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Neuromuscular Disorders

Neuromuscular Disorders. Faik Altıntas M.D. Orthopaedic and Traumatology. Neuromuscular Disorders. To operate correctly, muscles must be stimulated by electrical signals originated from brain and received from nerves Two major types of disorder are seen: 1). “Neuro” related:

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Neuromuscular Disorders

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  1. Neuromuscular Disorders Faik Altıntas M.D. Orthopaedic and Traumatology

  2. Neuromuscular Disorders • To operate correctly, muscles must be stimulated byelectrical signals originated from brain and received from nerves • Two major types of disorder are seen: • 1). “Neuro” related: • Problems with signal transmission in the nerves. • Problems getting the signal transferred from thenerve “across” to the muscle fibres (muscle cells). • 2). Muscle related: • Problems within the muscle itself.

  3. Neuromuscular Disorders • Cerebral Palsy • A group of disorders thta result from non-progressive brain damage during early devolepment • 1-2 /1000 live births • Known causes • Maternal toxemia • Perinatal anoxia • Kernicterius • Postnatal infections • Brain trauma • Birth trauma

  4. Neuromuscular Disorders • Cerebral Palsy • Main consequence is the development of neuromusclar incoordination, dystonia, weakness, spascity, convulsions,speech and vision disorders, mental retardition, behavioral problems • Spastic palsy %60 • Athetosis • Ataxia • Rigid • Hypotonic • Mixed

  5. Neuromuscular Disorders • Cerebral Palsy • Hemiplegia • Diplegia • Quariplegia

  6. Neuromuscular Disorders • Friedrich’s Ataxia • OR • Degeneration of the spinocerebellar and corticospinal tracts, posterior coloumn of the cord and part of cerebellum • Present 5-6 years old • Pes cavovarus, scoliosis, cardiac failure

  7. Neuromuscular Disorders • Spina Bifida • Congenital disorder • Lack of Folate • Failure of posterior vertebral arch fusion • Dysraphism • Usually in lomber spine • If neural elements involved, paralysis, loss of sensation and loss of sphincter control happens

  8. Neuromuscular Disorders • Spina Bifida • Spina Bifida Occulta • Mildest form • Only defect in laminae • May be defects on skin such as dimple, pit or hair • Most of them undiagnosed, few may have tethered cord, diastometamyelia, medullar lipomas

  9. Neuromuscular Disorders • Spina Bifida • Meningocele • The spinal cord and nerve roots remain in their normal position and duramater is open posteriorly and sac protrudes under skin • Most of them have no neurological abnormality, but hydrocephalus, infection and ulceration of the cycst are common

  10. Neuromuscular Disorders • Spina Bifida • Myelomeningocele • The spinal cord and nerve roots prolapse together with the meningeal sac • Hydrocephalus, paralysis under affected zone, multiple deformities ( hip dislocation, genu recurvatum, PEV, claw toes), CSF leakage are very common

  11. Neuromuscular Disorders • Poliomyelitis • Viral infection of the anterior horn cells of the spinal cord and brain stem • Asymetric flaccid paralysis of the affected muscle groups • Very rare after vaccination programs

  12. Neuromuscular Disorders • Poliomyelitis • Flue like symptoms • Pain and spasms for 2-3 days • Paralysis settled with in 6 months • Unbalanced,asymetric flaccid paralysis often with intact sensation

  13. Neuromuscular Disorders • Poliomyelitis • Isolated muscle weakness without deformity: quadriceps esp • Deformity • Flail Joint • Shortening

  14. Neuromuscular Disorders • Amyotrophic Lateral Sclerosis • Degenerative disease with unknown etiology • Progresive and incurable • Ends in wheelchair, death happens with respiratory weakness and aspiration pnomonia • Middle age patient with dysartria, difficulty in swallowing, clumpsy hand and limp weakness

  15. Neuromuscular Disorders • Spinal Muscular Atrophy • Heritable disorder • Widespread degeneration of anterior horn cells lading to progressive LMN weakness • Werdnig-Hoffmann disease most common type • Muscle weakness, paralytic scoliosis, hard to feed, difficult to breath • Death happens often 2 years of age

  16. Neuromuscular Disorders • Duchenne Muscular Dystrophy • X-linked heritable progressive disease : Only in boys • Defective gene p21 on X Chromosome, fails to produce Dystrophin • Most of the patients diagnosed when they start to walk • Difficulty in standing and walking/running • The muscles look bulky: pseudohypertrophia

  17. Neuromuscular Disorders • Duchenne Muscular Dystrophy • A characteristic method for climbing up his legs: Gower’s sign • By 10 years of age child cant walk again • By 20 years of age death comes by cardiac or respiratory failure

  18. THANK YOU

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