1/19. Hippocampal sclerosis in a two-year-old with temporal lobe epilepsy: case report with pathological confirmation.The case of a 2-year-old girl who underwent anterior temporal lobectomy for intractable epilepsy is described. Hippocampal sclerosis (HS) was present on histopathological examination. We believe this to be one of the youngest reported patients with pathologically confirmed HS. This has implications for the possible etiology of HS.- - - - - - - - - - ranking = 1keywords = sclerosis (Clic here for more details about this article) |
2/19. Corpora amylacea replace the hippocampal pyramidal cell layer in a patient with temporal lobe epilepsy.A 39-year-old woman, with a 23-year history of medically intractable seizures of the complex partial type, underwent a left anterior temporal lobectomy and hippocampectomy. Histologic examinations revealed a massive occurrence of corpora amylacea, neuronal loss, and gliosis in the pyramidal cell layer of the hippocampus. The distribution of corpora amylacea in this case was quite distinctive and unlike the patterns of "nonspecific" corpora amylacea formation seen in aging or other neurodegenerative conditions. The pathogenetic aspects of the accumulation of these peculiar bodies are discussed in relation to hippocampal sclerosis.- - - - - - - - - - ranking = 0.2keywords = sclerosis (Clic here for more details about this article) |
3/19. Clasmatodendrosis correlating with periventricular hyperintensity in mixed dementia.We report a 79-year-old woman with possible Alzheimer's disease and confluent periventricular white matter hyperintensities on magnetic resonance imaging in whom postmortem analysis unexpectedly demonstrated no periventricular demyelination or cerebral arteriosclerosis. However, astrocytes in the periventricular white matter exhibited clasmatodendrosis, defined as cytoplasmic swelling and vacuolation of astroglia, with beading of their dendrites. This finding represents a previously unrecognized correlate of periventricular white matter hyperintensities. Ann Neurol 2002;52:378-381- - - - - - - - - - ranking = 0.2keywords = sclerosis (Clic here for more details about this article) |
4/19. Sporadic amyotrophic lateral sclerosis with circumscribed temporal atrophy: a report of an autopsy case without dementia and with ubiquitinated intraneuronal inclusions.This report concerns an autopsy case of amyotrophic lateral sclerosis (ALS) with circumscribed temporal atrophy. The patient was a Japanese woman without hereditary burden who was 71-year-old at the time of death. She developed dysarthria and gait disturbance at age 69, followed by dysphagia. A neurological examination about 1 year 11 months after the onset of the disease revealed absence of character change and of dementia. Neuroradiological examination disclosed circumscribed atrophy of the anterior part of the right temporal lobe. The patient died of respiratory failure 2 years after the disease onset. No respirator administration was performed throughout the clinical course. Macroscopically, neuropathological examination showed circumscribed atrophy of the right first temporal gyrus. Histologically, there was neuronal loss in the cerebral cortex, including the first temporal gyrus, the parahippocampal gyrus, subiculum, amygdala, substantia nigra, brain stem motor nuclei, and anterior horns of the spinal cord, in addition to loss of Betz cells, obvious degeneration of the pyramidal tracts, and the presence of Bunina bodies. ubiquitin-immunoreactive intraneuronal inclusions were present in the hippocampal dentate granular cells, frontotemporal cortical layer II neurons, and motor neurons in the brain stem and spinal cord. Based on these clinicopathological findings and a review of the literature, we concluded that our case was atypical ALS without dementia, showing temporal lobe atrophy macroscopically, in addition to pathological hallmarks compatible with ALS with dementia. We also note the possibility that there is a forme fruste of ALS with dementia showing no overt dementia clinically.- - - - - - - - - - ranking = 1keywords = sclerosis (Clic here for more details about this article) |
5/19. Neurofilament inclusion body disease with early onset frontotemporal dementia and primary lateral sclerosis.A small number of patients have recently been described with a sporadic neurodegenerative disease, associated with the neuropathological finding of neurofilament-immunoreactive neuronal inclusions. The clinical and pathological spectrum of this new disease entity has yet to be fully defined. We describe an additional case of "neurofilament inclusion body disease" (NIBD) with several unusual features. This young woman, who suffered from rapidly progressive frontotemporal dementia (FTD) and features of primary lateral sclerosis (PLS), died at age 29. Neuropathological examination disclosed numerous neuronal cytoplasmic inclusions in many regions of the central nervous system. The inclusions varied in morphology with some being immunoreactive for ubiquitin while others showed strong positivity for neurofilament proteins. Intranuclear neuronal inclusions were also present. There was no significant tau or alpha-synuclein pathology. There was severe degeneration of the corticospinal tracts but lower motor neurons were normal in number and morphology. This case confirms that NIBD should be considered in the differential diagnosis of FTD, particularly in young patients. In addition, it extends the clinical phenotype of NIBD to include PLS and better defines the anatomical distribution and morphology of the pathological lesions.- - - - - - - - - - ranking = 1keywords = sclerosis (Clic here for more details about this article) |
6/19. Thalamic dementia: an example of primary astroglial dystrophy of Seitelberger.Pure thalamic dementia is highly uncommon and typically demonstrates widespread loss of neurons throughout the thalamus associated with reactive gliosis. This report describes an autopsy case in which there is widespread gliosis of subcortical white matter, focal hippocampal sclerosis and a unique proliferation of protoplasmic astrocytes in the thalamus, with limited bilateral focal loss of neurons. The alterations of the protoplasmic astrocytes consist of proliferation of perivascular feet surrounding blood vessels and velate sheets which surround individual neurons. It is proposed that the astrocytic alterations, or astrocytic dystrophy, constitute the primary and critical pathologic-change, sufficiently severe to produce dementia in the presence of a relatively intact neuronal population.- - - - - - - - - - ranking = 0.2keywords = sclerosis (Clic here for more details about this article) |
7/19. 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 = 1keywords = sclerosis (Clic here for more details about this article) |
8/19. Supranuclear paralysis preventing lid closure in amyotrophic lateral sclerosis.We report a case of amyotrophic lateral sclerosis (ALS) in which the ability to close the eyes on command or voluntarily, was lost in spite of retention of reflex activity. A electrophysiological study of the blink reflex revealed a prominent R1 component with normal latency, which confirmed that the blink reflex was exactly preserved and also suggested a hemispherical lesion. Postmortem examination disclosed prominent cortical and subcortical lesions of the precentral areas on both sides. These lesions seem to be very closely related to the inability to initiate lid closing.- - - - - - - - - - ranking = 1keywords = sclerosis (Clic here for more details about this article) |
9/19. Computerized tomographic findings and differential diagnostic considerations in subcortical arteriosclerotic encephalopathy (Binswanger's disease).This paper discusses the conditions which may be associated with diffuse periventricular hypodensity on CT. Of these conditions, subacute arteriosclerotic encephalopathy (Binswanger's disease) is a common differential diagnostic consideration. This condition results from ischemic demyelination with loss of axons and astrogliosis. CT usually shows diffuse bilateral confluent hypodensities in the cerebral white matter. This is most prominent and seen earliest in the periventricular region and may extend more diffusely into the centrum semiovale. It is important to differentiate this condition from normal pressure hydrocephalus and primary nonischemic demyelinating disease (multiple sclerosis).- - - - - - - - - - ranking = 0.2keywords = sclerosis (Clic here for more details about this article) |
10/19. Focal cortical dysplasia on magnetic resonance imaging: a case report.A case report of an 11 years old boy with new onset of a seizure disorder is presented. A computed tomography scan demonstrated a noncalcified, nonenhancing focal region of abnormal cortex. A magnetic resonance imaging scan delineated both an isointense area of abnormally thickened gyri and linear areas of abnormal high signal intensity in the subjacent white matter. A review of the radiologic and pathologic literature suggests that this lesion represents the entity focal cortical dysplasia as described by Taylor, et al. This abnormality is part of a spectrum of disorders including hamartomas (of tuberous sclerosis), focal cortical dysplasia and heterotopias.- - - - - - - - - - ranking = 0.2keywords = sclerosis (Clic here for more details about this article) |
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