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DR Medhat MRCP

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• Management :<br />

Riluzole<br />

1. Anti-glutamate drug.<br />

2. Used mainly in amyotrophic lateral sclerosis.<br />

3. Prolongs life by about 3 months<br />

4. Expensive<br />

Respiratory care<br />

1. Non-invasive ventilation (usually BIPAP) is used at night (better than<br />

riluzole).Studies have shown a survival benefit of around 7 months.<br />

NIV (BIPAP) has the greatest effect on survival in motor neuron disease .it`s better<br />

سؤال هام than Riluzole<br />

• Prognosis<br />

Poor: 50% of patients die within 3 years.<br />

Diseases of the muscles<br />

Inherited myopathies :<br />

1. Disorders of dystrophins<br />

- (Duchenne`s muscular dystrophy).<br />

2. Limb girdle muscular dystrophy.<br />

3. Facio-scapulo-humeral dystrophy.<br />

4. Myotonic dystrophy.<br />

Immune myopathy :<br />

1. Myasthenia gravis.<br />

Inflammatory myopathies:<br />

1. Polymyosistis.<br />

2. Dermatomyositis.<br />

3. Inclusion body myositis.<br />

Channelopathies :<br />

1. Periodic paralysis.<br />

Metabolic myopathies :<br />

1. Disorders of carbohydrate metabolism.<br />

2. Disorders of lipid metabolism.<br />

3. Mitochondrial myopathies.<br />

Wasted muscles in MND<br />

79

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