摘要:

We evaluated four autopsy cases of dentatorubropallidoluysian atrophy (DRPLA) which had been diagnosed clinically as Huntington's chorea based on choreic movements, mental symptoms, and family history. These cases showed cerebellar ataxia at an early stage and developed choreic movements including a ballistic element. Pathological examination after autopsy revealed findings of DRPLA in all cases, although each case appeared to be in a different stage. Comparison of the clinical signs with the pathological findings suggests that the cerebellar ataxia observed clinically may have been related to pathological changes in the dentate nucleus of the cerebellum, and choreic movements may have been related to the pallidoluysian system, in particular, the nucleus of Luys. Biochemical analysis using frozen brain tissue revealed a decrease in γ‐aminobutyric acid (GABA) in the substantia nigra similar to that seen in Huntington's chorea in three cases. The findings of this study help to clarify the pathological processes that are responsible for the motor disturbances seen in patients with DRP

ISSN:0919-6544    

DOI:10.1111/j.1440-1789.1996.tb00156.x

出版商:Blackwell Publishing Ltd    

年代:1996

数据来源: WILEY

摘要:

Two brains with corticobasal degeneration (CBD) were examined in order to elucidate the immunocytochemical and ultrastructural characteristics of argyrophilic threads (AT) and oligodendroglial cytoskeletal alterations. Gallyaspositive oligodendroglial perikaryal coiled bodies reacted positively with antibodies against tau and ubiquitin immunohistochemically, and consisted of 15 to 20 nm tubules with occasional irregular constrictions ultrastructurally. The present study demonstrated, for the first time, that (i) the tubular structure of the non‐physiological nature actually aggregated in the inner and outer loops of the myelin sheath, which corresponded to the histological AT; (ii) these tubules were 15 to 20 nm in diameter with occasional irregular constrictions ultrastructurally, coated with silver particles in Gallyas electronmicroscopy, positive for anti‐tau, and weakly positive for anti‐ubiquitin antibodies immunocytochemically; and (iii) the plasma membrane of the inner and outer loops that surrounded these abnormal tubules of AT was immunolabeled by both anti‐tau and anti‐ubiquitin antibodies. Argyrophilic threads and oligodendroglial perikaryal coiled bodies share the immunohistochemical, immunocytochemical, and electronmicroscopical characteristics. Therefore, it is considered that the common tau‐pathology may develop in the oligodendroglia from the perikarya throughout its process. In conclusion, the cytoskeletal pathology in oligodendroglial process is of significance in the pathoetio

ISSN:0919-6544    

DOI:10.1111/j.1440-1789.1996.tb00157.x

出版商:Blackwell Publishing Ltd    

年代:1996

数据来源: WILEY

ISSN:0919-6544    

DOI:10.1111/j.1440-1789.1996.tb00158.x

出版商:Blackwell Publishing Ltd    

年代:1996

数据来源: WILEY

摘要:

Tau‐immunopositive and Gallyas preparation‐positive inclusions were observed in the oligodendroglial and astrocytic cells, in addition to extensive neurofibrillary tangles (NFT) in the brains of Guam parkinsonism‐dementia complex (Guam PDC) patients who had been autopsied from 1979 to 1982. Crescent‐ or coil‐shaped intracytoplasmic inclusions were observed in the oligodendroglial cells, predominantly in the medial nucleus of the thalamus, motor cortex, globus pallidus, pyramis of the medulla oblongata and lateral funiculus of the spinal cord. Granular hazy cytoplasmic inclusions were observed in the astrocytic cells many in the amygdaloid nucleus, in the inferior olivary nucleus, and in the lateral funiculus in the cervical segment of the spinal cord. These granular hazy inclusions have ever been reported and the distribution pattern of the crescentkoiled inclusions in Guam PDC is different from that reported previously in other NFT‐ or argyrophilic grain‐forming diseases. These findings indicate that Guam PDC involves not only neurons but also oligodendroglial and astrocytic cells. The morphological differences from the other NFT‐forming diseases may provide further insight into the distinct etiopathogene

ISSN:0919-6544    

DOI:10.1111/j.1440-1789.1996.tb00159.x

出版商:Blackwell Publishing Ltd    

年代:1996

数据来源: WILEY

ISSN:0919-6544    

DOI:10.1111/j.1440-1789.1996.tb00160.x

出版商:Blackwell Publishing Ltd    

年代:1996

数据来源: WILEY

ISSN:0919-6544    

DOI:10.1111/j.1440-1789.1996.tb00161.x

出版商:Blackwell Publishing Ltd    

年代:1996

数据来源: WILEY