Inclusion body myositis
| Autor: | Pieter A. van Doorn, Jan J.G.M. Verschuuren, Umesh A. Badrising, C. G. Faber, Johannes C. Van Houwelingen, Aeiko E. de Jager, Marianne de Visser, Sjoerd G. van Duinen, Jessica E. Hoogendijk, Marion L.C. Maat-Schieman, Baziel G.M. van Engelen, Peter J. Koehler, Axel R. Wintzen |
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| Přispěvatelé: | Neurology, Radiology & Nuclear Medicine |
| Rok vydání: | 2005 |
| Předmět: |
Employment
Male Pediatrics medicine.medical_specialty Weakness Walking Myositis Inclusion Body Pharyngeal muscles Inflammatory myopathy Sex Factors medicine Humans Age of Onset Myopathy Creatine Kinase Myositis Aged Retrospective Studies Muscle contracture Aged 80 and over Neurologic Examination Human Movement & Fatigue [NCEBP 10] Muscle Weakness Electromyography business.industry Muscle weakness Middle Aged medicine.disease Neuromuscular development and genetic disorders [UMCN 3.1] Cross-Sectional Studies medicine.anatomical_structure Neurology Disease Progression Physical therapy Female Neurology (clinical) medicine.symptom Inclusion body myositis Deglutition Disorders business Functional Neurogenomics [DCN 2] |
| Zdroj: | Journal of Neurology, 252, 12, pp. 1448-54 Journal of neurology, 252(12), 1448-1454. D. Steinkopff-Verlag Journal of Neurology, 252, 1448-1454. D. Steinkopff-Verlag Journal of Neurology, 252, 1448-54 |
| ISSN: | 1432-1459 0340-5354 |
| DOI: | 10.1007/s00415-005-0884-y |
| Popis: | Contains fulltext : 48412.pdf (Publisher’s version ) (Closed access) The clinical features of inclusion body myositis (IBM) were of minor importance in the design of consensus diagnostic criteria, mainly because of controversial views on the specificity of signs and symptoms, although some authors reported "typical" signs. To re-assess the clinical spectrum of IBM, a single investigator using a standard protocol studied a cohort of 64 patients cross-sectionally. Symptom onset was before the age of 50 years in 20% of cases. Only a few patients (14 %) started with weakness other than that of quadriceps, finger flexor or pharyngeal muscles. The sequence of power loss was erratic, but onset of symptoms with quadriceps weakness predicted an earlier onset of dysphagia in older patients (>/= 56 years) compared with younger ones (< 56 years) (p = 0.02). Despite widespread weakness patients had favourable scores on three commonly used function scales and they kept their employment. Complete wheel-chair dependency was rare (3 %). A dominant characteristic was the anatomical distribution of afflicted muscles: ventral extremity muscle groups were more affected than dorsal muscle groups and girdle muscles were least affected, the latter preserving postural stability. Ankylosis, especially in extension of the fingers,was frequently present. Together with the sparing of intrinsic hand muscles it was helpful in the preservation of many skilful movements.IBM has a unique distribution of muscle weakness. Ankylotic contractures are common. We feel that their joint impact on daily functioning is characteristic for the disease. |
| Databáze: | OpenAIRE |
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