Main clinical features
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characterized by progressive dementia, spasticity, cerebellar ataxia with onset at around the fifth decade of life and cerebral amyloid angiopathy, non neuritic and perivascular plaques and neurofibrillary tangles as predominant pathological lesions
onset occurred between 40 and 60 years of age with early onset of spasticity with increased deep tendon reflexes and tone
MRI demonstrated extensive alteration of signal intensity in periventricular white matter, with sparing of U fibers, as well as abnormal signal intensity in brainstem and cerebellar white matter |