A case of early-onset cortical cerebellar atrophy with rhythmic skeletal myoclonus in the left upper limb

Ikuko Murakami, Takeshi Yamada, Hideo Hara, Takayuki Taniwaki, Masayuki Sasaki, Motohiro Kato, Jun Ichi Kira

Research output: Contribution to journalArticle

1 Citation (Scopus)

Abstract

A 19-year-old man developed progressive unsteady gait and speech disturbance two years ago. He recently noticed muscle twitches in the left limb. Neurological examination revealed scanning speech, saccadic ocular movement, ataxia of trunk and limbs predominant on the left side. Rhythmic myoclonus was present at rest around the left shoulder and arm, and amplified by raising the left arm. There was no myoclonus in the soft palate. MRI revealed only a cerebellar atrophy. This patient was diagnosed as having cortical cerebellar atrophy with rhythmic skeletal myoclonus (RSM). Tc-99m ECD SPECT showed a decrease in the blood flow of the right thalamus when the myoclonus was enhanced by raising the left upper limb, which suggests that the cerebello-thalamo-cortical system as well as Guillain-Mollaret triangle is involved in the development of RSM.

Original languageEnglish
Pages (from-to)754-757
Number of pages4
JournalClinical Neurology
Volume38
Issue number8
Publication statusPublished - Aug 1998

All Science Journal Classification (ASJC) codes

  • Clinical Neurology

Fingerprint Dive into the research topics of 'A case of early-onset cortical cerebellar atrophy with rhythmic skeletal myoclonus in the left upper limb'. Together they form a unique fingerprint.

  • Cite this

    Murakami, I., Yamada, T., Hara, H., Taniwaki, T., Sasaki, M., Kato, M., & Kira, J. I. (1998). A case of early-onset cortical cerebellar atrophy with rhythmic skeletal myoclonus in the left upper limb. Clinical Neurology, 38(8), 754-757.