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Resident morning report

Xuan KangPGY-2 Neurology

CC: weakness & vision issues


60 yo M hx of obesity s/p bariatric surgery & OSA.
16 days ago, food poisoning after eating KFC & droopy eyelid,
treated for bells palsy with acyclovir and steroid at day 4.
Day 6 had urinary/bowel incontinence, balance issues
Day 10: worsening vision problem (just blurry).
Continued worsening whole body weakness and numbness/tingling
down both arm & leg. Non-contrast CT head x2 unremarkable.
Saw in OSH ED, became unresponsive after 0.5mg dilaudid and
required narcan administration. Transferred for care.

Previous histories:
PMH: asthma, OSA, obesity.
PSH: bariatric surgery, incisional/ventral hernia repair,
cholecystecomy, left knee surgery.
Fx: HTN, diabetes and blindness; mom has stroke & heart
disease.
Sx: quit smoking in the 80s. Denies recreational drug use,
retired currently and worked in factory before, lives by
himself.
Medication: aspirin, zoloft 50mg, mirtazapine 15mg,
protonix, docusate & magnesium.

Exam:
Vitals: BP: 149/79, HR: 61, Ox: 96% RA, temp: Afebrile.
MS: Extremely dysarshric, can understand questions but doesnt follow
command consistently, require frequent stimulation to answer questions.
CN: follows finger movements, blink to threat, but restricted gaze in all four
directions and more prominent horizontal nystagmus. 6 mm pupil bilaterally
and sluggish to light, unable to close right eyelids and has left eyelid
drooping, right facial drooping.
Motor: increased tone of upper extremity, 4/5 BUE, BLE 2/5 (partially 2 nd to
pain).
Reflex: brisk upper extremity reflex (3+), 2+ RLE but 1+ LLE (has knee
surgery). Negative hoffman bilaterally, equivocal plantar reflex bilaterally.
Sensory: retract to pain equally in all four extremities.

Formulation of the case


Line 1: _yo M/F presents with (CC) for
(duration);
Line 2: Localization: Exam localizes
to
Line 3: differential diagnosis
including?
VITAMIN:
Vascular, inflammatory, toxic,
autoimmune, metabolic, infectious,
neoplastic, (congenital, degenerative,

Differential: VITAMIN
Vascular: Subacute brain stem stroke with edema;
Inflammatory: transverse myelitis vs. MS vs. NMO vs.
para-infectious inflammation.
Toxic: Botulism; heavy metal poisoning;
Autoimmune: GBS spectrum disease (weakness w/ recent
GI illness). But preserved reflex.
Metabolic: possible nutrition deficiency given Bariatric
surgery in the past.
Infectious: CNS infection
Neoplastic: Carcinomatosis meningitis (altered mentation,
multiple cranial nerve involvement) vs. neoplastic process
with brain metastasis.

At initial admission
Prior workup:
Non-con CT on day 12 and 14 unremarkable per report
non-contrast CT at OSH unremarkable at day of
admission.
Stool antigen positive for campylobacter on 11/9;
CBC: 12.8/12.9/40/285; BMP: 142/3.8/104/22/26/1/93;
Plan on admission:
MRI of brain for brain stem stroke & MRI spine to evaluate
spinal lesions.
VC/NIF given possible (but less likely) GBS.
LP evaluation of possible GBS vs. infection.
Lab workup for other etiology.

Clinical progression:
MRI was performed but was limited/not tolerated well and
was unremarkable.
Next morning patient suddenly became unresponsive and
staring straight, worsening EOMI and pupil non-reactive.
Brisk 3+ reflex except left patellar; Strength 2/5 for BUE
and 0/5 for BLE.
Stat CT head unremarkable.
Intubated for airway protection and admitted to TLC.

Lab studies:
CSF studies:
nuc: 16; RBC: 5000; glucose 55; protein 352;
CMV, EBV, VDRL, crypto, VZV, HSV, Lyme, WNV,
AFB, MAC, enterovirus, culture, HTLV negative.
lymphoma workup, NMDA, Oligoclonal band all
negative. Anti-Gq1b and paraneoplastic negative.

Serum studies:
BMP unremarkable. CBC: 22.4/13.4/42/232;
Blood culture, sputum culture, UA unremarkable.
Vitamin B1, 2, 6, 12 unremarkable.
UDS, heavy metal all unremarkable.

MRI brain: foci of enhancement involving the


left parietal lobe white matter, right frontal lobe
white matter, and left cerebellar tonsil are seen
in combination with abnormal enhancement
and T2-weighted/FLAIR hyperintensities in the
trigeminal, facial and vestibulocochlear nerves.
MRI Spine: there is enhancement of the nerve
roots of the cauda equina.
EMG: unremarkable for demyelinating
peripheral nerve process. However, concerning
for axonal injury/involvement.

Final diagnosis:
Bickerstaff encephalitis.
Due to white count and pleocytosis on CSF, Started on
acyclovir, ceftriaxone doxycyclline, vancomycin for
empiric coverage. And stopped after culture negative.
Plasmaphresis x7, patient had improved mental status,
EOM and 3/5 strength after 2 cycles of PLEX.
4+/5 strength throughout at discharge (~day 47).
Remains slurred speech, mental status intact, reactive
pupil intact, improved and almost intact EOMI.

Bickerstaffs Brainstem Encephalitis


Constellation of acute ophthalmo-plegia, ataxia and
altered sensorium.
Belongs to part of Anti-Gq1b syndrome. However,
negative studies DOES NOT PRECLUBE
DIAGNOSIS! (Only 66% of patient has positive AntiGq1b).
Majority preceded by infectious illness (92% of cases,
ex. Campylobacter Jejuni and Haemophilus inf).
BBE high in Japan comparing to western nations.

Presentation:
Altered sensorium is specific for BBE: as brain
stem reticular activating system is impaired.
Ophthalmoplegia: acutely in a symmetric and
progressive fashion.
Ataxia, dysarthria, hyper-reflexiainvolvement of
cerebellar/brain stem region;
Diagnosis is largely based on clinical features and
again anti-Gq1B is non-specific!

Differential diagnosis:
BBE vs. MFS: same spectrum.
MFS presents with areflexia consistent with a peripheral pathology.
Both disease has ataxia and ophthalmoplegia + ptosis, mydriasis and facial
palsy; but MFS does not have altered mentation.
Anti-Gq1b Ab seen more frequently in MFS than in BBE. (83% vs. 66%).

bulbar GBS (pharyngeal-cervical-brachial GBS variant):


ptosis, flexor weakness of UE and spares LE strength, sensation & reflex
mimicking botulism.

AMAN (acute motor axonal neuropathy);


Associated with Camylobactor;
More rapid progressive weakness than AIDP, symmetric proximal and distal
weakness without sensory disturbance.
Normal or brisk tenden reflex; GD1a and GM1 antibody positive.
Delayed recovery than AIDP.

Management:
Diagnosis:
Anti-Gq1b antibody found in 66% of patient; Anti-Gq1b
targets peripheral CN nerves
MRI shows abnormalities only in 30% cases.
EEG shows slow wave activities;
CSF shows elevated protein level and pleocytosis;
EMG shows axonal polyneuropathy;
Treatment:
IV steroid, plasmapheresis and/or IVIG.
Studies showed 66% showed complete remission 6
month after steroid, plasmapheresis and IVIG

Diagnosis/consideration in multiple
CN-medscape review.
Brainstem syndrome: with long tract sign,
gaze palsy, INO, crossed sensory syndromes,
vascular insults (stroke vs. bleedlateral
pons/medulla),
MS, ADEM
ependymoma,
brainstem encephalitis (bickerstaff encephalitis),
central pontine myelinolysis,
Arnold-Chiari Malformation,
Syringobulbia;

Chronic meningitis:
Infectious:
Bacterial: Lyme disease (second stage, CN 7, 2, 6, 8), TB (19%, CN VI),
syphilits; also Mycoplasma and pseudomonas
Fungal infection: cryptococcus. (also coccidiodes, histoplasma,
blastomycosis, aspergillus, mucomycosis, candida);
Viral: HIV (HIV-1, also HIV-2), HSV, EBV, CMV;
Parasitic: cysticercosis, chargas dz.
Non-infectious:
Granulomatous: Sarcoidosis (CN 2, 7, 8, 9, 10), Behcets
syndrome(ulcers + uveitis, CN 2 & 7);
Vasculitis: Wegner (CN 2, 6, 7, 8, also has renal); polyarteritis nodusa
(CN 3, 8);
Connective tissue disorder: RA, Sjogren, scleroderma, lupus.
Neoplastic: carcinomatosis (SCLC, melanoma, myeloblastic luekemia)
vs. lymphomatous ; Hematologic CA more likely has multiple CN;
Idiopathic hypertrophic cranial pachymeningitisincreased ICP, CN 512;

Extramedullary vascular phenomena;


Vertebro-basilar dolicho-ectasis (CN 3, 5, 6)
Fusiform aneurysm;
Carotid artery dissection/aneurysm (Horner,
lower CN)
Cavernous sinus thrombosis

Trauma: 12% of the cases;


Blunt trauma with MVA more common
Cavernous sinus fistula w/carotid a.: triad of
chemosis, pulsatile exophthalmos and audible
bruits.

Penetrating injury, Gunshot wounds;


Iatrogenic cause: CEA, LN biopsy;

Tumor:
More common: Nasopharyngela cancer (EBV, CN6 MC, 2, 5, and
12 at advanced stage);
Less common: metastasis, meningioma, lymphoma, myeloma,
Giant cell, hemangioblastoma,
If affecting all CN 6-12, tumor at Clivus.
At cerebellopontine angle (meningioma, epidemoid); CN V-X

Peripheral etiology:
Most common: GBS & MFS
PN: Diabetes, diphtheria, HIV, lyme, sarcoidosis, chemo
NMJ: Myasthenia gravis and botulism;
Myopathies with bulbar: Mitochondrial, oculopharyngeal
dystrophy.

Bone disorder:
Osteopetrosis (Albers-Schonberg/marble bone)
Pagets disease, Fibrous dysplasia.
Hyperostosis cranialis interna: AD, hyperostosis & osteosclerosis
of calvaria & base of skull, affecting CN 1, 2, 7, 8;

Diagnostic workup
MRI of brain with contrast (3D-FIESTA), CTA vs. MRA;
CT head/sinus/orbits, CT chest/abd/pelvis/PET for cancer eval.
Serum: ESR, CRP, CBC, CMP, ANA, ANCA, RF, ACD, Lyme, FTA, HIV,
Cryptococcus.
CSF: protein, cell count, glucose, culture, cytology (10-20cc), flow
cytometry, VDRL, Cryptococcus, Histoplasma, ACE, PCR (lyme,
CMV, EBV, TB), Ab (coccidiosis, blast mycosis, aspergillosis,
candida);
Meningeal biopsy: chronic meningitis, neoplastic, CNS vasculitis;
Yield higher when obtained from posterior fossa

Management
Rapid progressive symptoms: Empiric
therapy:
IF risk factor for TB, txt empirically,
If risk facto for fungal, txt with
fluconazole;
Non-infectious inflammatory, consider IV
solu-medrol 1g 3-5 days.
Different treatment based on the
diagnosis.