[Dura mater related Creutzfeldt-Jakob disease in Japan: relationship between sites of grafts and clinical features].
نویسندگان
چکیده
A nation wide survey demonstrated 129 case of CJD with cadaveric dura mater grafts in Japan to February 2007. These patients were identified to have received Lyodura during period between 1978 and 1991. Incubation period from grafting to the onset of symptoms varied from 16 months to 24.5 years. We conducted a retrospective review of the full medical records of 107 of these patients. Patients were divided into two groups by site of neurosurgical or orthopedic procedures (supratentorial vs. infratentorial). Hemiparesis or hemianopsia developed as an initial manifestation in 31.9% of 47 patients with supratentorial grafts but did not develop among nay of the infratentorial group (p<0.0001). Conversely, brainstem symptoms (nystagmus, diplopia, ipsilateral hearing loss, facial paresis or paresthesia) were noted in 25.0% of the infratentorial group, but were not seen in the supratentorial group (p<0.0001).
منابع مشابه
Creutzfeldt-Jakob disease and lyophilised dura mater grafts: report of two cases.
Two further cases of Creutzfeldt-Jakob disease (CJD) in association with cadaveric dura mater grafts are described. The clinical features of all such reported cases resemble more closely those of sporadic CJD, in contrast with kuru and the cases of CJD which have arisen after therapy with human pituitary-derived growth hormone. This observation may reflect the route of inoculation of the agent.
متن کاملProspective 10-year surveillance of human prion diseases in Japan.
We analysed the epidemiological data and clinical features of patients with prion diseases that had been registered by the Creutzfeldt-Jakob Disease Surveillance Committee, Japan, over the past 10 years, since 1999. We obtained information on 1685 Japanese patients suspected as having prion diseases and judged that 1222 patients had prion diseases, consisting of definite (n=180, 14.7%) and prob...
متن کاملPanencephalopathic type of Creutzfeldt-Jakob disease associated with cadaveric dura mater graft.
A 52 year old man with Creutzfeldt-Jakob disease who received a cadaveric dura mater graft 99 months before the onset is reported. The prion protein gene was homozygous for methionine at the polymorphic codon 129. Neuropathological examination disclosed a panencephalopathic type of Creutzfeldt-Jakob disease which was characterised by severe involvement of the cerebral white matter and cerebellu...
متن کاملGraft-related disease progression in dura mater graft-associated Creutzfeldt-Jakob disease: a cross-sectional study
OBJECTIVES Details of abnormal prion protein (PrP(Sc)) propagation in the human central nervous system (CNS) are unclear. To assess the spread of PrP(Sc) through the human CNS, we evaluated dura mater graft-associated Creutzfeldt-Jakob disease (dCJD) cases focusing on sites of grafting and dCJD pathological subtypes. DESIGN A cross-sectional study. SETTING nationwide surveillance data of hu...
متن کاملUpdate: Dura Mater Graft–Associated Creutzfeldt-Jakob Disease — Japan, 1975–2017
Creutzfeldt-Jakob disease (CJD) is a fatal neurodegenerative disorder that, according to the most well accepted hypothesis (1), is caused by replicating, transmissible, abnormal forms of a host-encoded prion protein (prions). Most CJD cases occur spontaneously (sporadic CJD) or are inherited (genetic CJD). Iatrogenic CJD can occur after exposure to prion-contaminated instruments or products in ...
متن کاملذخیره در منابع من
با ذخیره ی این منبع در منابع من، دسترسی به آن را برای استفاده های بعدی آسان تر کنید
عنوان ژورنال:
- Nihon rinsho. Japanese journal of clinical medicine
دوره 65 8 شماره
صفحات -
تاریخ انتشار 2007