The striatonigral and olivopontocerebellar systems are known to be vulnerable in multiple system atrophy (MSA), showing neuronal loss, astrogliosis, and alpha-synuclein-immunoreactive inclusions. MSA patients who displayed abundant neuronal cytoplasmic inclusions (NCIs) in the regions other than the striatonigral or olivopontocerebellar system have occasionally been diagnosed with variants of MSA. In this study, we report clinical and pathologic findings of MSA patients characterized by prominent pathologic involvement of the hippocampus. We assessed 146 consecutively autopsied MSA patients. Semiquantitative analysis of anti-alpha-synuclein immunohistochemistry revealed that 12 of 146 patients (8.2%) had severe NCIs in two or more of the following areas: the hippocampal granule cells, cornu ammonis areas, parahippocampal gyrus, and amygdala. In contrast, the remaining 134 patients did not show
AIMTo investigate factors, including psychosocial factors, associated with alcoholic use relapse after liver transplantation (LT) for alcoholic liver disease (ALD).METHODSThe clinical records of 102 patients with ALD who were referred to Nagoya University Hospital for LT between May 2003 and March 2015 were retrospectively evaluated. History of alcohol intake was obtained from their clinical records and scored according to the High-Risk Alcoholism Relapse scale, which includes duration of heavy drinking, types and amount of alcohol usually consumed, and previous inpatient treatment history for alcoholism. All patients were assessed for eligibility for LT according to comprehensive criteria, including Child-Pugh score, Model for End-Stage Liver Disease score, and psychosocial criteria.RESULTSOf the 102 patients with ALD referred for LT, seven (6.9%) underwent LT. One (14.3%) of these seven patients returned to heavy drinking, but that patient was able to successfully quit drinking following an immediate intervention, consisting of psychotherapeutic education and supportive psychotherapy, by a psychiatrist. A comparison between the transplantation/registration (T/R) group, consisting of the seven patients who underwent LT and 10 patients listed for deceased donor LT, and 50 patients who did not undergo LT and were not listed for deceased donor LT (non-T/R group), showed statistically significant differences in duration of abstinence period (P < 0.01), duration of heavy drinking (P < 0.05), adherence to medical treatment (P < 0.01), and declaration of abstinence (P < 0.05).CONCLUSIONPatients with ALD referred for LT require comprehensive evaluation, including evaluation of psychosocial criteria, to prevent alcoholic recidivism.
Globular glial tauopathy (GGT) is a recently proposed tauopathy characterized by the globular accumulation of four-repeat (4R) tau in the oligodendroglia (globular oligodendroglial inclusion (GOI)) and astrocytes (globular astrocytic inclusion (GAI)), in addition to deposition in neurons. Although it is proposed that GGT should be classified into three different neuropathological subtypes, previous reports have indicated that subclassification might be difficult in some cases. We report an autopy case of a 79-year-old man with behavioral variant frontotemporal dementia (bvFTD). He developed behavioral changes at 67 years of age and had auditory hallucinations and persecutory delusions at admission to a psychiatric hospital at 69 years of age. Neuropathologically, marked atrophy of the frontotemporal lobes and severe degeneration of the white matter and frontopontine tract were observed. The present case corresponded to GGT Type I, as numerous GOIs were observed, predominantly in the frontotemporal region. However, concurrent degeneration of the motor cortex and corticospinal tract suggest characteristics of Type II. Although the relationship between psychotic symptoms and GGT remains unclear, the present case demonstrates heterogeneity of GGT subtypes.
Huntington's disease (HD) is an autosomal‐dominant neurodegenerative disorder characterized by the presence of chorea, psychiatric symptoms, and dementia. Although motor symptoms are thought to be correlated with the degeneration of the striatum, there is little information regarding the neuropathological basis of psychiatric symptoms. The ventral part of the striatum is known as the nucleus accumbens (Acb) and is a region of interest as a responsible focus of psychiatric symptoms. The purpose of this study was to investigate the neuronal changes in the Acb and its relevance to psychiatric symptoms in HD. We investigated the brains of 16 HD patients (three patients presented psychiatric symptoms as the onset phenotype (HD‐P), 13 patients presented motor symptoms as the onset phenotype (HD‐M)) and four control subjects. The numerical cell densities for each of the large and small striatal neurons in the Acb, caudate nucleus and putamen were measured at three levels from the caudal to rostral region. As the result, the median small neuronal densities in all striatal regions in the HD brains were significantly lower than controls. Regarding the median small neuronal density in the caudate nucleus and putamen among the three levels, there were significant differences in the HD brains, but not in controls. The median large neuronal density in the Acb was significantly higher in the HD‐P than in the HD‐M, but there was no difference in the median small neuronal density between them. In the present study, we revealed that the Acb as well as the caudate nucleus were affected in HD brains. In terms of neuronal size and caudal to rostral levels, non‐uniform neurodegeneration was observed in the striatum of the HD brains. The pathological difference in the Acb between HD‐P and HD‐M may be one of the factors involved in the development of psychiatric symptoms.
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