[Familial akinesia-hypokinesia sequence (Pena-Shokier phenotype)]. / Familiäre Akinesie-Hypokinesie-Sequenz (Pena-Shokier-Phänotyp).

The Pena Shokeir phenotype (PSP) is characterised by multiple ankyloses, camptodactyly, facial dysmorphisms and lung hypoplasia with hydramnios. The basic neuromuscular defect leads, through a fetal hypokinesia-akinesia, to the development of this nonspecific phenotype and a respiratory insufficiency with early postnatal mortality. Severe central nervous anomalies are described in one-third of the reported cases. In this paper a foetus with PSP and 4 further foetuses with severe cerebral malformations and only discrete lung hypoplasia are described. It is not clear whether the cerebral malformations represent a primary or secondary developmental defect.

A reliable fluorescent stain for fungi in tissue sections and clinical specimens.

A simple and reliable staining technique is described using the fluorescent brightener Blankophor BA which binds specifically to fungal cell wall components. Potential diagnostic applications are shown.

[Spinal hemangioblastoma in infants with dysraphic malformation of the CNS]. / Das spinale Hämangioblastom im Säuglingsalter in Kombination mit dysraphischer Fehlbildung des ZNS.

Spinal hemangioblastoma in early infancy is an extreme rarity. In the case presented here such a hamartomatous tumor is combined with dysraphic malformation of the CNS in a male infant aged seven months. The morphological, epidemiological and clinical aspects are discussed with special reference to the possibility of congenital occurrence.

[Neuroanatomical correlates of the amnestic syndrome (author's transl)]. / Neuromorphologische Grundlagen des amnestischen Syndrom

An amnestic syndrome can be observed in several organic brain disorders. This paper tries to elucidate the anatomical substrate of this syndrome by investigation of the brain in 168 carefully examined cases. These included 55 cases of brain tumor, 8 of encephalitis, 55 of Wernicke encephalopathy, and 50 of Alzheimers disease or senile dementia. All these patients had shown a prominent amnestic syndrome. Despite the diversity of the organic brain pathology the anatomical findings in all cases were bilateral lesions of variable intensity in one or more of the following structures: Ammon's horn, fornix, and corpus mammillare. In conformity with the literature it is a assumed that preservation of these structures must be of great significance for the processes of mnemonic registration, integration, and recall. A bilateral lesion in all or some of these centers and their connections causes, as a rule, disturbances of memory. This conclusion, however, does not rule out the possibility that similar memory disturbances may occur following other organic lesions, localized elsewhere in the brain.