Primary lateral sclerosis presenting parkinsonian symptoms without nigrostriatal involvement

N. Mabuchi, H. Watanabe, N. Atsuta, M. Hirayama, H. Ito, H. Fukatsu, T. Kato, K. Ito, G. Sobue

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24 Citations (Scopus)


We encountered three patients with primary lateral sclerosis (PLS) showing bradykinesia, frozen gait, and severe postural instability, as well as slowly progressive spinobulbar spasticity. Cranial magnetic resonance (MR) imaging showed precentral gyrus atrophy. Central motor conduction was markedly prolonged or failed to evoke a response. Positron emission tomography (PET) showed significant reduction of [18F]fluoro-2-deoxy-D-glucose uptake in the area of the precentral gyrus extending to the prefrontal, medial frontal, and cingulate areas. No abnormalities were seen in the nigrostriatal system with PET using [18F]fluorodopa or [11C]raclopride or with proton MR spectroscopy. Thus, widespread prefrontal, medial, and cingulate frontal lobe involvement can be associated with the parkinsonian symptoms in PLS.

Original languageEnglish
Pages (from-to)1768-1771
Number of pages4
JournalJournal of Neurology, Neurosurgery and Psychiatry
Issue number12
Publication statusPublished - 12-2004
Externally publishedYes

All Science Journal Classification (ASJC) codes

  • Surgery
  • Clinical Neurology
  • Psychiatry and Mental health


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