11/25. MM2-thalamic-type sporadic Creutzfeldt-Jakob disease with widespread neocortical pathology.We report an autopsy case of MM2-thalamic-type sporadic Creutzfeldt-Jakob disease (sCJD) with widespread cerebral neocortical pathology. Initial symptoms were progressive insomnia and mental disturbance. magnetic resonance imaging revealed no high-signal intensity lesions on diffusion-weighted images and later showed gradually progressive cerebral atrophy. Periodic synchronous discharges and myoclonus were not observed. Upon neuropathologic examination, widespread cerebral neocortical involvement with fine vacuole-type spongiform change was observed. Severe degeneration with almost complete neuronal loss, tissue rarefaction, numerous fat-laden macrophages and hypertrophic astrocytosis of the medial thalamic nucleus was evident. The inferior olivary nucleus showed severe involvement with neuronal loss and hypertrophic astrocytosis. In the cerebellar cortex, moderate depletion of Purkinje neurons was evident, with no spongiform change in the molecular layer and no neuronal loss in the granule cell layer. immunohistochemistry for prion protein (PrP) revealed widespread synaptic-type deposits with some primitive plaque-type deposits in the cerebral neocortex, basal ganglia and cerebellar cortex. PrP deposition was also observed in the brainstem, particularly the tegmentum, substantia nigra and pontine nucleus, and spinal cord, particularly the posterior horn. In the medial thalamus and inferior olivary nucleus, PrP deposition was sparse. Analysis of the PrP gene showed no mutation but did show methionine homozygosity at polymorphic codon 129. Western blot analysis of protease-resistant PrP indicated the presence of type 2 PrP. We believe that this patient suffered from MM2-thalamic-type sCJD (sporadic fatal insomnia) with widespread cerebral neocortical pathology due to prolonged disease duration. The present case showed different patterns of spongiform degeneration and PrP deposition in the cerebral neocortex than those in previously reported MM2-thalamic-type sCJD cases.- - - - - - - - - - ranking = 1keywords = nucleus (Clic here for more details about this article) |
12/25. The distribution of Alzheimer's neurofibrillary tangles and gliosis in chronic subacute sclerosing panencephalitis.In two cases of clinically verified chronic subacute sclerosing panencephalitis (case 1, male, 15 years with a 9-year history; case 2, male, 20 years with a 9-year history) numerous Alzheimer's tangles (AT) were identified throughout the cerebral cortex (containing paired helical filament on electron microscopical examination). The brains were severely atrophic with hydrocephalus ex vacuo, occasional scattered microglial nodules, scant perivascular inflammatory infiltrates and demyelination. Only in case 1 were a few atypical intranuclear inclusion bodies noted. In the six-layered neocortex, a distinct distribution pattern of AT was observed; these lesions were mainly seen in laminae II, III and V (laminar distribution). The glial fibrillary acidic protein stain displayed extensive laminar gliosis mainly of the layers I, IIa, IV and VI; layers III and V, largely occupied by the AT, remained conspicuously spared from gliosis (especially the lamina III). gliosis was prevalent in the white matter which was atrophic and shrunk. In the hippocampus, the AT involved many pyramidal neurons and, in this layer gliosis was lighter than in the surrounding white matter. In case 2, AT were present in the nucleus of Meynert, hypothalamus and in raphe centralis of the upper brain stem. overall, the distribution of AT resembled that seen in Alzheimer's disease and aging; however, the senile plaques, vascular amyloidosis and granulovacuolar change were totally absent in both cases.- - - - - - - - - - ranking = 0.25keywords = nucleus (Clic here for more details about this article) |
13/25. Degeneration of the substantia nigra in familial amyotrophic lateral sclerosis.We have studied a large family with familial amyotrophic lateral sclerosis (FALS) and examined the brain and spinal cord of a 40-year-old male family member at autopsy three years after clinical onset of the disease. The most unusual finding was a severe degree of neuronal loss of the substantia nigra accompanied by gliosis and numerous pigment-laden macrophages. There was marked degeneration of the upper and lower motor neurons and the corticospinal tracts. In addition, there was an unusually severe degree of diffuse degeneration throughout the anterolateral columns of the spinal cord. The posterior columns and Clarke's nucleus were not involved. Nigral degeneration has rarely been reported in FALS. The findings in this case emphasize the great variability of morphologic changes encountered in FALS and raise the question of a relationship between FALS and extrapyramidal disorders such as Parkinson's disease. We conclude that careful postmortem examinations of further cases of FALS are needed to fully define the extent of degenerative changes in this disease.- - - - - - - - - - ranking = 0.25keywords = nucleus (Clic here for more details about this article) |
14/25. Brainstem findings in Huntington's disease. Possible mechanisms for slow vertical saccades.Quantitative, morphometric studies of the rostral interstitial nucleus of the medial longitudinal fasciculus (riMLF) were performed on the brains of four patients with Huntington's disease (HD) who had shown slow vertical saccades, and on the brains of three control subjects. Only one HD brain showed a statistically significant decrease in the number of larger neurons in the riMLF though all four brains showed non-specific gliosis. Taken with results from physiological and other clinical studies, the present data suggest that slow vertical saccades in HD are due, at least in part, to disordered inputs to the riMLF.- - - - - - - - - - ranking = 0.25keywords = nucleus (Clic here for more details about this article) |
15/25. The rostral mesencephalon in Parkinson's disease and Alzheimer's disease.The rostal mesencephalon at the level of the posterior commissure was studied by light microscopy in two patients with idiopathic Parkinson's disease, one patient with Alzheimer's disease, and one patient with senile dementia of Alzheimer's type. In the Parkinsonian cases, the rostral part of the nucleus of Edinger Westphal disclosed lewy bodies in 3% of the neurons, neurofibrillary degeneration in 2% of the neurons, and a 54% neuronal loss. In Alzheimer's disease, 2% of Edinger Westphal neurons contained neurofibrillary degeneration, whereas in senile dementia of Alzheimer's type only rare neurofibrillary degeneration was evident in this nucleus. Neuronal loss was not apparent in the nucleus of Edinger Westphal in either of the Alzheimer's cases. The pathologic changes observed in this presumably cholinergic nucleus resemble in some respects changes reported in the cholinergic centers of the basal forebrain in these diseases. In addition, the central gray matter and pretectal region in Parkinson's disease contained a patchy increase in astroglia, some with scant reactive cell bodies; however, lewy bodies were limited to that part of the central gray matter corresponding to the nucleus of Darkschewitsch. A few neurofibrillary tangles were present in the nucleus of Darkschewitsch in both diseases.- - - - - - - - - - ranking = 1.5keywords = nucleus (Clic here for more details about this article) |
16/25. A case of progressive supranuclear palsy with fibrillary gliosis of the midbrain and pontine reticular formation.An autopsy case of progressive supranuclear palsy with onset at age 53 and a clinical course of 7 years is reported. The clinico-pathological characteristics of the case were as follows: Fibrillary gliosis of the midbrain and pontine reticular formation corresponded clinically to personality changes: The patient had formerly been irascible and became extremely mild-mannered. He showed lack of spontaneity and initiative and slowing of thought processes, taking him longer than normal to find words and ideas. gliosis of the nucleus ventralis lateralis of the thalamus was secondary to the degeneration of the cerebellar dentate nucleus and the globus pallidus from which they receive fibers. neurofibrillary tangles were found in the cerebral cortex.- - - - - - - - - - ranking = 0.5keywords = nucleus (Clic here for more details about this article) |
17/25. multiple system atrophy with neuronal intranuclear hyaline inclusions. Report of a new case with light and electron microscopic studies.An 18-year-old girl died following a slowly progressive neurodegenerative disease of nine years duration. At 9 years of age, she developed intellectual deterioration associated with speech difficulty, pseudobulbar palsy and ataxia. The progression included spastic quadriplegia, anarthria, severe dysphagia, ophthalmoplegia, and pes cavus. There was no family history. The brain was uniformly small and the substantia nigra was not pigmented. Neuronal loss and gliosis involving globus pallidus, subthalamic nucleus, thalamic nuclei, brain stem, cerebellum, and spinal cord gave the picture of multisystem atrophy. Intranuclear hyaline inclusions were observed in numerous neurons of the central and peripheral nervous system. These were auto-fluorescent and were made up of intermingled straight filaments (8-9 nm in diameter). Only two previously reported cases showing these same inclusions are known. They are reviewed, compared, and discussed in relation to primary neuronal degenerations.- - - - - - - - - - ranking = 5.5281581680883keywords = subthalamic nucleus, subthalamic, nucleus (Clic here for more details about this article) |
18/25. Neuropathological findings in a case of coexistent progressive supranuclear palsy and Alzheimer's disease.A 66-year-old man was investigated for increasing forgetfulness and frequent falls. Following appropriate investigations, the cause was presumed to be senile dementia of the Alzheimer type. He was reviewed one year later and found to have impaired vertical gaze and a hoarse voice. Progressive dementia, nuchal rigidity, anarthria, and sphincteric incontinence developed subsequently. A diagnosis of progressive supranuclear palsy was made. He died four years after the initial assessment. Neuropathological examination revealed changes characteristic of progressive supranuclear palsy, and suggestive of Alzheimer's disease. Globose tangles, granulovacuolar bodies and gliosis were present in the midbrain, the pons, the dentate nucleus of the cerebellum, and the globus pallidus. Senile plaques and occasional neurofibrillary tangles and granulovacuolar bodies were found in the hippocampus, amygdala, and temporal cortex. The coexistence of two disease processes resulting in dementia is discussed.- - - - - - - - - - ranking = 0.25keywords = nucleus (Clic here for more details about this article) |
19/25. The neuropathological features of neuroacanthocytosis.In this article we describe the neuropathological changes in three patients with neuroacanthocytosis and review the neuropathology of the other eight cases reported in the literature. Macroscopically the brains showed enlargement of the lateral ventricles, especially the frontal horns. The most severely and consistently affected brain areas were the caudate nucleus and putamen, which were atrophic and showed by light microscopy marked neuronal loss and gliosis. Small and medium-sized striatal neurons were particularly depleted. The globus pallidus was almost as severely involved as the striatum. In some cases the thalamus, substantia nigra, and anterior horns of the spinal cord showed pathology, mainly neuronal loss and mild gliosis. Brain areas with no pathology included the subthalamic nucleus, cerebral cortex, cerebellum, pons, and medulla. The preservation of these areas may help in the neuropathological distinction of neuroacanthocytosis from Huntington's disease.- - - - - - - - - - ranking = 5.7781581680883keywords = subthalamic nucleus, subthalamic, nucleus (Clic here for more details about this article) |
20/25. Pallidoluysian atrophy: dystonia and basal ganglia functional anatomy.The clinical correlates of "pure" pallidoluysian atrophy are not well described. A 59-year-old man presented with 20 years of progressive generalized dystonia, dysarthria, gait disorder, supranuclear vertical gaze palsy, and bradykinesia. At autopsy there was severe bilateral atrophy of the external pallidum and subthalamic nucleus with neuronal loss and marked gliosis. This syndrome may epitomize the consequences of "pure" pallidoluysian atrophy. In this case, dystonia appears to occur in the setting of decreased excitation (increased inhibition) of medial pallidal neurons, a pathophysiologic condition common to several hyperkinetic states.- - - - - - - - - - ranking = 5.5281581680883keywords = subthalamic nucleus, subthalamic, nucleus (Clic here for more details about this article) |
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