PubMed Health⌕ Search
Home/Archive/PubMed

PubMed · 7090604

[Agyria--a clinico-pathological study (author's transl)].

J Rudel·R Warzok·D Schreiber·R Eulitz
View original source ↗Export metadata ↓

Abstract

Agyria is an extremely rare malformation of the brain caused by an arrest of neuronal migration towards the cortex. A case of nearly complete agyria is presented in a 2 8/12 year-old boy. The autopsy revealed complete absence of cerebral convolutions in the frontal, parietal and occipital lobes of both hemispheres and discrete convolutions in the temporal lobes. The cortex reached up to 22 mm and the central white matter was very thin, occipitally up to 2 mm. Additionally, hydrocephalus internus was observed. Clinical and pathoanatomical findings are presented and compared with other data published in the literature. The results are discussed in relation to a possible pathogenetic mechanism leading to this cerebral malformation.

Explore related subjects

AutopsyBrainChild, PreschoolFrontal LobeHumansHydrocephalusMaleOccipital LobeParietal LobeCase ReportsEnglish AbstractJournal Article

Keep this discovery

Explore connections, maps & timelines

BibTeXRIS

J Rudel, R Warzok, D Schreiber, R Eulitz. 1982. [Agyria--a clinico-pathological study (author's transl)].. https://pubmed.ncbi.nlm.nih.gov/7090604/

Cite the original work for its findings. Save a collection to share your selection of sources.

KEEP EXPLORING

Related citations

PubMed2007

Langerhans cell histiocytosis infiltration into pancreas and kidney.

An 18-month-old male presented with a swelling of the neck for 2 months. A presumptive diagnosis of Langerhans cell histiocytosis (LCH) was made on fine needle aspiration cytology from the lymph node. The child received chemotherapy. He remained well for around 10 months, when he represented with loose stools, cough, and respiratory distress. His condition deteriorated over a few hours culminating in death. A partial autopsy revealed LCH infiltration in liver, pancreas, and kidneys along with bronchopneumonia. The pancreatic and renal infiltration by LCH is extremely rare.

Autopsy↗
PubMed2007

Radiography after unexpected death in infants and children compared to autopsy.

BACKGROUND: Postmortem radiography may reveal skeletal and soft-tissue abnormalities of importance for the diagnosis of cause of death. OBJECTIVE: To review the radiographs of children under 3 years of age who had died suddenly and unexpectedly. To compare the radiological and autopsy findings evaluating possible differences in children dying of SIDS and of an explainable cause. MATERIALS AND METHODS: A total of 110 consecutive skeletal surveys performed between 1998 and 2002 were reviewed. All but one were performed before autopsy and comprised AP views of the appendicular and axial skeleton and thorax/abdomen, lateral views of the axial skeleton and thorax, and two oblique views of the ribs. Radiography and autopsy findings were compared. RESULTS: Causes of death were classified as SIDS/borderline SIDS (n = 52) and non-SIDS (n = 58), with one case of abuse. In 102 infants there were 150 pathological findings, 88 involving the chest, 24 skeletal, and 38 miscellaneous findings. The radiological-pathological agreement was poor concerning pulmonary findings. Skeletal findings were sometimes important for the final diagnosis. CONCLUSIONS: Radiography revealed many skeletal and soft-tissue findings. Pulmonary pathology was most frequently found, but showed poor agreement with autopsy findings. Recognizing skeletal findings related to abuse is important, as these may escape recognition at autopsy.

Autopsy↗
PubMed2007

Autopsy-proven Huntington's disease with 29 trinucleotide repeats.

Huntington's disease (HD) is a neurodegenerative disorder associated with expansion of CAG trinucleotide repeats in the huntingtin gene. A minimum of 36 CAG repeats is usually reported in patients with clinical features of HD; 30 to 35 repeats represent an intermediate range. Here we report a 65-year-old male with autopsy-proven HD and 29 CAG repeats.

Autopsy↗