PubMed · 6242985
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J J Askenasy. 1981. Sleep patterns in extrapyramidal disorders.. https://pubmed.ncbi.nlm.nih.gov/6242985/
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Five boys and two girls from a large consanguineous British Muslim family of Pakistani origin are described. All presented from infancy to early childhood with progressive moderate to severe developmental delay, postnatal microcephaly, spastic quadriplegia, refractory seizures, and visual handicap. Cerebrospinal fluid (CSF) pleocytosis was present in three children. Neuroimaging with computerized tomography on three boys and a girl showed generalized cortical atrophy, dilatation of the lateral, third, and fourth ventricles, widening of the surface CSF spaces, hypoplasia of the posterior fossa structures, and multiple and solitary calcifications in the cerebral cortex and punctate calcifications involving basal ganglia, cerebellum, and the Sylvian fissure. Histopathological examination of the brain from three boys and one girl confirmed generalized cortical and cerebellar atrophy with widespread calcifications within the cortical grey and white matter, the basal ganglia, the cerebellum, and in some areas along the capillaries. Investigations excluded a possible nongenetic cause. Parental consanguinity favor autosomal recessive inheritance. This appears to be a recognizable syndrome overlapping the syndrome of Aicardi and Goutières (MIM 225750).
Idiopathic cerebral calcification can be associated with a progressive neurologic disorder for which there is no known treatment. This report describes a patient with a familial form of this disorder presenting in middle age with progressive Parkinson-like features along with spasticity, dystonia, and ataxia. Disodium etidronate, a bisphosphonate, produced a two-fold improvement in the rate of his speech and gait, but did not affect his spasticity, dystonia, or ataxia. Quantitative analysis of cerebral calcification did not reveal any reduction in the amount of calcification, suggesting other possible mechanisms for this clinical improvement.
We review forms of diaschisis and their relationship with neurobehavioral and neuropsychological findings. The following forms of diaschisis are discussed: (1) cortico-cerebellar diaschisis; (2) cerebello-cortical diaschisis; (3) transhemispheric diaschisis; (4) cortico-thalamic diaschisis; (5) thalamo-cortical diaschisis; and (6) basal ganglia-cortical diaschisis. For each form, the neurobehavioral and neuropsychological findings are discussed. Diaschisis can be classified from the behavioral point of view as follows: (1) forms in which the metabolic effect at distance is not followed by neurobehavioral impairment; (2) forms in which the remote metabolic impairment could induce neurobehavioral and neuropsychological disorders; and (3) forms in which the role of the lesion itself and its remote metabolic effects on the production of neurobehavioral and neuropsychological abnormalities cannot be disentangled.