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J Neurol Sci. 2014 Nov 15;346(1-2):133-7. doi: 10.1016/j.jns.2014.08.009. Epub 2014 Aug 13.

Clinical features of Japanese patients with inclusion body myositis.

Author information

1
Department of Neurology, Graduate School of Medical Sciences, Kumamoto University, 1-1-1 Honjo, Kumamoto 860-8556, Japan.
2
Department of Neurology, Graduate School of Medical Sciences, Kumamoto University, 1-1-1 Honjo, Kumamoto 860-8556, Japan. Electronic address: y-stsh@kumamoto-u.ac.jp.

Abstract

BACKGROUND:

The incidence of sporadic inclusion body myositis (sIBM) has been much lower in Japanese than in Western populations. Because of a few reports on Asian populations, it is unclear whether the clinical characteristics of sIBM are identical in Caucasian and Japanese patients.

METHODS:

We compared 18 patients with sIBM, divided into 3 groups by age-of-onset, with previous cohort studies. We calculated the ΔIBM functional rating scale/time duration (ΔIBMFRS/Δtime) as an index of functional disability progression. Patients' electrophysiology was analyzed in relation to their clinical characteristics.

RESULTS:

The cohort was 83.3% male and showed uniform initial muscle weakness in the lower and/or upper limbs. An older age-at-onset was associated with a more rapid progression, and patients with a longer duration frequently showed F-wave abnormalities and findings of chronic denervation.

CONCLUSIONS:

The clinical characteristics of sIBM were relatively homogeneous beyond the ethnic differences. Aging might be a synergistic factor for the progression of sIBM pathology.

KEYWORDS:

Chronic denervation; Clinical characteristics; F-wave abnormality; Japanese cases; Motor neuron disease; Sporadic inclusion body myositis

PMID:
25152387
DOI:
10.1016/j.jns.2014.08.009
[Indexed for MEDLINE]

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