Acute neurological dysfunction associated with destructive lesions of the basal ganglia in children

Abstract

Three children ranging in age from 1 to 4 1/2 years suffered an acute illness with disturbance of consciousness followed by motor rigidity, loss of spontaneous movements and language, axial hypotonia, and a stereotyped response to any kind of stimulus. In two cases, images on computerized tomography were consistent with necrosis of the lenticular and caudate nuclei. The acute onset was followed by later improvement and stabilization. One child died, probably not as a direct consequence of the neurological disorder, autopsy showed bilateral necrosis of the putamina. We suggest that these three patients, together with two previously reported cases, constitute a clinically and radiologically recognizable subgroup among the heterogeneous disorder that produce bilateral striatal necrosis in children. The cause of the syndrome is unknown.

Imaging in influenza A encephalitis.

“…Localised hypodense lesions in the basal ganglia have also been described on computed tomography in encephalitis after infections with ECHO, mumps, measles, and Coxsackie viruses.18 20 Circumstantial evidence has implicated the influenza virus as the cause of the encephalitis lethargica epidemic of the 1920s.23 Similar isolated cases have been described from year to year often at the time ofinfluenza epidemics.2429 von Economo first described the illness, postulated a viral aetiology, and reported foci of inflammation in the grey matter of the midbrain and basal ganglia.30 The manifestations of encephalitis lethargica were variable but a number of clinical features have been proposed as major criteria to support the diagnosis.3' Our first case showed signs of basal ganglia involvement with choreoathetoid movements, ophthalmoplegia, and somnolence. Our second case exhibited akinetic mutism, central respiratory irregularities, and somnolence.…”

Localization of Basal Ganglia and Thalamic Damage in Dyskinetic Cerebral Palsy

“…37,38 The possible pathophysiologic route by which lentiform damage causes dystonia (the most common movement disorder in dyskinetic CP) can be hypothesized using two different models of basal ganglia motor function. In the classic rate-based model, two basal ganglia-thalamocortical pathways work in concert.…”

A type I interferon signature identifies bilateral striatal necrosis due to mutations inADAR1

“…BSN refers to the acute or subacute onset of a dystonic/rigid movement disorder associated with radiological evidence of symmetrical abnormalities in the corpus striatum and sometimes globus pallidus 3. These are apparent on CT as low density, and on MRI as high signal on T2 weighted and FLAIR sequences, and low signal on T1 weighted images.…”