Inclusion body myositis Neurology
Inclusion Body Myositits
Overview
• Most common inflammatory myopathy in the elderly
• Insidious onset of slowly progressive proximal and distal weakness (see fig 2)
• Usually after age 50 years old
• Men are more affected than women
Signs/Symptoms
• Early weakness and atrophy of the quadriceps (knee extensors); volar forearm muscles (wrist and finger flexors); and tibialis anterior (ankle dorsiflexors) muscles
• Asymmetrical muscle involvement
• Shoulder and hip girdle weakness
• Facial weakness ( 33% of patients)
• Dysphagia (50% of patients)
• Distal sensory peripheral neuropathy (30% of patients) (see fig 2)
• Quadricep muscle stretch reflexes are decreased if quadriceps atrophy is present
• Knee pain due genu recurvatum
Diagnosis
• Muscle biopsy
Treatment
• Supportive ( Usually refractory to immunosuppressive therapy intravenous gamma globulin therapy)
Prognosis
• Normal life expectancy
• Patients are usually incapacitated within 10 to 15 years of onset
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