NR AEAU
AU Finsterer,J.; Bancher,C.; Mamoli,B.
TI Giant visually-evoked potentials without myoclonus in the Heidenhain type of Creutzfeldt-Jakob disease
QU Journal of the Neurological Sciences 1999 Aug 1; 167(1): 73-5
PT journal article
AB In a 64-year old woman with progressive visual impairment for 4 weeks, probable Creutzfeldt-Jakob disease without myoclonus was diagnosed after rapidly progressive mental deterioration had also developed, and CSF and EEG showed characteristic findings. Pattern-reversal and flash visually-evoked potentials, recorded 5, 6, 7 and 8 weeks after onset, showed a maximum P100 latency of 210 ms, 8 weeks after onset, and a maximum N75/P100 amplitude of 33.1 microV, 5 weeks after onset. While the P100 latency progressively increased, the N75/P100 amplitude continuously decreased after reaching its maximum. In the Heidenhain type of Creutzfeldt-Jakob disease giant visually-evoked potentials may be recorded during the early stages of the disease, even in the absence of myoclonus. Visually-evoked potentials may prove useful in diagnosing Creutzfeldt-Jakob disease with atypical initial presentation.
MH Case Report; Creutzfeldt-Jakob Syndrome/diagnosis/*physiopathology; Evoked Potentials, Visual/*physiology; Female; Human; Middle Age; Myoclonus
AD Ludwig Boltzmann Institute for Research in Epilepsy and Neuromuscular Disorders, NKH Rosenhugel, Vienna, Austria. fij@2nr.nkr.magwien.gv.at
SP englisch
PO Niederlande