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Neuromuscular disorders Flashcards

Marfan's syndrome Arthrogyrposis Larsen's syndrome Freidreich's ataxia Charcot- marie Tooth Myelodysplasia ( spina bifidia)

1
Q

What is Marfan’s syndrome?

A
  • A connective tissue disorder associated with
    • long limbs
    • Skeletal Abnormalities
    • cardiovascular abnormalities
    • ocular abnormalities
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2
Q

What is the epidemiology of Marfan’s Syndrome?

A
  • incidence 1 in 10,000
  • no gender predilection
  • genetics
    • Autosomal Dominant
    • mutation in fibrillin-1 (FBN-1)= 90% of pts
    • location on chromosome 15 (locus CH 15q21)
    • multiple mutations identified
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3
Q

What is fibrillin?

A
  • A gylcoprotein common in many tissues
  • which is a structural component of elastin- containing microfibrils
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4
Q

Name any associated conditions?

A
  • Arachnodactyly
  • Scoliosis (50%)
  • Protrusio Acetabuli (15-25%)- centre edge angle >40o, medialisation of acetabulum past ilioschial line
  • ligamentous laxity
  • recurrent dislocations- patella, shoulder, fingers
  • Pes planovalgus

​non orthopaedic

  • Cardiac abnormalities
    • ​aortic root dilation
    • possible aortic dissection in future
    • mitral valve prolapse
    • superior lens dislocation (60%)
    • pectum excavatum
    • spontaneous pneumothoraces
    • dural ectasia (>60%)
    • meningocele
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5
Q

What is the signs and symptoms of Marfan’s syndrome?

A
  • ***Scoliosis first to be diagnosed
  • hx of ankle sprains

symptoms

  • asymptomatic in most cases

Signs

  • arm span > height ratio 1.05 ratio= Dolichostenomelia
  • arachnodactyly - long, thin toes and fingers
  • ligamentous hyperlaxity
  • scoliosis
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6
Q

What imaging is useful in Marfan’s syndrome?

A
  • Xrays
    • scoliosis series of spine
    • see scoliosis and kyphosis
  • MRI
    • prior to surgery
    • look for dural ectasia- widening ballooning of dural sac around spinal cord
    • these may cause headaches, leg pain or perineal pain
  • Cardiac evaluation prior to surgery
    • cardiac consultation & Echo
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7
Q

What is the tx of marfan’s Scoliosis?

A
  • Non operative
    • Bracing
    • early tx of mild curve
    • ineffective in most cases
  • Operative
    • ASF+ PSF w instrumentation
      • rapidly progressive curve in skeletally immature patient
      • large curve in skeletally mature pts
      • higher complication rate then idiopathic scoliosis
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8
Q

What is the tx of Marfan’s Protrusio?

A
  • Observe until severe symptoms develop
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9
Q

What is the tx of Marfan’s Joint laxity

A
  • Non operative
    • Observation and orthotics as indicated
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10
Q

What is the post op complications of scoliosis surgery for Marfan’s syndrome?

A
  • 10-20% incidence following scoliosis surgery
  • infection
  • failure of instrumentation
  • pseudoarthrosis
  • decompensation of curve
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11
Q

What is this?

A
  • Arthrogyroposis
  • A congential nonprogressive disorder involving Multiple Rigid Joints ( usually symmetrical) leading to severe limitation in motion
  • from the greek-curving of joints/ hook joints
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12
Q

What is the epidemiology and mechanism of Arthrogyroposis?

A
  • 1 in 3,000 live births
  • symmetry of contractures due to immobilisation in utero
    • neurogenic 90%
    • Myopathic 10%
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13
Q

What is the pathophysiology of Arthrogyroposis?

A
  • Exact mechanism unknown
  • Some mothers have serum antibodies inhibiting fetal acetylcholine-> a decreased no of anterior horn cells
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14
Q

Name associated condition of Arthrogyroposis?

A
  • Hip subluxation and dislocation
  • knee contractures
  • clubfoot
  • vertical talus
  • neuromuscular C shaped scoliosis- 33%
  • Fractures 25%
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15
Q

What is the prognosis of Arthrogyroposis?

A
  • 25% Non ambulatory
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16
Q

Describe the classification of Arthrogyroposis?

A
  • Type 1 - single localised deformity - e.g forearm pronated
  • Type 2- full expression
    • absent shoulder muscles, thin limbs, elbows extended, wrists flexed ulnarly deviated, intrinsic plus deformity of hands, adducted thumbs, no flexion creases
  • Type 3- full expression ( like type 2) with polydactylyl and involvement if non - neuromuscular systems
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17
Q

What are the signs of Arthrogyroposis?

A
  • Shoulders adducted and internally rotated- absent of shoulder muscles
  • elbows extended - no flexion crease
  • wristed flexed and ulnarly deviated
  • hands with intrinsics plus deformity
  • thumb adducted
  • hips flexed, abducted and externally rotated
    • subluxation/teratologic dislocations common
  • knees extended- most time flexed
  • clubfeet
  • normal intelligence, facies, sensation and viscers
  • range of motion- V limited involving all 4 extremities
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18
Q

What studies are preformed on a child with Arthrogyroposis?

A
  • At 3-4 months old
  • neurologial studies
  • enzyme tests
  • muscle biopsies
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19
Q

Describe the tx if upper limb deformities in Arthrogyroposis?

A
  • Goals
  • to allow optimial function to increase ability to drive an electric chair and use computer assisted devices
  • one elbow in extension for positioning & perinanal care, one elbow in flexion for feeding

Non operartive

  • Passive manipulation and serial casting
    • first line
  • Operative
    • Soft tissue releases, tendon transfers and osteotomies
    • consider after age 4 to allow independent eating
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20
Q

Describe the surgical tx of upper limb deformities in Arthrogyroposis?

A
  • Elbow extension
    • tx = triceps V-Y lengthening and posterior capsulotomy at 1.5-3 years
  • Wrist palmar flexion & ulnar deviation
    • tx=Flexor carpi ulnaris release, lengthening and transfer to wrist extensors, dorsal carpal closing
  • Thumb in palm contractures/syndactyly
    • Z plasty syndactylyl release
  • Finger deformity
    • PIP arthrodesis
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21
Q

Decribe the tx of hip subluxation and dislocation in Arthrogyroposis?

A
  • 68-80% pts present with hip subluxation/dislocation with Arthrogyroposis

Non operative

  • Observation alone
    • bilateral dislocations- contraversial
    • unilateral dislocation in older children
    • Pavlik Harness is CI

​​Surgical

  • Closed reduction
    • rarely successful
  • Medial open reduction & possible femoral shortening
    • ​for unilateral dislocations
    • may -> worse function if leads to hip flexion contracture because fo fixed flexion deformities worsen pts gait
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22
Q

Describe the tx of knee contractures in Arthrogyroposis?

A

Operative

  • Soft tissue releases- especially hamstrings
    • for flexion contracture >30o
    • best preformed early 6-9 months old
    • perform before hip reduction to assist maintainence of reduction
  • Femoral shortening thru guided growth (epiphysiodesis)
    • useful in conjunction with osteotomies
    • may not effectively correct chronic poor quads function
  • Supracondylar femoral osteotomy
    • may be needed to correct residual deformity at skeletal maturity
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23
Q

Describe the tx of clubfoot in Arthrogyroposis?

A
  • Non operative
    • Ponsetti casting
  • operative
    • soft tissue release
      • first line in rigid clubfoot
      • failed Ponsetti casting more flexible types
    • Talectomy vs triple arthrodesis
      • ​failed soft tissue release
      • triple arthrodesis in adolsecents
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24
Q

Describe the tx of vertical talus in Arthrogyroposis?

A
  • Operative
    • soft tissue release
      • lengthened of peroneals, achilles tendon
        • first line
    • talectomy
      • if deformities recur despite soft tissue release
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25
Q

What is Larsen’s syndrome?

A
  • A rare genetic disorder with characteristics findings of
  • Ligamentous laxity
  • abnormal facial features
  • multiple joint dislocations
    • dislocations include
      • Hips
      • Knees - usually bilateral
      • Shoulders
      • elbows-Radial head
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26
Q

Can you describe the epidemiology of larsen syndrome?

A
  • 1 in 100,00 births
  • Inhereitance pattern both
    • **Autosomal dominant **
      • ​Mutation in gene coding FILAMIN B
    • Autosomal recessive
      • Linked to Carbohydrate Sulfotransferase 3 deficiency
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27
Q

What are the associated conditions of Larsen syndrome?

A
  • Hand deformities
  • Scoliosis
  • Club feet
  • Cervical Kyphosis
    • may present extreme weakness 2ary to myelopathy
    • caused by HYPOPLASIA of cervical vertebra
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28
Q

What are the signs and symptoms of Larsen’s syndrome?

A
  • Pts are normal intelligence

Signs

  • Hypotonis
    • uncommon but maybe due to cervcial compression
  • Abnormal facial features
    • flattened nasal bridge
    • Hypertelorism
    • prominent forehead
  • Hands
    • Long cylindrical fingers that do not taper
    • wode distal phalanx at thumb
  • Elbows
    • bilateral radial head dislocations
  • Knees
    • Bilateral knee dislocations
  • Foot
    • Equinovarus
    • Equinovalgus
    • Clubfeet
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29
Q

What imaging is required in Larsen’s syndrome?

A
  • Xrays
    • AP and lateral C spine
      • during 1st yr of life
    • Ap and lateral of hips
      • US if less than 3/12
    • see hypoplasia of vertebra
    • cervical kyphosis w subluxation
    • hip dislocation
  • ​MRI
    • ​Cervical kyphosis
    • myelopathy
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30
Q

How is cervical kyphosis tx in larsen’s syndrome?

A
  • Operative
    • Posterior cervical fusion
      • for pt with significant kyphosis but no neuro
      • during first 18 months of life to prevent neurological deterioration
    • ​Anterior/posterior cervical decompression & fusion
      • ​for Cervical kyphosis with neurology
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31
Q

What is the tx for hip dislocation in Larsen’s syndrome?

A
  • Non operative
    • Closed reduction under anaesthesia
      • rarely successful
  • Operative
    • Open reduction of hip dislocation
      • for failed closed reduction
      • Decreased ROM 2ary to contractures
      • unilateral hip dislocation
      • bilat hip dislocation- controversial , perform early & only once
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32
Q

What is the tx of knee dislocations for Larsen’s syndrome?

A
  • non operative
    • Closed reduction and casting
      • rarely successful
  • Operative
    • Open reduction w femoral shrotening and collateral ligament excision
    • for those that remain unsatble after closed reduction
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33
Q

What is Fredreich’s ataxia?

A
  • Most common form of spinocerebellar degenerative disease
  • characterised by lesions in the
    • Dorsal Root Ganglia
    • Corticospinal tracts
    • Dentate nuclei in the cerebellum
    • sensory peripheral nerves
34
Q

What is the epidemiology of fredrich’s ataxia?

A
  • 1 in 50,000 births
  • onset usually between 7-25 years
  • age on onset related to no of GAA repeats
  • Autosomal Recessive
    • Repeat mutation-> lack of Frataxin gene
    • Mutation is GAA repeat at 9q13
35
Q

What is Fraxatin?

A
  • A mitochondrial protein involved in Iron metabolism and oxidative stress
  • It is a iron binding protein
  • Low levels-> increased iron levels in mitochondria_> damage to proteins
36
Q

Name associated conditions of fredrich’s ataxia?

A
  • Pes Cavovarus foot
  • Scoliosis
    • predictors of progression
      • onset of disease <10 yrs
      • onset scoliosis <15 yrs
  • Cardiomyopathy
    • cadiology evaluation b4 surgery
    • antioxidants ( Coenzyme Q) = to decrease rate of cadiac deterioration but have no effect on ataxia
37
Q

What is the prognosis of fredrich’s ataxia

A
  • Usually wheelchair bound by 30yrs
  • usually died by 50 yrs from CARDIOMYOPATHY, pneumonia, aspiration
38
Q

What are the signs and symptoms of fredrich’s ataxia?

A
  • Symptoms
    • Ataxia
      • Staggering wide based gait- spineocerebellar
  • Signs
    • Classic triad
      • Ataxia
      • Areflexia
      • Positive plantar response
    • Weakness
    • Nystagmus
    • Cavovarus foot
      • V high arch
      • Rigid deformity
      • assoc claw toes
    • Scoliosis
39
Q

What imaging is useful in fredrich’s ataxia?

A
  • Standing scoliosis series
  • AP and lateral of foot pes cavovarus present
40
Q

What do EMG studies show in pts with fredrich’s ataxia?

A
  • Show defects in motor and sensory with an increase in polyphasic potentials
  • nerve conduction velocities aer decreased in upper extremities
41
Q

What is the tx of cavovarus foot in fredrich’s ataxia?

A
  • No operative
    • Observation
      • only in non ambulatory pts
      • deformity is rigid and resistant to bracing
  • Operative
    • Plantar release, transfers +/- Metatarsal and calcaneal osteotomy
      • early disease in ambulatory pts
    • Triple arthrodesis
      • late disease nonambulatory pts
42
Q

What is the tx of scoliosis in fredrich’s ataxia?

A
  • Non operative
    • Observation
      • for curves <40 degrees without predictors of progression
  • Operative
    • PSF and instrumentation
      • Curves >60 degrees
      • rapid progression with positive predictors pf progression
      • usually not needs to be extended to pelvis
43
Q

What is charot marie tooth?

A
  • A hereditary motor sensory neuropathy
  • a demyelination disorder of the PNS
  • 2 forms resulting in
  • muscle weakness and sensory changes
  • Autosomal Dominant- most common
    • ​also autosomal recessive
    • X linked
  • ​Mutation
    • ​Duplications on chromosome 17
    • codes for peripheral myelin protein 22 ( PMP22)- gene mutations seen on PCR analysis
    • X linked Connexin 32
44
Q

What is the epidemiology of charot marie tooth?

A
  • 1 per 2,500
  • Most common inherited neurological disease
45
Q

What is the pathophysiology of charcot marie tooth?

A
  • Combination of motor and sensory disturbances as a result of nerve damage
    • Motor > than sensory
  • affected muscle become WEAK= TAP BI
    • Tibialis anterior
    • Peroneus Brevis
    • Intrinsics hand /foot
46
Q

What are the orthopaedic manifestations of Charcot marie tooth?

A
  • Pes cavus
  • Hammer toes
  • Hip dysplasia
  • Scoliosis
47
Q

Describe the 2 type of charcot marie tooth?

A
  • _Type 1 _
    • A demyelinating condition that slows nerve conduction velocity
      • Autosomal Dominant
      • Onset in 1st/2nd decade of life
      • most commonly-> Cavus foot
  • ​​Type 2
    • ​Direct axonal death by Wallerian degeneration
      • ​Usually less disabled cf 1
      • Onset 2nd decade of life
      • often-> FLACCID foot
48
Q

What are the symptoms & signs of charcot marie tooth?

A
  • Symptoms
    • Lateral foot pain
    • snesory deficits
    • Clumsiness
    • Frequent ankle sprains
    • difficulty climbing stairs
  • Signs
    • Rigid Cavovarus Foot ( similar ro Freidreich’s ataxia) w hammer toes/ clawing toes
    • atrophied EDB/EHB
    • Calf atrophy
    • weak Dorsiflexion & eversion = weak tib anterior and peroneals - drop foot during swing phase
    • lower limb Areflexia
    • Coleman block test
      • ​placed under lateral aspect of foot tests flexibility of hindfoot
      • rigid hindfoot won’t correct into neutral
    • ​Upper limbs= Intrinsic wasting of hand
49
Q

What do EMG studies show in charcot marie tooth?

A
  • Low nerve conduction velocities with prolonged distal latencies are noted of peroneal, ulnar and median nerves
50
Q

Describe the cavus foot deformity in charcot marie tooth?

A
  • Plantar flexed 1st ray= inital deformity- weak tib anterior over powered by peroneus longus-acts to plantarflex 1st Mt, tils hindfoot into varus
  • Peroneus longus normal overpowers weak Tibialis anterior=> Cavus foot PL>TA
  • Tibialis posterior normal overpowers weak Peroneus Brevis=> Varus and adduction forefoot TP>PB
    *
51
Q

What is the tx of charcot marie tooth cavovarus foot?

A
  • Non operative
    • Stretching, strengthening and orthotics
      • intial mx of young children
      • mobilisation and strengthening Tib Ant and peroneus Brevis
      • Orthotics
        • accommodative inserts
        • in flexible orthotics should post lateral forefoot and lateral heel
  • ​​​Surgery
    • ​Plantar fascia release, Tib post or peroneus longus transfer +/- TAL , +/- 1St Metatarsal Dorsiflexion osteotomy
    • for flexible hindfoot cavus deformity( Ncoleman block)
    • surgery when symptomatic or muscle weakness-> contracture
    • Post tibialis transfer dorsum of foot improve foot drop
    • Peroneus longus transfer to PB
  • Calcaneal valgus producing osteotomy
    • for RIGID Hindfoot
    • combined with above soft tissue releases + 1st MT osteotomy
  • Triple arthrodesis
    • for Severe Rigid Deformities
  • 1st MT osteotomy and transfer EHL to neck 1st MT
    • if hallux clawing combined with cavus foot
52
Q

How does clawing occur in charcot marie tooth feet?

A
  • Ankle dorsiflexion weakness may result in recruitment of toe extensors for assistance
  • In settiing of intrinsic muscle weakness, increased toe extensor activity-> claw toe deformity
53
Q

What is the tx of claw toes in charcot marie tooth?

A
  • Operative
    • Jones procedure
      • Symptomatic claw toe deformity
      • transfer of extensor of great and lesser toes thru bone to into metatarsal neck
      • goal is to increase contributors to ankle dorsiflexion and decrease clawing in order to relieve pain on the dorsum of the toes
54
Q

What are the characteristics of scoliosis in a pt with charcot marie tooth?

A
  • Left thoracic and kyphotic curve
55
Q

What is the tx of scoliosis in CMT?

A
  • Non operative
    • Bracing- rarely effective

​Surgery

  • Fusion and instrumentation
    • ​for progressive deformity
56
Q

What is myelodysplasia?

A
  • A group of congential abnormalities caused by failiure of spinal cord to completely close
57
Q

What is the epidemiology of myelodysplasia?

A
  • incidence 0.1-0.2%
  • risk factors
    • Folate deficiency
      • supplementation decrease risk by 70%
    • Maternal Hyperthermia
    • Maternal diabetes
    • Valproic acid
  • up to 10% have chromosomal abnormalities
    • trisome 13 or 18, triploid
58
Q

Name associated orthopaedic conditions of myelodysplasia?

A
  • pathological fractures
  • scoliosis
  • Kyphosis
  • Hip dysplasia- contracture/dislocations
  • Tibial torsion
  • knee contractures
  • Foot deformities
59
Q

What neurological manifestation of myselodysplasia exist?

A
  • Type 2 Arnold-Chairi malformations
    • most commonly associated congential abnormality
  • Hydrocephalus
  • Tethered cord
60
Q

What is the prognosis of myelodysplasia?

A
  • Depends on level of spinal segment involved
  • untx infants mortality 90-100%
  • Ability to ambulate
    • L3 or above - conined to wheelchair
    • L5 pt good prognosis for independence
61
Q

Can you describe one manifestation of myelosdysplasia?

A
  • IgE allergy to Latex- present in 20-70% cases
62
Q

What is the classification of myelodysplasia?

A
  • Spina bifidia occulta
    • defect in vertebral arch w confined cords and meninges
  • Meningocele
    • protruding sac without neural elements
  • Myelomeningocele
    • protruding sac with neural elements
  • Rachischisis
    • neural elements exposed with no covering
63
Q

What is the function al classification of myelodysplasia?

A

L2- non ambulatory

L3-

  • marginal household ambulator
  • risk hip dislocation
  • hip flexion- iliopsoas ( lumbar plexus/femoral n)
  • hip abduction (obturator n)

L4

  • household ambulator- key level as quads can work
  • Knee extension- quads- femoral n
  • ankle dorsiflexion/inversion-tibialis ant-deep peroneal n

L5

  • Community ambulators
  • Toe dorsiflexion- EHL-deep peroneal
  • Hip extension- deep peroneal n
  • Hip abduction- glut med & min- sup gluteal n

S1

  • ambulators
  • foot plantarlfexion- gastrosoleus- tibial n

S2

  • ambulators
  • toe plantar flexion-FHL - tibial n

S3/4

  • normal bladder and bowel
64
Q

What labs are useful in myelodysplasia?

A
  • alpha-fetaphrotein elevated in 75% children w spina bifida
65
Q

What is the epidemiology of pathological fx in myelodysplasia?

A
  • fx of long bones common due to Osteopenia
  • freq increases with higher level of the defect
  • common in hip and knee in children aged 3-7yrs
  • fx often confused w
    • infection
    • osteomyelitis
    • cellulitis
66
Q

What is the tx of path fx in myelodysplasia?

A
  • Short period of immobilisation
    • if fx in good alignment
    • avoid long-term casting
67
Q

Describe the epidemiology of scolioisis in myelodysplasia?

A
  • May result from
    • muscle imbalance (neurogenic) or
    • Congential malformation ( hemivetebra)
      • Defined as curve >20o
  • Higher the functional level the > incidence of scoliosis
  • 100% scoliosis rate defects in thoracic levels
  • consider cord tethering in rpaid progressive deformities
68
Q

What is the tx of myelodysplasia scoliosis?

A
  • Non operative- Bracing not effective
  • Surgery
    • ASF & PSF with Pelvix fixation
      • for progressive curve, most cases
      • ASF required due to dysplastic posterior elements that may impair posterior fusion
  • ​complications
    • High pseudoarthrosis rate
    • high incidence of infection 15-25%
      • due to poor soft tissue coverage of post spine
69
Q

What is the epidemiology of congential kyphosis in myelodysplasia?

A
  • Present in 10-15% with myelodysplasia
  • usually congential & progressive
  • Gibbus deformity may cause recurrent skin breakdown due ot pressure point when sitting
70
Q

What is the tx of congential kyphosis with myelodysplasia?

A
  • Operative
    • Kyphectomy w fusion and posterior instrumentation
      • for progressive deformity
      • sheck shunt function prior to kyphectomy
      • shunt failure during surgery may -> death
71
Q

What level is hip dislocation common in myelodysplasia and why?

A
  • L3
  • Unopposed hip flexion and adduction
  • L4 hip abduction
72
Q

What is the tx of hip abduction contracture in myelodysplasia?

A
  • it causes pelvic obliquity and scoliosis
  • proximal division of fascia lata and distal iliotibial band release
  • for contractures interfering with sitting/bracing
73
Q

What is the tx for hip flexor contractures?

A
  • common in thoracic and lumbar defects
  • for contractures >40o
  • surgery
    • anterior hip release with tenotomy of iliopoas, sartorius, rectus femoris, tensor fascia lata
74
Q

How are weak quads tx in myelodysplasia pts?

A
  • Common affecting children with myelodysplasia
  • tx= **Knee-ankle- foot orthotic **
75
Q

Tx of flexion contracture in myelodsyplasia?

A
  • not as important to tx in wheelchair bound pts
  • hamstring lengthening + post capsulomtomy
    • >20o of knee flexion contracture
  • Supracondylar extension osteotomy
    • older pts
    • those failed soft tissue proceedures
76
Q

Tx of knee extension contracture?

A
  • Less common than flexion contracture
  • tx with serial casting
  • for extension contracture limiting ambulation/sitting
  • goal is to reach 90o of flexion
77
Q

What is the tx of tibial torsion in myelodysplasia?

A
  • For children <5 years
    • Observation and orthotics
  • For children >5 yrs
    • Distal tibial derotational osteotomy
78
Q

What is the epidemiology of foot and ankle deformities in myelodysplasia?

A
  • v common
  • 60-90% incidence
  • due to high incidence of lower root involvment
79
Q

Can you describe the different types of foot abnormalities and consx tx?

A
  • L1/2= talipes equinovarus = HKAFO
  • L3= talipes equinovarus= KAFO
  • L4= Cavo varus= AFO
  • L5= Calcaneovalgus= AFO
  • S1= foot deformity= shoes
80
Q

What are the feature of clubfoot in a pt with myelodysplasia?

A
  • 30% incidence in myelodysplasia
  • Very rigid
  • insensate foot- different to idopathic clubfoot
81
Q

What is the tx of clubfeet in myelodysplasia?

A
  • serial casting
    • high complication rate
  • posteromedial lateral release
    • for failure of serial casting
    • perform when child is 12-18 months
82
Q

What is the tx of dorsiflexion deformity?

A
  • seen in L5 or sacral level patients
  • unopposed tibialis anterior causes dorsiflexion deformity
  • tx is posterior transfer of tibialis anterior tendon
    • for those who can’t achieve neutral foot with bracing

Paediatrics FCRS (27 decks)

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