Cases reported "Dementia"

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1/5. An autopsy case of spinocerebellar ataxia type 6 with mental symptoms of schizophrenia and dementia.

    We herein report the findings of an autopsy case of spinocerebellar ataxia type 6 (SCA6) which revealed a mild CAG-repeat expansion in the alpha1A voltage-dependent calcium channel (CACNL1A4) gene on chromosome 19p13. A 39-year-old man who showed slowly progressive mental disorders and gait ataxia was clinically diagnosed to have cortical cerebellar atrophy (CCA) and schizophrenia. None of his relatives revealed any symptoms such as spinocerebellar disease, however, his younger brother had shown some mental disorders. The patient eventually died at 52 years of age, and an autopsy was thus performed. The main histopathological findings included a severe neuronal cell loss of purkinje cells and inferior olivary nuclei. The number of purkinje cells in our case had decreased severely in comparison to that in either OPCA or age-matched control cases, and the purkinje cells in the cerebellar hemisphere were more affected than those in the cerebellar vermis. The neurons of the dentate nucleus and pontine nuclei were well-preserved, and no pathological changes were seen in cerebral cortices or basal ganglia. The clinicopathological findings were similar to those of late cortical cerebellar atrophy (LCCA), Holmes' cortical cerebellar atrophy (Holmes type) or SCA6 cases reported previously. Using genomic dna extracted from archival paraffin-embedded sections in the frontal lobe, cerebral basal ganglia and cerebellum, the identical mild CAG-repeat expansions in the CACNL1A4/SCA6 gene were revealed in all samples examined. These findings suggest that in cases with LCCA or Holmes type atrophy, we should thus examine the CAG-repeat expansions in the SCA6 gene, and the genomic dna extracted from paraffin-embedded sections was thus found to be useful in diagnosing SCA6 retrospectively.
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keywords = mental disorder
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2/5. Selective serotonin reuptake inhibitors for aggressive behavior in patients with dementia after head injury.

    head injury of any severity can result in acute and chronic neuropsychiatric symptoms. After head injury, aggressive behaviors can be disabling to victims and stressful to their families. When aggression is compounded by dementia, treatment can be more difficult. Psychotropic agents can attenuate aggressive behaviors associated with mental disorders. Three patients with dementia and chronic aggression after head injury responded favorably to selective serotonin reuptake inhibitors.
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keywords = mental disorder
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3/5. The concept and nosology of Heller's syndrome: review of articles and report of two cases.

    Based on a review of 60 historical cases of dementia infantilis and its synonyms (i.e., Heller's disease, Heller's dementia, Heller's syndrome and disintegrative psychosis) and a report of 2 new cases, the concept and nosology of Heller's syndrome, the most appropriate generic name for such conditions, were studied. In the age of onset, sex ratio, symptoms and a favorable prognosis in terms of life, a group of etiologically diverse conditions called Heller's syndrome differs from adult dementias and appears classifiable in pervasive developmental disorders according to DSM-III or DSM-III-R, even though its validity as a clinical syndrome remains to be studied.
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keywords = mental disorder
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4/5. Clinicopathological study on a case of neuro-Behcet's disease: in special reference to MRI, SPECT and neuropathological findings.

    A case of neuro-Behcet's disease with dementia and personality changes is described with magnetic resonance imaging (MRI), single photon emission tomography (SPECT) and neuropathological findings. MRI disclosed high signal areas in the cerebral white matter and the brain stem. SPECT showed a marked reduction of blood flow in the frontal cortex. Neuropathologically, multifocal necrotizing lesions with perivascular lymphocytic infiltration and glial proliferation were detected mainly in the brain stem, namely the midbrain and the pons. From these findings, it is suggested that mental disorders of neuro-Behcet's disease are related to the secondary dysfunction of the frontal cortex due to the damage of the subcortical structures, mainly the brain stem.
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keywords = mental disorder
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5/5. Normal pressure hydrocephalus: psychiatric findings before and after shunt operation classified in a new diagnostic system for organic psychiatry.

    23 patients with normal pressure hydrocephalus (NPH) underwent psychiatric examinations prior to and 80 days to 10 months after a ventriculo-peritoneal shunt operation. A global evaluation of the effect of the operation on the patients' mental symptoms indicated appreciable improvement in 10 cases and slight improvement in a further 4.8 patients were assessed as unchanged, and one as mentally deteriorated. The psychiatric analyses was based on a new diagnostic system created by two of the authors (G.L., H.M.). The patients manifested varying, often complex psychiatric symptom constellations with symptomatological components from two or more organic mental disorders. Before the operation a mild or a moderately severe somnolence-sopor-coma disorder (SSCD) was diagnosed in 10 cases. After the operation all these patients became free from symptoms of SSCD. This was the most unequivocal change in connection with the operation, and the elimination of all symptoms of SSCD was the single factor which most effectively contributed to the total therapeutical result in these patients. All 23 cases were considered to have symptoms of a more or less severe astheno-emotional disorder (AED) preoperatively. The degree of severity of this disorder could not be determined with satisfactory certainty in some of the patients with complex symptoms. Amongst the 17 cases where the preoperative symptomatology allowed for a reasonably precise calculation of the degree of severity of AED, 6 were assessed as markedly improved after the operation and 10 as largely unchanged. In one patient, symptoms of the AED increased when the postoperative course was complicated by a subdural haematoma. Symptoms of an emotional-motivational blunting disorder (EMD) were diagnosed in 5 cases before the operation. After the operation 3 of these patients were symptom free in this respect while 2 unchanged. Slight or moderately severe symptoms of Korsakoff's amnestic disorder (KAD) were before the operation found in 7 cases: at the postoperative examination 6 of these cases were improved, of which 4 were free from such symptoms; one was unchanged. According to our experience, confident prognoses concerning the effect of the shunt operation on symptoms of SSCD can be made preoperatively, while, for a particular patient, the therapeutic effect on AED, EMD and KAD is often difficult, or sometimes impossible, to foresee. This article contains three case reports which represent different forms and courses of the mental symptom patterns.
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