5) Neuromuscular Disease Flashcards
1
Q
Progressive neuromuscular diseases
A
- Peroneal muscular atrophy
- Hereditary spinocerebellar atrophy
- Hypertrophic interstitial neuritis
- Progressive muscular dystrophy
2
Q
Non-progressive neuromuscular disease
A
- Cerebral palsy
- Myelomeningocele
- Diastematomyelia
- Poliomyelitis
3
Q
Cerebral palsy
A
- Non-progressive disorder of the CNS resulting in impairment of motor function and originating in perinatal time frame
4
Q
Cerebral palsy etiologies
A
- Insult to CNS during perinatal or young childhood age
- Anoxic
- Vascular injury
- Toxin
- Metabolic
5
Q
Areas affected with cerebral palsy
A
- Cerebral cortex
- Base of brain
- Cerebellum
- Diffuse
- Patterns of muscles affected, not individual muscles
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 %
7
Q
Cerebral palsy diagnosis
A
- Perinatal complications
- Neonatal hypotonia
- Hypertonicity in first few months
- Delayed developmental milestones
- Abnormal reflexes
- Gait abnormalities: “Scissor gait”
8
Q
Cerebral palsy physical examination (hip)
A
- Flexor contracture
- Adductor contracture
- Femoral anteversion
- Subluxation / dislocation
- Degenerative joint disease
9
Q
Cerebral palsy physical examination (knee)
A
- Flexion contracture
- Internal tibial torsion
- Stiff knee
- Genu recurvatum
10
Q
Cerebral palsy physical examination (ankle/foot)
A
- Equinus
- Varus (pes cavus)
- Valgus (pes planus)
- Hallux valgus
- Flexion contractures
11
Q
Cerebral palsy conservative management
A
- Ankle foot orthoses
- U C B L
- Richie brace
12
Q
Cerebral palsy surgical management
A
- Tendon lengthening
- Tendon transfer
- Subtalar arthrodesis
- Triple arthrodesis
- MTPJ arthrodesis
- Metatarsal osteotomy (?)
- Digital arthroplasty / arthrodesis
13
Q
Myelomeningocele
A
- Developmental defect of the spinal column
- Failure of vertebral arch fusion
- Dysplasia of spinal cord
- Cystic distention and meningeal protrusion
14
Q
Myelomeningocele physical examination
A
- Flaccid paralysis of lower limbs
- Motor and sensory loss at and below the level of the lesion
15
Q
Myelomeningocele physical examinaton common deformities
A
- Pes calcaneus
- Calcaneovalgus
- Equino adductovarus
- Convex pes valgus
- Equinus, cavus, clawtoes
16
Q
Myelomeningocele management
A
- Orthoses for neuropathic feet
- Bracing
- Surgery
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
18
Q
Poliomyelitis physical examination common deformities
A
- Pes calcaneus
- Calcaneovalgus
- Equino adductovarus
- Convex pes valgus
- Equinus, cavus, clawtoes
19
Q
Poliomyelitis management
A
- Vaccine
- Bracing
- Orthoses
- Surgery
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
Weak anterior/posterior leg AFO prescription
- Leaf – spring AFO
26
Toe walking (uncompensated ankle equinus) AFO prescription
- Hinged AFO with plantarflexion stop
27
Fixed ankle equinus AFO prescription
- AFO in equinus with heel lift
28
Foot / ankle in flexible varus / valgus AFO prescription
- Tension band AFO
29
Roussy-Levy syndrome
- 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
Friedreich's Ataxia
- 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
Friedreich's ataxia physical examination
- 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
Friedreich's ataxia management
- Orthosis
- Bracing
- Surgery
- Death during second decade secondary to myocardial failure (interstitial myocarditis)
33
Dejerine-Sottas disease
- 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
Dejerine-Sottas disease physical examination
- Lower extremity muscular atrophy
- “Stocking glove” sensory deficit
- Difficulty walking
- Pes cavus
- Digital contractures
- Argyll – Robertson pupils
35
Dejerine-Sottas disease management
- Orthoses
- Bracing
- Surgery
36
Duchenne muscular dystrophy
- Progressive muscular dystrophy
| - X – linked recessive inheritance
37
Duchenne muscular dystrophy characteristics
- 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
Duchenne muscular dystrophy physical examination
- Proximal muscular weakness (Gower’s sign)
- Pseudohypertrophy of calf muscles
- Toe walking (later onset)
- Elevated CPK, serum aldolase
- Muscle biopsy definitive
39
Duchenne muscular dystrophy management
- Bracing
- Physical therapy
- Death by second decade secondary to cardiac / respiratory failure
40
W/A T.A.
- Clinical presentation = foot drop/slap
| - Compensation = steppage gait
41
W/A T.P.
- Clinical presentation = Ex. pronation
| - Compensation = flexor substitution
42
Spastic R.F.
- Clinical presentation = stiff knee gait
| - Compensation = circumduction
43
W/A gluteus medius
- Clinical presentation = Trendelenburg gait
44
Spastic gastrocnemius
- Clinical presentation = knee hyperextension
45
Ankle equinus
- Clinical presentation = early/absent HC/HO, TW
| - Compensation = Ex. pronation, ext. gait, genu recurvatum
46
W/A gluteus maximus
- Clinical presentation = lumbar lordosis
47
W/A quads
- Clinical presentation = buckling knee
| - Compensation = knee hyperextension
48
Tight/spastic adductors
- Clinical presentation = scissoring gait
