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Motor neurone
disease (Pathophysiology (Environmental trigger
(toxin,…
Motor neurone
disease
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Pathophysiology
Environmental trigger
(toxin, viral, stress, radiation)
Pathological process
(abnormal autoimmune, neuronal blood supply,
responses to hypoxia, ageing structural proteins,
ageing mitochondria, etc.)
Unknown; mostly sporadic,
some familial (AD in SOD-1)
Degeneration of motor neurons in brain, brainstem,
and spinal cord (UMN and LMN signs)
No sphincter impairment, sensory signs or
autonomic dysfunction
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Classification
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Progressive muscular atrophy
Uncommon (10%)
Anterior horn cells only, so only LMN
Distal muscles before proximal
Amyotropic lateral sclerosis (ALS)
Most common (50%)
Loss of motor cortex neurons and anterior horn cells
of spinal cord, so UMN and LMN signo
Primary lateral sclerosis
Loss of motor cortex lesions, so only UMN
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Diagnosis
Examination
Limbs
Tone: increased/decreased (UMN/LMN)
Power: weakness, may be distal>proximal
Reflexes: increased/decreased (UMN/LMN)
Coordination: intact
Sensation: intact
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Investigations
Bloods
FBC (anaemia, infection),
CRP/ESR (infection, autoimmune),
LFTs, TFTs (thyroid disease)
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Differentials
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Metabolic
Toxins e.g. lead, mercury
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Management
Conservative
MDT
MND nurse
Neurologist
PT, OT
SALT
Dietician
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Medical
Symptomatic
Spasticity
Anti-spasmodics
(e.g. baclofen, dantrolene)
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Drooling
Antisecretories
(e.g. amitriptyline,
atropine, hyoscine
Specific
Glutamate agonists
E.g.: riluzole
MOA: anti-glutamatergic;
prevent/reverse motor neuron loss
Outcome: prolong life 3m
SEs: vomiting, tachycardia, LFTs,
somnolence, headache, vertigo
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