Motor Neurone Disease

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Motor Neurone Disease

  1. 1. Motor neurone disease Dr. Osman Sadig Bukhari
  2. 2. <ul><li>- IN MND there is progressive degeneration of </li></ul><ul><li>1- anterior horn cells in za spinal cord i.e. </li></ul><ul><li>motor neurones </li></ul><ul><li>2- motor nuclei of za lower cranial nerves </li></ul><ul><li>3- neurones in za motor cortex and the </li></ul><ul><li>corticospinal & corticpbulbar pathways . </li></ul><ul><li>- Usually sporadic Of unknown cause ? </li></ul><ul><li>- degeneration genetically is programmed </li></ul><ul><li>- oxidative neuronal damage </li></ul><ul><li>- aggregation of abn neuronal protein </li></ul><ul><li>- Familial in 5% due to mutation in za gene on chromosome 21 </li></ul>
  3. 3. <ul><li>- No evidence of infective, traumatic or toxic caus </li></ul><ul><li>- Prevalence: 5/100 000/ year </li></ul><ul><li>- Slight male preponderance </li></ul><ul><li>- Onset middle life. </li></ul><ul><li>- Sensory system not involved </li></ul>
  4. 4. <ul><li>Patterns of MND:- </li></ul><ul><li>1- Progressive muscular atrophy : LMN </li></ul><ul><li>signs in za limbs i.e. ant. horn cell degenerat </li></ul><ul><li>2- Progressive bulbar & pseudobulbar palsy : </li></ul><ul><li>lower cranial nerve nuclei & their supranuclear </li></ul><ul><li>connections are involved. </li></ul><ul><li>3- Amyotrophic lateral sclerosis (ALS): mixed </li></ul><ul><li>upper & lower motor neurone signs in the </li></ul><ul><li>limbs. </li></ul><ul><li>4- Primary lateral sclerosis :- disorder confined to </li></ul><ul><li>UMN. Usually there is terminal pseudobul pals </li></ul>
  5. 5. <ul><li>Progressive muscular atrophy </li></ul><ul><li>- Wasting & weakness often begins in small </li></ul><ul><li>muscles of za hands & spreads proximally. </li></ul><ul><li>- Start unilaterally but soon za opposite </li></ul><ul><li>side is involved. </li></ul><ul><li>- There is muscle cramps & Fasciculation is common. </li></ul><ul><li>- Signs include:- wasting & weakness </li></ul><ul><li>- wide spread fasciculations </li></ul><ul><li>- absent tendon reflexes - exaggerated reflexes if C/S </li></ul><ul><li>tracts are involved. </li></ul>
  6. 6. <ul><li>Amyotrophic lateral sclerosis </li></ul><ul><li>Here there is : </li></ul><ul><li>- Progressive spastic quadriparesis or paraparesi </li></ul><ul><li>- Exaggerated tendon reflexes </li></ul><ul><li>- Extensor planter </li></ul><ul><li>- Muscle wasting & fasciculations </li></ul><ul><li>- Bulbar & pseudobulbar palsy follow eventually. </li></ul>
  7. 7. <ul><li>Progressive bulbar & pseudobulbar palsy </li></ul><ul><li>Presents with: </li></ul><ul><li>- Dysphagia </li></ul><ul><li>- Dysarthria </li></ul><ul><li>- Nasal regurgitation of fluids & chocking </li></ul><ul><li>- difficulty in coughing </li></ul><ul><li>- Signs:- wasted fasciculating tongue in bulb pals </li></ul><ul><li>- contracted spastic tongue, spastic weak </li></ul><ul><li>palate & exaggerated jaw jerk in </li></ul><ul><li>pseudobulbar palsy. </li></ul><ul><li>- NO ophthalmoplegia, cerebellar or </li></ul><ul><li>extra pyramidal signs & sphincter disturbances occur late </li></ul>
  8. 8. <ul><li>Primary lateral sclerosis </li></ul><ul><li>There is : </li></ul><ul><li>- progressive quadriparesis wz signs of UMN </li></ul><ul><li>- terminal pseudobulbar palsy. </li></ul><ul><li>Note in MND </li></ul><ul><li>- No sensory loss </li></ul><ul><li>- Extra ocular muscles not involved </li></ul><ul><li>- Sphincters remain intact till late </li></ul><ul><li>- No intellectual impairment in most cases </li></ul>
  9. 9. <ul><li>Investigations </li></ul><ul><li>- Diag is clinical. </li></ul><ul><li>- EMG confirms denervation </li></ul><ul><li>-Sensory & motor conduction velocities are norm </li></ul><ul><li>- imaging of spinal cord & brain to exclude focal </li></ul><ul><li>brain & spinal cord disease </li></ul><ul><li>- CSF is normal </li></ul><ul><li>Diff diag </li></ul><ul><li>- spinal cord comp - motor neuropathy </li></ul><ul><li>- motor Ca neuropathy - diabetic amyotrophy </li></ul><ul><li>- bulbar MG - spinal muscular atrophy of adul </li></ul>
  10. 10. <ul><li>Management </li></ul><ul><li>- Psychotherapy </li></ul><ul><li>- Physiotherapy </li></ul><ul><li>- Speech therapy </li></ul><ul><li>- Occupational therapy </li></ul><ul><li>- walking aids & wheel chairs </li></ul><ul><li>- NG & gastrstomy feeding in bulbar palsy </li></ul><ul><li>- Nerve growth factor promising </li></ul><ul><li>- Riluzole, which reduces presynaptic release of </li></ul><ul><li>glutamate slows progression of ALS & bulb pals </li></ul><ul><li>- Remission is unknown & no TR alters outcome </li></ul>
  11. 11. <ul><li>Course of MND </li></ul><ul><li>- Disease starts focally & gradually become </li></ul><ul><li>widespread. </li></ul><ul><li>- Most die within 3-5 years </li></ul><ul><li>- Young pts & those wz bulbar palsy have </li></ul><ul><li>rapid course </li></ul><ul><li>- Cause of death are resp infection, resp failure </li></ul><ul><li>and complications of immobility. </li></ul><ul><li>- Remission unknown. </li></ul>
  12. 12. <ul><li>Spinal muscular atrophies </li></ul><ul><li>- Genetically determined disorders of spinal </li></ul><ul><li>(? Cranial) motor neurones </li></ul><ul><li>Slowly progressive symmetrical muscle wasting </li></ul><ul><li>and weakness + fasciculation. </li></ul><ul><li>1- Acute infantile type (Werdnig -Hoffman) </li></ul><ul><li>2- Chronic childhood type ( Kuglberg – </li></ul><ul><li>Welander). </li></ul><ul><li>3- Chronic adult type </li></ul><ul><li>- Should be differentiated from muscular dystro, </li></ul><ul><li>hereditary neuropathies & MND. </li></ul>

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