Huny_00@yahoo.

com
Explain the neuromuscular trigger points = Drugs that cause Myositis Missense = late onset
disease concept: hyperesthesia) RIPS)
1. It doesn't matter whether 6. Shoulder girdle Rifampin Clues for Duchene’s
you hurt a nerve or hurts most (hard INH Muscular dystrophy
muscle you get time combing hair, Prednisone X-linked recessive
weakness reaching up in Statins Problem w dystrophin (part
2. Diaphragm failure cabinet, waving Any long acting Rx of muscle protein)
causes problems with bye) Pseudohypertrophy calf
inspiration  decreased 7. Visceral cancer Disease that causes myositis Gower’s sign-back muscles
PO2  less ATP  (Colon) 1. Hypothyroidism weak, use legs to get up
Low Energy State 8. Caused by 2. Cushing disease Waddling gait- transfer
3. MC Symptoms are Rifampin, INH, weight to one hip at a time
Weakness and SOB Statins, Prednisone, Infection that causes bc spinal muscles weak
4. MC Signs are Hypothyroidism myositis Onset < age 5
Tachypnea and Dyspnea 9. Treated with Trichinella Spiralis Nonsense or Frameshift =
5. MC Infections are Amitryptyline early onset
Pulmonary and UTI 10. a/w Temporal Management chronic pain?
6. MC Cause of death is Arteritis Amitryptyline and exercise Defect in Duchene's
not heart failure because 11. Multiple trigger (to avoid m atrophy) muscular dystrophy?
the heart has points Absence of dystrophin
autonomics. respiratory 12. Muscle pain when Temporal arteritis (rule of protein
failure moving 60s)? Tx? Complication?
7. Other symptoms 13. Heliotropic (deep >60yo (3) Clues for Becker's or
(Constipation, Urinary purple rash on ESR >60 Duchene's muscular
retention, Impotent, eyelids aka Tx-Prednisone > 60 mg dystrophy?
hypotensive) Gottran’s Sign) Granulomatous = T cells & Duchene's (< 5 y.o)
8. Diaphragm failure 14. Throbbing temporal macrophages Becker's (>5 y.o)
causes problems with HA Complication – blindness Waddling gate
inspiration  decreased 15. Tx of most (b/c ophthalmic artery) Gower's sign (use hands to
PO2  vasodilation of inflammatory **Visual complaints  IV walk up own legs)
vessels in brain  myopathies steroids first then bx Calf Pseudohypertrophy
Headache No visual complaints  bx
9. Low PO2  1. Myositis (mcc first Cause of the
vasoconstriction to lung hypothyroid, Rx’s) pseudohypertrophy seen in
 Pulm HTN  2. Polymyositis Muscular Dystrophies the calves of Duchene's or
RVH S4 3. Dermatomyositis Becker's?
10. Elevated labs = ESR, 4. Fibrositis Genetic inheritence Duchene's
WBC, myoglobin, AST, 5. Fibromyalgia (Tx- Mitochondrial  mom to all Fat deposition in the muscles
ALT, Aldolase exercise, physical kids. Leihs (chronic fatigue) of the calf
therapy, and Lebers (blind). Red
Give the associated amytriptyline) fibers. Heteroplasmy (ppl Clues for Myotonic
inflammatory myopthay 6. Polymyalgia have different variations) Dystrophy
1. One Muscle Hurts Rheumatica AR- AaAa (25% recessive Increased muscle tone 
2. Several muscles 7. Dermatomyositis 67% carrier), enzyme break down of muscles
Hurt. Difficulty (colonoscopy bc of AD – structural. (if one Attacks facial muscles
rising from chair. risk of cancer) parent has it 50% likely to Bird-beak face
3. Muscle pain with 8. Myositis get, if both have it 75% get Cannot let go after shaking
heliotropic (purple 9. Fibromyalgia (b/c it) hands (increased muscle tone
or violacious on eye high incidence of Decreased penetrance (have = myotonic)
lids or knuckles) depression) gene but not shown) Trinucleotide repeats
rash  r/o 10. Polymyalgia Variable expression – ppl Dx- clinically will sustain a
malignancy (colon Rheumatica show differences contraction
ca therefore do 11. Fibromyalgia XLR- variable. Boys.
colonoscopy) 12. Fibrositis Females are carriers. Trinucleotide repeats
4. Inflammation in 13. Dermatomyositis Incidence given = chance of 1. Fragile x
muslce insertions 14. Temporal Arteritis getting it. 2. Frederick’s ataxia
(hurts w/ mov’t) 15. Steroids 3. HD
5. Pain of Muscle and Clue for Becker’s 4. Myotonic dystrophy
Muscle insertions Onset > age 5 5. Prader willi
all the time (tender milder
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X- linked Recessive
(Enzyme) deficiency dz?
1. HGPRT
2. G6PD
3. CGD
4. Pyruvate
Dehydrogenase
5. Hunter’s
6. Fabry’s
7. Adrenoleukodystrophy
Neuropathies
Ascending paralysis two
weeks after a URI (viral
gastroenteritis)?
Pathology? AKA?
Pathophysiology?
Symptoms? Tx?
Guilliann Barre
Autoimmune
MC associated bug- camp
bacter jejuni)
Polyrediculoneuropathy
(autoimmune attack on
peripheral nerves)
T-cell mediated myelin
injury
Starts in legs
Ascending paralysis
Tx-IVIG and plasma
exchange
Descending paralysis?
ALS
Tick paralysis (acute)
Neuropathy in glove and
stocking (hands and feet at
same time) distribution?
Chemical responsible?
Diabetic Neuropathy
Sorbitol (aldol reductase
pathway, neurons turn
glucose to sorbitol can’t use
anymore. sugar trapped
somewhere  swelling)
Disease with Lancinating,
shooting, stabbing pain?
DOC? CNS?
Syphillis (tertiary)
Carbemazepine (DOC)
Attacks dorsal columns 
tabes dorsalis (position,
vibration, touch)
Argyle Robertson pupil
(constricts to accommodation
(near) but not to light. Slow
to respond to light. From
edinger westfal nucleus)
What is another name for
Guillianne Barre?
Inflammatory
Polyrediculoneuropathy
2 muscle types least
affected by neuromuscular
dz? Why?
Smooth muscles and cardiac
muscles because they have
autonomics
How do you differentiate
between Myasthenia
Gravis and Myasthenia
Syndrome? Endrephonium
MOA?
MG gets weaker with
contractions. Stronger with
Edrophonium Test.
MS gets stronger with
contractions. Weaker with
Edrophonium Test.
What is the defect in
Myasthenia Gravis?
Demographics? W/U?
Autoimmune attack against
Ach receptors
Anti-Ach R Ab  worse as
day goes by
Middle aged female with
ptosis or gets weaker as day
goes by
Thymoma associated with
every autoimmune dz except
GRAVEs dz  CT
Treatment of MG?
Neostigmine (ACHesterase
Inhibitor)
What is the defect in
Myasthenia Syndrome?
autoimmune
The Sarcoplasmic reticulum
is slow in sequestration of
Calcium leading to stronger
muscle contractions 
stronger as day goes by.
Ab to presynaptic Ca
Small cell carcinoma of lung
= Paraneoplastic syndrome
Dx myasthenia syndrome?
EMG
Stair step decline -MG
Stair step rise -MS
Another name for
Myasthenic Syndrome
Lambert Eaton Syndrome
What cancers are
associated with the
following diseases:
1. Myasthenia Syndrome
2. Myasthenia Gravis
3. Dermatomyositis
1. Small cell of the lung
2. Thymoma
3. Visceral Cancers (MC
colon cancer)
MG? Dx tests? F/u?
autoimmune
Ab to Ach receptor
Dx- Ach receptor Ab test or
EMG (decrement response to
repetitive stimulation)
F/u – other autoimmune dz,
chest CT (thymoma)
Diagnose MG by Rx? Tx?
Edrophonium (shortest
acting) –Achesterase
inhibitor  parasympathetics
Give weight and can lift 
temporary relief
Neostigmine and steroids
Imaging for Myasthenia
Gravis?
CT scan to r/o thymoma  if
present surgery to remove
Ach esterase inhibitors?
Edrophonium-Dx MG
Neostigmine -DOC
Pyridostigmine- 2nd line
Physostigmine-too much
CNS s/e
Irreversible
(noncompetitive)
Achesterase inhibitors?
Tx?
Organophosphates (end in
phate and thion)
Symptoms parasympathetic
Atropine
Huny_00@yahoo.com
A Myasthenia Gravis
patient treated with
Neostigmine presents with
weakness, what is the
course of action?
Determine if this is worsened
MG or cholinergic crisis
(desensitized receptor) due to
Neostigmine.
Repeat edrophonium test
If symptoms improve,
increase Neostigmine
since MG worsened
If symptoms worsen 
cholinergic crises,
Administer Atropine and
decrease Neostigmine
dose since it’s a
cholingeric crisis
Anticholinergic drugs?
1. Atropine- DOC heart
block, cholinergic crisis, dry
up mucous secretions before
surgery
2. Ipratropium – block cGMP
3. Tiatropium – block cGMP
4. Glycopyrolate-dry up
secretions
5. Benztropine
6. Trihexyphenidyl
S/E of anticholinergics
sympathetic except + HOT
DRY SKIN
If pt has eye pain after using
drugs  glaucoma (dilates)
C/I in obstructive lung dz (bc
dry up secretions therefore
can’t cough up secretions)
Pathophysiology of MS?
Findings in CSF on LP?
 Anti-myelin Ab
 Myelin basic protein in
CSF (LP)
What structure does MS
like to attack? What is this
called?
 Medial Longitudinal
Fasciculus
 Internuclear
opthalomoplegia (medial
rectus palsy on
conjugate lateral gaze)
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What is responsible for the
halo vision (see periphery)
seen in multiple sclerosis?
 Optic neuritis
 Wax and wanes
Profile of MS? Sx? Dx?
early presentation?
 Middle aged woman w/
vision problems
 Scattered neurological
deficits**
 MRI-demyelinated
plaques asymmetric
 LP-myelin basic protein
in CSF
 Trigeminal neuralgia
Middle aged woman with
optic neuritis (any eye
problem)
Multiple Sclerosis
Middle aged woman with
ptosis
Myathenia Gravis
MS Equivalent in children
(5-10yo)
Metachromatic
Leukodystrophy
Arylsulfatase deficiency
MS and Metachromatic
leukodystrophy
Attacks optic nerve (optic
neuritis  patient sees
periphery bc central vision
gone)
Attacks medial longitudinal
fasciculus (CN6 and CN3 not
connected) internuclear
ophthalmoplegia  ask
patient to look to one side
(CN3 for medial rectus
doesn’t respond but lateral
rectus CN6 does therefore
one eye will stay centered)
What is the treatment for
Multiple Sclerosis and all
autoimmune neuropathy?
#1 Steroids (stop
inflammation)
#2 IV gammaglobulins 
block immunoglobulins
#3 Plasmaphoresis (spin
plasma and cells effecting
patient). When severe
#4 INF β
Lower motor neuron dz
Tx autoimmune
neuropathies?
Steroid (avoid in Guil Bar)
IV gammaglobulins (block
Abs) when weakness
significant
Plasmapheresis (when
respiratory compromised)
LMN diseases
3 neuromuscular diseases
with fasciculations ?
Werdnig Hoffman
Polio
ALS
fasciculations are a specific
sign of what?
Lower motor neuron Damage
Fasiculations in a newborn
Werdnig Hoffman (floppy
baby)
Floppy baby syndrome
1. Hypothyroidism < 1 yo
2. Botulinum if mention
Honney molasses
3. Werdnig Hoffman
Fasciculation in a middle
aged male
ALS
Fasciculation 2 weeks after
Gastroenteritis
Polio
Middle aged man with
descending paralysis?
Tracts?
Amyotrophic Lateral
Sclerosis
(UMN & LMN lesion)
Slowly progresses
Corticospinal tract (UMN)
and ventral/anterior Horn
(LMN)  loss of all motor
fibers
No sensory involvement
Head and neck  trunk
Tx-Riluzole (blocks voltage-
activated Na channels) 
increases life span
Asymmetrical paralysis 2
weeks after diarrhea
(gastroenteritis)
Polio (< 2y.o.)
Fasciculations in a neonate
Werdnig Hoffman (no
anterior horn motor neurons)
Floppy baby (also seen with
hypothyroidism and
botulism)
Cerebellar
3 neuromuscular diseases
that cause cerebellar signs
in 5-10 yo?
1. Ataxia Telengectasia
2. Fredrick Ataxia
3. Adrenal Leukodystrophy
Clues for Ataxia
Telangiectasia
 Cerebellar signs in 5-
10yo
 Spider veins on skin
 IgA deficiency (diarrhea
+ respiratory illness)
 Increase incidence of
DNA breakage 
increased risk of skin
cancer
Clues for Fredrick’s Ataxia
Cerebellar signs in 5-10yo
Scoliosis and Retinitis
Pigmentosa
(pigments on the retina)
Trinucleotide repeats
Increase incidence of
hypertrophic myopathy
What structures does
Fredrieck’s Ataxia affect?
Dorsal Columns
(position/vibration) and
SpinoCerebellar
(balance/depth) pathway
What structure does
adrenal leukodystrophy
affect?
Cortex
Babinski spasticity
Huny_00@yahoo.com
Clues for Adrenal
Leukodystrophy
Carnitine shuttle defective 
Problem metabolizing long
chained fatty acids from
cytoplasm to mitochondria
(seen on bx)
CAT1 on cytoplasmic side
Babinski
Electrolyte abnormality
Adrenal Failure
(insufficiency)
Early white matter problem
(leukodystrophy)
Dx- muscle bx
Cerebral Palsy
1. Spastic diplegia
2. Spastic hemiplegia
3. Choreathetosis
4. Atonic
Definition for Cerebral
Palsy
Permanent neurological
damage suffered < 21 yo
What can cause Chorio
Athetotic CP?
Kernicterus
CP which affects Legs
more than arms
Spastic Diplegia (e.g. CMV,
hydrocephalus )
Midline cortex involved
CP which affects one side
of body more than the
other
Spastic hemiplegia (e.g.
stroke, tumor, etc…)
HSV attacks one temporal
lobe at a time
Toxoplasmosis- parietal lobe
CP which affects the Basal
Ganglia
Chorio Athetotic
Kernicterus (high bilirubin)
If bilirubin > 25 need
exchange transfusion bc
worried about attack to basal
ganglia
CP where the patient has
No tone to the body
Atonic
Frontal cortex involves
corticospinal tract
3 of 4

Infection that can cause
spastic hemiplegia
CMV
Huny_00@yahoo.com
Tx: rest, ice compression (to
prevent inflammation),
elevate

What can cause spastic Best initial test for

hemiplegia? polymyositis/
Stroke dermatomyositis
CPK and aldolase
Muscle physiology
H band- Myosin-heavy chain Tx for DM/PM
I band- actin only Glucocorticoids
A band- actin and myosin
Sarcomere- from one z line Tx fibromyalgia
to another Exercise, milnacipran,
M contraction  Z lines duloxetine, pregabalin
closer, force increases, TCA
tension increases, overlap
increases
Areas of nonoverlap decrease
(I and H band)
CPK found  at M line

CPK? Released when?

Reserve form of energy for
muscles
CPK released when M
breaks down  rises with all
muscle disease

Naturally high CPK races

AA and latinos, anyone with
high activity

Where T Tubules found?

AI jxn-skeletal
Z line- cardiac

Length of maximal overlap

in muscles?
2.2 microm

Muscle strain?Tx?
overstretch or tearing muscle
Tx: rest, heat (increase blood
flow), NSAIDs, M relaxants

Baclofen?
NSAID for back pain due to
GABA (inhibits m)

Cyclobenzaprine?
Anticholinergic activity
(relaxes m. b/c muscle
contraction is cholinergic)

Joint sprain? Tx (RICE)?

Tear tendon or ligament

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