Biomedical subjects

E J Cochran

Publications and source records attributed to E J Cochran.

26 records · Page 2Linked to original sources

PubMed1996

Familial Creutzfeldt-Jakob disease with a five-repeat octapeptide insert mutation.

We report a familial form of Creutzfeldt-Jakob disease, associated with a unique insert mutation of the PRNP gene in an American family of Ukrainian origin. Ten family members exhibited early age at onset and long-duration illnesses characterized primarily by personality changes, cognitive impairment, and spasticity. The proband, presenting at age 42 years, exhibited a fairly stable, nonprogressive course over 7 years, followed by precipitous decline and death in the eighth year. Other affected family members exhibited marked clinical heterogeneity. Each tested affected member had an insert mutation consisting of five extra octapeptide repeats between codons 51 and 91 of the PRNP gene on chromosome 20. Examination of two autopsy cases showed classic spongiform change, neuronal loss and astrocytosis in one case, and minimal pathologic abnormality in the other case. This report documents a new insert mutation of the PRNP gene, and confirms the early age of onset, characteristically prolonged clinical course, and clinical and pathologic heterogeneity seen in such mutations.

Adult↗

PubMed1995

Autopsy practices at CERAD and Alzheimer disease center sites: a survey of neuropathologists.

A questionnaire, collecting information on methods used at neuropathology core facilities for handling of brain autopsies on dementia and control subjects, was sent to 29 CERAD (Consortium to Establish a Registry for Alzheimer's Disease) and/or Alzheimer's Disease Center (ADC) sites. Neuropathologists shared their successes and concerns in response to questions regarding administrative, technical, and neuropathological procedures for brain autopsies and tissue banking. Adequacy of fiscal support and staff, scarcity of control cases, and logistical aspects of autopsy coordination emerged as common problems. Successful strategies included the designation of dedicated autopsy coordinators, enhanced relationships with community hospital pathologists and funeral homes, and increased multilevel educational efforts on the importance of the autopsy.

Alzheimer Disease↗

PubMed1994

Cerebrospinal fluid from patients with Parkinson's disease alters the survival of dopamine neurons in mesencephalic culture.

We have previously demonstrated that extracts of striatal tissue from patients with Parkinson's disease (PD) increase the survival of dopamine neurons in mesencephalic cultures relative to striatal extracts from control patients. In the present study, ventricular cerebrospinal fluid (vCSF) from patients with PD, Alzheimer's disease (AD), and age-matched controls was similarly assessed. vCSF samples were separated into > 10-kDa and < 10-kDa fractions. Cultures incubated with the > 10-kDa fractions from PD and AD patients contained 73 and 13%, respectively, more tyrosine hydroxylase immunoreactive neurons than cultures incubated with vCSF from age-matched controls. This trophic activity was positively correlated with the trophic activity present in striatal extracts from the same patients. The < 10-kDa vCSF fractions from all patient groups inhibited culture growth. These data suggest that the trophic environment in the striatum is altered in PD and can be successfully monitored in CSF.

Aged↗

PubMed1994

Severe panencephalic Pick's disease with Alzheimer's disease-like neuropil threads and synaptophysin immunoreactivity.

We report a case of a 69-year-old woman with an 11-year history of progressive dementia, who was found at autopsy to have classic Pick's disease pathology, as well as extensive paired helical filament -- immunoreactive neurities, with few Alzheimer's disease (AD)-like neurofibrillary tangles and no senile plaques. Both Pick bodies and neuritic degeneration were extensive in the neocortex and hippocampal complex. In addition, synaptophysin immunostaining of the hippocampal complex showed a bilaminar pattern of immunoreactivity in the outer molecular layer as previously described in AD.

Alzheimer Disease↗

PubMed1982

Transient global amnesia after cerebral angiography. Report of seven cases.

Because of a case of typical transient global amnesia following cerebral angiography, the records of all patients undergoing cerebral angiography during a seven-year period were reviewed. Six other cases were discovered. All patients had either transfemoral catheter or brachial angiograms performed immediately before the development of amnesia. Spasm of a vertebral artery was noted in one case. None had clinically obvious seizures. All recovered within 24 hours. This only recently reported complication of cerebral angiography is consistent with a vascular etiology of the syndrome of transient global amnesia when not associated with angiography, although a direct toxic effect of the contrast material cannot be excluded.

Acute Disease↗

PubMedDate not supplied

Pathological changes in frontal cortex from biopsy to autopsy in Alzheimer's disease.

We evaluated the change in density of total senile plaques, plaque subtypes, and neurofibrillary tangles, from biopsy to autopsy in left frontal cortical sections from four patients with clinically typical Alzheimer's disease (AD). Comparisons were made on sections stained with modified Bielschowsky and Thioflavin S. In two cases, comparisons were also made on tissue stained with a monoclonal Alz-50 antibody and an antiserum to A beta (beta-amyloid protein). Despite a marked decline in mental status over several years of follow-up clinical evaluations, there was no consistent significant change in numerical density of plaques or tangles among the four cases. However, we did find fewer primitive plaques in the autopsy specimens. These results from longitudinally evaluated persons with typical AD suggest that although plaques and tangles may serve as adequate markers of the presence of AD, their numerical density within a single neocortical region may not reflect dementia severity. This conclusion supports the results of recent cross-sectional studies on the progression of pathology among persons with AD.

Alzheimer Disease↗

PubMedDate not supplied

Pathological glial tau accumulations in neurodegenerative disease: review and case report.

Abnormal deposits of tau protein accumulate in glia in many neurodegenerative diseases. This suggests that in some instances the disease process may target glial tau, with neuronal degeneration a secondary consequence of this process. In this report, we summarize the pattern of glial tau pathology in various neurodegenerative disorders and add original findings from a case of sporadic frontotemporal dementia that exhibits astrocytic tau pathology. The neurodegenerative diseases span the spectrum of relative neuronal and glial tau involvement, from disorders affecting only neuronal tau to those in which abnormal tau deposits are found only in glia. From this, we conclude that glial tau can be a primary target of the disease process, and that this can lead to neuronal degeneration.

Aged↗

PubMedDate not supplied

White matter changes: neurobehavioral manifestations of Binswanger's disease and clinical correlates in Alzheimer's disease.

Although white matter lesions (WMLs) are among the most common structural neuroimaging changes found on computed tomography and magnetic resonance imaging of older persons with dementia, their presence should not be misconstrued as proof that vascular disease is causing or contributing to the dementia. We report the results of several studies examining the neurobehavioral manifestations of persons meeting explicit operational criteria for Binswanger's disease (BD) and the clinical correlates of white matter changes in persons with autopsy-proven Alzheimer's disease (AD). The findings suggest that relative to persons with AD of comparable dementia severity, persons with BD have less profound impairments in episodic memory, more depressive symptomatology and a more variable rate of cognitive decline; among persons with AD, some WMLs are associated with incontinence and gait disturbance, but they do not appear to contribute to dementia severity.

Alzheimer Disease↗