caa a22 4500 445807059 CHVBK 20180317145159.0 cr unu---uuuuu 170323e20110101xx s 000 0 eng 10.1007/s00415-010-5694-1 doi (NATIONALLICENCE)springer-10.1007/s00415-010-5694-1 Pruritus in familial Creutzfeldt-Jakob disease: a common symptom associated with central nervous system pathology [Elektronische Daten] [Oren Cohen, Joab Chapman, Hedok Lee, Zeev Nitsan, Shmuel Appel, Chen Hoffman, Hanna Rosenmann, Amos Korczyn, Isak Prohovnik] Pruritus, a common feature of animal prion diseases such as scrapie, is rarely reported in humans with Creutzfeldt-Jakob disease (CJD), and its anatomical background is not well defined. The present study was undertaken to carry out a methodical prospective search for the prevalence of pruritus in CJD patients and investigate its anatomical substrate by MRI. The study group included consecutive familial and sporadic CJD patients carrying the E200K PRNP mutation followed up in a longitudinal prospective study between the years 2005 and 2008. Pruritus was prospectively screened for and diffusion-weighted imaging (DWI) was used to correlate brain diffusion abnormalities with pruritus in CJD patients. Pruritus was present in 6/31 (19.35%) patients with familial disease (fCJD) and in none of the patients with sporadic disease (sCJD). Pruritus was a presenting symptom in one patient and evolved during the course of the disease in the other five patients. The pruritus was generalized in three patients, regional in two and localized in one patient. It was transient in one patient and continued throughout the disease in five patients. DWI showed that pruritus was significantly associated with reduced diffusion in the several areas known to be affected by CJD, but most significantly in the midbrain periaqueductal grey matter. Pruritus is relatively common in patients with familial CJD carrying the E200K mutation. Our findings point to a central origin that involves damage to the inhibitory gating mechanism for itch in the periaqueductal grey matter. Springer-Verlag, 2010 Creutzfeldt-Jakob disease nationallicence Prion nationallicence Pruritus nationallicence Periaqueductal grey matter nationallicence Cohen Oren Department of Neurology, The Sagol Center for Neuroscience, Chaim Sheba Medical Center, 52621, Tel-Hashomer, Israel aut Chapman Joab Department of Neurology, The Sagol Center for Neuroscience, Chaim Sheba Medical Center, 52621, Tel-Hashomer, Israel aut Lee Hedok Departments of Psychiatry and Radiology, Mount Sinai Medical Center, New York, NY, USA aut Nitsan Zeev Department of Neurology, The Sagol Center for Neuroscience, Chaim Sheba Medical Center, 52621, Tel-Hashomer, Israel aut Appel Shmuel Department of Neurology, The Sagol Center for Neuroscience, Chaim Sheba Medical Center, 52621, Tel-Hashomer, Israel aut Hoffman Chen Department of Radiology, Chaim Sheba Medical Center, Tel-Hashomer, Israel aut Rosenmann Hanna Hadassah University Hospital, Jerusalem, Israel aut Korczyn Amos Sieratzki Chair of Neurology, Tel Aviv University, Tel-Aviv, Israel aut Prohovnik Isak Departments of Psychiatry and Radiology, Mount Sinai Medical Center, New York, NY, USA aut Journal of Neurology Springer-Verlag 258/1(2011-01-01), 89-95 0340-5354 258:1<89 2011 258 415 https://doi.org/10.1007/s00415-010-5694-1 text/html Onlinezugriff via DOI 1 research-article jats NATIONALLICENCE 856 40 https://doi.org/10.1007/s00415-010-5694-1 text/html Onlinezugriff via DOI NATIONALLICENCE 700 1- Cohen Oren Department of Neurology, The Sagol Center for Neuroscience, Chaim Sheba Medical Center, 52621, Tel-Hashomer, Israel aut NATIONALLICENCE 700 1- Chapman Joab Department of Neurology, The Sagol Center for Neuroscience, Chaim Sheba Medical Center, 52621, Tel-Hashomer, Israel aut NATIONALLICENCE 700 1- Lee Hedok Departments of Psychiatry and Radiology, Mount Sinai Medical Center, New York, NY, USA aut NATIONALLICENCE 700 1- Nitsan Zeev Department of Neurology, The Sagol Center for Neuroscience, Chaim Sheba Medical Center, 52621, Tel-Hashomer, Israel aut NATIONALLICENCE 700 1- Appel Shmuel Department of Neurology, The Sagol Center for Neuroscience, Chaim Sheba Medical Center, 52621, Tel-Hashomer, Israel aut NATIONALLICENCE 700 1- Hoffman Chen Department of Radiology, Chaim Sheba Medical Center, Tel-Hashomer, Israel aut NATIONALLICENCE 700 1- Rosenmann Hanna Hadassah University Hospital, Jerusalem, Israel aut NATIONALLICENCE 700 1- Korczyn Amos Sieratzki Chair of Neurology, Tel Aviv University, Tel-Aviv, Israel aut NATIONALLICENCE 700 1- Prohovnik Isak Departments of Psychiatry and Radiology, Mount Sinai Medical Center, New York, NY, USA aut NATIONALLICENCE 773 0- Journal of Neurology Springer-Verlag 258/1(2011-01-01), 89-95 0340-5354 258:1<89 2011 258 415 Metadata rights reserved Springer special CC-BY-NC licence nationallicence BK010053 XK010053 XK010000 NATIONALLICENCE NATIONALLICENCE NL-springer