Rheumatoid arthritis is a systemic disease that can potentially affect any organ. Neurologic complications in particular are well recognized, causing distinct increase in morbidity and mortality. Multidisciplinary conservative therapies include disease activity control with disease-modifying antirheumatic drugs, glucocorticoids, and new biological agents, in addition to symptomatic treatments such as rest, restriction of activity, moist heat, NSAIDs, gentle massage, etc. Neurologic complications of newer biological therapies and immunosuppressive drugs represent an increasing clinical challenge. Cervical collar, physical therapy, and occupational therapies also have a role. Survival rate is significantly decreased in patients with rheumatoid arthritis and neurologic complications.
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• Patients with rheumatoid arthritis have a reduced survival rate in general.
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• Rheumatoid arthritis of the cervical spine is a common complication that is often under-appreciated.
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• Neurologic complications related to rheumatoid arthritis affect the central nervous system, peripheral nervous system, muscles, and neuromuscular junction.
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• Neurologic complications of newer biological therapies and immunosuppressive drugs are an increasing concern.
Historical note and terminology
Neurologic complications of rheumatoid arthritis have been known for many years. In the 19th century Piters and Villard (107) and later Bannatyne (08) described peripheral neuropathy due to rheumatoid arthritis. Bannatyne described infiltration of small round cells in the nerve sheath, in the perivascular region, and among nerve fibers, as well as thickening of intima of blood vessels with encroachment on the vascular luminal wall (08). In 1942 Freund and colleagues reviewed the earlier reports on the rheumatoid arthritis neuropathy and reported “perineuritic nodules” in autopsied specimens of rheumatoid arthritis patients (45). These nodules consisted of chronic inflammatory cells in peripheral nerves. However, the significance of these observations was unknown until Ball described the presence of systemic arteritis that included peripheral neuropathy, which could complicate rheumatoid arthritis (07). Three years later, Hart and colleagues reported 10 rheumatoid arthritis patients with peripheral neuropathy and attributed it to diffuse arteritis (54). Neuropathy, therefore, was considered as an important complication of rheumatoid arthritis. Lastly, Ferguson and Slocumb indicated that development of peripheral neuropathy in rheumatoid arthritis has prognostic values (43). In their view, rheumatoid arthritis patients with peripheral neuropathy due to vasculitis had decreased survival compared to patients without neuropathy. With introduction of modern neurology in the last 25 years, other neurologic complications of rheumatoid arthritis such as cerebral vasculitis and cervical myelopathy have also been recognized.