NeuroMuscular Disorders

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NeuroMuscular Disorders

• Upper motor neuron lesion
• ? spastic paralysis
• Lower motor neuron lesion
• ? flaccid paralysis
• Muscular Dystrophy

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Grading of muscle power

• 5- full power
• 4- strong but less than normal (mild weakness)
• 3- weak but can act against gravity
• 2- not enough power to act against gravity
• 1- minor flickering of muscle
• 0 total paralysis

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Effect of paralysis

• Instability occur when opposing muscles are
  equally weak or paralysed
• Flexor-extensor, abductor adductor
• The joint become floppy or flail
• Deformity when one group of muscles overpowers
  its antagonist Unbalanced paralysis
• Shortening paralysed limb fail to grow
• Growth and nutrition depend on intact nerve
  supply
• Paralysed limb fail to grow

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Principles of treatment

• Assessment of the whole patient
• Mental capacity
• Loss of sensation in a limb
• Can he use the limb if deformity corrected
• Instability can be corrected by
• Splints
• Joint fusion (Arthrodesis)

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• Deformity can be corrected by
• Stretching of muscles and ligaments
• Tendon transfer
• Tendon lengthening
• Osteotomy
• Shortening corrected by bone lengthening

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Cerebral palsy

• Non-progressive cerebral dysfunction
• Originates before the CNS has matured
• Due to
• Perinatal anoxia (hypoxia)
• Trauma to brain or its blood vessels
• Kernicterus ( severe neonatal jaundice )
• Infection of brain or meninges

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General features

• Incidence 0.5 2 / 1000 births
• History of difficult labour, early severe
  jaundice or perinatal hypoxia (Cyanosis)
• Early symptoms are
• Difficulty in sucking or swallowing
• Mother notice the baby is stiff or has
  incoordinated (clumsy) movements
• Delayed development of milestones (normally the
  child hold up his head at 3 months and walk at
  one year)
• Intelligence may be impaired
• May suffer from epileptic fits

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Limb signs

• Features of upper motor neuron lesion
• Spastic child
• Exaggerated reflexes
• Imbalance cause deformities
• Adduction, internal rotation of shoulder
• Flexion elbow
• Pronation forearm
• Flexion wrist
• Adduction of thumb

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• Lower limb
• Flexion, adduction hip ? scissor gait
• Flexion knee
• Equinus foot stand or walk on tip of toes
• Could be
• Hemiplegia (half side of body affected) 1/3
• Diplegia (lower limbs affected more than upper
  limbs) 1/3
• Quadriplegia (four limbs affected) 1/3

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Treatment

• Mental training
• Education
• Speech therapy
• Physical therapy
• Teach new patterns of
• Temporary splints
• Passive exercises to increase range of movement
• Operation to prevent or correct deformity
• Tendon surgery release or lengthening
• Bone surgery osteotomy
• Physiotherapy with all the above

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Spina Bifida

• Simply it means Failure of closure of the two
  halves of neural arch of the vertebrae.
• Usually occur at the lumbar or lumbosacral region
• Classified into two types
• Closed spina bifida skin closed over the defect
  in neural arch
• Open spina bifida skin opened with protrusion
  of meninges and / or spinal cord

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Pathology

• Defect of vertebral arch at the lumbar or
  lumbosacral area of spine. Could be
• Just a Defect in spinal canal bones
  posteriorlycalled Spina Bifida Occulta
• Protrusion of meninges through the defectcalled
  Meningocoele
• Protrusion of spinal cord with the meninges
  called Myelo-meningocoele

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• Cause unknown
• Geographic variation present (certain areas has
  more spina bifida than others)
• Poor families more incidence
• Familial incidence
• If one parent affected then a child has 5 risk
  of affcetion
• Estimation of certain protein (alpha fetoprotein)
  in amniotic fluid of fetus shows if fetus
  affected or not
• Many of those babies die soon because of
  complications

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Closed spina bifida

• Also called spina bifida Occulta (hidden)
• Skin is intact over the defect
• But it is abnormal skin
• Dimple
• Hairy patch at the area
• Large pigmented navus
• Small rudimentary tail
• Xray
• Show a gap in neural arch posteriorly
• Widening of spinal canal

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Neurological deficit

• May be no neurological deficit if only bone
  defect.
• If Meninges or spinal cord protrude through the
  defect but remain under skin ?
• Closed Meningocoele
• Closed myelomeningocoele
• If cord is affected ? neurological deficit
• Weakness of lower limb
• Loss of Sensation in limb
• Trophic ulcers of lower limb
• Bladder function disturbance

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Treatment

• Occulta No neurological deficit No treatment
  needed
• If Neurological deficit present
• Operation by neurosurgeon
• Replace the cord meninges in the spinal canal
• Close the defect
• Rehabilitation afterwards
• Exercises
• Massage
• Training of walking
• Bladder training
• Care for the skin from trophic ulcers

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Open spina bifida

• Skin defect in addition to bone defect
• Meninges protrude to outside
• Spinal cord may also protrude with meninges

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Clinical

• Translucent cystic mass in midline of back
• Neurological defect of lower limb
• Associated congenital deformities such as
• Congenital Dislocation of Hip
• Congenital Dislocation of foot (Club foot)

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Treatment

• Combined clinic of neurosurgeon, orthopedic
  surgeon, urologist an paediatrics
• But the key figure is the
• Physiotherapist,
• Occupational therapist,
• Psychotherapist
• Social therapist
• Splint maker
• parents understanding and support

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• Skin closure within 48 hours
• Deformities
• Stretching
• Physiotherapy
• Splints
• Bladder care and training
• Operations to correct deformities
• The key is
• Gentle manipulation
• Intermittent splinting
• Avoid forceful manipulation or stretching

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