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The mystery of IBM and hIBM: Why do the two have completely different mechanisms despite similar names?
As we age, many people experience varying degrees of muscle weakness and discomfort. Isolated inclusion body myositis (IBM) is the most common inflammatory muscle disease in the elderly, with symptoms usually starting in one's forties. The disease is characterized by progressive muscle weakness and atrophy and is often confused with hereditary inclusion body myopathy (hIBM). Although the names of the two are similar, there are fundamental differences in pathological mechanisms and clinical manifestations.
The key difference between IBM and hIBM is the meaning of the "M": the "M" in IBM stands for "myositis", while the "M" in hIBM stands for "myopathy."
Although these two diseases share the common characteristics of muscle atrophy and weakened strength, their causes and development are completely different.
Symptoms and effects
The progression of IBM is usually slow and asymmetrical, with patients gradually becoming limited in their motor function. Initial symptoms may include frequent stumbling and difficulty climbing stairs, and in many cases patients experience falls, often due to premature quadriceps involvement. Patients may also have limited finger dexterity. For example, the ability to turn a doorknob or hold a key may be significantly reduced.
As the disease progresses, patients may need to rely on walking aids, such as crutches or wheelchairs, to cope with increasing weakness and loss of mobility.
Potential reasons
As for the cause of IBM, there is currently no conclusion. Some researchers have suggested that it may be caused by the interaction of certain genes and environmental factors. Research into the disease has led to two main hypotheses: one suggests that the cause may be related to an abnormal response of the immune system, while the other points to the aging of muscle tissue and the accumulation of abnormal proteins associated with the disease.
According to research, IBM may be involved in viral infection, similar to the situation with the HTLV-1 virus, but there is no confirmation. Even so, it is possible that the immune system's response may lead to further muscle deterioration.
Diagnosis and differential diagnosis
The diagnosis of IBM often relies on muscle biopsy, and some patients may have significantly elevated creatine kinase (CK) levels. Electrophysiological testing may reveal a wide range of abnormalities. During the diagnosis process, common misdiagnoses include misdiagnosing IBM as polymyositis. Compared with polymyositis, the progression of IBM is slow and the treatment effect is limited.
Current management methods
As of 2019, there is no effective treatment option for IBM. Experts often advocate for supportive care that focuses on preventing falls. Physical therapy is a necessary means to help patients adapt to life and activities, but the lack of consensus on exercise guidance still requires further exploration.
Social and cultural influences
In social culture, there are also some well-known figures such as musician Peter Frampton, who was diagnosed with IBM in 2019, highlighting the impact of this disease on personal lives. In addition, the protagonist in the movie "Father Stu" also suffered from a similar illness, making more people face up to this disease that is not yet fully understood.
With in-depth research on IBM and hIBM, can we find more effective treatment options in the near future, thereby improving the quality of life of patients?
