Biomedical subjects

C M Hulette

Publications and source records attributed to C M Hulette.

42 records · Page 3Linked to original sources

PubMedDate not supplied

Gliomatosis cerebri: cytologic and autopsy findings in a case involving the entire neuraxis.

We describe the case of a 7-year-old girl who was clinically diagnosed as having a pontine glioma based on magnetic resonance imaging studies. Neoplastic cells were identified upon cytologic examination of cerebrospinal fluid. Autopsy studies revealed an anaplastic astrocytoma (WHO grade III) diffusely infiltrating the cerebral hemispheres, brain stem, cerebellum, leptomeninges, and spinal cord to the level of the conus medullaris. The Ki-67 labeling index focally approached 30%. Although many of the neoplastic cells displayed elongated twisted nuclei reminiscent of microglia, these cells stained intensely for glial fibrillary acidic protein, supporting an astrocytic origin. Unusual features of this case of gliomatosis cerebri include involvement of the entire central neuraxis, correlation with pre-mortem lumbar puncture cytology, and a markedly elevated Ki-67 labeling index.

Astrocytoma↗

PubMedDate not supplied

Adult onset Niemann-Pick disease type C presenting with dementia and absent organomegaly.

A 39-year-old female presented to the Bryan Memory Disorders Clinic at Duke University with a 7-year history of an atypical progressive dementia, mildly impaired vertical gaze, dysarthria and mild ataxia. There was no evidence of organomegaly by clinical examination or by radionuclide liver/spleen scan. Brain biopsy disclosed a neuronal storage disorder characterized by ballooned neurons filled with oligo-lamellar cytosomes and lipid droplets. Cultured skin fibroblasts had diminished sphingomyelinase activity and impaired cholesterol esterification, although peripheral leukocyte sphingomyelinase activity was normal. Two years after biopsy, follow-up examination revealed marked progression of vertical gaze paralysis and ataxia. This case expands the clinical spectrum of Niemann-Pick disease type C by presenting in adulthood with subtle neurologic abnormalities; no visceromegaly and profound dementia.

Adult↗

PubMedDate not supplied

Lobar atrophy without Pick bodies.

Four patients from the Kathleen Price Bryan Brain Bank with clinical Pick's syndrome are presented. Thorough neurological evaluation revealed no evidence of a movement disorder. The brains showed marked knife-blade type atrophy of the frontal and temporal lobes with relative sparing of the superior temporal gyrus and parietal and occipital lobes. There was marked caudate atrophy in all four. Histologically there was severe neuronal loss and gemistocytic astrocytosis in the involved areas with marked myelin pallor in the deep white matter and subcortical gliosis. There was sometimes marked spongiform change in cortical layer 2. There was severe neuronal loss and gliosis of the caudate nucleus. The gross and microscopic features were characteristic of Pick's disease except that careful search failed to uncover either Pick's bodies or Pick's cells. Review of the literature revealed that fronto-temporal cortical and caudate atrophy with clinical features of Pick's disease has received many different names including Pick's disease type C, Pick's disease type II, progressive subcortical gliosis, presenile glial dystrophy, long duration Creutzfeldt-Jakob disease, frontal lobe degeneration, dysphasic dementia, and dementia lacking distinctive histologic features. Nevertheless, the morphologic findings in the present cases so closely resemble Pick's disease that they may well represent endstage Pick's disease. In our experience, such cases account for a significant proportion of non-Alzheimer disease dementia.

Aged↗

PubMedDate not supplied

Rasmussen's syndrome: a study of potential viral etiology.

Rasmussen's syndrome (RS) is a devastating constellation of severe, unilateral focal motor epilepsy resistant to anticonvulsant therapy, followed by ipsilateral neurological deficits and neuropathological evidence of a chronic encephalitis occurring primarily in the pediatric population. Recent reports have identified viral genomic material for cytomegalovirus (CMV) and Epstein-Barr virus (EBV) using in situ hybridization (ISH) in brain tissue of RS patients. We studied 10 biopsy- and resection-specimens from seven patients using biotinylated double-stranded DNA probes to CMV, herpes simplex virus (HSV) and EBV to confirm these results. Two patient samples were also evaluated by electron microscopy and one using standard immunoperoxidase techniques. We were unable to identify any evidence of viral material utilizing one of these techniques. There was some nonspecific localization of crystalline material by in situ hybridization over nuclei, which may account for literature reports of positivity, although one cannot be sure since photomicrographs were not included in these reports. Although the neuropathological morphology of the identifiable lesions in resected specimens is consistent with that seen in other viral encephalitides, our findings fail to support the role of CMV, HSV, or EBV as the etiology and sole factor in the development of Rasmussen's syndrome.

Child↗

PubMedDate not supplied

An infant with macrocephaly, abnormal neuronal migration and persistent olfactory ventricles.

An asphyxiated male infant was delivered by cesarean section at 38 weeks gestation. The patient was macrocephalic with clinical evidence of fetal immobility syndrome. He died aged two days. Autopsy revealed excessive brain weight, pachygyria, enlarged olfactory tracts and hypoplastic optic nerves. Microscopy revealed evidence of impaired neuronal migration throughout the cortex. The olfactory bulbs showed persistence of the primitive olfactory ventricles and absence of olfactory glomeruli. There was persistence of the fetal connection between lateral and olfactory ventricles. The lateral geniculate nuclei, inferior olivary nuclei and dentate nuclei of the cerebellum were abnormally formed. Relevant aspects of fetal brain development and clinical neuropathologic syndromes are discussed.

Abnormalities, Multiple↗

PubMedDate not supplied

Pathologic assessment of cerebellar atrophy following acute lithium intoxication.

Lithium carbonate is a widely used pharmacologic agent for acute bipolar disorder, long term prophylaxis of mania in a bipolar patient, and prevention of "manic overshoot" with an antidepressant in acute depression in a bipolar patient. Although clinical neurological associations with lithium overdose have been-established, there has been a dearth of reports of pathologic changes related to lithium toxicity. We report a case of a 52-year-old Black female with bipolar disorder who had been treated with lithium for over 5 years and who expired 24 days after presenting in a stuporous state with an elevated lithium level of 3.2 mEq/l. Postmortem neuropathologic examination revealed severe cerebellar atrophy of the internal granule and Purkinje cell layers with attendant Bergmann gliosis presumably resulting from chronic lithium use and toxicity. There was also Alzheimer type II cell change in the thalamus and lentiform nuclei possibly due to terminal uremia. In summary, this is a unique case which appears to illustrate cerebellar atrophic changes related to lithium therapy and acute lithium intoxication.

Acute Disease↗