Rinsho Shinkeigaku (Clinical Neurology)

Case Report

A case of progressive ataxia and palatal tremor (PAPT) with ear clicks

Hiroyuki Sumikura, M.D.1), Tatsusada Okuno, M.D., Ph.D.1), Masanori P. Takahashi, M.D., Ph.D.1), Katsuya Araki, M.D.1), Kazuo Kitagawa, M.D., Ph.D.1) and Hideki Mochizuki, M.D., Ph.D.1)

1)Department of Neurology and Cerebrovascular Diseases, Osaka University Hospital

A 71-year-old man noted clicking sounds in the ear. At the age of 75, he developed progressive unsteadiness of gait and became unable to walk without assistance at the age of 76. There was no family history of neurologic illness. Neurological examination revealed truncal ataxia and 1-2 Hz rhythmic palatal tremor, which persisted during sleep. Consistently, brain magnetic resonance imaging showed mild cerebellar atrophy and increased signal intensity of bilateral inferior olivary nuclei on T2-weighted image. progressive ataxia and palatal tremor (PAPT) has recently been described as a rare sporadic neurodegenerative disease and the features of our case consistent with those of PAPT. However, for correct diagnosis of PAPT, multiple system atrophy, spinocerebellar ataxia, progressive supranuclear palsy or adult-onset Alexander's disease should be carefully ruled out.
Supplemental Video(Members Only)
Full Text of this Article in Japanese PDF (2027K)

(CLINICA NEUROL, 53: 224|228, 2013)
key words: palatal tremor, ataxia, PAPT, ear clicks

(Received: 22-May-12)