title: Giant visually-evoked potentials without myoclonus in the Heidenhain type of Creutzfeld-Jakob disease.
abstract: In a 64-year old woman with progressive visual impairment for 4 weeks, probable Creutzfeld-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 Creutzfeld-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 Creutzfeld-Jakob disease with atypical initial presentation.
authors.affiliation: Ludwig Boltzmann Institute for Research in Epilepsy and Neuromuscular Disorders, NKH Rosenhügel, Vienna, Austria. fij@2nr.nkr.magwien.gv.at
authors.forename: J
authors.initials: J
authors.lastname: Finsterer
authors.forename: C
authors.initials: C
authors.lastname: Bancher
authors.forename: B
authors.initials: B
authors.lastname: Mamoli
mesh_terms: D007562:Creutzfeldt-Jakob Syndrome / Q000175:diagnosis / Q000503:physiopathology*; D005074:Evoked Potentials, Visual / Q000502:physiology*; D005260:Female; D006801:Humans; D008875:Middle Aged; D009207:Myoclonus