Search PubMed⌕ Search

Biomedical subjects

F C Stam

Publications and source records attributed to F C Stam.

At least 37 records · Page 2Linked to original sources

[Do immunological factors play a role in the pathogenesis of Alzheimer's disease?].

Immunological injury is frequently thought to be a critical factor in Alzheimer's disease. In this review we discuss the immunological indices in serum and cerebrospinal fluid of patients with dementia of the Alzheimer type and subsequently the nature of cerebral amyloid. We conclude that the results of the immunopathological research program have not contributed to the understanding of the pathogenesis of Alzheimer's disease. Finally, we discuss the problems of neurobiological investigations concerning patients with dementia.

Aged↗

Leucoencephalopathy after inhaling "heroin" pyrolysate.

47 patients with spongiform leucoencephalopathy but no other consistent abnormalities, except brown pigmentation of the alveolar macrophages in the lungs, are described. 11 patients have died. Epidemiological studies indicate at the cause of the illness the inhalatory use of poisoned heroin vapours (pyrolysate). The heroin is primarily sold on the black market in Amsterdam. The Netherlands. Over 170 suspect heroin samples were collected for analysis of the possible poisonous factor. Although suspect, none of the samples could be unambiguously related to the observed illness. Chemical, toxicological, and histopathological investigations have not so far revealed the nature of the poisonous factor, but several neurotoxic agents that are known to cause comparable leucoencephalopathies have been ruled out. This appears to be the first manifestation of the poisonous potential of the unknown causative factor.

Adolescent↗

Immunoglobulins and complement factors in senile plaques. An immunoperoxidase study.

Immunoperoxidase techniques were used to obtain information about the possible presence of serum factors in senile plaques. We found only in plaques consisting of an amyloid core surrounded by a corona of degenerating neurites small amounts of IgG and light chains (kappa and lambda). These immunoglobulins were principally localized in the corona and not in the central amyloid core. Further it was found that all plaques contain the complement factors C1q, C3b, C3c, C3d and C4. Senile plaques lacked C5, C3 pro-activator and properdin. The possible significance of these findings in the genesis of the senile plaques and amyloid formation is discussed.

Alzheimer Disease↗

Familial lissencephaly with extreme neopallial hypoplasia.

Two siblings, male and female, with identical lethal brain malformation are described. Their anomaly is characterized by very low brain weight, lissencephaly, wide ventricles and thin neopallium (colpocephaly) varying in thickness between 0.2 and 3 mm. The neocortex is four layered as in classic lissencephaly. Brainstem and cerebellar anomalies are more extensive than in cases hitherto described in detail. No extracranial malformation is found. The parental karyotypes are normal. The relationship to previously reported familial cases of lissencephaly and several inherited syndromes featuring lissencephaly is discussed. The present family may represent a severe expression of previously described autosomal recessive lissencephaly without extracranial anomaly or may represent a new genetic lissencephaly syndrome.

Brain↗

Autosomal dominant multicore disease.

Two girls and their mother with multicore myopathy are described. The cores consisted of Z band disorganisation and decreased or absent enzyme activity. Only one case has clinical signs of myopathy. Muscle enzyme activity was elevated in the two children. The mode of inheritance was autosomal dominant.

Adolescent↗

Congenital multiple angiomatosis with brain involvement.

A case of congenital multiple angiomatosis with cerebral involvement is presented. Postmortem investigation showed identical angiomas on skin and brain. The cerebral vascular tumor has caused altered vascularization of the hemisphere, resulting in a development arrest of the corpus callosum and a fetal appearance of the parietal and temporal vessels.

Agenesis of Corpus Callosum↗

On the relationship between germinal layer haemorrhage and telencephalic leucoencephalopathy in the preterm infant.

The brains of 54 deceased preterm infants were retrospectively studied for signs of germinal layer haemorrhage (GLH) and telencephalic leucoencephalopathy (TLE). On the basis of presence or absence of iron laden macrophages (siderophages) the cases with germinal layer haemorrhage were sub-classified as either "old" or "fresh" haemorrhage. Cases without GLH (non-GLH) were used as a control group. The distribution of ages at death in the non-GLH group was such that comparison to both fresh GLH cases and old GLH cases was possible. The distribution of pregnancy duration in GLH and non-GLH cases was similar. Statistical evaluation showed that old GLH cases were more often associated with TLE as either fresh GLH or non-GLH cases. By comparison of the three groups it is shown that differences in age at death do not explain the higher incidence of TLE in the old GLH group compared to the fresh GLH group. Hydrocephalus was found in the majority of old GLH cases. From a neuropathological view this complication is unlikely to account for the excess of TLE-lesions in that group. A logic explanation of the findings in this study is that the association between GLH and TLE is due to a common origin: asphyxia. The relatively low incidence of TLE in fresh GLH cases may be due to early death precluding detection of TLE. It is advocated that studies on GLH or TLE be comprehensively based on both phenomena to further the understanding on how they contribute to death or handicap.

Asphyxia Neonatorum↗

Congenital muscular dystrophy and cerebral dysgenesis in a Dutch family.

A Dutch sibship is described consisting of a girl and a boy affected by the same disease. Both suffered from hydrocephalus and severe generalized weakness with death at 2 days and 4 months respectively. Full autopsy was done on the boy and this revealed a lissencephalic, partly polymicrogyric, neocortex, a bridge of grey matter linking the cerebral hemispheres before and over the lateral ventricles, neocortical dysplasia with subcortical neuronal heterotopic masses, generalized white matter gliosis, also involving the long fibre tracts and generalized vascular proliferation. The cerebellum showed generalized polymicrogyria. Also true hydrocephalus was found presumably related to a malformed aqueduct. Muscle biopsy revealed severe changes, consistent with congenital muscular dystrophy. Representative sections from the girls autopsy revealed an identical pattern of abnormalities. The described pattern fits descriptions of Fukuyama's cerebromuscular dystrophy.

Brain↗

Presenile dementia--a form of Lafora disease.

The autopsy findings on a 60-year-old man with progressive disturbances of gait, presenile dementia and incontinence, showed Lafora bodies in numerous ganglion cells of the cerebral cortex and in many nuclei of the brain stem. Histochemical analysis of the Lafora bodies revealed the presence of a polysaccharide-protein complex containing phosphate groups. The case closely resembled the one described by Suzuki et al. It is suggested that this type of presenile dementia may be a presenile form of Lafora disease.

Ataxia↗

Pigment in the lining of nasopalatine duct cysts: report of two cases.

In a review of 20 nasopalatine duct cysts, two cases were encountered with a pigmented epithelial lining. In the literature we have found one similar case only. The pigment in our cases was shown to be melanin. The authors suggest that the melanin-containing epithelial cells are derived from Jacobson's organs and, therefore, most likely should be considered olfactory epithelium.

Adult↗

Tuberous sclerosis and dysplasia of the corpus callosum. Case report of their combined occurrence in a newborn.

A neuropathological study is presented of a case showing the association of tuberous sclerosis of the brain and dysplasia of the corpus callosum as well as omphalocele and malrotated colon. No signs of tuberous sclerosis were found in the internal organs. From a review of the literature this appears to be the fourth case report of tuberous sclerosis and dysplasia of the corpus callosum. The association with omphalocele, to our knowledge, has not yet been reported.

Agenesis of Corpus Callosum↗

Paraganglioma of cauda equina.

After a brief introduction to the problem of the nature and localisation of sympathetic and parasympathetic paragangliomas a new case of paraganglioma of cauda equina is reported.

Adult↗