Asymmetric Motor Disturbance and Hemineglect in Patients with Semantic Dementia Takuya YOSHIMURA 1 , Osamu YOKOTA 2,3 , Yoshikatsu FUJISAWA 1 , Hidenori YOSHIDA 3 , Seishi TERADA 3 , Shigetoshi KURODA 3,4 , Ken SASAKI 1 1Kinoko Espoir Hospital, Kasaoka, Japan 2Clinical Neuroscience Research Group, University of Manchester, Manchester, UK 3Department of Neuropsychiatry, Okayama University Graduate School of Medicine, Dentistry and Pharmaceutical Sciences 4Jikei Hospital Keyword: Semantic dementia , Pyramidal sign , Asymmetric parkinsonism , Hemineglect , TDP-43 pp.163-171
Published Date 2010/2/15
DOI https://doi.org/10.11477/mf.1405101572
  • Abstract
  • Look Inside
  • Reference
  • Cited by

 We report the two patients who presented with semantic dementia and subsequent asymmetric motor disturbance characterized by pyramidal signs and parkinsonism. The first patient initially developed semantic memory impairment with respect to word meanings, common objects, and faces at the age of 64 years. She subsequently exhibited oral tendency, altered eating behavior, and hypersexuality. Sixteen years after the onset asymmetric motor disturbance occurred in the left extremities. Neurological examination revealed a Babinski sign on the left foot, rigidity in the left extremities, and left-sided hemineglect. Computed tomography revealed right side-predominant severe temporofrontal atrophy and moderate atrophy in the right precentral gyrus and inferior parietal lobule. The second patient initially exhibited semantic deficits with respect to word meanings and common objects at age 39 years and developed motor disturbance in the right extremities 12 years later. Neurological examination revealed heightened tendon reflex and rigidity in the right extremities. Magnetic resonance imaging demonstrated severe left side-predominant temporo-frontal atrophy and severe atrophy in the basal ganglia. Recent clinicopathological studies indicate that both semantic dementia and asymmetric motor disturbances including pyramidal signs and parkinsonism may be predictors of sporadic frontotemporal lobar degeneration with TDP-43-positive inclusions (FTLD-TDP) rather than Pick's disease. Although the pathological basis of clinical FTLD patients is composed of various pathological disease entities including Alzheimer's disease with atypical cerebral atrophy, given the previous findings, at least sporadic FTLD-TDP, rather than Pick's disease, is a plausible pathology in the present cases. Clinicians should assess motor function in FTLD patients throughout the course carefully. Whether occurrence of hemineglect is associated with the specific pathological disease entity is unclear at preset.

Copyright © 2010, Igaku-Shoin Ltd. All rights reserved.


電子版ISSN 1882-126X 印刷版ISSN 0488-1281 医学書院