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Motor Neurone Disease (Examination (Completion (Speech (Bulbar: nasal,…
Motor Neurone Disease
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Differential
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Mixed UMN and LMN Signs
- MND
- Ataxia, Friedrich’s
- SCDC: B12
- Taboparesis
Classification
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Progressive Muscular Atrophy: 10%
Anterior horn cell lesion → LMN signs only
Distal to proximal
Better prognosis cf. ALS
Primary Lateral Sclerosis: 30%
Loss of Betz cells in motor cortex → mainly UMN signs
Marked spastic leg weakness and pseudobulbar palsy
No cognitive decline
Viva
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Mx
General
MDT: neurologist, physio, OT, dietician, specialist nurse, GP, family
Discussion of end-of-life decisions
E.g. Advanced directive, DNAR
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Supportive
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Spasticity: baclofen, botulinum
Prognosis
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Worse prog: elderly, female, bulbar involvement