According to the World Health Organization (WHO), the following are the criteria for definite sCJD[30]
Diagnosed by standard neuropathologic techniques
and/or immunocytochemically
and/or Western blot-confirmed protease-resistant prion protein
and/or presence of scrapie-associated fibrils
Criteria for probable sCJD are as follows:
Progressive dementia
At least two of the following four symptoms:
Myoclonus
Pyramidal/extrapyramidal symptoms
Visual or cerebellar symptoms
Akinetic mutism
Positive EEG (periodic epileptiform discharges) findings and/or a positive 14-3-3 protein result and < 2-year disease duration
No alternative diagnosis suggested
Criteria for possible sCJD are as follows:
Progressive dementia
At least two of the following four symptoms:
Myoclonus
Pyramidal/extrapyramidal symptoms
Visual or cerebellar symptoms
Akinetic mutism
No supportive EEG findings
Researchers believe that DW-MRI should be appropriately considered among the diagnostic tests because it is noninvasive and has higher sensitivity and specificity than biopsy.[23,24,25,26,27,28,29]
Treatment of prion diseases remains supportive; no specific therapy has been shown to stop the progression of these diseases. Antidementia drugs should be avoided. Pentosan polysulfate, flupirtine, and doxycycline are being investigated for treatment of CJD. The prognosis is dismal; it is an invariably fatal disease, and approximately 90% patients die within 1 year of symptom onset.[31]
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Any views expressed above are the author's own and do not necessarily reflect the views of WebMD or Medscape.
Cite this: Sumaira Nabi, Sadaf Fayyaz, Shahzad Ahmed. A 49-Year-Old Man With Forgetfulness and Gait Impairment - Medscape - Jun 27, 2017.
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