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  • 1
    ISSN: 1432-0533
    Keywords: Key words Fukuyama-type congenital muscular ¶dystrophy ; Cerebellar degeneration ; Dentate nucleus ; Thalamus ; System degeneration
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract We report a male autopsy case of Fukuyama-type congenital muscular dystrophy (FCMD), with unusual neuropathological findings. The patient was a Japanese man aged 26 years at the time of death. He had shown severe psychomotor retardation and muscular dystrophy since early infancy, and was diagnosed as having FCMD at the age of 5 years. He died of respiratory failure. The main neuropathological finding was extensive cerebral and cerebellar cortical dysplasia, characteristic of this disorder. In addition, degeneration of the cerebellar efferent pathway, including the dentate nucleus, superior cerebellar peduncle, and red nucleus, and that of the lateral thalamic nucleus were observed. These findings suggest the possibility that the long survival can clarify the latent neurodegeneration in the cerebellum and thalamus in FCMD, in addition to congenital malformations. The system degeneration should be carefully evaluated in the pathological examination of this disorder.
    Type of Medium: Electronic Resource
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  • 2
    Electronic Resource
    Electronic Resource
    Springer
    Journal of hepato-biliary-pancreatic surgery 1 (1994), S. 222-228 
    ISSN: 1436-0691
    Keywords: abnormal pancreatico-choledocho-ductal junction ; common channel ; biliary tract carcinoma
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract The reliability of radiological diagnosis of abnormal pancreatico-choledocho-ductal junction (APCDJ) in comparison with findings of gross dissection of the pancreatic choledocho-ductal junction was studied in 8 patients, based on comparison between radiologic and macroscopic findings of the junction in 99 patients who had received pancreatoduodenectomy. APCDJ, that is the joining of the common bile duct and the pancreatic duct in the pancreatic parenchyma extraduodenally, was identified in 5 patients both radiologically and macroscopically. Macroscopically, the length of their common channel ranged from 20 to 32 mm. with a mean of 29 mm. In the other three patients, APCDJ was misdiagnosed radiologically, for the following reasons. In a patient with pancreatic carcinoma, a rather long common channel, 15 mm in length, while ran in the submucosal layer of the duodenum, was though to be APCDJ on radiologic examinations. In a patient with bile duct carcinoma, an abnormal closing between the common bile duct and the pancreatic duct due to carcinomatous infiltration of the pancreas led to a radiologication misinterpretation for APCDJ. In the last patient, APCDJ was identified only macroscopically, not radiologically, because of a relatively short common channel and the location of common bile duct carcinoma. Thus, APCDJ was clearly identified in patients with a long common channel, whereas it was difficult to identify in patients with a short common channel.
    Type of Medium: Electronic Resource
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