PedAM
Pediatric Disease Annotations & Medicines
| Disease | dementia |
| Phenotype | C0026884|mutism |
| Sentences | 2 |
| PubMedID- 23341815 | Sporadic creutzfeldt-jakob disease (scjd) is a prion disease presenting as rapidly progressive dementia that may lead to akinetic mutism [1, 2]. |
| PubMedID- 26089671 | According to the world health organization diagnostic criteria for probable diagnosis of cjd, the presence of at least one typical feature on the eeg and 14-3-3 positivity in csf samples or at least two criteria of myoclonus, visual disturbances, cerebellar, pyramidal, or extrapyramidal findings, and akinetic mutism, together with progressive dementia, are required.7 zerr et al found that 14-3-3 protein is 94% sensitive and 84% specific for cjd.8 steinhoff et al found that periodic biphasic or triphasic synchronized sharp wave complexes had 64% sensitivity and 91% specificity for eeg examination during the intermediate or terminal phase.9 therefore, although our patient presented with atypical abnormal facio-brachio-crural movement and hyponatremia, which was suggestive of anti-lgi1 encephalitis, he met the diagnostic criteria for cjd. |
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