Cytoplasmic body myopathy masquerading as motor neuron disease.

Abstract:

:Cytoplasmic body myopathy (CBM) is characterized by proteinaceous inclusion bodies in muscle tissue. A 43-year-old woman presented with rapidly progressive weakness and dysphagia. Electromyography (EMG) elsewhere demonstrated lower-limb chronic partial denervation. Muscle biopsy showed fiber size variation without diagnostic features. A diagnosis of possible motor neuron disease was made and the patient was commenced on riluzole. Subsequently, the patient's condition stabilized, prompting reassessment. Repeat EMG demonstrated no features of denervation and was more suggestive of a myopathic process. Review of the original muscle biopsy showed cytoplasmic bodies. The case highlights a further diagnostic possibility in the assessment of patients with "possible" motor neuron disease. The clinical features of CBM are briefly reviewed.

journal_name

Muscle Nerve

journal_title

Muscle & nerve

authors

Krishnan AV,Pamphlett R,Burke D,Wills EJ,Kiernan MC

doi

10.1002/mus.20115

subject

Has Abstract

pub_date

2004-11-01 00:00:00

pages

667-72

issue

5

eissn

0148-639X

issn

1097-4598

journal_volume

30

pub_type

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