Generalized myoclonus as a prominent symptom in a patient with FTLD-TDP

摘要

Myoclonus is a hyperkinetic movement disorder characterized by sudden, brief, shock-Like, and involuntary jerks caused by muscular contractions or interruptions of tonic muscle activity [1]. It could be a part of clinical picture of several neurodegenerative disorders, especially in Creutz-feldt-Jakob disease (CJD), tauopathies (cortico-basal degeneration or progressive supranuclear palsy), Alzheimer's disease (AD) and in synucleinopathies, such as dementia with Lewy bodies [2]. We report on a patient who developed generalized asymmetric myoclonus associated with frontotemporal lobar degeneration with TDP-43 positive inclusions (FTLD-TDP). A 77-year-old man developed apathy and loss of initiative as the first and predominant signs of his disease in September 2010. In December 2010, hypophonia, dysarthria, and expressive aphasia developed gradually (language comprehension was not affected). In January 2011, cerebellar gait disorder and myoclonus of the upper extremities occurred that soon spread to the neck and eyelids. In February 2011, mutism and swallowing problems developed.