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Inclusion Body Myositis

Definition
Inclusion body myositis (IBM) is one of a group of muscle diseases known as the inflammatory myopathies, which are characterized by chronic, progressive muscle inflammation accompanied by muscle weakness.  The onset of muscle weakness in IBM is generally gradual (over months or years) and affects both proximal (close to the trunk of the body) and distal (further away from the trunk) muscles.  Muscle weakness may affect only one side of the body.  Falling and tripping are usually the first noticeable symptoms of IBM.

For some individuals, the disorder begins with weakness in the wrists and fingers that causes difficulty with pinching, buttoning, and gripping objects.  There may be weakness of the wrist and finger muscles and atrophy (thinning or loss of muscle bulk) of the forearm muscles and quadricep muscles in the legs.  Difficulty swallowing occurs in approximately half of IBM cases.  Symptoms of the disease usually begin after the age of 50, although the disease can occur earlier.  IBM occurs more frequently in men than in women.

Prognosis

IBM is generally resistant to all therapies and its rate of progression appears to be unaffected by currently available treatments.

Treatment

There is no cure for IBM, nor is there a standard course of treatment.  The disease is generally unresponsive to corticosteroids and immunosuppressive drugs.  Some evidence suggests that intravenous immunoglobulin may have a slight, but short-lasting, beneficial effect in a small number of cases.  Physical therapy may be helpful in maintaining mobility.  Other therapy is symptomatic and supportive.

Source: https://www.ninds.nih.gov/

March 30, 2019
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