1/44. Sporadic Creutzfeldt-Jakob disease presenting as major depression.We describe a 68-year-old white woman who initially had symptoms of major depression and was admitted to a psychiatric hospital where she had electroconvulsive therapy. With failure of psychiatric treatment and subsequent rapidly progressive dementia, she had left frontal brain biopsy. The biopsy revealed spongiform changes, the hallmark of Creutzfeldt-Jakob disease (CJD). We report a case of sporadic CJD with unusual initial presentation of psychiatric symptoms.- - - - - - - - - - ranking = 1keywords = white (Clic here for more details about this article) |
2/44. Creutzfeldt-Jakob disease: magnetic resonance imaging findings.Rapidly progressive dementia in an adult with findings of bilateral, symmetric high signal intensity on T2-weighted sequences and normal findings on T1-weighted sequences predominantly in the deep grey matter is suggestive of Creutzfeldt-Jakob disease (CJD). The peripheral cortex may be involved, as it was in the present case. The absence of subcortical periventricular white matter high signal intensity suggests that symmetric high signal intensities within the basal ganglia and cortical grey matter are more likely to be due to a degenerative process rather than due to ischaemia, infection or tumour.- - - - - - - - - - ranking = 1keywords = white (Clic here for more details about this article) |
3/44. 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 = white (Clic here for more details about this article) |
4/44. Amyotrophic form of Creutzfeldt-Jakob disease with rapid course in 82-year-old man.The authors present a case of Creutzfeldt-Jakob disease in 82-year-old man. Besides the onset of the disease in the elderly and short survival time (8 weeks), other uncommon clinical and morphological features also characterized our case. An evident amyotrophic syndrome, confirmed in morphological findings, developed soon after the CJD onset. The spongiform change also observed within the white matter of cerebral hemispheres allowed us to diagnose the 'panencephalopathic' form of CJD.- - - - - - - - - - ranking = 1keywords = white (Clic here for more details about this article) |
5/44. Infantile spongiform leukoencephalopathy: clinical and neuropathologic findings.A 10-month-old male with spongy leukoencephalopathy is presented. neurologic manifestations included feeding difficulties, horizontal nystagmus, and spasticity at 5 months of age. His head circumference was within the normal range. Radiologic examination demonstrated a diffuse white matter disorder. There was no detectable biochemical abnormality. He followed a neurologically progressive course. Neuropathologic findings revealed characteristic vacuolar changes in the white matter located immediately under the cortex with spongy alterations of the entire subcortical white matter, including intense astrocytic gliosis and marked vascular hyperplasia. Tissue of the matrix was destroyed in the deep white matter to form cystic areas of degeneration. White matter myelin development was severely disturbed compared with that of a normal infant of the same age. Cortical neuronal cells were preserved and did not reveal any specific abnormalities. Electron microscopic examination revealed that each vacuole in the white matter was covered by several layers of myelin structures, and intralamellar splits of white matter myelin were observed. These neuropathologic findings are also observed in some known inherent metabolic disorders. The present patient, however, did not demonstrate any metabolic abnormalities. These findings suggested a new genetic disorder of myelin metabolism.- - - - - - - - - - ranking = 6keywords = white (Clic here for more details about this article) |
6/44. MR spectroscopic pulvinar sign in a case of variant Creutzfeldt-Jakob disease.We report MR spectroscopic findings in a patient hospitalized with biopsy-proven variant Creutzfeldt-Jakob (vCJD) disease. N-acetyl aspartate was markedly decreased in the postero-medial part of the thalami (pulvinar) but was not diminished in the parieto-occipital white matter and cortical grey matter. These observations, which are in accordance with the pathological findings in this disease, suggest that MR spectroscopy, a highly sensitive method for the detection of subtle brain metabolic dysfunction, could be of interest for the diagnosis, prognosis and therapeutic follow-up of vCJD.- - - - - - - - - - ranking = 1keywords = white (Clic here for more details about this article) |
7/44. Creutzfeldt-Jakob disease with congophilic kuru plaques: CT and pathological findings of the cerebral white matter.In a patient whose Creutzfeldt-Jakob disease with congophilic kuru plaques that was proved at necropsy, the early brain CT showed low-density areas in the cerebral white matter before cortical atrophy and ventricular enlargement became apparent. Subsequently, there occurred diffuse white matter lucency and severe brain atrophy. At necropsy, there was severe white matter destruction which was more prominent than cortical neuronal loss. Serial CT scans were of great value for demonstrating the early and predominant changes in the cerebral white matter.- - - - - - - - - - ranking = 8keywords = white (Clic here for more details about this article) |
8/44. Coexistence of CJD and Alzheimer's disease: an autopsy case showing typical clinical features of CJD.The present report concerns an autopsy case of CJD showing typical clinical features of CJD. The patient was a Japanese woman without hereditary burden or dementing disorder anamnesis who was 70-years-old at the time of death. She developed gait disturbance at age 68, followed by memory impairment, visual disturbance, and myoclonus. A neurological examination approximately 2 months after the disease onset revealed akinetic mutism, in addition to periodic synchronous discharges on electroencephalogram. Serial neuroradiological examinations disclosed progressive atrophy of the brain. She died of bronchopneumonia 25 months after the disease onset. The brain weighed 560 g (cerebrum 490 g, brainstem with cerebellum 70 g). Macroscopically, neuropathological examination showed prominent atrophy of the cerebrum, caudate nucleus, and cerebellum, in addition to necrosis of the cerebral white matter, compatible with panencephalopathic CJD. Histologically, there was neuronal loss with or without spongiform change in the cerebral cortex, parahippocampal gyrus, amygdala, striatum, pallidum, thalamus, pontine nucleus, and cerebellar granule cells, in addition to diffuse synaptic-type prion staining in the cerebrum and cerebellum. Furthermore, senile plaques, compatible with definite Consortium to establish a registry for Alzheimer's disease rank Alzheimer's disease, and neurofibrillary changes of the limbic system, consistent with stage IV of Braak's classification, were found. Based on these clinicopathological findings and a review of the published literature, it is concluded that there were two forms of coexistence of CJD and Alzheimer's disease in the same patient.- - - - - - - - - - ranking = 1keywords = white (Clic here for more details about this article) |
9/44. CT appearance of panencephalopathic and ataxic type of Creutzfeldt-Jakob disease.We present a proven case of Creutzfeldt-Jakob disease with severe involvement of white matter and cerebellum. Serial CT showed a unique pattern consisting of discrete cerebral and cerebellar atrophy, diffuse white matter hypodensity, and progressive enlargement of lateral and fourth ventricles.- - - - - - - - - - ranking = 2keywords = white (Clic here for more details about this article) |
10/44. Creutzfeldt-Jakob disease with severe involvement of cerebral white matter and cerebellum.We describe a patient with Creutzfeldt-Jakob disease (CJD) of the ataxic and panencephalopathic type. Postmortem examination revealed the characteristic lesions of CJD in the grey matter and profound white matter involvement was seen with immunocytochemical techniques. Ultrastructural white matter lesions were identical to those described in experimentally transmitted CJD. There was marked loss of cerebellar granule cells with virtual disappearance of parallel fibres, but purkinje cells were only slightly reduced. Electron microscopic studies revealed extensive degenerative changes including cytoplasmic vacuoles in both cell types. silver methods disclosed massive impregnation of white matter and striking abnormalities of purkinje cells consisting of hypertrophy and flattening of thick dendritic branches, reduction in the number of terminal branchlets, segmentary loss of spines and polymorphic spines. These findings show the extensive involvement of all three cerebellar cortical layers and the reactive plasticity of purkinje cells to deafferentiation. They favour the hypothesis that demyelination represents a primary lesion of the white matter.- - - - - - - - - - ranking = 8keywords = white (Clic here for more details about this article) |
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