NR AWFH
AU Geschwind,M.D.; Haman,A.; Torres-Chae,C.; Raudabaugh,B.J.; Devereux,G.; Schardein,K.; Miller,B.L.
TI CSF findings in a United States sporadic CJD cohort
QU International Conference - Prion 2006: Strategies, advances and trends towards protection of society - 3.10.-6.10.2006, Torino, Italy, Lingotto Conference Centre - Poster sessions DIA-16
PT Konferenz-Poster
AB To determine the CSF profile and diagnostic sensitivity of CSF proteins in a large US sCJD cohort. As a major prion research center in the US, we have been referred more than 800 potential CJD cases over about the past five years. Data on these patients is stored in a clinical relational database. This database was queried for various CSF findings including cell count (n=132), protein (n=140), IgG index (n=34), oligoclonal bands (n=38), 14-3-3 (n=131), neuron specific enolase (NSE; n=33), total tau (t-tau; n=19) in patients with probable and definite sporadic CJD. T-tau was positive if >1200 pg/ml. NSE was positive if >20 ng/ml. 14-3-3 was measured primarily by western blot (NPDPSC, Cleveland, Ohio). 14-3-3 Diagnosis of probable CJD was based on modifications to WHO 1998 criteria, 2 of the following six sets of symptoms 1. Extrapyramidal/pyramidal 2. Cerebellar 3. Visual 4. Other focal cortical signs (e.g., aphasia, apraxia, neglect) 5. Myoclonus 6 Akinetic mutism AND a positive MRI or EEG. 14-3-3 was not used for diagnosis. CSF protein level was elevated in 42% of cases (range of 0-123, median 47 & mean 50 mg/dl). WBCs (>6 per HPF) and OCBs were each elevated in 8% of cases, while only 1 subject had an elevated IgG index. The 14-3-3 had a sensitivity of only 50% (47% for definite sCJD and 52% for probable sCJD). T-tau had a sensitivity of 68% (78%for definite and 60% for probable sCJD), while NSE was the most sensitive CSF marker at 73% (84% for definite and 71% for probable sCJD). Our results are in contrast to other published data that suggest high sensitivity of 14-3-3 and t-tau for sCJD. Interestingly, the only protein marker used in the WHO criteria, 14-3-3, had the lowest diagnostic sensitivity in our study. These proteins should be used with caution. The specificity of these markers is being evaluated in a cohort of non-prion rapidly progressive dementias.
AD Memory & Aging Center, Department of Neurology, University of California, San Francisco, USA. E-mail: mgeschwind@memory.ucsf.edu
SP englisch
PO Italien