Progressive encephalomyelitis with rigidity is one of the Stiff Man Plus Syndromes.
Course: subacute, with survival usually less then 3 years
Cause: paraneoplastic, post-viral, idiopathic
Pathology: demyelination that spares the corticospinal tracts
Clinical features:
(1) widespread rigidity
(2) myoclonus with painful muscle spasms
(3) long tract signs
(4) brainstem signs
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Specialty: Immunology/Rheumatology
ICD-10: ,
