Creutzfeldt-Jakob disease in an adolescent

Progressive Neurological Deterioration in a 14-Year-Old Girl

“…This is the youngest case of diagnosed CreutzfeldtJakob disease on record. Previously, the youngest reported case was that of a 16-year-old boy [11]. Most cases present in middle or old age, as do patients with Gerstmann-Straussler-Scheinker disease [21].…”

“…This illness usually has a fairly rapid course, death occurring within weeks or months following ini tial presentation, but a 2-year course is certainly possi ble. SSE is usually a disease of middle or late life, but rare instances of onset in the 2nd or 3rd decade of life have been reported [10,11], Unlike SSPE and progres sive rubella panencephalities, this illness resists defini tive laboratory confirmation. CSF examination is usually normal, and abnormalities which may occur on CT, EEG and other tests are not specific, although peri odic brief paroxysmal discharges are noted on EEG in most patients [12].…”

“…At the end stage there may be only widespread neuron loss and gliosis, as reported in some cases of 'Alpers' disease'. Diagnosis of spongiform encephalopathy must rely on awareness, supplemented where possible by transmission to labora tory animals [11].…”

Clinical and laboratory features of 14 young Chinese probable sCJD patients

“…Despite being rare, young sCJD cases have been repeatedly reported since 1980s. [12][13][14] An large-scale study of the CJD surveillance from 1970 to 1996 in UK has proposed 25 (3.8%) young sCJD patients who died under 40 year-old out of 658 cases. 15 A prospective study based on the national mortality data from 1979 to 1994 in USA has illustrated 41 young patients (1.13%) below 40 year-old out of 3642 CJD cases.…”

Epidemiologic implications of Creutzfeldt-Jakob disease in a 19 year-old girl

“…Epidemiologic investigation of the present case excluded a familial component, and provided no evidence for iatrogenic or natural case-to-case transmission, or of other environmental sources of viral contamination. Young patients such as this one serve to emphasize the obscurity that still sourrounds the epidemiology of CJD, and invite serious reconsideration of the possibilities of transmission by undetected virus carriers, or of the agent as a natural resident of human cells, replication of which might be triggered by non-infective (e.g., traumatic or mutational) environmental events.Creutzfeldt-Jakob disease ( C J D ) i s u n u s u a l before the age of 30 years, and e x t r e m e l y r a r e in adolescence (2, 21,27,28,29). We r e p o r t here an o t h e r w i s e typical ,CJD illness in .a 19 year-old girl, with special e m p h a s i s on the epide.miological aspects of a c o m p a r a t i v e l y short and uncomglicated life story.…”