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90 Cards in this Set
- Front
- Back
HALLMARK OF MUSCLE DISEASES
(exceptions) |
slowly PROGRESSIVE PROXIMAL muscle weakness
- Myotonic Dystrophy = distal muscles are more involved - Metabolic myopathies = exertional weakness/pain - Inflamm. myopathies = subacute (wk-months) |
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Types of Muscular Dystrophies
All hereditary. No cure |
1. Dystrophinopathies: Duchenne & Becker's MD
2. Myotonic Dystrophy: Most common adult MD 3. Limb Girdle MD 4. FSH MD |
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Types of Inflammatory Myopathies
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PRIMARY
1. Polymyositis: subacute progressive weakness in proximal muscles 2. Dermatomyositis 3. Inclusion Body Myositis (IBM) VS. ASSOCIATED WITH OTHER DISEASES |
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Metabolic Myopathies
- clinical |
Weakness/Pain upon EXERTION
"coca-cola" urine = myoglobinuria (muscle breakdown) - can be slowly progressive or chronic weakness *disease may be due to damage at any metabolic level* |
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Mitochondrial Myopathies
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Defects in ETC or ox-phos impair ATP synthesis
LOOKS: - fixed eyes - Ptosis -Retinitis pigmentosa - Myoclonic epilepsy - Myopathic/proximal weakness |
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DUCHENNE MUSCULAR DYSTROPHY (DMD)
- genetics - pathophys - complications 30/100,000 --> 1/3 cases = new mtts |
GENETICS: X-linked Recessive (Xp21) --> no dystrophin to link actin to sarcolemma/ECM --> excess Ca2+ influx --> muscle fiber necrosis
Complications RESP: hypovent 2' to m. wkness and kyphoscoliosis ORTHO: kypho & contractures CARD: Tachy, 90% abnl EKGs CNS: IQ is 1 SD below mean |
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DMD
- CLINICAL |
CLINICAL
- Dx 2-3 yo - Rapidly prog (die by 20 yo) - Delayed motor milestone (walk by 18 mo instead of 12) - Wide, broad gait & lordosis - Gower sign: "downward dog" - plantar flexors (tip-toes) and ankle inversion is intact, use hands on knees - Gastrocnemius (pseudo)hypertrophy - Braces by 10 yo --> wheelchair |
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DMD
- Dx - Tx |
DIAGNOSE:
1. Genetics Test: Xp21 2. Elevated Creatine Kinase 3. EKG abnl 4. EMG - myopathic pattern 5. Muscle biospy - no dystrophin stain TREATMENT: - Prednisone/Corticosteroids (controversial due to side effects) - braces - spine stabilization sx - portable vent |
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BECKER MUSCULAR DYSTROPHY
3 / 100,000 --> 10% = new mtts |
similar to DMD but more benign & 10x LESS frequent.
- Later onset (5-15 yo) - Slower prog (many live >20 yo) - Similar complications (not IQ) - Same tests to diagnose: some dystrophine staining will occur in BMD |
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MYOTONIC DYSTROPHY (MyD)
- Genetics - Pathyphys - Dx most common in adults: 13/100,00 |
Autosomal Dominant: chrom 19q - expansion of a CTG (trinucleotide) rpt
MyD gene = Thr-Ser Protein Kinase modulating ion channel fxn *Anticipation: Increased severity with successive generations (due to increased # of CTG rpts). DIAGNOSE: 1. NORMAL CK 2. EMG:myotonic discharges 3. Slit lamp: cataracts 4. EKG: Cardiac conduction defects |
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MyD
- Clinical - Assc'd |
Range in severity (even w/in family)
- Face: GLUM, fish mouth, ptosis, temporalis wasting - Nasal voice (m. wknes) - Weak SCMs - DISTAL mm are more affected than PROXIMAL: ex// can't open a jar or foot drop = tripping ASSC'D: 1. Cardiac conduction abnls --> complete heart block poss 2. Mental retard (esp in congenital cases) 3. RESP: hypovent 2' to m. wkness AND CENTRAL hypovent 4. Cataracts 5. Frontal balding. 6. Endocrine Dysfxn: insulin resistance & decreased testosterone/atrophy in males |
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Myotonia
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Seen in MyD - Inability to quickly relax after contracting a muscle
test with grip or eye closure - can also be percussion induced (napkin ring tongue) |
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FACIOSCAPULOHUMERAL DYSTROPHY (FSH)
- genetics - dx 1 / 100,000; 1/3 may be asymptomatic |
Autosomal Dominant - Chrom 4
- Normal or slightly high CK - EMG = myopathic pattern - genetic counseling |
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FSH CLINICAL FEATURES
**doesn't decrease life expectancy** |
Onset: teens/20s - middle age
1st. Facial Weakness = glum, can't smile, whistle, close eyes completeles 2nd. Shoulder girdle wkness usually reason for hospital visit - scapular winging - can't lift heavy stuff or pushups - scapular WINGING - rides up over shoulder (looks like fat trap) - deltoids are SPARED - foot-drop = trips/falls - Range in severity - Slowly progressive **sometimes assc'd with sensorineural heaing loss & vascular retinal dz** |
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MYASTHENIA GRAVIS
- pathophys - LOCATION in NMJ - Assc'd condictions **INTERMITTENT, Need to see same doctor multiple times to notice** |
NMJ, autoimmune dz
Path: Abs destroy the POST-synaptic NICOTINIC Ach-R - NORMAL release of ACh Assc'd with: Hyperthryoidism, Thymoma, Young females, old males (>40) |
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MG
- Clinical |
1. FATIGUE, esp at the end of the day or exertional
- sensation & reflexes are normal 2. OCULAR WEAKNESS: - diplopia, ptosis (BUT PUPIL IS NEVER INVOLVED - musc-R) - "curtain sign." - **pure ocular MG** 3. BULBAR WEAKNESS: - dysarthria: voice fatigues - dysphagia - NECK FLEXOR WKNESS - facial weakness - Myasthenic crisis = SOB & resp failure 4. EXTREMITY WEAKNESS: -worse in proximal muscles - exercise intolerance - weakness as day progresses - examine with sustained resistance |
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MG
- DX -TX #1 test = blood test for ACh-R Abs. |
DX:
1. Tensilon (ACHase Inhib, edrophonium) Test: look for improved ptosis or ocular weakness post-injection 2. Repetitive stimulation on nerve conduction study ---> decremental response 3. Blood test for Ach-R Abs (in 85%) but just cuz u have Abs doesn't mean u have dz. TX: 1. Chest CT (thymoma 2. Mestinon (pyridostigmine): oral, longer-acting "Tensilon" - also acts on muscarinic-R = stomach cramps ( & CN 3,7,9,10) - Nicotinic-R = fasciculations (BOARDS) 3. ORAL STEROIDS: if mestinon fails 4. Azathioprine (Imuran) = immunosuppressant (decreases the effective dose of the steroids) - takes 6 mos to see 5. IVIg & plasmapharesis - expensive -acute crisis |
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DERMATOMYOSITIS
- PATHOPHYS - SUMM OF DISTINGUISHING FEATURES |
PATH:
- Autoimmunes - Primary site of injury is microvasculature (capillaries): C'-Mediated Vasculopathy of unknown etiology SUMM: 1. Skin rash 2. Adults & kids 3. Joint contractures 4. Perifascicular atrophy 5. Inflamm cells = B cells |
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DERMATOMYOSITIS
- PATHOPHYS - SUMM OF DISTINGUISHING FEATURES |
PATH:
- Autoimmunes - Primary site of injury is microvasculature (capillaries): C'-Mediated Vasculopathy of unknown etiology SUMM: 1. Skin rash 2. Adults & kids 3. Joint contractures 4. Perifascicular atrophy 5. Inflamm cells = B cells |
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DERMATOMYOSITIS
- clinical features |
1. SKIN - violaceous scaly lesions over malar face + eyelids (periorbital edema); heliotropic
- Also over extensor surfaces - Cutaneous calcinosis can occur 2. Muscle weakness - subacute, proximal more - reflexes are preserved - 1/3 have dysphagia - resp mm are usually NOT involved 3. Myalgias: achy pain. more common in kids w/ dz 4. Joint contractures 5. Mild ^ CA risk |
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DERMATOMYOSITIS
- DX - TX |
DX:
1. Blood: HIGH CK 2. EMG: small motor unit potentials with fibrillations potentials 3. MUCLE BIOPSY - PERIFASCICULAR ATROPHY (BOARDS) - INFLAMM CELLS = B CELLS TX: 1. STEROIDS: very effective 2. other immunosuppressant 3. IVIg |
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DISTINGUISHING B/W INFLAMM MYOPATHIES
|
1. Derm: rash & B-cells & perifascic. atrophy
2. Poly: T-cells; rule out all others 3. IBM: Also Tcells - HEREDITARY - inclusion bodies on muscle biopsy |
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POLYMYOSITIS
- PATH - clincial |
PATH:
cell-mediated/Tcell attack against muscle fibers (autoimmune) CLINICAL: Similar to dermatomyositis but NO SKIN RASH - subacute wkness, more proximal - dysphasia - myalgias in 1/3 pts. |
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POLYMYOSITIS
- DX - TX |
DX:
not as specific; rule out all others first - High CK - EMG same as derm - Muscle biopsy: Inflamm t cells with active muscle fiber necrosis ( need all of the above findings + clinical to make the dx) TX: - HIGH DOSE STEROIDS - IMMUNOSUPPRESSANTS - IVIg |
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SIDE EFFECTS OF LONG-TERM PREDNISONE USE (and other high dose steroids)
**always taper a steroid drug off** - best if transitioned to QOD therapy ASAP |
1. Steroid induced diabetes
2. Osteoporosis (rpt bone scans needed) 3. Adrenal suppression: wean off drug so you don't cause Addison's dz 4. Cataracts 5. Weight gain & Cushing's 6. Fragile skin; easy bruising 7. Mood fluctuations: euphoria & mania 8. Insomnia 9. Peptic ulcers 10. Lowered immunity with susceptibility to infxn and reduced wound healing. |
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INCLUSION BODY MYOSITIS (IBM)
- CLINICAL - PATH proximal = distal |
VERY VERY SLOW PROGESSION (MO-YR)
CLINICAL: - Age > 50 - Male > female - slowly prog wkness: esp quads & finger flexors: legs buckle, can't make fist - NO MYALGIA - dysphagia in 1/3 - mild Periph neuropathy on EMG PATH: similar to polymyositis (Tcells) - also HEREDITARY |
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IBM
- DX -TX |
DX:
- mildly elevated CK (slow prog) - EMG (nonspecific) - MUSCLE BIOPSY: INCLUSION BODIES (intranuclear tubulofilamentous) tx: no known effective tx. |
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NMJ diseases
- time span |
MG and LE = slowly progressive
- MG is post synpatic - LE is presynaptic Ca channels Botulism = rapid, acute - postsynaptic - both musc and nicotinic receptors affected - FIXED DILATED pupils |
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LAMBERT-EATON SYNDROME
- pathophys - presentation |
Paraneoplastic syndrome
- Autoimmune rxn against PRE-SYNaptic voltage gated cA2+ CHANNELS = NO ACH release CLINICAL: - Slow prog weakness, fatigue that starts w/ lower legs - also autonomic dysfxn - ABSENT reflexes --> brief sustained mm axn = transient ^ in strength & reflex may appear - ocular & bulbar symptoms are less obvious |
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LES
- DX -TX |
DX:
#1. Blood test for Abs 2. EMG: INCREMENTAL response TX: #1. Be vigilant in looking for small cell lung CA (LES usually precedes it) - CHEST CT every 6 mo. |
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BOTULISM
- PATH - clinical presentation |
C. botulinum spores
- esp in black tar heroin or honey for infants, home canned goods - block ACH release at pre-syn PRESENT: - RAPID, DESCENDING weakness (vs. GBsyn) BOARDS: FIXED, DILATED PUPILS (usu only seen in coma pts) + fast diffuse weakness + ocular/bulbar wkness |
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BOTULISM
- DX -TX |
DX:
Stool culture for bug & toxin assay emg: incremental response tx: mechanical vent - remove toxin (gastric lavage) or wound tx. - abx & botulinum antitoxin |
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65 yo male smoker presenting w/ fatigue, dry mouth, constipation and leg weakness starting about 6 months ago.
- no reflexes on pE |
LES
- do blood test & chest ct for CA |
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Entire family except for 5 mo. old baby presents to ED with weakness all over starting at about 7pm that night. They live in a rural area about an hour away. Squinting during exam - sensitive to light. fixed, dilated pupils upon exam
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Botulism
- check for resp. failure / need for vent - give antitoxin & gastric lavage & abx |
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Consequences of myelin diseases in adults vs. children
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adults: psych problems, dementia. sensory disturbances, etc.
children: MENTAL RETARDATION!! |
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MULTIPLE SCLEROSIS
- classical - Devic's Disease = neuromyelitis optica - Acute MS (Marburg's) |
FOUR FEATURES:
- multiple myelinoclastic lesions of CNS - does NOT involve PNS myelin - Protracted clinical course - Periods of clinical relapse & remission * axons are preserved in early stages * - classic MS = Charcot's MS - Devic's: lesions only at SC, optic chiasm, & optic nerves |
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MS
- LESIONS/plaques |
RANDOM DISTRIBUTION OF LESIONS IN CNS
- UL OR BL - preference for periventricular areas *gray matter can also be involved* EARLY PLAQUES: - poorly demarcated & soft - NO axonal damage - minor degree of remyelination & reactive astrocytosis LATE: - well demarcated & firm (reactive astrogliosis) - +axonal damage - OG cells are now extinct --> no more remyelination |
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MS PATHOLOGY
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Immune-mediated process (mostly cell-mediated)
- WBCs populate the VR spaces around venules of an early lesion - foamy macros clean up destroyed myelin - plaques fuse |
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MS CLINICAL PRESENTATION
**UNEXPLAINED UL VISION LOSS** |
WAX & WANE
1. Weakness & paresthesias in >1 limb 2. Diplopia / UL loss of vision 3. Incoordination, vertigo 4. Painful muscle spams, CN5 Neuralgia 5. cognitive impariment, depression 6. seizures - incontinence (sc) - nystagmus |
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MS
- epidimiology - genetics - dx |
Female > male
young adult (20-40) Ave survival: 20-25 yr Genetic: Assc'd with HLA groups (HLA-DR2) - 15-20X > RISK in 1st degree relatives - strong genetic component LABS: - MRI is best to view plaques - Evoked potential: slowing of conductivity - CSF: increased Ig & oligoclonal bands |
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PERIPHERAL NEUROPATHY
- symptoms - signs *motor, sensory, autonomic* **absent ankle jerks are common in >70yo **IBM - flexors are more affected** |
SYMPTOMS
- SENSORY: "stocking-glove" distribution of distal dysesthesias. * feet are affected first since their axons are longer * - MOTOR: distal weakness, first EXTENSORS - AUTONOMIC: orthostasis (dizzy), impotent, gastroparesis, skin&hair changes SIGNS: -SENSORY: distal STT (more-so)or Post columns affected (fingers & toes affected first) - MOTOR: distal extensor weakness (lower ext); atrophic mm. * check bulk of the intrinsic hand muscles & extensor digitorum brevis - ABSENT STRETCH REFLEXES (LMN); esp ankle jerks - AUTONOMIC: orthostatic hypotension |
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DISTRIBUTION IN PERIPHERAL NEUROPATHIES
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1. Symmetrical generalized polyneuropathies
- distal --> proximal - stocking glove - MOST neuropathies 2. MULTIFOCAL NEUROPATHIES: - ASYMM weakness - usually 2' vasculitis (mononeuritis multiplex) --> ischemic infarct in individual mm. 3. FOCAL NEUROPATHIES (mononeuropathies): - entrapment/compression syndromes - FOCAL loss of myelin --> conduction block (but myelin can be replaced in the PNS readily) ex//carpal tunnel, ulner or peroneal neuropathy |
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DIABETES
- PN TYPE |
SYMMETRICAL GENERALIZED NEUROPATHY
- most common cause of neuropathy |
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CHRONIC ALCOHOLISM
- PN type |
common cause of PN
- symmetrical sensorimotor symptoms * severe burning pain is common symptom (small-fiber PN) * TAKE A GOOD ETOH HISTORY - CHECK FOR VITAMIN DEFICIENCY (b12, b6) |
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VITAMIN B DEFICIENCIES
- PN TYPE **always check vitamin levels on patient with peripheral neuropathy** |
B12 DEFICIENCY: degen of SC white matter pathways: posterior columns & CST
B6 TOXICITY: megadoses of B6 can cause severe sensory NEURONopathy - lesion in DRG --> severe sensory ataxia & gait disturbance - vibration, proprioception & gait ataxis **loss of myelin on large nerves --> trip, fall a lot |
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HYPOTHYROID NEUROPATHY VS. HYPERTHYROID
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HYPO:
- sensorimotor PN & carpal tunnel - burning distal pain --> motor involvement - slowed AXONAL TRANSPORT - tx hypothyroidism and PN will improve HYPER: - SUBACUTE leg weakness - LOSS of reflexes - mild sensory findings **associated findings are key to these two dzs* |
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ANTIRETROVIRAL AGENTS & PN
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HIV MEDS COMMONLY CAUSE PN, don'tneed to know specifics
- most are painful small fiber PNs - mimics the PN caused by HIV virus |
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ANTITUBERCULOUS AGENTS & PN
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giving INH w/o B6 --> sensorimotor PN
pyridoxine = B6 |
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ANTIMICROBIAL AGENTS
- flagyl - macrodnatin |
METRONIDAZOLE (FLAGYL)
- given over a looong period of time = sensorimotor PN & encephalopathy (confusion, sleepy) NITROFURANTOIN (MACRODANTIN): - used to tx chronic UTIs - sensorimotor PN usually within 6 wks of starting tx - SEVERE WEAKNESS is possible. |
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PHENYTOIN & PN
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PHENYTOIN (DILANTIN)
- 50% get aymptomatic loss of reflexes - well known to cause PN |
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LEAD NEUROPATHY
|
KIDS: chronic / acute confusion
ADULTS: - confusion - PN: motor > sensory - GI - weightloss, anemia, fatigue - renal dysfxn - SELECTIVE RADIAL NEUROPATHY (Frankenstein) tx: penicillamine (chelating agent) |
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ARSENIC POISONING & PN
*in contaminated well water* |
*similar to GBS b/c weakness can get better after a few weeks
- but Arsenic PN is due to SEVERE AXON LOSS ( NOT DEMYEL) SYMPTOMS: - GI (if high dose) - Tachy, hypotension, encephalopathy - PN is acute, severe weakness (days - weeks) - motor > sensory, - areflexia chronic exposure to low levels: - MEE'S LINES (transverse white) - hyperpigmentation, - hyperkeratosis - alopecia - constitutional symptoms - sotkcing glove PN burning & pain tx w/ penicillamine |
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ARSENIC POISONING & PN
*in contaminated well water* |
*similar to GBS b/c weakness can get better after a few weeks
- but Arsenic PN is due to SEVERE AXON LOSS ( NOT DEMYEL) SYMPTOMS: - GI (if high dose) - Tachy, hypotension, encephalopathy - PN is acute, severe weakness (days - weeks) - motor > sensory, - areflexia chronic exposure to low levels: - MEE'S LINES (transverse white) - hyperpigmentation, - hyperkeratosis - alopecia - constitutional symptoms - sotkcing glove PN burning & pain tx w/ penicillamine |
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VASCULITIS ASSOCIATED NEUROPATHY
|
MOST COMMON CAUSE OF PN IN PATIENTS WITH CT DISORDERS
= MULTIFOCAL PN - ischemic infarct of vasa nervorum - ASYMMETRICAL - individual nn are involved * polyarteritis nodosa * - system manifestation of vasculitis = sick all over - most common vasculitis of PNS - high sedimentation rate tx: high dose steroids & oral cyclophosphamide (immunosuppressive tx) |
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AUTOIMMUNE DZ & PN
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SLE & SJOGREN'S = DEMYLINATING PN
- sjogren's also has sensory NEURONopathy RHEUMATOID ARTHRITIS & SCLERODERMA: entrapment neuropathies |
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GUILLAIN-BARRE SYNDROME
(acute inflamm demyelinating polyradiculoneuropathy - AIDP) *radiculopathy = pain* **campylobacter jejuni = more severe case & diarrheal illness |
most common cause of ACUTE (days), FLACCID paralysis
- Botulism also causes flaccid paralysis Immune-mediated - preceded by viral illness (fever): similar to...? - Abs against the infxn attach PNS myelin instead (including nn roots) SYMPTOMS & SIGNS: - SENSORY: distal paresthesias = 1st sympton, pain, large myel fibers more affected - MOTOR: MOST HAVE LIMB WEAKNESS **ASCENDING PARALYSIS* lasting < 3 weeks - PROXIMAL > distal - cn 7 = BL facial weakness - AREFLEXIA (LMN) - autonomic things (esp if needs to be admitted) *resp failure might happen within weeks1-2* |
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GBS
- DIAGNOSIS - TX GBS is monophasic (only 1x) vs. CIDP |
DX:
- CSF: albuminocytological dissoc (high protein, normal inflamm cells) due to intense demyel - EMG: asymm demyel with conduction block TX: - Admit ASAP in early phase (resp fail fear - peak is 1-2 wks) - Plasma exchange & IVIg *3-5% still die* |
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MILLER-FISHER VARIANT OF GBS
- similarites - differences - testing |
Milder GBSyn
TRIAD: ophthalmoplegia, ataxia, areflexia Time frame: same as GBS (3-4 wks) anti-GQ1b Abs |
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CIDP = CHRONIC INFLAMM DEMYELINATING POLYRADICULOPATHY
- compare to MS & GBS - symptoms - dx & tx |
Demyelination of PNS (MS = CNS)
- only 1/3 have flu symptoms first - onset: 30s-50s SLOWLY progressive (have it for months b4 seeing Dr.), lifelong disease - intermittent relapses * motor, vibration, proprioception aff'd = big myel'd neurons * - AREFLEXIA (LMN dz) DX: same as GBS: elevated csf protein & EMG = asymm demyelination w/ conduction blocks TX: Steroids, plasmapharesis, IVIg, azathioprine (immunosuppress) |
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CHARCOT-MARIE-TOOTH (CMT)
- genetics - onset **2/3 of hereditary motor & sensory neuropathies |
Hereditary peripheral neuropathy
*extensive family hx* - autosomal dom (chrom17) - onset: teens-20s Mostly motor nn - BL stork legs (atrophy) & foot drop - high arches & hammer toes - slow, progressive DX: NCS & genetics (slow conduction velocity) |
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HEREDITARY NEUROPATHY WITH SUSCEPTIBILITY TO PRESSURE PALSIES
(HNPP) |
autosomal dominant (chrom17)
- FOCAL areas of demyelination due to PRESSURE *episodic, recurrent pressure palsies* ex// peroneal n. at fibular head, ulnar n. at elbow *(superficial at these points) |
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IDIOPATHIC SENSORY PN
- DX - SCREENING * 1/3 of PNs seen in clinic* |
DX OF EXCLUSION
- mainly numbness/pain in toes/feet - common w/ ^ age - no foot drop |
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HERPES ZOSTER NEUROPATHY
(SHINGLES) |
vIRUS HIDES IN SENSORY N. GANGLION
* post-herpetic neuralgia (PHN) = SEVERE pain in dermatome burning, electric pain - radiculopathy & CN palsies too tx: acyclovir STAT - gabapentin, tricyclic antidepressants, narcotics |
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FOCAL MONONEUROPATHIES
- carpal tunnel - unlar - radial - peroneal **focal loss of myelin 2' compression --> conduction block** |
CTS: MEDIAN N.
- numbness of digits 1-3 - nocturnal awakening (MUST ask if it's worse at morning or night?) - abductor pollicis brevis can atrophy = lateral thenar eminence - proximal pain in shoulder - pregnant women ULNAR: - mostly motor/weakness - numbness of digits 4-5 - avoid elbow resting RADIAL: extensors affected = frankenstein - SAT. NIGHT PALSY: spiral groove (triceps spared) - Honeymooner's: axilla (triceps affected) - lead tox PERONEAL: FOOT DROP - most common entrapment in legs - dont' cross legs - numbness/sensory loss bw 1-2 toes |
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NERVE CONDUCTION STUDY
- indications for use - results *EMG = NCS & NEE |
ONLY USE EMG FOR PNS & muscle conditions - LMN
NCS tells you: - amplitude & area = estimate of amt of functioning n & muscle - distal latency: stimulus --> recording - conduction velocity: from latencies at 2 dif pts SENSORYCS: - same things **NMJ and muscle disorders will have NORMAL SNAPs - but SNCS are more senstiive than motor |
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NCS RESULTS
- axonal neuropathies - demyelinating neuropathies |
AXONAL: axon/soma death
- DECREASED CMAP/SNAP amplitude ex// diabetes *wallerian degen: proximal n. injury = distal degen* DEMYELINATING: - slower conduction velocities - prolonged distal latencies *conduction block: FOCAL drop in amplitude (only proximal to lesion/block) ex// entrapments & acquired demyelinating PNs (GBS) |
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NEE
- fibs -fasc |
evaluates spontaneous activity & voluntary motor units
FIBRILLATIONS: assc'd with injury to axon (only single axon/muscle fiber dying) FASCICULATIONS: muscle twitch - disorders of AHCs (like ALS) *popcorn sounds* Voluntary motor unit potentials: big amplitude & increased duration |
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CERVICAL RADICULOPATHIES
- FINDINGS |
• neck pain
• radiating pain down the arm • numbness, tingling and sensory loss in the involved dermatome • weakness in the involved myotome |
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REFLEXES
- c5,6,7, 8 |
• Biceps: C5,6; upper trunk, lateral cord, musculocutaneous nerve
• Brachioradialis: C5,6; upper trunk, posterior cord, radial nerve • Triceps: C6-8; upper/middle/lower trunk; posterior cord, radial nerve |
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DESCENDING CST
- location - arrangement - pathway |
Dorsolateral SC; UMN
- synapses on AHC Pathway: Motor cortex --> CST --> pyramidal decussation @ medulla --> LCST (lateral funiculus) - sacral (legs) are lateral/outer - cervical fibers = medial BIG, MYELINATED NEURONS |
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STT
- sensory type - pathway - synapses |
ASC PAIN & TEMP
- Ventrolateral portion of SC - SMALL, unmyelinated neurons PATHWAY: DRG --> ipsilat zone of lissauer --> ventral white commissure (cross) --> VPL of thalamus |
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Posterior columns
- sensory type - pathway |
BIG, MYELINATED periph nn
- asc. vibration & proprioception Fasciculus Gracilis: medial; legs - below T6 Fasciculus cuneatus: lateral; arms - above T6 - opposite of CST PATHWAY: (does NOT synapse in sC) DRG --> Ipsi Fasiculus gracilis/cuneatus --> 1st synapse = nucleus g/c --> CROSS = Int. arcuate fibers --> contralat VPL |
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DSCT
- location - what |
Lateral white matter of SC
- dorsal spinocerebellar tract - ascending: sends infro from muscle 2 cerebellum *accurate movement coordination* |
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SC DISEASES W/ MAJOR UMN FINDINGS
VS. |
UMN:
1. Compressive myelopathy: spondylolysis, disc herniation, CA (intra/extra-medullary) 2. Acute SC Trauma 3. Transverse Myelitis 4. AIDS myelopathy 5. Ant. Spinal A. occlusion (stroke) |
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SC DISEASES W/ MAJOR LMN FINDINGS
|
Poliomyelitis:
#1 cause of ACUTE flaccid paralysis ASYMM: - Weakness - atrophy - severe cramps - FASCICULATIONS** (2' loss of AHC) NO REFLEXES (LMN) |
|
COMPRESSIVE MYELOPATHY:
cervical cord involvement is most likely **Brown-sequard syndrome = boards** |
BELOW lesion: spastic, BL UMN findings
- brisk reflex, babinski, ankle clonus AT level of lesion: - Pain & radicular symtpoms - LMN findings Lhermitte's sign: electrical sensation down the spine w/ neck flexion *2' posterior column * - also loss of vibration & proprioception = balance & walking probs - BOWEL & BLADDER DYSFXN TOO! |
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BROWN-SEQUARD SYNDROME
|
tYPE OF COMPRESSIVE MYELOPATHY
ONLY 1/2 of SC is involved CROSS FINDINGS: - Ipsi loss of vibration & propioception - Ipsi spasticity & weakness - Contra loss of pain & temp **usually only CST & STT involved** |
|
ACUTE SPINAL CORD TRAUMA
- incidence - locations - presentation |
men in their 20s
Atlantoaxial joint, low cervical SC, thoracolumbar & lumbosacral regions Partial or complete paraplegia or quadriplegia w/ loss of sensation - below level of the lesion **SPINAL SHOCK: UMN findings might not happen right away (may take 1day) - common w/ ACUTE sc etiology tx: high dose steroids & sx |
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TRANSVERSE MYELITIS
- incidence - symptoms **most likely involves thoracic cord** |
YOUNG PT W/ MS
ACUTE inflamm (doesn't follow vasculature) HALLMARK: UMN findings in legs - spastic leg wkness - brisk reflexes *diminished sensation below lesion AT level of lesion: tight, band-like sensation (2' ventral white comm inflamm) + bowel & bladder dysfxn *SPINAL SHOCK & flaccid wkness possible* |
|
anterior spinal artery occlusion
(stroke) *boards* |
ANTERIOR SC = CST & STT
- diabetes & HTN - MID-THORACIC SC (watershed) *above a. of Adamkiewicz ACUTE ONSET: - paraplegia --> UMN findings in legs - lose STT below lesion **POST COLUMNS PRESERVED** STILL GET AN MRI |
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AMYOTROPHIC LATERAL SCLEROSIS
(ALS) - INCIDENCE - FINDINGS 55-75 yo (underreported) *Normal sensory & cognition* **FASCICULATIONS + WKNESS** |
UMN & LMN findings & PROGRESSIVE wkness
UMN: - Spastic wkness in ext. (CST) - Dysarthria & Dysphagia (CBT) (PSEUDOBULBAR PALSY) - Emotional lability LMN: - FASCICULATIONS** (AHC) - Bulbar palsy: dysarthria & dysphagia - wkness worse w/ exertion = presenting symptom DIE FROM RESP. FAIL (brisk reflexes can become NO reflex 2' super atrophy) |
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FALS
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Familial ALS
- autosomal dominant - 10 yr earlier onset |
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50 yo male presents to ER with burns on his hands and forearm. Despite 2nd degree burns, reports them as non-painful. on PE, nonresponsive to pain & temp BILATERALLY. progressive - nonacute
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SYRINGOMYELIA & CENTRAL SC SYNDROMES
- AFFECT VENTRAL WHITE COMMISURE (esp cervical SC) *STT: pain & temp * can progress to AHC & CST --> weakness *bilateral*** if it extends to the medulla, bulbar symptoms can occur |
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CENTRAL SC TUMORS
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similar symptoms as myelopathy & syringomyelia
(STT - BL loss of pain& temp) **SACRAL SPARING: last to be affected - lie outermost in STT |
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VIT B12 DEFICIENCY & NEURO DYSFXN
60 yo pts with malabsorption problems |
Megaloblastic anemia & glossitis, vitiligo
- elevated methylmalonic acid & homocysteine levels SUBACUTE COMBINED DEGEN (degen of SC & periph nn) 1. Gait abnormality (lose proprio) 2. Posterior column dysfxn 3. Distal PN (stocking glove) & temp sensory loss 4. UMN findins: CST |
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tabes dorsalis
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LATE LATE LATE findings of neurosyphilis
- POSTERIOR COLUMNS AFFECTED 1. SEVERE GAIT PROBLEM: sensory ataxia - Slapping gait 2. Severe radicular-type pain (shooting back pain) - dorsal nerve roots 3. bowel & bladder dysfxn |
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POSTERIOR SPINAL A. OCCLUSION/STROKE
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ACUTE LOSS of vib/prop BELOW level of lesion
- STOMPING/SLAPPING gait - NO WEAKNESS, NO PAIN - NORMAL TEMP SENSATION *posterior columns only* |
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HEREDITARY SPASTIC PARAPARESIS
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"UNCOMPLICATED HSP"
- GAIT disturbance - SPASTIC lower ext. weakness - urinary urgency *usu autosomal dominant* |
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AMN
ADRENOMYELONEUROPATHY |
AMN: check family hx
X-LINKED: MEN ONLY 1. onset: 30-40 yo 2. PROGRESSIVE spastic paraparesis 3. "pure" AMN: no brain involvement; better prognosis dx: - elevated VLCFAs (peroxisomal fatty acid oxidation) - MRI: atrophy w/o edema |
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STATIN MYOPATHY
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HMG-CoA REDUCTASE INHIBITORS
- MYALGIAS - MYOPATHY w/o weakness (rare: severe rhabdomyolysis) |