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Motor Neurone Disease
Motor Neurone Disease
Motor Neurone Disease
Motor Neurone Disease
Motor Neurone Disease
Motor Neurone Disease
Motor Neurone Disease
Motor Neurone Disease
Motor Neurone Disease
Motor Neurone Disease
Motor Neurone Disease
Motor Neurone Disease
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Motor Neurone Disease

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

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