Primary Progressive Multiple Sclerosis (PPMS) indicates progressive disease from disease onset.

Clinical features:

(1) >=1 year of disability progression (increasing objectively documented neurological disability independent of relapse)

(2) >= 2 of the following

(2a) >= 1 T2 hyperintense lesions characteristic of MS in >= 1 of the following brain regions: periventricular, cortical/juxtacortical. or infratentorial

(2b) >= 2 T2 hyperintense lesions in the spinal cord

(2c) presence of CSF specific oligoclonal bands


The patient may experience periods of stability, fluctuations and superimposed relapses.


Differential diagnosis:

(1) secondary progressive MS, with initial relapsing-remitting course followed by progression

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