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Inclusion body myositis

2007; Elsevier BV; Linguagem: Inglês

10.1016/s0072-9752(07)86013-1

2212-4152

Michael R. Rose, Robert C. Griggs,

Genetic Neurodegenerative Diseases

This chapter discusses inclusion body myositis (IBM). It is usually a slowly progressive myopathy that causes considerable morbidity but does not directly cause mortality. The lack of any effective treatments makes it a challenge to manage. Inclusion-body myopathy is usually of late onset with more than 80% of cases occurring over the age of 50 years. Nearly all cases of IBM are sporadic. Cases have been described of siblings and twins with a phenotype identical to sporadic inclusion body myositis and with inflammation in their muscle biopsies. The presentation of typical IBM reflects the insidious onset and progression of the disease and the often stereotyped pattern of muscle involvement. The first symptom is often one of sudden, unprovoked falls without loss of consciousness. Despite the lack of facial symptoms in a third of cases examination shows mild weakness of eyelid or lip closure that is not a feature of inflammatory myopathies such as polymyositis and dermatomyositis. Muscle biopsy is essential for a definitive diagnosis of IBM. The diagnosis of IBM is based upon a combination of clinical, laboratory, and histological data. The increasing understanding of the degenerative aspects of IBM opens up a further range of therapeutic options.