Neurological signs and operative indication by agenesis of the perisylvian region: a study of 13 operated cases.

The article reports on 13 cases of agenesis of the perisylvian region in patients aged 2 to 53 years. The agenesis occurred in the temporal operculum in 7 cases, in the frontal operculum in 5 cases and the whole fronto-parieto-temporal region in one case. The agenesis was always associated with space requiring arachnoidal cysts. Symphyses and obstruction of the flow of the basal CSF spaces were also seen. In 85% of the patients were males. The patients tend to decompensate after light cerebrocranial trauma, and in many cases surgery reveals a subdural haematoma which fills the aplastic region, due to detachment of the exposed cerebral veins effecting basal drainage. In the remaining cases, surgery was performed because of general signs of cerebral compression, exarcebation of an existing disease associated with attacks, or because of the space occupying character of the concomitant arachnoidal cysts. In patients wit agenesis of the perisylvian region without space occupying arachnoidal cysts, surgery is not performed for the time being, instead, regular neurological and CAT-Scan control is effected.

[Opercular aplasia (author's transl)]. / Die Aplasie der perisylvischen Region.

The article reports on 11 cases of opercular aplasia in patients aged 5 to 44 years. Aplasia occurred to a greater extent in the frontal operculum than in the temporal operculum, and was always associated with space-requiring arachnoidal cysts. Symphyses and obstruction of the flow of the basal CSF spaces were also seen. In 91% of the cases, the aplasia was localized on the left side, 91% of the patients were males. The patients tend to decompensate after a cerebrocranial trauma, and in many cases surgery reveals a subdural haematoma which fills the aplastic region, due to detachment of the exposed cerebral veins effecting basal drainage. In the remaining cases, surgery was performed because of general signs of cerebral compression, exacerbation of an existing disease associated with attacks, or because of the space-occupying character of the concomitant arachnoidal cysts. In patients with opercular aplasia without space-occupying arachnoidal cysts, surgery is not performed for the time being; instead, regular neurological and CT control is effected.

Medical audit and reliability control of cranial computer tomography.

A long-term documentation is presented which serves different quality-control mechanisms in computer tomography (CT) reporting. Developed by co-operation between different departments of the Hannover Medical School it has been based on 1000 CT reports and the experiences gained from them. The modular straightforward design makes the system applicable for routine documentation as well as for scientific investigations. It allows easy statistical analysis of the data gathered and can be used as a differentiated master file for special investigations, for example pattern-recognition procedures on the original data matrices. With regard to medical audit it allows the patients who really need it, access to the limited CT capacity.

The value of computed tomography in cerebral syphilis.

The computed tomographic findings of three patients with cerebral syphilis, including cerebral gumma, which regressed completely under penicillin therapy, syphilitic angiitis with cerbral infarction, and syphilitic cerebral atrophy, are reported. CT is unable to provide specific diagnostic data for these conditions. The etiology can be clarified only by taking into consideration the clinical findings and course, the serological results, and the result of therapy.

[Psychosocial intellectual development of children with infantile cystinosis and cerebral atrophy (author's transl)]. / Psychosozial-intellektuelle Entwicklung bei Kindern mit infantiler Zystinose und Hirnatrophie.

The psychosocial and intellectual development of 4 boys with nephropathic cystinosis and brain atrophy documented by cranial computerized tomography was investigated by use of biographical data and psychological tests (HAWIK, Deutscher Rechtschreibtest). Inspite of the brain atrophy the patients showed low-normal intellectual capacities and mainly average school performance. There were no psychosocial abnormalities correlated to the primary metabolic disease. However, renal dwarfism led to mascotism requiring psychotherapy.

Evidence for cerebral involvement in nephropathic cystinosis.

Cranial computerized tomography (CCT) of 3 children with nephropathic cystinosis and chronic renal failure (CRF) revealed a hydrocephalus internus and externus. In two boys the findings consisted of bilateral dilatation of the ventricular system and of the subarachnoid space; in one boy the alterations were mainly unilateral. The children had repeated convolsions which could not be explained by deterioration of renal function. Their neurological condition was otherwise normal. In six non-cystinotic patients with chronic renal failure, CCT showed normal anatomical structures. The possibility is discussed that the hitherto unknown pathogenetic mechanism of cystinosis leads to diffuse cerebral atrophy, resulting in internal and external hydrocephalus.