Neuropathology : official journal of the Japanese Society of Neuropathology
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Case Reports
Chronic superior sagittal sinus thrombosis with phlebosclerotic changes of the subarachnoid and intracerebral veins.
An autopsy case of chronic thrombotic occlusion of the superior sagittal sinus (SSS) with an unusually protracted clinical course is reported. The patient was an 84-year-old man without any predisposing conditions for thrombosis. ⋯ The hemorrhagic infarction was comprised of the aggregation of numerous minute foci of coagulation necrosis associated with petechial hemorrhages. Marked phlebosclerotic changes were observed in the subarachnoid and intracerebral veins, which were considered to be reactive changes of the venous walls against a persistent elevation in peripheral venous pressure caused by thrombosis of the SSS.
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Three cases of Wernicke's encephalopathy in nonalcoholic patients diagnosed by postmortem examination were reported to improve the recognition of this disease. All three cases were male, ages ranged from 33 to 73 years old. All the cases had a clinical history of malnutrition but no history of chronic alcoholism. ⋯ Neuronal losses were found only in the medial nucleus of the thalamus and inferior olive, myelin staining demonstrated demyelination and gliosis in those areas. The diagnosis of Wernicke's encephalopathy was made. In combination with the reviewed literature, our cases suggest that Wernicke's encephalopathy can occur not only in patients with alcohol abuse, but also in those who have suffered thiamine deficiency due to metabolic and nutritional disorders.
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A 9-year-old Japanese girl received a cadaveric dura mater graft during surgery following a head injury with brain contusion. She continued to do well, but when she became 19-years-old, she gradually showed a violent character and was treated in a psychiatric hospital. Another 6 years later, 200 months after the procedure, she developed a progressive gait ataxia, which subsequently led to her death within 10 months of onset. ⋯ However, our detection of an asymmetrical increase in the MRI-derived images of pulvinar nuclei has not been previously observed in other atypical cases of dCJD. Because atypical dCJD cases share several clinicopathological features with those of vCJD, and because asymmetrical hyperintense signals in the pulvinar have been observed in some neuropathologically confirmed vCJD cases, we had some difficulty in a differential diagnosis between atypical dCJD and vCJD. This is the first atypical dCJD case showing a pulvinar high signal compared with all other basal ganglia on MRI.
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Biography Historical Article
In memory of Dr Hirotsugu Shiraki (1917-2004).
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Amyotrophic lateral sclerosis with dementia (ALS-D) is a non-Alzheimer-type dementia characterized by both frontotemporal degeneration and motor neuron disease and marked by ubiquitin-positive, tau- and alpha-synuclein-negative intraneuronal inclusions and dystrophic neurites. New neuropathological diagnostic criteria for ALS-D are proposed on the basis of the present investigation of 28 autopsy cases. Clinical features included those of typical ALS-D, primary lateral sclerosis, atypical ALS with frontotemporal atrophy and atypical Pick's disease without Pick's bodies. ⋯ The distribution pattern and density differed between neuronal inclusions and dystrophic neurites and correlated with clinicopathological phenotypes. Therefore, the ALS-D spectrum may be broader than that previously recognized, extending to primary lateral sclerosis, atypical ALS and to atypical Pick's disease without Pick bodies. Further investigation is needed to determine the characteristics of the ubiquitinated component in ALS-D.