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Podopediatrics > 5) Neuromuscular Disease > Flashcards

5) Neuromuscular Disease Flashcards

1
Q

Progressive neuromuscular diseases

A
  • Peroneal muscular atrophy
  • Hereditary spinocerebellar atrophy
  • Hypertrophic interstitial neuritis
  • Progressive muscular dystrophy
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2
Q

Non-progressive neuromuscular disease

A
  • Cerebral palsy
  • Myelomeningocele
  • Diastematomyelia
  • Poliomyelitis
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3
Q

Cerebral palsy

A
  • Non-progressive disorder of the CNS resulting in impairment of motor function and originating in perinatal time frame
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4
Q

Cerebral palsy etiologies

A
  • Insult to CNS during perinatal or young childhood age
  • Anoxic
  • Vascular injury
  • Toxin
  • Metabolic
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5
Q

Areas affected with cerebral palsy

A
  • Cerebral cortex
  • Base of brain
  • Cerebellum
  • Diffuse
  • Patterns of muscles affected, not individual muscles
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6
Q

Cerebral palsy classification

A
  • Spastic (cortical): 50 %
  • Athetosis (base of brain): 25 %
  • Ataxic (cerebellar): 7 %
  • Tremor (base of brain): 1 %
  • Rigid (diffuse): 7 %
  • Flaccid (cortical): 7 %
  • Mixed: 10 %
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7
Q

Cerebral palsy diagnosis

A
  • Perinatal complications
  • Neonatal hypotonia
  • Hypertonicity in first few months
  • Delayed developmental milestones
  • Abnormal reflexes
  • Gait abnormalities: “Scissor gait”
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8
Q

Cerebral palsy physical examination (hip)

A
  • Flexor contracture
  • Adductor contracture
  • Femoral anteversion
  • Subluxation / dislocation
  • Degenerative joint disease
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9
Q

Cerebral palsy physical examination (knee)

A
  • Flexion contracture
  • Internal tibial torsion
  • Stiff knee
  • Genu recurvatum
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10
Q

Cerebral palsy physical examination (ankle/foot)

A
  • Equinus
  • Varus (pes cavus)
  • Valgus (pes planus)
  • Hallux valgus
  • Flexion contractures
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11
Q

Cerebral palsy conservative management

A
  • Ankle foot orthoses
  • U C B L
  • Richie brace
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12
Q

Cerebral palsy surgical management

A
  • Tendon lengthening
  • Tendon transfer
  • Subtalar arthrodesis
  • Triple arthrodesis
  • MTPJ arthrodesis
  • Metatarsal osteotomy (?)
  • Digital arthroplasty / arthrodesis
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13
Q

Myelomeningocele

A
  • Developmental defect of the spinal column
  • Failure of vertebral arch fusion
  • Dysplasia of spinal cord
  • Cystic distention and meningeal protrusion
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14
Q

Myelomeningocele physical examination

A
  • Flaccid paralysis of lower limbs

- Motor and sensory loss at and below the level of the lesion

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15
Q

Myelomeningocele physical examinaton common deformities

A
  • Pes calcaneus
  • Calcaneovalgus
  • Equino adductovarus
  • Convex pes valgus
  • Equinus, cavus, clawtoes
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16
Q

Myelomeningocele management

A
  • Orthoses for neuropathic feet
  • Bracing
  • Surgery
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17
Q

Poliomyelitis

A
  • Viral infection which occurs during childhood (contaminated water)
  • Virus attacks anterior horn cells
  • Some degree muscle wasting and flaccid paralysis
  • Rare: < 1000 cases/ year in USA
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18
Q

Poliomyelitis physical examination common deformities

A
  • Pes calcaneus
  • Calcaneovalgus
  • Equino adductovarus
  • Convex pes valgus
  • Equinus, cavus, clawtoes
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19
Q

Poliomyelitis management

A
  • Vaccine
  • Bracing
  • Orthoses
  • Surgery
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20
Q

Peroneal muscular atrophy

A
  • Charcot – Marie – Tooth disease
  • Genetic disorder (autosomal recessive, sex-linked recessive, autosomal dominant)
  • Progressive degeneration of the peripheral nerves, motor nerve roots, and spinal cord
  • Demylenation with loss of anterior horn cells
21
Q

CMT physical examination

A
  • Symptoms begin between ages 5 -15
  • Lower extremity affected initially, followed by upper extremity
  • Atrophy of all lower leg musculature with exception of tibialis posterior
  • Wasting of plantar and palmar intrinsic muscluature
  • Absent DTR’s
22
Q

CMT physical examination findings

A
  • Flaccid paralysis
  • Drop foot deformity
  • Champagne glass legs
  • 80 % pes cavus
  • 20 % pes planus
23
Q

CMT management

A
  • Orthoses
  • Bracing (ankle – foot orthosis); Dorsiflexion-assist AFO
  • Surgery (posterior tibialis transfer to dorsum of foot), arthrodesis
24
Q

AFO presentation

A
  • Dropfoot deformity with stable knee: Hinged AFO

- Dropfoot deformity with unstable knee: Fixed AFO

25
Q

Weak anterior/posterior leg AFO prescription

A
  • Leaf – spring AFO
26
Q

Toe walking (uncompensated ankle equinus) AFO prescription

A
  • Hinged AFO with plantarflexion stop
27
Q

Fixed ankle equinus AFO prescription

A
  • AFO in equinus with heel lift
28
Q

Foot / ankle in flexible varus / valgus AFO prescription

A
  • Tension band AFO
29
Q

Roussy-Levy syndrome

A
  • Form fruste of CMT disease
  • Abortive form of Friedreich’s ataxia
  • Clinical presentation similar to CMT
  • Static tremor of hands present (not in CMT)
30
Q

Friedreich’s Ataxia

A
  • Progressive hereditary spino – cerebellar ataxia
  • Genetic: autosomal recessive, exact etiology unknown
  • Degeneration of spino – cerebellar tracts, corticospinal tracts, posterior columns
  • Atrophy of purkinjie cells and dentate nuclei in the cerebellum
31
Q

Friedreich’s ataxia physical examination

A
  • Symptoms begin at 7 – 10 years old
  • Unsteady gait may be initial complaint
  • Upper extremity usually affected initially (difficulty holding fork, writing)
  • Thoracic scoliosis
  • Lower extremity muscular weakness (peroneal)
  • Pes cavus
32
Q

Friedreich’s ataxia management

A
  • Orthosis
  • Bracing
  • Surgery
  • Death during second decade secondary to myocardial failure (interstitial myocarditis)
33
Q

Dejerine-Sottas disease

A
  • Hypertrophic interstitial neuritis; chronic familiar polyneuropathy of childhood and adolescence
  • Possibly a variant of tabes dorsalis
  • Exact etiology unknown
  • “Onion bulb” formation around nerves due to proliferation of Schwann cells
34
Q

Dejerine-Sottas disease physical examination

A
  • Lower extremity muscular atrophy
  • “Stocking glove” sensory deficit
  • Difficulty walking
  • Pes cavus
  • Digital contractures
  • Argyll – Robertson pupils
35
Q

Dejerine-Sottas disease management

A
  • Orthoses
  • Bracing
  • Surgery
36
Q

Duchenne muscular dystrophy

A
  • Progressive muscular dystrophy

- X – linked recessive inheritance

37
Q

Duchenne muscular dystrophy characteristics

A
  • X-linked recessive genetic disease
  • Progressive muscle degeneration
  • Proximal muscles affected initially
  • Children wheel chair – bound by teenage years
  • Death during twenties due to cardiac / respiratory muscle failure
38
Q

Duchenne muscular dystrophy physical examination

A
  • Proximal muscular weakness (Gower’s sign)
  • Pseudohypertrophy of calf muscles
  • Toe walking (later onset)
  • Elevated CPK, serum aldolase
  • Muscle biopsy definitive
39
Q

Duchenne muscular dystrophy management

A
  • Bracing
  • Physical therapy
  • Death by second decade secondary to cardiac / respiratory failure
40
Q

W/A T.A.

A
  • Clinical presentation = foot drop/slap

- Compensation = steppage gait

41
Q

W/A T.P.

A
  • Clinical presentation = Ex. pronation

- Compensation = flexor substitution

42
Q

Spastic R.F.

A
  • Clinical presentation = stiff knee gait

- Compensation = circumduction

43
Q

W/A gluteus medius

A
  • Clinical presentation = Trendelenburg gait
44
Q

Spastic gastrocnemius

A
  • Clinical presentation = knee hyperextension
45
Q

Ankle equinus

A
  • Clinical presentation = early/absent HC/HO, TW

- Compensation = Ex. pronation, ext. gait, genu recurvatum

46
Q

W/A gluteus maximus

A
  • Clinical presentation = lumbar lordosis
47
Q

W/A quads

A
  • Clinical presentation = buckling knee

- Compensation = knee hyperextension

48
Q

Tight/spastic adductors

A
  • Clinical presentation = scissoring gait

Podopediatrics (6 decks)

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