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Peds PM&R > Muscle Disorders > Flashcards

Muscle Disorders Flashcards

1
Q

Infantile Botulism

A

Continued intraintestinal production of toxins after ingestion of spores

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2
Q

Foodborne Botulism

A

Preformed toxin is ingested in a single episode

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3
Q

How does C. botulinum flourish in infant?

A

Infant GI tract as low oxygen and low acid d/t lack of protective bacterial flora so C.botulinum can flourish

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4
Q

What does Eteric toxin of C. botulinum do?

A

Causes intestinal motility and progressive paralysis d/t Ach release at NMJ

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5
Q

Risk factors for Botulism

A

Honey in infants
Weaning from breast feeding to foods
March & November
Aminoglycosides potentiate weakness at NMJ

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6
Q

Clinical features of Botulism

A 
Constipation
Bulbar and extremity weakness
Dysphagia
Weak cry
Resp compromise
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7
Q

Onset of Botulism

A

18-36 hours after consumption

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8
Q

Resolution of Botulism

A

Weeks-months with supportive care

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9
Q

EMG findings in Botulism

A

Reduced amp
Increased + sharp waves
Fibrillations
Single fiber EMG showed increased jitter and blocking

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10
Q

Nerve conduction in Botulism

A

Reduced compound motor amplitude

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11
Q

Treatment of Botulism

A

Botulism Immune Globulin IV

Supportive care

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12
Q

Becker Muscular Dsytrophy (BMD)

A

More mild than Duchenne

Progressive limb-girdle pattern of weakness, calf hypertrophy and loss of ambulation after age 15yo

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13
Q

Becker Muscular Dsytrophy (BMD) Inheritence

A

X-linked on dystrophin gene Xp21

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14
Q

Cause of death in Becker Muscular Dsytrophy (BMD)

A

Respiratory or cardiac dz by age 30yo

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15
Q

Becker Muscular Dsytrophy (BMD) Exam

A 
Symmetric weakness (hip girdle/quad>upper limb)
Preserved neck flexion
Pseudohypertophy of calves 
Gower's sign
Toe-walking w/ heel cord contractures
Dec or absent reflexes
Intact sensation
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16
Q

Becker Muscular Dsytrophy (BMD) Testing

A

CK 5-1,000x ULN
DNA tesing
Muscle bx with stain to quantify dystrophin

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17
Q

Congenital muscular dystrophy (CMD)

A

Perinatal muscle weakness w/ hypotonia
Joint contractures
ABN muscle bx

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18
Q

Congenital muscular dystrophy (CMD) Inheritance

A

Autosomal recessive

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19
Q

Congenial myotonic muscular dystrophy

A 
Muscle weakness and wasting
Myotonia
Cataracts 
Cardiac conduction problems
Restrictive lung dz
Cognitive impairment
Inc risk for diabetes
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20
Q

Duchenne Muscular Dystrophy

A

neuromuscular dz with progressive loss of strength in hips, pelvic area, thighs and shoulders

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21
Q

Duchenne Muscular Dystrophy Inheritance

A

X-linked recessive
Chr Xp21
96% w/ frameshift mutation

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22
Q

Duchenne Muscular Dystrophy CF

A

Muscle weakness in boys by age 3yo (proximal>distal)
Calves are enlarged
Obesity by 10yo
Dilated Cardiomyopathy

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23
Q

Cause of death in Duchenne Muscular Dystrophy

A

Respiratory or cardiac failure by 15-25 years

Life prolonged 6-25 years with resp support

24
Q

Duchenne Muscular Dystrophy Testing

A

CK 5-100x normal
Xp21 deletion, duplication, small/point mutation
Muscle bx: dystrophin absent staining

25
Q

Emery-Deifuss Muscular Dystrophy (EDMD)

A

Hereditary myopathy

26
Q

Emery-Deifuss Muscular Dystrophy (EDMD) CF

A

Contractures of elbows, posterior neck and ankles
Muscle weakness and wasting
Cardiac dz (arrhythmia and CM)

27
Q

Facioscapulohumeral Muscular Dystrophy (FSHD)

A

3rd MC MD

Slow progression of weakness, mainly facial and shoulder girdle muscles followed by leg, thigh and hip girdle weakness

28
Q

Facioscapulohumeral Muscular Dystrophy (FSHD) Inheritance

A

Autosomal dominant

Reduction in D4Z4 repeats on 4q35

29
Q

Myasthenia Gravis

A

NMJ w/ defect in proteins required for neuromuscular tansmission or auto-Ab to the nicotinic Ach rec at the NMJ

30
Q

Myasthenia Gravis CF

A 
Weakness that improves with rest
Ptosis
Diplopia
Dysphagia
Dysphonia
Resp involvement
Proximal muscle weakness
No sensory, bowel or bladder inolvement
31
Q

Myasthenic crisis

A

Exacerbation of sx to require ventilator support

Triggered by fever, infection, medications or stress

32
Q

Medications that cause Myasthenic crisis

A 
Aminoglycosids
Macrolides
Beta blockers
ACE-I
Quinidine
Lidocaine 
Procainamide
CNS dugs ( phenytoin)
Antirheumatics-chloroquine 
D-Penacillamine
Prednisone
33
Q

Myasthenia Gravis Tx

A 
AchE inhibitors
Immunosuppression (steroids, azathioprine, cyclosporine, cyclophosphamide)
Plasma exchange
IVIG
Thymectomy (some cases)
34
Q

Poliovirus

A

Enterovirus
RNA virus
PV1 MC, also PV2 and PV3

35
Q

Polio vaccines

A

Sabin and Salk vaccines contain Ag from all 3 polio viruses

Salk is dead virus, only one used in US

36
Q

Polio transmission

A

Fecal oral

Peak in summer and fall

37
Q

Pathogenesis of Polio

A

Virus enters GI tract and lymphatic cells
Viremia w/ flu like sx
Spreads to neurons w/ invasion to anterior horn cells
Death of AHC leaves skeletal muscle w/o trophic factors to maintain muscle

38
Q

Polio CF

A 
Asymmetric paralysis of muscles
Preservation of sensation
Muscle pain
Loss of reflexes
Constipation/difficulty voiding
39
Q

Polio EDX

A

Loss of axons
Preservation of sensory conductions
Positive sharp waves and fibrillations
Late EDX: large amp motor units d/t reninnervation

40
Q

Spinal Muscular Atrophy

A

Group of neurodegenerative d/o characterized by progressive symmetric weakness and atrophy d/t loss of anterior horn cells of the SC and motor cranial nerve nuclei V, VII, IX, X, XI and XII

41
Q

Inheritance of Spinal Muscular Atrophy

A

Autosomal recessive on Chr 5q SMN (survival motor neuron) gene

42
Q

How is Spinal Muscular Atrophy classified?

A

Age of onset and disease severity

43
Q

Type of Spinal Muscular Atrophy

A

SMA I: acute infantile, Werdnig-Hoffman
SMA II: chronic infantile, intermediate
SMA III: chronic juvenile, Kugelberg-Welander
SMA IV: adult onset

44
Q

SMA I History

A

Onset birth to 6 mo
Never sits independently
Death usually prior to 2 yo, later in some cases esp w/ technology

45
Q

SMA II History

A

Onset 6-18 mo
Will sit but never walk
Death MC in 20-30’s

46
Q

SMA III History

A

Onset >18 yo
Walks independently
May have normal lifespan

47
Q

SMA IV History

A

Onset mid-30’s
Slowly progressive weakness
Transitioning to WC dependence over 20 years
Normal life expectancy

48
Q

SMA Exam

A

Mild facial weakness w/ sparing of extraocular muscles
Tongue fasciculation’s and/or poor suck
Frog-leg positioning w/ abd breathing
Scoliosis SMA II> III
Joint contractures
Wide-based Trendelenburg gait if ambulatory
Sensation intact
Fine tremor in hands
Dec muscle tone and bulk, proximal>distal
Reduced or absent reflexes
Normal to above normal intelligence

49
Q

SMA Labs

A

Serum CK normal to 2 x normal

50
Q

SMA EMG Findings

A 
Spontaneous potentials
Fasciculations in SMA II and III
Large amp
Long duration
Polyphasic motor unit action potentials (MUAP)
51
Q

Red flags in SMA

A

Severe metabolic acidosis may occur during illness or fasting
Resolves w/ IVF in 2-4 days

52
Q

Moyamoya Syndrome

A

Congenital constriction of cerebral arteries, esp internal carotid artery, w/ collateral circulation that appears like a “puff of smoke” on ateriography

53
Q

Hemiparesis recovers ___ to ___.

A

Proximally to distally

54
Q

Torticollis

A

Neck deformity w/ shortening of SCM muscles resulting in limited neck rotation and lateral flexion.

55
Q

Torticollis results in a ___ to the affected side and ___ to the contralateral

A

Head tilt

Rotation

56
Q

Sandifer’s Syndrome

A

Associated GERD and torticollis

Peds PM&R (15 decks)

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