Inclusion body myositis. Clinical features and clinical course of the disease in 64 patients.
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 (> or = 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 skillful 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.
Badrising
Umesh A
UA
Dept. of Neurology, Leiden University Medical Centre, Leiden, The Netherlands. u.badrising@zhsdirksland.nl
Maat-Schieman
Marion L C
ML
van Houwelingen
Johannes C
JC
van Doorn
Peter A
PA
van Duinen
Sjoerd G
SG
van Engelen
Baziel G M
BG
Faber
Carin G
CG
Hoogendijk
Jessica E
JE
de Jager
Aeiko E
AE
Koehler
Peter J
PJ
de Visser
Marianne
M
Verschuuren
Jan J G M
JJ
Wintzen
Axel R
AR
D017668:Age of Onset; D000368:Aged; D000369:Aged, 80 and over; D003402:Creatine Kinase / Q000097:blood; D003430:Cross-Sectional Studies; D003680:Deglutition Disorders / Q000209:etiology; D018450:Disease Progression; D004576:Electromyography / Q000379:methods; D004651:Employment; D005260:Female; D006801:Humans; D008297:Male; D008875:Middle Aged; D018908:Muscle Weakness / Q000209:etiology; D018979:Myositis, Inclusion Body / Q000453:epidemiology / Q000378:metabolism / Q000503:physiopathology*; D009460:Neurologic Examination / Q000379:methods; D012189:Retrospective Studies; D012737:Sex Factors; D016138:Walking
D003402:Creatine Kinase