Biomedical subjects
Publications and source records attributed to R C Kim.
At the age of 36, this patient's clinical picture satisfied the criteria for the diagnosis of benign intracranial hypertension. After an essentially symptom-free interval, she developed new symptoms 17 years later that proved to be the result of a associated with glial-lined cavities in the midbrain and malignant glioma in the splenium. The possibility of a relationship between these disparate events is considered.
We studied a case of cerebral capillary telangiectasis in infancy. The patient, who had received treatment with intramuscular corticotropin for hypsarrhythmia, suffered disseminated intravascular coagulation and died on the 66th day of life. Neuropathological examination disclosed the presence of two fresh hemorrhages of moderate size within the cerebral hemispheres, one of which seemed to have originated from a capillary telangiectasis within the left frontoorbital white matter.
A woman with active systemic rheumatoid disease had striking neuropsychiatric manifestations; a rheumatoid nodule within the choroid plexus was the sole neuropathological finding. Although we found only one other report of findings of this type in rheumatoid disease, we suspect that this phenomenon may not be as rare as originally believed, and that it may represent another mechanism by which behavioral changes develop in this disorder.
Patients with active rheumatoid disease may develop encephalopathy, myelopathy, peripheral neuropathy, and myopathy through a variety of tissue mechanisms. Brain involvement is usually characterized by the formation of rheumatoid nodules or by the development of vasculitis or its complications, and there is evidence to suggest that the trapping of immune complexes within the choroid plexus may be important in pathogenesis. Structural damage to the spinal cord and lower brain stem, on the other hand, most commonly results from narrowing of the bony canal, leading either to direct compression of neural tissue or to compromise of its vascular supply. The appearance of peripheral neuropathy generally signifies the presence either of inflammatory epineurial arterial disease or entrapment by neighboring anatomical structures. Skeletal muscle dysfunction may be due to vasculitis, myositis, or denervation atrophy. Both systemic and local anatomical factors, therefore, are of importance in determining the manner in which different parts of the nervous system may be affected in rheumatoid disease.
A family is described in which 4 of 10 siblings developed a dementing illness that culminated in death within five to six years of onset. The pathological findings in 3 members were strikingly similar, and consisted of widespread nerve cell loss and astrocytosis within the cerebral cortex, status spongiosus within the outer cortical layers and, in 2, nerve cell loss and astrocytosis within the dorsomedial nucleus of the thalamus. It is concluded that the disorder described in this report does not conform precisely to any of the currently recognized categories of familial dementing disease.
A 72-year-old hypertensive man had focal seizures and mild memory loss. At autopsy, there were many lacunar infarcts, particularly in the neostriatum. Rust-brown discoloration and spheroid formation within the globus pallidus and pars reticulata of the substantia nigra resembled Hallervorden-Spatz disease, in which more detailed analysis of the striatum may be worthwhile.
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In a 23-year-old primigravida with no prior history of rheumatic fever, choreiform movements developed late in the third trimester, and she died in a state of hyperthermia 14 days later. Results of neuropathological examination showed, as the basis for the chorea, nerve cell loss and astrocytosis within the striatum (especially the caudate nucleus). Anaylsis of the case in conjunction with a review of the literature strongly suggests that chorea gravidarum should be regarded as a syndrome rather than a specific disease entity.
A 63-year-old woman developed progressive rheumatoid disease complicated by a confusional state as well as persistent cerebrospinal fluid pleocytosis and hypoglycorrhachia. Neuropathological examination revealed extensive rheumatoid lesions in the cranial dura, falx, and choroid plexus. The choroid plexus has not been affected in any of the 16 previously reported cases of pathologically verified rheumatoid disease of the central nervous system. The findings in this patient support the observations of others regarding the role of the choroid plexus in development of CNS manifestations in systemic immune complex disease.
Three fatal cases of heat stroke were encountered in the central New York area over a two-month period. Although in each instance one or more predisposing factors were identified, exertion clearly played a role in its development in only one case. In addition to laboratory and postmortem findings indicative of dysfunction of many organ systems, there was morphological evidence of widespread damage to skeletal muscle.
A 16-year-old boy was stricken with a progressive neurologic disorder characterized primarily by dementia progressing to severe neurologic debility in 12 months and death 28 months following the first symptoms. Pathologic examination showed a spongiform encephalopathy, consistent witha clinical diagnosis of Creutzfeldt-Jakob disease (CJD). The noteworthy features of the case are the age of onset, the somewhat prolonged course an the amount of white matter change. These are discussed within the frame of reference of CJD and the spongiform encephalopathies of infancy and childhood. Animal inoculation studies employing post-mortem embalmed brain as inoculum are currently in progress to determine the transmissibility of this patient's disease.
The specific activity of protein L-isoaspartyl methyltransferase, an enzyme implicated in the metabolism of damaged, isoaspartate-containing proteins, has been measured in postmortem samples of parietal cortex from 30 individuals (19 with Alzheimer's disease and 11 controls). Methyltransferase specific activity was positively correlated with age at death, increasing by 2.9 pmol/min/mg of protein for every ten years of age (r = .51, p less than 0.005). This correlation was significant in the control and Alzheimer's disease groups alike. Specific activity also appeared to be about 15% higher in females than in age- and diagnosis-matched males (p less than 0.05). No significant differences were observed between age- and sex-matched Alzheimer patients and controls, suggesting that a deficiency in this enzyme is not responsible for the accumulation of abnormal proteins in Alzheimer's disease.
A marked and significant reduction of protease nexin-1 (PN-1) and PN-2/amyloid beta protein precursor (A beta PP) was observed in selected regions of Alzheimer's disease (AD) brains as compared to those of aged-matched controls. Correlative analysis indicated a relationship between PN-1 reduction and the severity of pathologic alterations. A statistically significant inverse correlation was noted between the level of PN-1 activity and the density of tau-positive dystrophic neurites in the hippocampus. In view of the ability of thrombin and PN-1 activity to regulate neurite outgrowth, it is possible that abnormal thrombin and PN-1 interactions may play a role in dystrophic neurite formation. The presence of clusters of dystrophic neurites around the capillaries suggests that blood-brain barrier (BBB) dysfunction may enhance such abnormal interactions. The decrease in PN-2/A beta PP levels in AD brains could possibly contribute to neuronal degeneration in AD in view of the ability of PN-2/A beta PP to protect neurons against the toxic effects of the A beta.
To understand the extent and specificity of astrocyte pathology in sporadic frontotemporal dementia (FTD), we examined several FTD cases for molecular and morphologic characteristics of astrocyte degeneration. We quantified reactive and degenerating astrocytes in sections of frontal, temporal, parietal, and occipital cortex identified using glial fibrillary acidic protein (GFAP) immunoreactivity, terminal deoxynucleotidyl transferase (TdT) labeling, and morphological characteristics and compared them with nondemented, age-matched control brains. Conventional and confocal microscopy revealed that a subpopulation of GFAP(+) astrocytes exhibited positive TdT labeling and beading of their processes in the frontal, temporal, and parietal cortices in 5 of 7 FTD cases that also exhibited gliosis. This morphology was reproduced in cultured astrocytes using ischemic insults. Degenerating astrocytes in FTD correlated inversely with cerebral blood flow as measured by single photon emission computed tomography (SPECT) analysis of (133)Xe inhalation (r = 0.55, p < 0.05). Furthermore, areas of significant astrogliosis corresponded to areas of SPECT hypoperfusion, suggesting that astrocytes may be affected by or perhaps have a causal role in the disturbances of cerebral perfusion in FTD.