Progressive Neurological Deterioration in a 14-Year-Old Girl

Berman, Peter H.; Davidson, George S.; Becker, Laurence E.

doi:10.1159/000120361

Cited by 15 publications

(8 citation statements)

References 30 publications

(37 reference statements)

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“…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.…”

Section: Commentsmentioning

confidence: 99%

Clinical and laboratory features of 14 young Chinese probable sCJD patients

Shi

Xiao

Cao

et al. 2017

Prion

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ABSTRACT. Sporadic Creutzfeldt-Jakob disease (sCJD) occurs frequently in the relatively older population, mainly in the groups of 60-69 and 70-79 year-old. Since 2006 when China performed national CJD surveillance, 14 young probable sCJD patients below 40 year-old were identified, counting for 1.93% of all probable sCJD cases. The clinical features of young probable sCJD cases, including the onset feature, the presence of sCJD-associated signs and the clinical duration, are indistinguishable from those of older patients. Special sCJD-associated abnormalities on EEG and MRI were noticed in 7 and 10 cases. CSF 14-3-3 was positive in 7 cases. CSF RT-QuIC showed positive reactive curves in 9 cases, with short lag phases. PRNP sequencing did not find any mutation. Due to low rate of brain autopsy in China, performances of other CJD-associated examinations as much as possible are extremely important for the distinguish diagnosis of young probable sCJD patients.

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Section: Commentsmentioning

confidence: 99%

Clinical and laboratory features of 14 young Chinese probable sCJD patients

Shi

Xiao

Cao

et al. 2017

Prion

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“…Numerous eponymous clinical variants of sporadic CJD have long been recognized (6, 59, 74), although recent prospective CJD surveillance studies have found that the boundaries between these clinical subgroups are frequently ill-defined (77,82). Most cases of CJD occur in adults aged 60-65, although it has been recognized for many years that a wide age range can be affected, from teenagers to patients in their ninth decade (10,16,19,53,64,67,82). The UK CJD Surveillance Project has found in recent years an apparently increasing number of CJD cases in individuals aged 75 and over, which appears to be a consequence of increased diagnostic vigilance in the elderly (76,82).…”

Section: Introductionmentioning

confidence: 99%

Review: Creutzfeldt‐Jakob Disease

Ironside¹

1996

Brain Pathology

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The clinicopathological spectrum of Creutzfeldt-Jakob disease has recently been extended by the identification of an apparently new variant of this disorder in the UK. The hypothesis that this new disorder is causally related to the transmissible agent responsible for bovine spongiform encephalopathy has prompted re-evaluation of the relationships between human and animal transmissible spongiform encephalopathies, and reinforced the need for adequate experimental models to investigate this field. Recent experimental transmission studies of bovine spongiform encephalopathy into primates have supported the hypothesis, and reinforced the central role of neuropathology in the investigation of this group of diseases. Whilst clearly of distinctive etiology, Creutzfeldt-Jakob disease shares both clinical and neuropathological features with other human dementias; renewed evaluation of potentially similar disease mechanisms in these devastating conditions might provide new information of both scientific and therapeutic value.

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“…Five cases of sCJD in teenagers have been reported previously from the USA,3 France,4 Canada,5 Poland6 and Germany 7. The characteristics of all seven cases are summarised in table 1.…”

Section: Discussionmentioning

confidence: 99%

“…A young age at death may also occur in kuru, iatrogenic CJD and genetic forms of human prion disease 2. However, there have previously been five case reports of sCJD in teenagers, dying at or younger than age 20 years, from the USA,3 France,4 Canada,5 Poland6 and Germany 7. Therefore, young age cannot be regarded as diagnostic of vCJD.…”

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confidence: 99%