Numerous Neurofibrillary Tangles Research Articles

Long survival in Fukuyama congenital muscular dystrophy: occurrence of neurofibrillary tangles in the nucleus basalis of Meynert and locus ceruleus.

Three patients with Fukuyama congenital muscular dystrophy (FCMD), who died aged 23, 29 and 34 years, are reported. There was extensive brain malformation, but the most severe pattern of cortical dysplasia was absent in one case and in the other two localized bilaterally to small areas near the occipital poles. In two cases, numerous neurofibrillary tangles were observed in the locus ceruleus and nucleus basalis of Meynert. Electron microscopy revealed paired helical filaments, with a maximal width of about 25 nm and regular constrictions at approximately 80 nm intervals. The occurrence of neurofibrillary tangles, which is unknown in younger patients, suggests the presence of degenerative processes in the brains of the older patients with FCMD.

PATHOLOGICAL EVIDENCE FOR NEUROTRANSMITTER DEFICITS IN DOWN'S SYNDROME OF MIDDLE AGE

The number of nerve cells was counted and the volume of their nucleolus measured in the nucleus basalis of Meynert, the locus caeruleus, the dorsal motor vagus, the dorsal tegmental nucleus and the substantia nigra of seven patients with Down's syndrome, six of whom were over 50 years of age and showed within their cerebral cortex and hippocampus numerous senile plaques and neurofibrillary tangles. When compared with mentally-able age matched controls the youngest patient with Down's syndrome showed no substantial alterations in either number or nucleolar volume in any of these cell types. The six middle-aged patients showed significant loss of cells from nucleus basalis, locus caeruleus, dorsal motor vagus and dorsal tegmental nucleus but no loss from substantia nigra; nucleolar volume was, however, reduced in all five cell types. A similar pattern of change was seen in the six patients with Alzheimer's disease at that age. It is calculated that in both Down's syndrome and Alzheimer's disease of middle age there are similar degrees of damage to cholinergic, noradrenergic and serotonin neurotransmitter systems.

Reduced somatostatin-like immunoreactivity in cerebral cortex from cases of Alzheimer disease and Alzheimer senile dementa.

Both Alzheimer's disease and senile dementia of the Alzheimer type (AD/SDAT) are progressive dementias characterized neuropathologically by the presence in the cerebral cortex of numerous neurofibrillary tangles and neuritic plaques. We use the abbreviation AD/SDAT to denote all such cases, irrespective of age of onset. Studies of neurotransmitter-related parameters in autopsied brain tissues from patients with AD/SDAT have, to date, been confined to five putative transmitter systems. Acetycholine-releasing neurones seem to be most markedly and consistently affected, as judged by the extensive reductions in choline acetyltransferase (ChAT) and acetylcholinesterase activities that have been reported. Despite numerous studies, there is no consistent evidence for the involvement of neurones releasing dopamine, noradrenaline, serotonin, or gamma-aminobutyric acid in AD/SDAT, nor for loss of muscarinic cholinergic receptors. Thus, the involvement of cholinergic neurones in AD/SDAT seems to be specific. However, the possible involvement of neurones using other chemicals as transmitters has yet to be explored. The recent recognition of the existence of so-called 'peptidergic neurones' in the mammalian brain (for review see ref. 8) and the availability of radioimmunoassay (RIA) techniques for studying these peptides, have led us to begin a systematic investigation of neuropeptides in autopsied brain tissue from cases of AD/SDAT, and from neurologically normal individuals. We report here results obtained with a RIA for somatostatin, showing that somatostatin-like immunoreactivity in the cerebral cortex is reduced in tissue from AD/SDAT patients.

Huntington disease associated with Alzheimer disease.

The clinicopathological study of a case of Huntington disease with early severe dementia is presented. The pathological findings were those of Huntington disease (atrophy with neuronal loss and astrocytosis in the neostriatum) and Alzheimer disease (neuronal loss with numerous senile plaques and neurofibrillary tangles in the neocortex). Ultrastructural study of neurofibrillary tangles showed that they were composed of twisted tubules similar to those found in Alzheimer disease.

Motor neurone disease with neurofibrillary tangles in a Brazilian woman

The occurrence of neurofibrillary tangles in both the cerebral cortex and brain stem is typically seen in the Guam type of amyotrophic lateral sclerosis, but is exceedingly rare in the classical form of the disease. Only 3 cases of sporadic amyotrophic lateral sclerosis with such histopathologic features have so far been reported, all in the United States. A 49-year-old Brazilian woman had an 18-month history of amyotrophic lateral sclerosis involving predominantly the left-sided extremities, with prominent bulbar signs. Autopsy disclosed moderate to severe loss of motor neurones in the hypoglossal nuclei and anterior spinal horns, absence of pyramidal tract demyelination, depigmentation of the substantia nigra and numerous neurofibrillary tangles in the hypothalamic region, parahippocampal gyrus, reticular substance of the mesencephalon and pons and in some brain stem nuclei. The topographical distribution of these changes was closely similar to that of Guamanian amyotrophic lateral sclerosis.

Contribution to clinical pathology of progressive supranuclear palsy (subcortical argyrophilic dystrophy): On the distribution of neurofibrillary tangles in the basal ganglia and brain-stem and its clinical significance

A new case of progressive supranuclear palsy is reported, and the pathological findings in 2 cases of this disease are compared. We consider the marked accentuation of argyrophilic dystrophy (neurofibrillary tangles) in the basal ganglia and the brain-stem to be the basic lesion of this disease and in our view atrophy is a secondary phenomenon. The presence of numerous neurofibrillary tangles in the brain-stem reticular formation is considered to be responsible for dementia of a mild or moderate degree, often accompanied by night delirium and confusion.