1/16. Creutzfeldt-Jakob disease (CJD) in a case of suspected chronic heavy metal poisoning.We describe a patient who died of suspected heavy metal poisoning after a nine-month history of rapidly worsening dementia. autopsy at a forensic-pathological institute established the postmortem diagnosis of sporadic Creutzfeldt-Jakob disease (CJD) based on demonstration of the proteinase-resistant prion protein (PrPsSc) in Western-Blot on native brain tissue. Microscopic examination of the macroscopically largely inconspicuous brain revealed marked spongiform changes in the gray matter--mainly affecting the cerebral cortex, nucleus caudatus, and putamen--with confluent vacuoles. Patchy or perivacuolar deposits of PrPSc were found as well as granular PrPsc deposits. The cerebellum contained focal PrPsc deposits. There was an astrogliosis in the white matter and a proliferation of microglia in the gray matter with a simultaneous clear reduction in neuronal elements. The differential diagnosis is discussed, as is the potential risk to those performing autopsy on forensic cases with a clinical picture of rapidly progressing dementia, especially in cases where a prion disease is not initially suspected.- - - - - - - - - - ranking = 1keywords = putamen (Clic here for more details about this article) |
2/16. PET findings and neuropsychological deficits in a case of Fahr's disease.In a case of Fahr's disease with frontal lobe type dementia and hyperkinetic-hypotone syndrome, functional changes were investigated using positron emission tomography (PET) with (18)F-fluorodeoxyglucose (FDG) as a tracer. Computed tomography showed bilateral calcifications in the putamen and globus pallidus consistent with the diagnosis of Fahr's disease and a frontally pronounced brain atrophy. In contrast, reduced glucose uptake in PET was not only confined to the areas mentioned above, but extended to the temporal and parietal cortices, bilaterally. These functional changes corresponded to the neuropsychological deficits observed, i.e. disturbed selective attention and cognitive flexibility, verbal perseverations, and declarative memory deficits. It is suggested that functional changes may precede cerebral atrophy in Fahr's disease and may reflect deficits in functional circuits, which involve both the basal ganglia and the frontal, parietal, and temporal lobes.- - - - - - - - - - ranking = 1keywords = putamen (Clic here for more details about this article) |
3/16. frontotemporal dementia with cerebral intraneuronal ubiquitin-positive inclusions but lacking lower motor neuron involvement.A case of frontotemporal dementia with cerebral intraneuronal ubiquitin-positive, tau- and alpha-synuclein-negative inclusions is reported. A 50-year-old female patient exhibited mental changes; however, no clinical evidence of motor neuron disease was detected in her 11-year history. Neuronal loss and spongiform changes were mainly found in the frontotemporal cortices. Degeneration of the pyramidal tract was observed. Depletion of Betz cells was observed, whereas motor neurons of the hypoglossal nuclei and spinal anterior horn were well preserved. Immunohistochemically, intraneuronal ubiquitin-positive, tau and alpha-synuclein -negative inclusions were present in the small neurons of the dentate gyrus, frontal cortices and putamen. Neither Betz cells nor the anterior horn cells contained any inclusions. Fragmentation of the golgi apparatus was visible only in 2.2% of anterior horn cells. A large number of tau-positive glial structures lacking argyrophilia were seen in the area of the frontopontine tract in the cerebral peduncle. The pyramidal tract lesions of the present case may be based on frontal lobe degeneration with spread of lesions to the motor cortex. Except for the pyramidal tract lesions, our case is similar to cases of motor neuron disease-inclusion dementia.- - - - - - - - - - ranking = 1keywords = putamen (Clic here for more details about this article) |
4/16. Rapidly progressive autosomal dominant parkinsonism and dementia with pallido-ponto-nigral degeneration.We describe a family with nearly 300 members over 8 generations with 32 affected individuals who have an autosomal dominant neurodegenerative disease characterized by progressive parkinsonism with dystonia unrelated to medications, dementia, ocular motility abnormalities, pyramidal tract dysfunction, frontal lobe release signs, perseverative vocalizations, and urinary incontinence. The course is exceptionally aggressive; symptom onset and death consistently occur in the fifth decade. Positron emission tomographic studies with [18F]6-fluoro-L-dopa (6FD) were performed in 4 patients and 7 individuals at risk for development of the disease. All affected subjects had markedly reduced striatal uptake of 6FD (p less than 0.001). All individuals at risk had normal striatal uptake, but high 6FD uptake rate constants were noted in 3 of the 7 studied. autopsy findings revealed severe neuronal loss with gliosis in substantia nigra, pontine tegmentum, and globus pallidus, with less involvement of the caudate and the putamen. There were no plaques, tangles, lewy bodies, or amyloid bodies. This kindred appears to represent a neurodegenerative disease not heretofore described. We propose the following name for this new genetic disease: autosomal dominant parkinsonism and dementia with pallido-ponto-nigral degeneration.- - - - - - - - - - ranking = 1keywords = putamen (Clic here for more details about this article) |
5/16. Diffuse cerebral white matter T2-weighted hyperintensity: a new finding of general paresis.General paresis (parenchymatous neurosyphilis) is a rare disease, and in recent years the number of papers published on the magnetic resonance imaging findings has been limited. The findings are as follows: cerebral atrophy; mesiotemporal T2 hyperintensity; ventriculomegaly; pathological T2 hypointensity of the globus pallidus, putamen, the head of the caudate nucleus and thalamus. We present a new finding, diffuse cerebral white matter T2 hyperintensity, observed in a patient with general paresis with a 5-year history of progressive dementia.- - - - - - - - - - ranking = 1keywords = putamen (Clic here for more details about this article) |
6/16. ubiquitin-positive frontotemporal lobar degeneration presenting with progressive Gogi (word-meaning) aphasia. A neuropsychological, radiological and pathological evaluation of a Japanese semantic dementia patient.A patient with progressive anomia and alexia with agraphia for kanji (Japanese morphograms) is described. The patient showed a deficit in single-word comprehension and on-reading (a type of reading that conveys phonetic value) dominance in kanji reading, i.e. on-preceding (pronouncing first with on-reading, irrespective of its preferred reading) and kun-deletion (inability to recall and recognize kun-reading [another type of reading that conveys meaning]) when reading a single-character kanji. These features were due to loss of lexico-semantic information and thus the patient was regarded as having progressive Gogi (word-meaning) aphasia by Imura, a Japanese manifestation of semantic dementia. Macroscopically, neuropathological examination disclosed atrophy of the left frontotemporal lobe with accentuation in the anterior portion of the temporal lobe. Histologically, there was neuronal loss in the cerebral cortex, hippocampus, parahippocampal gyrus, amygdala, caudate nucleus, and putamen. ubiquitin-immunoreactive neuronal inclusions were present in the hippocampal dentate granular cells. This case demonstrates that progressive Gogi aphasia is semiologically identical to semantic dementia, and our patient clinicopathologically resembled those of Rossor et al. [Rossor, M.N., Revesz, T., Lantos, P.L., Warrington, E.K. Semantic dementia with ubiquitin-positive tau-negative inclusion bodies. brain 2000; 123: 267-76.] and Hodges et al. [Hodges, J.R., Davies, R.R., Xuereb, J.H., Casey, B., Broe, M., Bak, T.H., et al. Clinicopathological correlates in frontotemporal dementia. Ann Neurol 2004; 56: 399-406.].- - - - - - - - - - ranking = 1keywords = putamen (Clic here for more details about this article) |
7/16. Sporadic onset Creutzfeldt-Jacob disease: interesting MRI observations.We describe a 60-year-old woman with "probable" sporadic Creutzfeldt-Jacob disease (CJD) who manifested with two months history of rapidly progressive dementia and abnormal behavior, speech and gait abnormality, excessive sleepiness and myoclonic jerks. scalp EEG showed diffuse slowing of background activity to delta range and triphasic sharp wave complexes occurring periodically twice in one-second interval. magnetic resonance imaging (MRI) of brain revealed high signal intensity on T2 weighted image (T2WI) and fluid attenuated inversion recovery sequences in the caudate and putamen bilaterally. diffusion weighted images showed bilateral symmetric hyperintense signals in the caudate and putamen. The role of MRI in the diagnosis of CJD is discussed.- - - - - - - - - - ranking = 2keywords = putamen (Clic here for more details about this article) |
8/16. Membranous lipodystrophy: MR imaging appearance of the brain.Five patients with membranous lipodystrophy (lipomembranous polycystic osteodysplasia with progressive dementia) underwent magnetic resonance (MR) imaging of the brain. T2-weighted MR images showed atrophied cerebral white matter with dilated ventricles; increased signal intensity of the white matter; and decreased signal intensity of the thalamus, putamen, caudate nucleus, and cerebral cortex. Although each single finding is not specific, the combination of the above MR findings when coupled with skeletal lesions strongly suggests this rare disease.- - - - - - - - - - ranking = 1keywords = putamen (Clic here for more details about this article) |
9/16. Down's syndrome in middle age. Topographical distribution and immunoreactivity of brain lesions in an autopsied patient.A 45-year-old patient with Down's syndrome was autopsied. The brain, weighing 800 g, was small in size, and serial sections revealed generalized gyral atrophy and ventricular dilatation. In the gray matter, there was diffuse neuronal degeneration characterized by numerous neurofibrillary tangles (NFTs), senile plaques and frequent amyloid angiopathy. Histochemical and electron microscopical analyses of these lesions showed no qualitative difference from those in Alzheimer's disease. A topographical study of NFTs showed that they were numerous in the limbic system and cerebral neocortex. Various numbers of NFTs were seen in the olfactory bulb, thalamus, medial geniculate body, innominate substance, putamen, caudate, pallidum, central gray, reticular formation, certain midline nuclei of the brainstem, substantia nigra, red nucleus and dorsal vagal nucleus. This distribution pattern was not different qualitatively from that in Alzheimer's disease, and such a similarity was especially evident in the olfactory bulb, where many tufted and mitral cells as well as anterior olfactory nucleus cells showed NFTs. These common features of brain pathology in Down's syndrome and Alzheimer's disease may be due to a specific gene defect in both diseases.- - - - - - - - - - ranking = 1keywords = putamen (Clic here for more details about this article) |
10/16. Topography of Pick body distribution in Pick's disease: a contribution to understanding the relationship between Pick's and Alzheimer's diseases.A case of Pick's disease with the onset at 51 years old and a 15-year clinical course was studied. The brain, weighing 740 g, showed striking atrophy limited to the frontal and temporal lobes and inferior parietal lobules of both sides. The topographic distribution of Pick bodies (PBs) was investigated; PBs were present most densely in the amygdaloid, hippocampus, innominate substance, posterior cingulate and insula, inferior parietal lobule, posterior inferior temporal gyrus, and fusiform and lingular gyri. They were present less densely in the anterior frontal and temporal gyri, occipital gyri, caudate, hypothalamus, claustrum, putamen, pallidum, and olfactory bulbs and tubercles. Their presence was sparse in the pre- and post central gyri and superior parietal lobule. The calcarine and cerebellum were spared. The tectum and central grey of the midbrain, red nuclei, substantia nigra, locus ceruleus, superior central nuclei, tegmental reticular nuclei, pontine nuclei, dorsal vagal nuclei, and arcuate nuclei, were also severely affected. This distribution pattern of PBs was very similar to that of Alzheimer's neurofibrillary tangles in Alzheimer's disease, except for the pontine and arcuate nuclei. Electron microscopy of PBs disclosed two component fibrils: smooth-surfaced straight tubular filaments with a diameter of 15nm /- 3 and no periodic constrictions, and long-period (160nm) constricted fibrils. The morphology of these two fibrils of PBs, respectively resembled the straight filaments and paired helical filaments in Alzheimer's neurofibrillary tangles.- - - - - - - - - - ranking = 1keywords = putamen (Clic here for more details about this article) |
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