Sporis D, Habek M, Mubrin Z et al. Cog Behav Neurol 2007: 20:138-140.
Authors claim there are two forms: a vasculitis type with strokelike manifestations and seizures, and a diffuse progressive type with dementia and psychiatric manifestations. Authors present a 38 year old woman (type 2) with a history of RA, and depression, and a history of hypothyroidism (not mandatory for HE), with acute psychosis with auditory hallucinations and bizarre behavior, a CSF pleocytosis (37 WBC, normal protein) normal memory, EEG showing slowing. Behavioral exam abnormal calculations, constructional praxis, and attention. CSF showed 12 cells. MRi showed punctuate lesions in left frontal gyrus. Treatment with methylprednisoline 1000 mg per day for three days followed by prednisone 50 mg per day for one month resulted in resolution of all symptoms and EEG,
Authors discuss that half of patients have MRI abnormalities that can mimic tumor, granuloma, infection, stroke, degeneration, or leukodystrophy. Several patients had other autoimmune abnormalities including RF, or p ANCA. Etiology of condition is not known.
Authors claim there are two forms: a vasculitis type with strokelike manifestations and seizures, and a diffuse progressive type with dementia and psychiatric manifestations. Authors present a 38 year old woman (type 2) with a history of RA, and depression, and a history of hypothyroidism (not mandatory for HE), with acute psychosis with auditory hallucinations and bizarre behavior, a CSF pleocytosis (37 WBC, normal protein) normal memory, EEG showing slowing. Behavioral exam abnormal calculations, constructional praxis, and attention. CSF showed 12 cells. MRi showed punctuate lesions in left frontal gyrus. Treatment with methylprednisoline 1000 mg per day for three days followed by prednisone 50 mg per day for one month resulted in resolution of all symptoms and EEG,
Authors discuss that half of patients have MRI abnormalities that can mimic tumor, granuloma, infection, stroke, degeneration, or leukodystrophy. Several patients had other autoimmune abnormalities including RF, or p ANCA. Etiology of condition is not known.
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