Esquizencefalia de labio cerrado bilateral: a propósito de un caso / Schizencephaly of bilateral closed lip: speaking of one case

La esquizencefalia es un desorden de la migración neuronal, caracterizada por hendiduras de sustancia gris en los hemisferios cerebrales que se extienden desde la superficie pial a los ventrículos laterales. Esta patología es el resultado final de una amplia variedad de factores genéticos, tóxicos, metabólicos y de agentes infecciosos que ocurren durante un período crítico del desarrollo cerebral. Esta malformación puede ser unilateral o bilateral y puede ser dividida en dos subtipos: de ¨labios cerredos" o tipo I, o de "Labios abierto" o tipo II. Presentamos el caso de una paciente demenina de 37 años de edad con epilepsia y hallazgos neurorradiológicos de esquizencefalia de "labios cerrados" bilateral

Schizencephaly is a rare developmental of neuronal migration, characterized by congenital clefts spanning the cerebral hemisphere from the pial surface to the lateral ventricle and lined by cortical gray matter. The lesion is the final result of a variety of etiologies including genetic, toxic, metabolic and infectious agents during a critical period of the development of the brain. This malformation can be unilateral or bilateral and may be divided in two subtypes: "closed tips" or type I and "open tips" or type II. We present 37 years old female patient with epilepsy and neuroradiological findings of bilateral "closed lips" schizencephaly

Cortical sulcal enlargement in catatonic schizophrenia: a planimetric CT study.

To determine whether patients with catatonic schizophrenia have specific alterations in brain morphology, internal (ventricles) and external (frontal, temporal, parieto-occipital) components of the cerebrospinal fluid (CSF) spaces were examined morphometrically. Planimetric measurements of computed tomographic (CT) scans from 37 patients with catatonic schizophrenia, 28 patients with hebephrenic schizophrenia, and 39 patients with paranoid schizophrenia, all diagnosed according to DSM-III-R criteria, were compared with separate age- and sex-matched non-psychiatric control groups, respectively. The areas of the frontal sulci, the parieto-occipital sulci, the inter-hemispheric fissure, and the lateral and third ventricles were measured separately for the right and left hemispheres. Catatonic patients showed significant enlargements in almost all CSF spaces, especially in the left fronto-temporal area which, in addition, correlated significantly with illness duration. Hebephrenic patients showed selective enlargements in left temporal and left/right lower frontal cortical sulci, whereas paranoid schizophrenic patients showed no enlargements but significant correlations between left temporal cortical sulcal volume and illness duration. Alterations in temporal cortical areas were present in all three sub-types of schizophrenia. In addition to temporal alterations, hebephrenic schizophrenia was characterised by lower frontal (i.e. orbitofrontal) enlargement. Catatonic schizophrenia, the most severe sub-type with regard to clinical symptomatology and brain pathology, showed fronto-parietal cortical alterations.

Limbic pathology in schizophrenia: the entorhinal region--a morphometric study.

The volume of the entorhinal region is significantly reduced in postmortem brains of schizophrenics compared with controls (p less than 0.017). In addition, a significant reduction of neurons (p less than 0.017), but no significant increase in absolute glial cell numbers, is found. These results are consistent with the hypothesis that structural changes in the medial temporal lobe of schizophrenics may be developmental in origin.

Hippocampal pyramidal cell orientation in schizophrenia. A controlled neurohistologic study of the Yakovlev collection.

The finding of marked disorganization of the hippocampal pyramidal cell layer in the brains of schizophrenic patients has recently been reported. The present study was undertaken to determine whether similar abnormalities could be found in the brains of a population of schizophrenic patients, most of whom were never exposed to neuroleptics. Though statistical analysis of 2808 pyramidal cells failed to reveal significantly greater disorganization in a group of seven alleged schizophrenic brains than in brains of age-matched, nonpsychotic controls, the data suggest a relationship between the degree of pyramidal cell disarray and the severity of behavioral impairment due to psychosis. The implications and pitfalls in interpreting these findings are discussed.

Cell loss in the hippocampus of schizophrenics.

To investigate whether volume reduction of the hippocampal formation of schizophrenics, as described previously, is paralleled by loss of neurons and fibre systems, tissue volumes and cell numbers of all parts of the hippocampal formation in post mortem brains of 13 schizophrenics and 11 age-matched controls belonging to the Vogt collection were determined. Volumes of the whole hippocampal formation (P less than 0.01), the whole pyramidal band (P less than 0.001) and the hippocampal segments CA1/CA2 (P less than 0.01), CA3 (P less than 0.005), CA4 (P less than 0.01) were decreased, whereas no significant volume reduction of the alveus and fimbria hippocampi and prosubiculum/subiculum could be found. The perforant path showed a trend towards volume reduction (P less than 0.1). The absolute number of pyramidal cells (tissue volume X cell density) was diminished in CA1/CA2 (P less than 0.05), CA3 (P less than 0.05) and CA4 (P less than 0.05), but was not significantly changed in the prosubiculum/subiculum, the presubiculum/parasubiculum and the granular cell layer of the dentate fascia. Pyramidal cell loss in CA1/CA2, CA3, CA4 was more distinct in the paranoid patients than in catatonics. The findings are discussed with respect to current hypotheses of limbic dysfunction in schizophrenia.

A catatonic syndrome resulting in death.

A patient is described who presented with post-partum catatonic schizophrenia and who later became comatose and died with hyperthermia. The differential diagnosis of catatonic stupor is notoriously difficult. The clinical picture somewhat resembled Stauder 's lethal catatonia, doubtless a syndrome secondary to various encephalopathies rather than a disease sui generis . The differential diagnosis is discussed with special emphasis on herpes virus encephalitis and the rare but very important neuroleptic malignant syndrome, which is often unrecognized but for which specific treatment may be available.

The diencephalon in schizophrenia: evidence for reduced thickness of the periventricular grey matter.

To find out whether ventricular enlargement in schizophrenia, as demonstrated by neuroradiological methods, is caused by an atrophy of ventricle surrounding diencephalic structures, volume measurements and linear measurements of the whole thalamus, all large thalamic subnuclei and some extrathalamic brain parts were carried out on serial sections of post mortem brains belonging to the Vogt collection. The only significantly diminished parameter of this study was the thickness of the periventricular grey matter surrounding the third ventricle, while the volume and linear measurements of the whole thalamus and all large thalamic subnuclei were not significantly changed. The findings are discussed with respect to current hypotheses of diencephalic dysfunction in schizophrenia.