ALS-Plus Syndromes and ALS-Mimic Syndromes must meet the clinical, electrophysiological and neuroimaging criteria for Clinically Possible, Clinically Probable or Clinically Definite ALS. The predominant presentation in ALS-Plus Syndromes and ALS-Mimic Syndromes is that seen in sporadic ALS, but includes one or more features such as:
- (1) Geographic clustering
- including disorders seen in the Western Pacific, Guam, Kii Peninsula, North Africa, Madras, etc.
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- (2) Extrapyramidal signs
- bradykinesia; cogwheel rigidity; tremor; familial or sporadic
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- (3) Cerebellar degeneration
- spinocerebellar abnormalities; familial or sporadic
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- (4) Dementia
- familial or sporadic; frontal lobe type; Creutzfeldt-Jacob amyotrophic form
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- (5) Autonomic nervous system involvement
- clinically significant abnormal cardiovascular reflexes; bowel or bladder control problems; familial or sporadic
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- (6) Objective sensory abnormalities
- decreased vibration; sharp/dull discrimination; blunting of cold sensation; familial or sporadic
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- (7) Ocular movement abnormalities
- supranuclear; nuclear; familial or sporadic
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- (8) ALS mimics
- delayed post-poliomyelitis; multifocal motor neuropathy with or without conduction block; endocrinopathies; lead intoxication; infections
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