of inspiratory and expiratory
muscles along with pharyngeal weakness that results in decreased inspiration, increased
atelectasis, poor cough with decreased secretion mobilization, and high risk of aspiration.
As a result, patients have elevating CO2 levels. Routine care includes assessment of FVC
(sitting and supine), MEP and MIP, end tidal CO2, and respiratory symptoms with
initiation of Noninvasive ventilation (NIV) when either the FVC drops to 50% expected,
MIPs are below -60, CO2 is elevating, or the patient is experiencing symptoms of dyspnea.
Remember that NIV should not be utilized within a half hour of eating as food in the
mouth can be dislodged into airways. Additionally, if secretions are a problem or
expiratory pressures have dropped—pulmonary clearance protocols should be adopted.

In general then, when an ALS patient is seen in the ER, keep in mind that he is likely
compromised from the pulmonary standpoint.
A minor cold in an ALS patient can be life threatening when the URI adds even minimal
increases in secretions that must be mobilized.

Check an ABG, CXR, VC, and CBC, SMA20 to look at respiratory status, evidence of
aspiration or pneumonia, hydration status and nutritional status. Take a history that looks
for evidence of respiratory compromise including daytime sleepiness, yawning, morning
headaches, fatigue, or shortness of breath. Listen to the lungs and check respiratory rate,
temp along with a complete exam. Assess the use of accessory muscles. Look for evidence
of paradoxical breathing, accessory muscle use and speech difficulties due to respiratory
compromise.

Ascertain what respiratory devices are used in the home.

Depending on your findings initiate hydration (remember if the patient is dehydrated,
once hydrated the infiltrate will be more obvious), and consider clearance modalities (see
below) and NIV with a pulmonary consult.

Start antibiotics if there is any evidence of infection.

Start pulmonary clearance techniques if there are increased secretions and atelectasis:

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Nebulizer treatments to loosen secretions followed by a percussion vest to mobilize the
secretions to the large airways. Given the weak cough and to prevent secretions blocking
large airways use a coughalator to mobilize the secretions to the upper airways where
they can be suctioned.
Keep the patient hydrated or secretions will be tenacious.
You can use meds for siallorhea but remember they are often anticholinergic
(hyosciamine, atropine, tricyclics, transderm scopolamine, robinul) leading to dry mouth,
thickened secretions in the lung, blurred vision, and confusion in elderly (especially if they
are already compromised with a URI)

If the patient has not had the pneumovac or flu vaccine administer them.

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 LUMBAR PUNCTURE

Indications:
● Emergent: Subarachnoid Hemorrhage, Meningitis, Encephalitis , AIDP / GBS,
ADEM / Demyelinating disease
● Routine: Normal Pressure Hydrocephalus, Benign Intracranial Hypertension, CIDP,
Obtain Cytology, Monitor for chemotherapy, Metabolic disease
Contraindications:
▪ Elevated intracranial pressure with focal signs
▪ Mass effect or midline shift
▪ Platelets < 50K or INR > 1.4
▪ Aspirin, Plavix, Brilinta
▪ Infection over site or anywhere between the CSF and skin where you’d insert
needle
Checklist:
● Head imaging to evaluate for midline shift and above contraindications
● Review Previous Xray Spine/Abd/Chest for hardware or arthritis evaluation
● Platelets and Coags
● Obtain consent
● LP Kit and sterile gloves
● Optional extras: lidocaine, sterile gloves, needle
*Opening pressure is only accurate if measured while patient is in the lateral decub
position with legs extended

Specific Testing:
● Cell Count Tube 1 & 4
● Protein & Glucose
● Viral:
o PCR useful for HSV 1 and 2, VZV, RT-PCR for enteroviruses, HIV-1 RNA,
EBV DNA (seen in primary CNS lymphoma in HIV pts), West Nile DNA,
CMV, HTLV 1 and 2, HHV-6 DNA.
o Intrathecal anti-borrelial antibody for CNS Lyme testing.
● Bacterial:
o Gram stain/culture

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 o Bacterial antigen testing: Neisseria, Strep pneumo, H influenzae type B,
group B strep, E. coli.
o Syphilis testing: VDRL can be false-positive (most common reason is old
age). 10% of elderly individuals age 80 or older have a low titer false
positive VDRL.
▪ If there’s a nonreactive CSF VDRL, an elevated CSF WBC and
protein concentration & a reactive serum treponemal test are
sufficient to treat for neurosyphilis
● Fungal and spirochete:
o PCR useful for HSV 1 and 2, VZV, RT-PCR for enteroviruses, HIV-1 RNA,
EBV DNA (seen in primary CNS lymphoma in HIV pts), West Nile DNA,
CMV, HTLV 1 and 2, HHV-6 DNA.
o Intrathecal anti-Borrelial antibody is the best test for CNS Lyme testing.
o Cryptococcal antigen testing is highly specific and sensitive.
o India Ink / Cryptococcal culture
o Fungal Culture
● Cytology w/ cytospin (try to get large volume and deliver to lab on a weekday
before 4pm)
● Cruetzfeldt-Jakob: 14-3-3 (can be false positive in HSV encephalitis, TB meningitis,
and degenerative disorders)
● CSF Multiple Sclerosis panel
o Immunoglobulin G (IgG) Index: [CSF IgG / CSF Albumin] / [Serum IgG /
Serum Albumin] all in mg/dL
▪ Must have get serum sample for IgG and albumin the same day as
LP
▪ MUST CONVERT ALBUMIN to mg/dL since it is usually reported as
g/dL for formula to work. If albumin=3.7g/dL convert to
3700mg/dL and use in formula.
▪ Increased levels of CSF IgG due to excess production of IgG either
within the central nervous (e.g.MS, neurosyphilis, AIDP, subacute
sclerosing panencephalitis system) or leakage of plasma proteins
into the CSF (e.g.inflammation or trauma).
▪ The index is independent of the activity of the demyelinating
process

83
 ▪ IgG index is positive (elevated) in approximately 80-90% of MS
patients
▪ Normal IgG/albumin in CSF is 0.00-0.21, and in serum is 0.0-0.4
o Oligoclonal bands:
▪ Bands of immunoglobulins produced by B-lineage cells
▪ Positive if there are two or more bands in the CSF that are not
present in the serum. Therefore, a matched serum sample is
required.
▪ Oligoclonal banding in CSF is also positive in approximately 80%
of patients with MS.
▪ The use of CSF index plus oligoclonal banding has been reported
to increase the sensitivity to over 90%
o Myelin Basic Protein: indicate demylination and isn’t specific for MS
o NMO Antibody: Tested in Devic’s

The Traumatic Tap

● Send tubes 1 and 4 for the cell count. If RBC do not decrease by tube 4, & if
xanthochromia→ concern for SAH.
● There should be 1 WBC for every 700-1000 RBC.
● CSF protein concentration will be increased by 1 mg/dl for every 1000 RBC.
● VDRL and PCR for EBV may be falsely positive. PCR for HSV may be falsely
negative.

Isolated Protein Elevation

● The upper range for normal of the lumbar CSF protein is 58 mg/dl.
● Increased CSF protein is a nonspecific abnormality seen in with any disruption of
blood-brain-barrier.
● Differential for elevated protein: meningitis, abscess, neurosyphilis, demyelinating
disease (MS, Guillian-barre), tumor or leptomeningeal carcinomatosis, cerebral
hemorrhage, and post-seizure.
● The highest proteins (in the 2-3 gram range) are due to low CSF flow with partial
obstruction, often tumors.

CSF Pressure

84
 ● Opening pressure should be measured in all patients with headache or AMS, on
their side with legs extended.
● Normal OP: 11 cmH20 in infants, 15cmH2O in children, 18cmH2O in adults, and
25cm H2O in obese adults.
● Differential for low pressure (rare): spontaneous dural tears, prior LP, h/o head
trauma.
● Differential for high pressure: meningitis, tumors, pseudotumor cerebri,
intracranial hemorrhage.

CSF Findings in Meningitis

Test Bacterial Viral Fungal Tubercular
Opening pressure Elevated Usually Variable Variable
normal
WBC >1000 per mm3 <100 per Variable Variable
mm3
Cell differential Mostly PMNs Mostly Mostly Mostly
Lymphs Lymphs Lymphs
Protein Elevated Normal to Elevated Elevated
high
CSF: serum glucose Normal to very Normal Low Low
ratio low

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 ACUTELY ELEVATED INTRACRANIAL PRESSURE

Herniation Syndromes:
1. Subfalcine herniation: cingulate gyrus herniates
under falx cerebri
a. No clear clinical signs; occasional ACA
infarcts causing leg weakness
2. Central hernitation: downward displacement of
brainstem
a. If mild, CN VI palsy
b. If severe, uncal herniation features
3. Transtenotrial/uncal herniation: medial
temporal lobe/uncus displaced through
tentorium
a. Blown pupil, hemiplegia, coma
4. Cerebellar tonsils: respiratory failure, labile BP, death
Causes: mass lesions, hemorrhage, edema

Results: Decreased cerebral blood flow/ischemia because of poor cerebral perfusion

pressure (CPP = MAP-ICP)

General Symptoms:
● Headache, nausea/vomiting
● Altered mental status or irritability
● Visual problems: papilledema (within hours to days), vision loss, diplopia (due to
CN VI palsy), scotoma
● Cushing’s triad: hypertension, bradycardia, irregular respirations
Management:
*Goal: To reduce ICP and maintain CCP >50mmHg so that cerebral blood flow is
maintained
1. Immediately elevate HOB to 300 and keep head straight to keep jugular vein
unobstructed.
2. Intubate and hyperventilate to pCO2 of 25-30mHg

86
3. Start IV Mannitol (1g/kg bolus, then 0.25mg/kg q6hrs) or hypertonic saline.
Maintain serum osmolarity of 300-310, as well as blood pressure and volume
status.
4. Call neurosurgery for possible ventricular drain
5. If other measures fail, last resort options: hemicraniectomy, barbituate
coma, empiric steroids to reduce swelling, hypertonic bulle

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 ACUTE HEADACHE SYNDROMES

Presentation:
1. Meningitis: diffuse, photo/phono, meningismus, N/V, fever, malaise, irritability
2. Encephalitis: like meningitis, plus altered mental status, seizures, focal features
3. Subarachnoid hemorrhage: sudden-onset, WHOL, 10/10, meningismus
4. Pseudotumor cerebri (BIH): diffuse, photo, tinnitus, vision loss, V1 palsy + wt
gain/VitA/meds
5. Carotid dissection: periorbital/auricular throbbing (carotidynia), Horner’s, TMB,
focal features
6. Temporal Arteritis: achy, jaw claudication, myalgias, ESR>age/2 +10, wt loss
7. Hypertensive encephalopathy: frontal/occipital, throbbing, +/- confusion
8. Venous sinus thrombosis: diffuse or focal, seizure, meningismus
9. Optic neuritis/myositis: painful eye movements +/- vision loss, F>M
10. Migraine: moderate unilateral, F>M, pulsatile, photo/phono, N/V, transient visual
obscurations, scintillating scotomas, fortification spectra, +family history,
+menstrual, +triggers. Subtypes: common (no aura, 50%), classic (aura, 50%),
complicated (focal features, <5%), retinal (monocular vision loss), vertiginous,
acephalgic (aura without headache)
11. Cluster: severe unilateral, M>F, daily x wks-months, photo/phono, autonomic
features (Horner’s, tearing, rhinorrhea)
12. Tension-type: bilateral, pressure, “hatband”, shoulder/neck tightness
13. Occipital Neuralgia: unilateral over occipital region, to eye, lasts seconds
14. Paroxysmal Hemicrania: like clusters in women, indomethacin-responsive
15. SUNCT syndrome: “short-lasting, unilateral, neuralgiaform, conjunctival injection
and tearing”

Workup
● R/O meningitis/encephalitis: HCT then LP (determine
aseptic/viral/bacteria/fungal/etc.), broad tx.
● R/O SAH: Head CT then LP. (HCT less likely to see blood after 48-72 hrs)
● R/O Pseudotumor: HCT then LP. Check OP>20 in decubitus position.
● R/O dissection: MRI/A neck c/s gad with T1 fat sats, add MRI brain to r/o
infarction

88
 ● R/O temporal arteritis: TA bruit/tenderness/pulselessness, if ESR elevated then
steroids and biopsy
● R/O hypertensive encephalopathy: watch BP, if focal or AMS then MRI to see
RPLS.
● R/O VST: HCT with contrast (“empty delta sign”) vs MRV
● R/O optic neuritis/myopathy: HCT then MRI brain/orbits c/s gad, CK, admit for IV
steroids for ON
● R/O acute migraine: tension-type continuum: consider HCT if new HA syndrome

When to order MRV with MRI

● Pregnant or post pregnancy with new onset headaches
● New onset visual complaints and worst headache (pseudotumor-type picture
with loss of b/l peripheral vision- concern that pseudotumor is actually high
pressure caused by thrombus)
● Any patient with headache and papilledema. 
● Any headache patient who has neurological findings
● Any patient at risk for thrombus (hypercoagulable pt: oral contraceptives, recent
long flight and has been dehydrated, cancer patient, autoimmune history).

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 MIGRAINE ACUTE TREATMENT
ER Cocktail: Toradol 30mg iv, Decadron 10mg iv, Depakote 1g iv, Magnesium 1gram IV
and Benadryl 50mg iv
If refractory try Reglan 10mg iv or Compazine 10mg iv and Depakote 1g IV

DHE Protocol:
Tests:
● Baseline EKG, reviewed by MD before starting medications
● CBC with differential, Basic Metabolic Panel, Liver Function Tests, Magnesium,
Phosphorus
● Beta HCG in a female patient who is still menstruating
● Daily EKG
Test dose:
● Diphenhydramine (Benadryl) 25mg IVPB
● Once diphenhydramine complete, prochlorperazine (Compazine) 10mg IVPB
● Wait 30 minutes
● DHE test dose of 0.5mg IVP (over 2 minutes)
● Monitor Blood pressure 5 minutes after DHE. If BP changes by < or > 20 mm at
anytime after DHE, or pulse changes by < or > 20mm, notify MD to lower DHE dose for
next round.
Maintenance dose: every 8 hours
● Diphenhydramine (Benadryl) 25mg IVPB Q8hours
● Once diphenhydramine complete, prochlorperazine (compazine) 10mg IVPB
Q8hours
● Wait 30 minutes
● DHE 1mg IVP (over 2 minutes) Q8 hours
● Monitor Blood pressure 5 minutes after DHE. If BP changes by < or > 20 mm at
anytime after DHE, or pulse changes by < or > 20mm, notify MD to lower DHE dose for
next round.
PRN Meds:
● Diphenhydramine 25mg IVPB Q8hours prn extrapyramidal side effects
● Cogentin 1mg IVPB Q8 hours prn extrapyramidal side effects

90
 ABORTIVE ORAL HEADACHE TREATMENT
STEROIDS Medrol dose pack

Prednisone taper 6mg to 4mg to 2mg

NSAIDS MOA: Block neurogenic inflammation by a direct effect on dural blood vessels. Inhibition of COX1 or
COX2, preventing prostaglandin synthesis. Interfere with cell adhesion. Some decrease the synthesis of
ASA leukotrienes by inhibiting 5-lipooxygenase.
Naproxen Indications: Chronic paroxysmal hemicranias, menstrual migraines, hemicrania continua, exertional
Ibuprofen migraine, benign organic cephalgia, ice pick HA
Indomethacin
Diclofenac Adverse effects: Gastric toxicity (d/t suppression of prostaglandin synthesis via COX1 inhibition).
Ketorolac Contraindications: GI blood loss, renal dysfunction, worsening HTN, colitis

Notes: Use ASA, naproxen, ibuprofen, and diclofenac for non-disabling HA. Ketorolac IM for
disabling HA. AAC more effective than plain Tylenol, ASA, or NSAID. Diclofenac very effective.
Triptans MOA: Selective serotonin agonist
Indication: Moderate to severe disabling migraine
Sumatriptan
Zolmitriptan Adverse effects: Dizziness, heaviness, neck pain, dysphoria – usually abate within 45 min
Naratriptan Contraindications: Ischemic heart dx (CVD), Prinzmetal’s, uncontrolled HTN, vertebrobasilar
Rizatriptan migraine
Almotriptan
Eletriptan Notes: Good in combo with naproxen. Lower risk for rebound than with Ergots. Check EKG in
Frovatriptan patients over 40 or with risk factors prior to initiating. Sub-cat of tension HA who develop migraine
will benefit from triptans, but plain tension HAs do not. Take off if uncontrolled BP >140/90.

Antiemetics MOA: Antidopaminergic

Indications: Good for nausea AND can help decrease headache
Metoclopramide
Proclorperazine Adverse effects: Drowsiness, sedation (most frequent). Acute dystonic reactions, akathisia (rare)
Chlorpromazine
Notes: Metoclopramide+NSAID/triptan more effective than monotherapy. Zofran is not effective.
Ergotamine MOA: Stimulates arterial and venous smooth muscle constriction via serotonin receptors. Inhibit
reuptake of noradrenaline at sympathetic nerve endings
Take up to 6 of the Indication: Moderate to severe migraine
1mg tabs in 24 hrs
no more than Adverse effects: Nausea and vomiting; rebound headache
2x/week Contraindications: Renal/hepatic failure, pregnancy, HTN, sepsis, CAD, PVD

Notes: Inferior to triptans. May need to pre-treat for nausea. Methergine is an ergotamine derivative.
DHE MOA: Weak arterial vasoconstrictor but potent venoconstrictor
Indication: Moderate to severe migraine
1 spray into each
nostril, repeat in 10 Adverse effects: Nausea, vomiting
min and again in 2 Contraindications and notes similar to Ergots. Lower risk for rebound than Ergots.
hours
Notes: Works well in combo with methergine
Dopamine Blocking Zyprexa qhs (up to 20mg) x5 days, tell them to start with 5mg tab, take one every
hour until they fall asleep or headache resolves or they max out on 4 tabs, next night
take whatever it took the first night all at once. Counsel on weight gain and sedation.
The myriad other SE of zyprexa are minimal with a 5d course .

MIGRAINE PROPHYLACTIC TREATMENT

TCAs Amitriptyline MOA: Antidepressant. Increases synaptic NE or serotonin by

91
 Notriptyline inhibiting high-affinity re-uptake

Starting dose: 10-30mg QHS Adverse effects: Sedation, dry mouth, metal taste, constipation,
Up to: 150mg dizziness, confusion, tachycardia, palpitations, blurred vision, urinary
retention

Notes: If too sedating, switch from amitriptyline to nortriptyline.

Give in AM if they have insomnia/nightmare.
SSRI/SNRI Indications: Helpful for co-morbidity of depression

Fluoxetine Adverse effects: SNRIs – causes more nausea and are more activating. Weight gain (but less SE than TCA)
Venlafaxine
Topiramate (Topamax) Adverse effects: Parethesia, fatigue, nausea, anorexia, abnormal taste,
weight loss, somnolence, difficulty concentrating, anxiety, renal
Start: 25mg/day, increase by 25mg calculi
weekly, to 50mg BID
Up to: 200mg/day Contraindications: Acute myopia with secondary angle closure
glaucoma (can occur – need to discontinue med immediately)
Valproic Acic (Depakote) MOA: Increases GABA by inhibiting its degradation, turns off firing
of 5-HT neurons which cause head pain
Anti-
Starting dose: 500mg nightly
epileptics
Up to 1500mg nightly AE: Nausea, vomiting, GI distress – self-limiting and less common if
written as divalproex sodium vs sodium valproate. Tremor, alopecia,
weight gain.
Gabapentin Adverse effects: Dizziness, giddy, drowsy
600-1800mg/day

Levetiracetam Adverse effects: Psychosis

1000-3000mg/day over BID
Beta Propranolol (160-240mg/day start MOA: Inhibition of B1 mediated mechanisms resulting in inhibition
blockers with 40mg) of NE release
Timolol, Atenolol, Metoprolol,
Nadolol Contraindications: Migraine with aura if clear stroke risk exists.
Don’t use with Raynaud’s or asthma. Or DM due to masked
hypoglycemia symptoms
Calcium Verapamil MOA: Block 5-HT release, interfere with neurovascular
channel Diltiazem (Cardizem) inflammation, interfere with initiation and propogation of spreading
blockers Nifedipine (Procardia) depression

Adverse effects: Constipation, dizziness, nausea, hypotension, HA,

edema, AV block

Alternative Feverfew MOA: Nonspecific NE, 5-HT, bradykinin, prostaglandin, and

50-200mg/day acetylcholine antagonist
Adverse effects: Mouth ulceration
Riboflavin MOA: Plays a role in improving oxygen metabolism possibly
200mg BID impaired in migraines
Adverse effects: Diarrhea and polyuria
Butterbur 50-75mg BID
HEADACHE IN PREGNANCY
Primary Headaches

92
 ● Migraine: In general, retrospective studies have suggested that migraine
frequency and severity decreased during pregnancy, particularly during the 2 nd
and 3rd trimesters. A prospective study of 49 patients showed 79% had a
complete remission of headaches by the third trimester. By one month
postpartum, 50% had recurrence of migraine.
● Tension headaches: No change in frequency during pregnancy.
Secondary Headaches
● Pseudotumor cerebri: see other section
● Pre-eclampsia: new hypertension>140/90, proteinuria>300mg/dL, headache may
precede development of serious neurologic sequalae (ie. Eclampsia with seizures)
● SAH: Incidence may be higher in pregnancy (between 0.9-5/10,000); during the
six weeks postpartum risk of SAH increases 28 fold.
● Cerebral Venous Thrombosis: Occurs in 1/2500- 10,000 deliveries; especially
likely if associated hypercoagulable state (protein C/S or antithrombin III
deficiency, antiphospholipid antibody syndrome). Can occur upto 2-8 weeks
postpartum as well.
● Postdural Puncture/Non postdural puncture: can occur with epidural and spinal
anesthesia (CSF leak, pneumocephalus, other complications-subdural hematoma,
aseptic/septic meningitis).
Workup:
● Worrisome features: new-onset headache, change in pattern, visual changes,
speech abnormalities, focal neuro signs/symptoms, meningeal signs, trauma,
seizure, history of malignancy.
● Detailed headache history and neurologic ROS, medications, diet.
● Vitals, neuro exam (including fundoscopic exam)
● Labs (urinalysis, pre-eclampsia labs)
● Imaging: Avoid gad if at all possible. If you suspect SAH then non-contrast head
CT (sensitivity of 90% in first 24hr of onset); it takes 50-100 Head CTs to reach a
concerning level of radiation exposure (5 rads). If you suspect venous sinus
thrombosis then MRI/MRV (although if necessary, a CT venogram can be done).
The national radiological protection board advises against MRI in the first
trimester, though this isn’t supported by evidence.
● LP: if imaging reveals no hemorrhage and the suspicion remains high, lumbar
puncture is the only way to exclude SAH. Also indicated if suspicious of
meningitis.

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Treatment in Pregnancy
Abortive:
● Step one: Tylenol. 
● Step two: Excedrin tension type headache- which is caffeine and tylenol, no
aspirin
● Step 3 is percocet or vicodin- safe during pregnancy and one of the few times we
recommend use of an opiod. 
● If they come to the ER, you can give 1
o 2 Liters normal saline, which often helps and
o 1 gram magnesium sulfate, which may help. 
o Sometimes we use IV or po steroids, but only after speaking with their OB
(whom you may curbside) because safety depends on which trimester
they are in. 
● Nerve blocks using lidocaine only, that is safe in every trimester.
● ALL OTHER MEDS NEED TO BE APPROVED BY PT’s OB
Prophylactic:
● hydration and daily excercise with good rest to avoid frequent headaches.
● labetelol is OK - class B but check with attending.

94
 VERTIGO
History: Acute or insidious onset? Duration? Positional? Other symptoms (n/v, hearing
loss, tinnitus, HA, ataxia)
Physical Exam: Otoscopy, nystagmus, Dix-Hallpike, head impulse test (or cold calorics
Workup: head CT, or if high suspicion brain MRI
Management:
● BPPV: Epley maneuver, symptomatic treatment (meclizine/clonazepam prn),
reassurance
● Labyrinthitis: symptomatic treatment, outpatient follow-up
● Meniere’s disease: low-salt diet, long term management with diuretics,
symptomatic treatment
● Stroke: Admit to hospital! Stroke w/u
Localizing Vertigo: Peripheral Vs. Central Lesions
Nystagmus VORs Differential Onset Hearing loss
Labyrinthitis Over hours No
P
e
r Acute, episodic No
i Unidirectional, BPPV
p fatiguable,
Abnormal
h +latency,
e adaptable. Gradual,
Meniere’s disease Yes
r episodic
a
l Gradual,
Acoustic Neuroma Yes
progressive

C Cerebellar lesion
hyperacute No
e (stroke, tumor)
Can be multi-
n
directional,
t Normal
Not fatiguable Vertebrobasilar Possible
r hyperacute
No latency ischemia (int. auditory a.)
a
l
Seemungal, Barry and Bronstein, Adolfo. Practical Approach to Acute Vertigo. Practical Neurology 2008;8:
211-221

95
 Dix-Hallpike Maneuver (#1 & #2)
● Sit pt upright w/ legs extended
and head rotated 45 deg
● Lie pt down quickly, head
extended 20 deg past horizontal
● Observe for nystagmus for 45 sec
(in bppv starts after 10 secs, is
torsional, fast toward lowered ear,
and fatiguable)
Epley Maneuver (#1-#5)
● Do Dix Hallpike but at #2 hold
head in position for 30 secs
● Turn head 90 deg toward
affected side for 30 sec
● Turn head another 90 deg so pt is
nearly face down
● Sit pt back up abruptly and keep
pt upright
● If sx improved, pt should remain at upright at 45 deg for 24hr
● If still symptomatic, repeat procedure
Head impulse test
● Have pt fixate and rotate head abruptly
● If pos/abnml, pt will lose fixation and correct w/ saccade

ogy Today:Volume 8(11)5 June 2008pp 1,10-11

96
 DIAGNOSTIC CRITERIA FOR BRAIN DEATH
(AAN guideline update for brain death)

Checklist for determination of brain death Prerequisites (all must be checked)

● Coma, irreversible and cause known
● Neuroimaging explains coma
● CNS depressant drug effect absent (if indicated toxicology screen; if barbiturates given, serum
level < 10 µg/mL)
● No evidence of residual paralytics (electrical stimulation if paralytics used).
● Absence of severe acid-base, electrolyte, endocrine abnormality
● Normothermia or mild hypothermia (core temperature > 36°C)
● Systolic blood pressure 100 mm Hg
● No spontaneous respirations
Examination (all must be checked)
● Pupils nonreactive to bright light
● Corneal reflex absent
● Oculocephalic reflex absent (tested only if C-spine integrity ensured)
● Oculovestibular reflex absent
● No facial movement to noxious stimuli at supraorbital nerve, temporomandibular joint
● Gag reflex absent
● Cough reflex absent to tracheal suctioning
● Absence of motor response to noxious stimuli in all 4 limbs (spinally mediated reflexes are
permissible)

Apnea testing (all must be checked)

● Patient is hemodynamically stable
● Ventilator adjusted to provide normocarbia (PaCo2 34–45 mm Hg)
● Patient preoxygenated with 100% FiO2 for > 10 minutes to PaO2 > 200 mm Hg
● Patient well-oxygenated with a PEEP of 5 cm of water
● Provide oxygen via a suction catheter to the level of the carina at 6 L/min or attach T-piece
with CPAP at 10 cm H2O
● Disconnect ventilator
● Spontaneous respirations absent
● Arterial blood gas drawn at 8–10 minutes, patient reconnected to ventilator
● PCO2 60 mm Hg, or 20 mm Hg rise from normal baseline value
OR:
● Apnea test aborted

97
Ancillary testing (only 1 needs to be performed; to be ordered only if clinical examination cannot
be fully performed due to patient factors, or if apnea testing inconclusive or aborted)
● Cerebral angiogram
● HMPAO SPECT
● EEG
● TCD

98
 PERIPHERAL NEUROANATOMY

ACTION TESTED ROOTS NERVES MUSCLES

CRANIAL
Closure of eyes,pursing of lips,showing
teeth CN 7 Facial Orbicularis oculi and oris
Levator palpebrae,
Elevation of eyelids, movement of eyes CN 3,4,6 Oculomotor, troclear, abducens extraocular
Closure and opening of jaw CN 5 Motor Trigeminal Masseters, Pterygoids
Protrusion of the tongue CN 12 Hypoglossal Lingual
Phonation and swallowing CN 9/10 Glossopharyngeal, vagus Palatal, laryngeal, pharyngeal
CN 11 &
Elevation of shoulders, turning of head cerv. Spinal accessory Trapezius, SCM
BRACHIAL
Adduction of extended arm C5/6 Brachial Plexus Pectoralis Major
Fixation of Scapula C5/6/7 Brachial Plexus Serratus Anterior
Initiation of abduction of arm C5/6 Brachial Plexus Supraspinatus
External rotation of flexed arm C5/6 Brachial Plexus Infraspinatus
Abduction and elevation of arm to 90 deg. C5/6 Axillary Nerve Deltoid
Flexion of supinated forearm C5/6 Musculocutaneous Biceps/Brachialis
Extension of forearm C6/7/8 Radial Triceps
Extension (radial) of wrist C6 Radial Extensor carpi radialis longus
Flexion of semipronated arm C5/6 Radial Brachioradialis
Adduction of flexed arm C6/7/8 Brachial Plexus Latissimus Dorsi
Supination of forearm C6/7 Posterior Interosseous Supinator

99
 PERIPHERAL NEUROANATOMY

ACTION TESTED ROOTS NERVES MUSCLES

CRANIAL
Closure of eyes,pursing of lips,showing teeth CN 7 Facial Orbicularis oculi and oris
Oculomotor, troclear,
Elevation of eyelids, movement of eyes CN 3,4,6 abducens Levator palpebrae, extraocular
Closure and opening of jaw CN 5 Motor Trigeminal Masseters, Pterygoids
Protrusion of the tongue CN 12 Hypoglossal Lingual
Phonation and swallowing CN 9/10 Glossopharyngeal, vagus Palatal, laryngeal, pharyngeal
CN 11 &
Elevation of shoulders, turning of head cerv. Spinal accessory Trapezius, SCM
BRACHIAL
Adduction of extended arm C5/6 Brachial Plexus Pectoralis Major
Fixation of Scapula C5/6/7 Brachial Plexus Serratus Anterior
Initiation of abduction of arm C5/6 Brachial Plexus Supraspinatus
External rotation of flexed arm C5/6 Brachial Plexus Infraspinatus
Abduction and elevation of arm to 90 deg. C5/6 Axillary Nerve Deltoid
Flexion of supinated forearm C5/6 Musculocutaneous Biceps/Brachialis
Extension of forearm C6/7/8 Radial Triceps
Extension (radial) of wrist C6 Radial Extensor carpi radialis longus
Flexion of semipronated arm C5/6 Radial Brachioradialis
Adduction of flexed arm C6/7/8 Brachial Plexus Latissimus Dorsi
Supination of forearm C6/7 Posterior Interosseous Supinator
Extension of proximal phalanges C7/8 Posterior Interosseous Extensor digitorum
Extension of wrist (ulnar side) C7/8 Posterior Interosseous Extensor carpi ulnaris
Extension of proximal phalanx of index fing. C7/8 Posterior Interosseous Extensor Indicis
Abductor pollicis longus and
Abduction of thumb C7/8 Posterior Interosseous brevis
Extensor pollicis longus and
Extension of thumb C7/8 Posterior Interosseous brevis
Pronation of forearm C6/7 Median Nerve Pronator Teres
Radial flexion of wrist C6/7 Median Nerve Flexor Carpi Radialis
Flexion of middle phalanges C7/C8/T1 Median Nerve Flexor Digitorum Superficial
Flexion of proximal phalanx of thumb C8/T1 Median Nerve Flexor Pollicis Brevis
Opposition of thumb against fifth finger C8/T1 Median Nerve Opponens Pollicis
Extension of middle phalanges of
index/middle fingers C8/T1 Median Nerve First, second lumbricals
Anterior Interosseous
Flexion of terminal phalanx of thumb C8/T1 Nerve Flexor Pollicis Longus
Flexion of terminal phalanx of 2nd and 3rd Anterior Interosseous
fingers C8/T1 Nerve Flexor Digitorum Profundus
Flexion of distal phalanges of ring and little C7/C8 Ulnar Flexor Digitorum Profundus

100
 fingers
Adduction and opposition of fifth finger C8/T1 Ulnar Hypothenar
Extension of middle phalanges of ring and
little fingers C8/T1 Ulnar Third/ Fourth lumbricals
Adduction of thumb against second finger C8/T1 Ulnar Adductor Pollicis
Flexion of proximal phalanx of thumb C8/T1 Ulnar Flexor Pollicis Brevis
Abduction and adduction of fingers C8/T1 Ulnar Interossei
CRURAL
Hip flexion from semiflexed position L1/2/3 Femoral Iliospoas
Hip flexion from externally rotated position L2/3 Femoral Sartorius
Extension of knee L2/3/4 Femoral Quadriceps Femoris
Adductor longus, magnus,
Adduction of Thigh L2/3/4 Obturator brevis
Superior
Abduction and int. rotation of thigh L4/5/S1 Gluteal Gluteus medius
Inferior
Extension of thigh L5/S1/S2 Gluteal Gluteus Maximus
Biceps, semitendinosis,
Flexion of knee L5/S1/S2 Sciatic semimembranosis
Peroneal
Dorsiflexion of foot (medial) L4/5 (deep) Anterior Tibial
Peroneal Extensor digitorum longus and
Dorsiflexion of toes (proximal and distal phalanges) L5/S1 (deep) brevis
Peroneal
Dorsiflexion of great toe L5/S1 (deep) Extensor hallucis longus
Peroneal
Eversion of foot L5/S1 (superficial) Peroneus longus and brevis
Plantar flexion of foot S1/S2 Tibial Gastrocnemius, soleus
Inversion of foot L4/5 Tibial Tibialis Posterior
Flexion of the toes (distal phalanges) L5/S1/S2 Tibial Flexor digitorum longus
Flexion of the toes (middle phalanges) S1/S2 Tibial Flexor digitorum brevis
Flexion of great toe (proximal phalanx) S1/S2 Tibial Flexor hallucis brevis
Flexion of great toe (distal phalanx) L5/S1/S2 Tibial Flexor hallucis longus
Contraction of anal sphincter S2/3/4 Pudendal Perineal muscles

101
 LOCALIZATION TIPS
Disc Root Motor Fx. Sensory Reflex
C5/6 C6 Biceps/wrist ext. Medial arm & hand Biceps
C6/7 C7 Triceps/wrist flex Middle finger Triceps
C7/T1 C8 Finger flex Lat. Hand Finger
L3/4 L4 Quadriceps Med. Calf Knee
L4/5 L5 Dorsiflexors Med. Foot
L5/S1 S1 Plantar Flexors Lat. Foot Ankle

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 ROOT Vs. NERVE LESION IN EXTREMITIES

BOTH ROOT ONLY NERVE ONLY

C7 vs. radial Weakness in Wrist flexor weakness Weakness of
triceps and wrist Middle finger sensory loss brachioradialis and thumb.
extensors Index finger sensory
Loss of triceps changes
reflex
C8 vs. Weakness of flexor Flexor digitorum profundus No flexor digitorum
median digitorum weak profundus weakness.
superficialis (in ring and little finger) Sensory loss confined to
Sensory loss in 5th digit, the first three digits and
heel, and median forearm heel of the hand
T1 vs. ulnar Intrinsic hand Weakness of adductor Weakness of flexor carpi
weakness pollicis brevis ulnaris
Pain in shoulder/axilla Pain in the ring and little
finger
L5 vs. Foot drop Little weakness of foot Foot eversion weakness,
common eversion, sensory change in Intact inversion
peroneal buttocks

SPINAL CORD LESIONS

Localization Symptoms and Signs
Craniocervical Junction Neck pain, head tilt, down-beat nystagmus, UMN weakness x 4
Cervical Spinal Cord Neck pain, LMN in UE at level of lesion + B/L UMN below, sensory level, bowel/bladder
Thoracic Spinal Cord Back pain, radicular signs, spastic paraparesis, sensory level, bowel/bladder
Cauda Equina Syndrome Low back and perineal pain, flaccid paraparesis, areflexic bowel/bladder
Conus Medullaris Areflexic bowel/bladder, minimal LE weakness, perineal sensory loss
Anterior Cord Syndrome Motor and spinothalamic tract (pain/temp) with spared position sense, bowel/bladder
Central Cord Syndrome Suspended sensory level (pain/temp), UMN weakness below, intact position sense
Ipsilateral UMN weakness and position sense below lesion, contralateral pain and
Brown-Sequard Syndrome sensory loss below lesion

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104
 HIGH YIELD NERVE FACTS
● Median Nerve innervates the following muscles of the hand:
o Lumbricals I and II
o Opponens Pollicis
o Abductor Pollicis Brevis
o Flexor Pollicis Brevis, Flexor Carpi Radialis, and Flexor Digitorum
Superficialis
● C7 dermatome- middle finger
● Arm abduction: 1st 15 degrees: supraspinatus (suprascapular nerve; C5,C6)
▪ Up to 120 degrees: Deltoid (axillary nerve C5,C6)
▪ Beyond 120 degrees: Trapezius (spinal accessory nerve)
● Suprascapular nerve (C5/6) supplies the supraspinatus (arm abduction) and
infraspinatus (external rotation at the shoulder with the elbow flexed)
● Long thoracic nerve (C5/6/7) = serratus anterior = scapular winging when injured.
● Most common cause of iliopsoas abscess = TB
● Meralgia paresthetica is a lateral femoral cutaneous neuropathy (L2/3) often seen
in acute weight gain (obesity/pregnancy) and/or obstetric surgery
● L5 radiculopathy vs. peroneal neuropathy: foot drop is present in both; in
peroneal neuropathy foot inversion is intact (tibialis posterior)
● Innervation of Biceps Femoris; Long head = femoral nerve; short head = common
peroneal nerve above the knee; this muscle flexes the leg at the knee
● Crutches can cause posterior cord or radial nerve injury
Polyneuropathy differential diagnosis “DANG THERAPIST”
(Adapted from U of R “Neurology Survival Guide” by Eric Hanauer)
● Diabetes
● Alcohol
● Nutritional: B12, folate, B6 defic/excess, Vit E, niacin
● GBS and other inflammatory demylinating PN: CIDP, MMN
● Toxins: metals, antibiotics (dapsone, chloroquine, macrobid, ethambutol, flagyl,
INH, ddI, ddC, chloramphenicol), AEDs (phenytoin), chemo (cisplatin, etoposides,
taxol, vincristine), immunosuppression (cyclosporin, tacrolimus, gold,
thalidomide, colchicine), other (amiodarone, amphetamine, heroin, hydralazine,
disulfuram, NO, B6, CN, tryptophan)
● Hereditary (and metabolic hereditary disorders)
● Entrapment (and hereditary predisposition to pressure palsies)

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● Rheum: connective tissue disease, vasculitis, sarcoid
● Amyloid
● Porphyria
● Infection: viral, bacterial, parasitic
● Systemic: uremia, endocrine, heme-onc, hepatic
● Tumor: compression, invasion, paraneoplastic, and treatment related (XRT, meds)
Neuropathy differential diagnosis
Acute: GBS, vasculitis, porphyria, toxins (organophosphates, arsenic, thalidomide),
tick, drugs
Stepwise (mononeuritis): Vasculitis (PAN, Sjogrens, Churg-Strauss, Wegener’s,
hypersensitivity, cryoglobulinemia, SLE, RA, isolated PNS vasculitis), DM, CIDP,
infectious (Lyme, HIV, HepC, cryos, leprosy), infiltration (sarcoid, granuloma,
leukemia/lymphoma)
Predominantly Motor: lead, porphyria, hereditary, GBS/CIDP, MMN, dysproteinemia
Pure Sensory: paraneoplastic (anti-Hu), Sjogren, Toxic (cisplatin, B6), HIV, anti-
sulfatide Ab, Friedreich’s, idiopathic
Predominantly Autonomic: GBS, DM, Amyloid
Small Fiber Neuropathy: DM, amyloid, toxic (EtOH), hereditary, Tangier, Fabry, AIDS,
idiopathic

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 NEURO-ONCOLOGY

Most Common tumors

In adults: Metastasis, as for primaries: GMB, meningioma and astrocytoma.
In Children: Astrocytoma, medulloblastoma, ependymoma
Common Metastasis to the Brain:
● Lung, Breast, adenocarcinoma of the colon, renal cell Ca, and melanoma
● Metastasis that tend to bleed: Melanoma, Thyroid Cancer , Renal cell Ca,
Choriocarcinoma, Retinoblastoma
● Breast and Lung cancer most commonly bleed but due to frequency of cancer not
its tendency to bleed
Cerebellopontine Angle Masses
vestibular schwannoma (75%); meningioma (10%), epidermoid (5%), facial nerve
schwannoma (4%), aneurysm (vertebral, basilar, PICA), stem glioma; arachnoid
cyst, paraganglioma, hematogenous metastasis, subarachnoid seeding, lipoma,
hemangioma, choroid plexus papilloma, ependymoma, desmoplatic
medulloblastoma
Suprasellar Masses
● Sellar/Parasellar neoplasm - pituitary adenoma/Sarcoid
● Aneurysm /Arachnoid cyst
● Teratoma
● Craniopharyngioma
● Hamartoma of tuber cinereum/Histiocytosis/Hypothalamic glioma
● Meningioma / Metastasis (to infundimbulum)
● Optic nerve glioma - erodes sphenoid - visual loss
● Epidermoid or Dermoid inclusion cyst
Calcified Tumors
● Metastases- chondrosarcoma, mucinous adenocarcinoma, breast, ovarian,
stomach cancers, ostesarcoma
● Primary CNS tumors- astrocytomas, choroid plexuspapilloma,
craniopharyngioma, ependymoma, ganglioma, meningioma, neurocytoma,
oligodenroma, pineal region tumors

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 PARANEOPLASTIC SYNDROMES

Recognized Ab Clinical Syndrome Antigens Bound Associated Tumors

Anti-Hu Encephalmyelitis, Sensory RNA binding proteins SCLC, Neuroblastoma,
(ANNA-1) neuropathy (HuD, HuC, HEL-N1) Prostate Ca
Anti-Ri (ANNA- Opsoclonus, Ataxia CNS neuronal RNA- Breast Ca, Ovarian,
2) binding proteins SCLC
ANNA-3 Encephalomyelitis, Purkinje cell nuclear SCLC
Neuropathy antigen
Anti-Yo (PCA-1) Cerebellar degeneration Purkinje cell cdr2 Ovarian, Breast Ca
protein
Anti-Ma Cerebrellar/brainstem, Ma-1- SCLC, Ma-2 and Testicular germ cell Ca
limbic encephalitis Ma-3
Anti-Recoverin Retinopathy Recoverin (Ca binding SCLC
protein)
Anti-Tr Cerebellar ataxia Purkinje cell protein Hodgkin’s Lymphoma
Anti-CRMP-5 Chorea, Optic neuropathy Collapsin response SCLC, Thymoma
mediator protein

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Anti-VGCC Lambert Eaton, Myasthenic P/Q type voltage SCLC
syndrome gated calcium channel
Anti- Stiff Peroson Syndrome C-terminus of synaptic SCLC and Breast Ca
Amphiphysin vesicle protein
Anti-VGKC Neuromyotonia, seizures, Voltage gated K Thymoma
limbic encephalitis channel
Anti-MAG Polyneuropathy Waldstroms
Macroglobulinemia
Anti-AChR Ab Myasthenia gravis Acetyl-choline Thymoma
receptor
Anti- NMDA Encephalopathy NMDA Ovarian/uterine
cancer
Adapted from Battaller & Dalmau, Paraneoplastic Syndromes, approaches to diagnosis and treatment,
Seminars in Neurology, 2003,23(2) 215-224

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 PARKINSON’S AND PARKINSON’S PLUS DISEASES

Discriminating Features Consistent Features Variable Features

Idiopathic Severe neuronal loss and Asymmetric signs & Depression
Parkinson depigmentation in the symptoms at onset Dementia
Disease substantia nigra, corpus Pronating-supinating 4- Dysarthria
striatum, locus ceruleus; 7Hz tremor at rest, Drooling
Lewy bodies at autopsy bradykinesia, rigidity, Dysphagia
postural instability, gait Hypophonia
difficulty, handwriting
disturbance
Drug-induced History of exposure to Extrapyramidal signs With chronic use
parkinsonism neuroleptics, following drug exposure of neuroleptics,
metoclopramide, Absense of significant predominance of
reserpine, or resting tremor tardive dyskinesia
tetrabenazine Associated akathisia
Vascular History or neuroimaging Gait disturbance, Dementia
Parkinsonism evidence of multiple pseudobulbar palsy, Rarely, tremor
strokes spasticity, hyperreflexia,
Babinskis
Postencephalitic Neurofibrillary tangles History of encephalitis Facial tics
parkinsonism and marked gliosis; with high fever, delirium, Myoclonus
absence of Lewy bodies or coma Oculogyric crises
at autopsy Parkinsonian syndrome Bizarre postures
Ophthalmoplegia and gaits
Multiple System Neuronal loss in central Extrapyramidal signs Reduced sweating
Atrophy (Shy- autonomic neurons, Orthostatic hypotension Iris atrophy
Drager substantia nigra, caudate, and syncope Bladder
syndrome) locus ceruleus, dorsal Cerebellar signs dysfunction
mostor nucleus of vagus, Pyramidal signs Amyotrophy
inferior olives,
cerebellum, and lateral
horns of spinal cord at
autopsy
Striatonigral Marked degeneration of Similarity to PD with Corticobulbar and
degeneration the striatum (especially greater degree of rigidity corticospinal signs
putamen) rather than Mild or absent tremor
substantia nigra; absence Rapid course

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 of Lewy bodies at autopsy
Progressive Atypical neurofibrillary
supranuclear tangles and neuronal
palsy degeneration in pons,
dorsal midbrain, and
globus pallidus at autopsy
Corticobasal Enlarged achromatic
ganglionic neurons in cerebral
degeneration cortex, particularly in
frontal and parietal lobes;
striatal and nigral
neuronal degeneration at
autopsy
Parkinsonism- Chamorro population of
dementia-ALS Guan
complex Neurofibrillary tangles
mainly in substantia
nigra, but also anterior
horn cells and
corticospinal tract at
autopsy
Table 17.2 Parkinsonism; Clinical Adult Neurology; Bloom and David
Poor response to
levodopa
Pseudobulbar palsy, gait
unsteadiness, and
supranuclear
ophthalmoplegia
(especially vertical gaze)
Extrapyramidal rigidity,
particularly in axial
musculature
Unilateral at the onset
with marked rigidity-
dystonia in the involved
arm
Cortical signs of apraxia,
cortical sensory loss and
alien limb phenomena
Parkinsonism with severe
progressive dementia
and motor neuron
disease
Predominantly affects
males
Dementia, tremor,
corticospinal and
corticobulbar signs
Modest response
to levodopa
Hesitant speech,
gait disturbance
and occasional
action tremor
Usually no
response to anti-
Parkinsonian meds
Motor neuron
signs may be
upper or lower in
nature

Differentiating Parkinsonian Syndromes

PD DLBD MSA PSP CBGD
Symmetric deficit + + +++ +++ --
Axial rigidity + ++ ++ +++ +
Limb dystonia + + + + +++
Postural instability ++ + ++ +++ +

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Supranuclear gaze palsy -- -- -- +++ +
Dysautonomia + + +++ -- --
Early visual hallucinations -- +++ + + --
Dementia + +++ ++ ++ +
Levodopa response +++ + + -- --

Medications for PD:

Variations of Levodopa: Sinemet IR, SInemet CR, Stalevo, Rytary, Carbidopa/Levodopa

Intestinal Gel (Duopa) – THESE MEDICATIONS SHOULD NEVER BE STOPPED ABRUPTLY.
Even if patient is NPO, they need to get these medications, stopping these medications
will impact swallowing, mobility, heart rate and blood pressure

Dopamine Agonists: Requip (ropinerole), Mirapex (pramipexole), Neupro Patch, Apokyn

Injection – THE MEDICATIONS SHOULD NEVER BE STOPPED ABRUPTLY. This can lead to
dopamine agonist withdrawal syndrome. If they can not take meds by mouth or PEG then
switch to Neupro Patch. There is no direct coversion from oral dosing to patch – please
call Dr. Jill Farmer to discuss even if she is not on service or covering the patient.

MAOB inhibitors: Rasageline (Azilect), Selegeline (Eldapryl) – these can be stopped

abruptly, but they do not need to be held for anesthesia

COMT inbibitors: COMTAN (entacapone) – used with each dose of sinemet up to 5 times a
day, this can be stopped abruptly, this cannot be crushed

It is of utmost importance that when a parkinson’s patient comes into the hospital that
you get a detailed history of dosage, how many pills per dose, how frequently are the
taking their medications per day, what is the hour interval between doses and do they
take any additional doses overnight. Write the order EXACTLY as they are taking their
home medications. If there are ever any questions, call Dr. Farmer.

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113
ALS PEG Tube Placement Admissions

Prior to any PEG placement, respiratory status needs to be addressed. FVC should be at
least 25% expected. If it has been borderline prior to admission, pulmonary should
evaluate the patient pre-PEG. If pulmonary status is stable then patients may be
scheduled for the PEG followed by admission.

In this case,Often patients will come to floor after having procedure performed by GI. If
not already placed, consult GI unless there is a reason to consult surgery or IR for
alternative method of feeding tube placement. This occurs due to anatomical issues.

Post-Peg admissions are This is usually a one or two night admission. The justification for
an admission for PEG tube is based on the refeed phenomenon in which ALS patients are
refed calories which are metabolized to CO2 producing a burden on already compromised
respiratory systems.

NPO until cleared by GI for any po intake. Make sure fluids are ordered. Check chart or
call GI fellow so diet and then tube flushes and then tube feeds can be started asap.
Typically the tube is not used for 12-24 hours followed by only hydration and then
increasing percentages of formula.
Order IV fluids
May need a speech and swallow evaluation – although most of these patients have had
this and are already on modified diets.
Ascertain the caloric needs and nutritional state with an albumin, prealbumin, 24 hour
Urine Urea Nitrogen and a metabolic cart, the metabolic cart is ordered as a pulmonary
function

Consult nutrition and check chart for their tube feed recommendations.
Pain control with Tylenol, NSAIDs, or narcotics as needed. Be aware of respiratory
depression with narcotics.
Most of these patients will be on NIPPV – check with patient for their NIPPVbipap setting
and if they have not brought their own machine this will need to be ordered. Consult
respiratory therapy to set up NIPPVbipap per patients home protocol.
May need in-exufflator, suction, and nebulizers ordered (See section on Respiratory Care
in ALS).

114
Routine CBC, CMP, albumen and prealbumin in morning post op.
DVT/GI prophylaxis

ALS Respiratory Care

Remember that in ALS there is progressive weakness of inspiratory and expiratory

muscles along with pharyngeal weakness that results in decreased inspiration, increased
atelectasis, poor cough with decreased secretion mobilization, and high risk of aspiration.
As a result, patients have elevating CO2 levels. Routine care includes assessment of FVC
(sitting and supine), MEP and MIP, end tidal CO2, and respiratory symptoms with
initiation of Noninvasive ventilation (NIV) when either the FVC drops to 50% expected,
MIPs are below -60, CO2 is elevating, or the patient is experiencing symptoms of dyspnea.
Remember that NIV should not be utilized within a half hour of eating as food in the
mouth can be dislodged into airways. Additionally, if secretions are a problem or
expiratory pressures have dropped—pulmonary clearance protocols should be adopted.

In general then, when an ALS patient is seen in the ER, keep in mind that he is likely
compromised from the pulmonary standpoint.
A minor cold in an ALS patient can be life threatening when the URI adds even minimal
increases in secretions that must be mobilized.

Check an ABG, CXR, VC, and CBC, SMA20 to look at respiratory status, evidence of
aspiration or pneumonia, hydration status and nutritional status. Take a history that looks
for evidence of respiratory compromise including daytime sleepiness, yawning, morning
headaches, fatigue, or shortness of breath. Listen to the lungs and check respiratory rate,
temp along with a complete exam. Assess the use of accessory muscles. Look for evidence
of paradoxical breathing, accessory muscle use and speech difficulties due to respiratory
compromise.

Ascertain what respiratory devices are used in the home.

Depending on your findings initiate hydration (remember if the patient is dehydrated,
once hydrated the infiltrate will be more obvious), and consider clearance modalities (see
below) and NIV with a pulmonary consult.

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Start antibiotics if there is any evidence of infection.
Start pulmonary clearance techniques if there are increased secretions and atelectasis:
Nebulizer treatments to loosen secretions followed by a percussion vest to mobilize the
secretions to the large airways. Given the weak cough and to prevent secretions blocking
large airways use a coughalator to mobilize the secretions to the upper airways where
they can be suctioned.
Keep the patient hydrated or secretions will be tenacious.
You can use meds for siallorhea but remember they are often anticholinergic
(hyosciamine, atropine, tricyclics, transderm scopolamine, robinul) leading to dry mouth,
thickened secretions in the lung, blurred vision, and confusion in elderly (especially if they
are already compromised with a URI)

If the patient has not had the pneumovac or flu vaccine ad

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 EEG Rooms With Remote Access

When connecting somebody to continuous EEG in EMU, ICU, SICU, or MICU

make sure the following rooms are used as they have remote EEG viewing
capacity.

EMU:
1467-1471

CCU
2101-2103

MICU
1255-1260
1268-1269
1278-1290

Surgical ICU
08
0811-0812

OR
12-14

117
 Morning Report and Sign Out

 Morning sign out will take place in the 7th Floor Conference Room at
7:30AM

 It is the responsibility of the overnight resident to print an updated patient

list for the floor and consult team and bring this list to conference room by
7:30AM.

 Updated patient list will be used to assign new patients and follow up on old
patients.

 Sign out for the floor and consult team must happen in the conference room
and finish before starting morning report at 8:00AM.

 On Tuesday and Thursday morning, morning report will be done by the

overnight resident on call.

118