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NEUROLOGY (path) > Spinal cord diseases/Peripheral nerve/Myopathy > Flashcards

Spinal cord diseases/Peripheral nerve/Myopathy Flashcards

1
Q

Spinal Muscular Atrophy: SMA-1

A 
Pathophysiology: SMA1 infantile: manifests within 3 months (autosomal recessive)
Clinical: 
- hypotonia
- difficulty suckling, swallowing
-atrophy, fasciculations of the tongue
-absent plantar response
-wasting/weakness of extremities
- kyphoscoliosis

Dx/Rx:death by age 3

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2
Q

Spinal Muscular Atrophy: SMA-2

A

Pathophysiology: SMA2 intermediate: latter half of first year of life (autosomal recessive)
Clinical: gradually progressive proximal muscle weakness
Dx/Rx: supportive

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3
Q

Spinal Muscular Atrophy: SMA-3

A

Pathophysiology: SMA3 juvenile: hereditary vs. sporadic (autosomal recessive)

Clinical: gradually progressive proximal muscle weakness
Dx/Rx:

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4
Q

Amyotrophic Lateral Sclerosis

A

Pathophysiology:

  • umn and lmn signs in bulbar + spinal cord distribution
  • SOD1 mutation

Clinical:

  • bulbar involvement: dysphagia, dysarthria, wasting/fasciculations of tongue
  • UE/LE weakness: fatiguability, weakness, stiffness, twitching, wasting, cramps

Dx/Rx:

  • riluzole: blocks glutaminergic transmission
  • PEG tube for dysphagia
  • fatal within 3-5 years
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5
Q

Poliomyelitis

A

Pathophysiology: RNA picornavirus, fecal-oral transmission

Clinical:

  • prodrome: fever, myalgia, malaise
  • weakness, asymmetric, focal, or unilateral
  • DECREASED tone/reflexes
  • CSF: increased pressure, pleocytosis

Dx/Rx:

  • dx: stool culture
  • rx: supportive care
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6
Q

Diphtheric Polyneuritis

A

Pathophysiology:

  • corynebacterium diphtheria infection as URI or in skin wound
  • neuropathy 2/2 neurotoxin elaboration

Clinical:

  • palatal weakness
  • impaired pupillary responses
  • sensorimotor polyneuropathy
  • +/- respiratory paralysis

Dx/Rx:

  • diphtheria antitoxin
  • penicillin/azithromycin
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7
Q

Porphyria

A

Pathophysiology: attacks precipitated by drugs: barbiturates, estrogen, sulfonamides

Clinical:

  • colicky abdominal pain preceding neurologic involvement
  • +/- acute confusion/convulsions
  • weakness 2/2 polyneuropathy that is symmetric
  • decreased reflexes
  • fever, tachycardia, hyponatremia, peripheral leukocytosis

Dx/Rx:

  • dx: increased porphobiliogen and d-aminolevulinic acid in the urine
  • rx: IV dextrose to suppress heme pathway, propanolol to control tachycardia/hypotension
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8
Q

Lead toxicity

A

Pathophysiology: often occupational exposure

Clinical:

  • acute encephalopathy in kids 2/2 ingestion
  • painless peripheral neuropathy in arms > legs in adults
  • anemia, constipation, abdominal pain, nephropathy

Dx/Rx: EDTA

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9
Q

Multifocal motor neuropathy

A

Pathophysiology:

  • progressive asymmetric weakness with EMG evidence of demyelination
  • 2/2 antiglycolipid antibodies (anti-GM1 IgM)

Clinical:

  • pure motor multineuropathy beginning in arms
  • insidious onset with chronic course
  • conduction block on EMG

Dx/Rx: cyclophosphamide

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10
Q

Bell’s palsy

A

Pathophysiology:

  • LMN facial weakness without widespread CNS disease
  • common in pregnancy, DM

Clinical: facial weakness often preceded by pain around the ear

Dx/Rx: corticosteroids

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11
Q

Myasthenia Gravis

A

Pathophysiology:

  • a/w thymoma, thyrotoxicosis, RA, SLE
  • F>M
  • 2/2 immune mediated decrease in # of functioning Ach receptors
  • -> 80% v. nicotinic receptor; can have MuSK ab (muscle specific kinase)

Clinical:

  • exacerbated by infection
  • slowly progressive course
  • ptosis, diplopia, limb weakness with diurnal variation
  • extraocular muscle invovlement (90%)
  • sustained muscle activity temporarily increases weakness

Dx/Rx:

dx: anticholinesterase trial (edrophonium?)
rx: neostigmine, thymectomy, corticosteroids, azathioprine, myfortic (mycophenolate)

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12
Q

Eaton-Lambert Myasthenic syndrome

A

Pathophysiology:

  • antibodies against presynaptic voltage-gated calcium channels
  • a/w neoplasm

Clinical:

  • proximal muscle weakness
  • spares ocular muscles!

Dx/Rx:

dx: ab titers
rx: corticosteroids, azathioprine, Ca screening

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13
Q

Botulism

A

Pathophysiology:

  • prevents release of Ach at NMJ and autonomic synapses
  • 2/2 home canned foods
  • A, B, E toxin

Clinical:

  • fulminating weakness 12-72h after ingestion
  • diplopia, ptosis, facial weakness, dysphagia, respiratory failure
  • weakness in limbs
  • blurry vision, dry mouth, hypotension

Dx/Rx: antitoxin A, B,E

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14
Q

Duchenne Muscuar Dystrophy

A

Pathophysiology:

  • X-linked disorder
  • M>F
  • symptomatic onset by age 5
  • 2/2 absent/reduced dystrophin

Clinical:

  • early sx: toe walking, waddling, inability to run
    • Gower’s sign, +/- cardiac problems, +/- MR (mental retardation?)
  • pseudohypertrophy of the calves
  • increased creatine kinase
  • wheelchair bound by age 12

Dx/Rx: prednisone

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15
Q

Becker Muscular Dystrophy

A

Pathophysiology:

  • X-linked
  • symptomatic onset around 11 y
  • normal dystrophin levels, but ABNORMAL protein

Clinical:

  • early sx: toe walking, waddling, inability to run
    • Gower’s sign, +/- cardiac problems, +/- MR
  • pseudohypertrophy of calves
  • increased CK
  • wheelchair by age 12

Dx/Rx:

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16
Q

Myotonic dystrophy I?

A

Pathophysiology:

  • AD inheritance
  • manifests in 20s and 30s
  • 2/2 expanded CTG repeat on chromosome 19 myotonin protein kinase

Clinical:

  • myotonia (abnormal stiffness) + weakness/wasting in distal muscles
  • +/- cataracts, frontal balding, DM, cardiac abnormalities, ptosis, dysphagia, testicular atrophy, insulin resistance, cognitive changes

Dx/Rx:

  • increased CK (mild)
  • quinine sulfate
  • procainamide
  • phenytoin (myotonia)
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17
Q

Polymyositis

A

Pathophysiology:

  • onset at any age with variable rates
  • muscle fibers that express MHCI are invaded by CD8 T cells resulting in necrosis
  • commonly associated with other autoimmune syndrmes

Clinical:

  • muscle pain, tenderness
  • weakness, wasting in proximal limbs
  • a/w raynaud’s phenomenon
  • increased CK

Dx/Rx: prednisone

18
Q

Dermatomyositis

A

Pathophysiology:

  • microangiopathy of skin, muscle
  • lysis of capillaries 2/2 activation and deposition of complement –> muscle ischemia

Clinical:

  • associated w cancer
  • heliotrope rash, Gottron’s papules
  • increased CK
  • cardiac conduction problems, myocarditis, CHF, etc.
  • ILD (interstitial lung disease) in patients with Jo-1 antibodies

Dx/Rx: prednisone

19
Q

Inclusion body myositis

A

Pathophysiology: M>F; onset s/p 50 yo

Clinical:

  • painless proximal muscle weakness with lower>upper extremities
  • a/w autoimmunity, DM, peripheral neuropathy

Dx/Rx:

20
Q

Polymyalgia Rheumatica

A

Pathophysiology:

  • F>M; age > 50
  • variant of giant cell arteritis

Clinical:

  • muscle pain/stiffness in head and neck
  • h/a, anorexia, weight loss, fever
  • increased ESR

Dx/Rx: DRAMATIC response to steroids

21
Q

Acute necrotizing myopathy

A

Pathophysiology: 2/2 heavy binge drinking

Clinical:

  • muscle pain, weakness, dysphagia
  • muscles are swollen, tender, and weak
  • proximal weakness that may be asymmetric or focal
  • increased CK +/- myoglobinuria

Dx/Rx: Etoh abstinence

22
Q

Stiff person syndrome

A

Pathophysiology:

  • rare, sporadic
  • may be a/w autoimmune disorders, diabetes
  • glutamic acid decarboxylase (GAD) antibodies

Clinical:

  • tightness, stiffness, and rigidity of axial and proximal limb muscles with superimposed spasms
  • distinguish from tetanus by absence of lockjaw

Dx/Rx:

  • baclofen
  • vigabatrin
  • sodium valproate
  • gabapentin
23
Q

Neuromyotonia

A

Pathophysiology:

  • rare, sporadic vs. paraneoplastic vs. autosomal dominant
  • can be 2/2 voltage-gated K+ channel antibodies if acquired

Clinical:

  • continuous muscle stiffness
  • rippling muscle movement (myokymia)
  • delayed relaxation

Dx/Rx: phenytoin or carbamazepine

24
Q

Malignant Hyperthermia

A

Pathophysiology:

  • autosomal dominant
  • defect of ryanodine receptor gene on Chr 19
  • results in excitation-contraction coupling
  • precipitated by NMJ blocking agents (succinylcholine) or inhalational anesthetics

Clinical:

  • rigidity
  • hyperthermia
  • metabolic acidosis
  • myoglobinuria

Dx/Rx:

  • cessation of offending agent
  • Dantrolene
25
Q

Upper motor neuron lesion

A
  • weakness
  • spasticity
  • increased reflexes
  • babinski present
  • (-) atrophy
26
Q

Lower motor neuron lesion

A
  • weakness/paraparesis
  • fasciculations
  • hypotonicity
  • decreased reflexes
27
Q

Cerebellar dysfunction

A
  • hypotonia
  • decreased reflexes
  • ataxia, dysarthria
  • gait disturbance
  • eye mvmt problems
28
Q

Neuromuscular transmission

A
  • normal or decreased tone
  • normal or decreased reflexes
  • (-) sensory symptoms
  • patchy weakness distally
29
Q

Myopathic disorder

A
  • proximal weakness
  • no muscle wasting
  • normal plantar responses
  • no sensory loss
  • increased CK
30
Q

Total spinal cord transection

A

Pathophysiology:

  • acute stage
  • spastic plegia
  • terminal

Clinical:

  • flaccid paralysis, (-) tendon reflexes, sensory loss, urinary/fecal retention
  • brisk reflexes, extensor plantar responses
  • bladder/bowel regain function
  • flexor/extensor spasms in legs

Dx/Rx:

  • immobilization
  • corticosteroids
  • baclofen
31
Q

Multiple Sclerosis

A
  • F > M; age of onset 20-46
    Pathophysiology:
  • lesions separated in space/time
  • HLA-DR2
  • immune mediated attack vs. myelin antigens
  • relapsing/remitting vs. secondarily progressive

Clinical:

  • focal weakness, numbness, tingling, optic neuritis, diplopia
  • relapses triggered by infection, pregnancy, etc.
  • CSF: oligoclonal bands, lymphocytosis

Dx/Rx:

  • 2+ CNS lesions at 2 diff times
  • MRI
  • rx: interferon b-1A to prevent relapses
  • corticosteroids for acute rx of relapses
32
Q

ADEM

A

Pathophysiology:

  • single episode that develops s/p viral illness (varicella, measles, chickenpox)
  • perivascular demyelination w/ associated inflammatory rxn

Clinical:

  • HA, fever, confusion, +/- seizures
  • CSF: pleocytosis, normal glucose/protein

Dx/Rx:

33
Q

Epidural abscess

A

Pathophysiology:

  • sequelae of skin infection, sepsis, osteomyelitis, IVDU, trauma, etc.
  • 2/2 S.aureus, Strep pneumo, gram neg bacilli

Clinical:

  • fever, backache, tenderness
  • spinal root distribution of pain
  • neurologic emergency

Dx/rx:

  • MRI with contrast
  • abx + sx
34
Q

Vacuolar Myelopathy

A

Pathophysiology:

  • vacuolation of white matter most pronounced in lateral/posterior columns
  • thought to be 2/2 HIV-infection of cord
  • comorbid AIDS/dementia

Clinical:
- early incontinence, leg weakness, ataxia, ED, paresthesias, spasticity, (+) Babinski, decreased vibration/position sensation

Dx/rx:

35
Q

HTLV-1

A

Pathophysiology:

  • tropical spastic paraperesis
  • transmitted in breast milk, sex, blood

Clinical:

  • spastic paraperesis
  • decreased position/vibration sense
  • bowel/bladder dysfunction

Dx/rx:

36
Q

Tetanus

A

Pathophysiology:

  • clostridium tetani
  • elaborates neurotoxin that is transported retrograde to spinal cord
  • toxin interferes with release of NT

Clinical:

  • trismus (lockjaw)
  • difficulty swallowing
  • facial spasm (risus sardonicus)
  • muscle spasms/ rigidity
  • +/- increased CK, myoglobinuria
Dx/rx:
PPx: tetanus toxoid
Rx: debridement
- tetanus Ig if high risk wound
- penicillin or metronidazole for infection
37
Q

Spinal cord infarction

A

Pathophysiology:

  • 2/2 anterior spinal artery territory
  • s/p trauma, aortic aneurysm, polyarteritis nodosa, hypotension

Clinical:

  • acute onset flaccid paraparesis
  • evolves into spastic paraperesis
  • pain/temp lost
  • position/vibration retained

Dx/rx: symptomatic

38
Q

AVM

A

Pathophysiology:
- usually involve the lower part of the cord –> motor/sensory disturbances in the legs

Clinical:

  • may present with subarachnoied hemorrhage or myelopathy
  • bruit audible over the spine

Dx/rx:

  • MRI appearance suggests dx
  • extramedullary lesions treated with embolization and excision
39
Q

Cervical spondylosis

A

Pathophysiology: 2/2 chronic disk degeneration with herniation and secondary calcification

Clinical:

  • pain/stiffness in the neck, arms, +/- motor deficits
  • present with neck pain and limitation of head movement
  • affects C5/C6 nerve roots

Dx/rx:

dx: x-ray
rx: cervical collar

40
Q

Spinal tumor

A

Pathophysiology: intramedullary [ependymoma] vs. extramedullary [neurofibroma, meningioma]

Clinical:

  • insidious onset of symptoms
  • radicular pain
  • motor symptoms may develop
  • CSF: xanthrochromia, increased protein

Dx/rx:

NEUROLOGY (path) (8 decks)

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