Progressive subkortikale Gliose

Abstract

A case of autoptically verified progressive subcortical gliosis (PSG) is reported. The 79 year old woman developed subacutely a right sided hemisyndrome and a cerebellar syndrome. Generalized action myoclonus of the left leg evolved into left sided Epilepsia partialis continua and dementia appeared. After a 6 month course the patient died of aspiration pneumonia. There was no indication of alcoholism or HIV-dementia neither clinically nor at autopsy. Morphologically the brain showed a diffuse proliferation of astrocytes in the subcortical white matter, thalamus, basal ganglia, brain stem and cerebellum. A severe neuronal dropout was found in medial thalamic neurons but Wernickes encephalopathy was ruled out. 21 cases of PSG confirmed by autopsy were found in the literature. Clinics, neuropathology and classification of PSG is discussed.

The neuropathology of chromosome 17‐linked dementia

“…ential involvement of the ventral striatum is most likely associated with the pathology in the association cortices and neocortical limbic structures [25, 261. The present disease is sufficiently different from AD, Parkinson's disease (PD), and amyotrophic lateral sclerosis (ALS) that they need not be considered in the differential diagnosis. Ch-17D shares clinical features with Picks disease [32-351, hereditary dysphasic dementia (HDD) [32], lobar atrophy 136, 371, pallidopontonigral degeneration (PPND) [38], progressive subcortical gliosis (PSG) [9, [39][40][41][42], frontal lobe degeneration (FLD) [43,441,CBD 145,461, mesolimbocortical dementia [47], diffuse L e y body disease (DLBD) , and parkinsonism-ALS and related disorders [51][52][53]. Ch-17D [I] and familial PSG 141 are the first frontal lobe dementia syndromes that have been linked to chromosome 17, and differ from known genetic loci for other dementing disorders, parkinsonism, or motor neuron disease [54][55][56][57].…”

Progressive Hemiparesis in Frontal Lobe Degeneration

“…There is 1 case of a 79-year-old woman who presented with right hemiparesis and cerebellar ataxia, but soon developed action myoclonus and rapidly progressive dementia. At autopsy, intense white matter gliosis was found, while neuronal loss was limited to the medial thalamic nuclei [34], In one of Neumann's [35, case 6] original cases, latestage hemiparesis was described. A further patient [36, case 2] showed flexion contractures of both arms and increased muscle tone with hyperreflexia of both legs.…”