[Early infantile autism and excessive aerophagy with symptomatic megacolon and ileus in a case of Ehlers-Danlos syndrome].

A case of Ehlers-Danlos syndrome is reported, which was associated with mental retardation and early infantile autism. The patient died at the age of 19 by ectasy of the stomach as the result of extreme aerophagy causing a megacolon and by compression so finally resulting in a mechanical ileus. Investigation of the cerebellum revealed a significant rarefication and diminuation of the Purkinjè cells as well as the cells of the stratum granulare in the lobuli VI and VII, obviously due to a genetically determined malformation.

[Vascular (arteriosclerotic) and Alzheimer's dementia in neurologic-psychiatric patient sample].

Despite the obviously more important degeneratively conditioned dementias, particularly of the Alzheimer-type, dementias due to vascular disorders are still of considerable relevance. They are even predominant among autopsies in a neurological-psychiatrical hospital, showing, however, a decreasing tendency. Though this cannot reflect the real incidence of a disease, because many cases of dementia die in geriatric or nursing homes, mostly not autopsied, exact epidemiological examinations of autopsy-cases seem to be advisable, limited temporarily and locally for economic reasons.

[Encephalomyocarditis syndrome (EMC syndrome)].

Among 28 autopsy-cases with EMC had been none diagnosed clinically before. Typically we found an interstitial myocarditis and perivenous encephalitis with a preferred participation of the brain stem, eventually resulting in a central failure. There were only 5 cases with meningitis.

[Neuropsychiatric and neuropathologic findings in an adult case of cri-du-chat syndrome (Lejeune syndrome, crying cat syndrome)].

It is reported on a mental retarded female, observed from 3-33 years of age, which demonstrated even in adulthood with long lasting shrill crying the clinical leading symptom of Lejeune syndrome and further typical symptoms with inhibition of physical maturation, important delayed motoric and mental development, microcephaly resp. characteristic craniofacial dysmorphia, anomalies of extremities, muscular hypotonia and susceptibility to infection. Psychically there were a striking affective instability and irritability, neuropathologically pachygyria and multiple cortical heterotopias.

[Leigh disease. Morphology and clinical aspects].

Morphologico-topographic differences, especially with regard to size and distribution of foci, as well as equalities of the histomorphological pattern are shown in an infantile and in a juvenile case of Leigh's disease. Almost identical microscopic findings in the area of the olives in the medulla oblongata seem to be of special importance. The typical symptoms at the beginning of the disease with predominance to muscular hypotonia and the bulbar paralytic final stage might at least enable a tentative diagnosis even without evidence to the basic enzymatic defect, mostly in pyruvate metabolism, and changes in mitochondria in muscles and the central nervous system.