Neurologic Symptoms of Rheumatologic Conditions Elucidated

VBCR - October 2014, Volume 3, No 5 - Rheumatology Update
Rosemary Frei, MSc

A brief review of the neurologic­al symptoms occurring in people with rheumatologic diagnoses seeks to assist clinicians in differentially diagnosing conditions (Curr Neurol Neurosci Rep. 2014;14:456-469). In the article, Amre Nouh, MD, Department of Neurology, Loyola University Medical Center, Maywood, IL, and colleagues, also discuss ways to distinguish between neurotoxicity from medications used to treat rheumatic disease and neurologic manifestations of rheumatologic conditions.

Rheumatologic diseases and some of their treatments can involve many parts of the nervous system ranging from the meninges to the peripheral nervous system, according to the study authors. Neurologic symptoms may even be the presenting feature of some rheumatologic conditions. “The neurologic manifestations of rheumatic disorders and their treatments are some of the most challenging conditions to diagnose,” Sean Ruland, DO, Associate Professor and Medical Director, Neuroscience Intensive Care Unit, Department of Neurology, Loyola University Chicago, Stritch School of Medicine, and senior author of the review, told Value-Based Care in Rheumatology.

Neurologic Signs and Symptoms

Among the rheumatologic conditions that are frequently accompanied by neurologic symptoms is systemic lupus erythematosus (SLE), they explained. Central nervous symptom manifestations are the presenting feature in approximately one-quarter of SLE patients, according to one study they cited (Joseph FG, et al. Neurology. 2007;69:644-654). Moreover, more than half of SLE patients have headaches, up to 15% have recurrent seizures, and 10% to 20% have mood disorders.

Dr Ruland and colleagues also described the neurologic symptoms that can be present in patients with rheumatoid arthritis, Sjögren’s syndrome, scleroderma, immunoglobulin-4–related disorders, idiopathic inflammatory myositis, sarcoidosis, and spondyloarthropathies. For example, peripheral neuropathies occur in 22% to 27% of people with Sjögren’s syndrome and central nervous system manifestations in 0.3% to 48%. The former include length-dependent sensorimotor polyneuropathy, mononeuritis multiplex, small-fiber neuropathy, and a pure sensory neuronopathy, which is an asymmetric non–lengthdependent neuropathy usually affecting arms more than legs. The latter include aseptic meningitis, demyelination, vasculitis, acute cerebellitis, seizures, and cognitive impairment.

In addition, 19% to 40% of patients with scleroderma have neurologic symptoms. Fifty-seven percent of those with neurologic involvement have distal axonal sensorimotor peripheral neuropathy, and respond poorly to treatment, the study authors noted; 15% to 17% of patients have myositis. Other neurologic syndromes include brachial plexopathy and entrapment neuropathies.

They also describe in detail the neurologic symptoms that can accompany systemic vasculitis and idiopathic inflammatory myositis. There are many forms of vasculitis, including giant cell arteritis, Takayasu’s arteritis, polyarteritis nodosa, Kawasaki’s arteritis, microscopic polyangiitis, and granulomatosis with polyangiitis, each with distinct presentation, clinical signs and diagnosis, and treatment.

Efficient Diagnosis and Treatment Possible

The 4 types of idiopathic inflammatory myositis are polymyositis, dermatomyositis, immune-mediated necrotizing myopathy, and inclusion body myositis. Dr Ruland and co­authors provided details on the dif­ferences between these conditions, ranging from age—inclusion body myositis, for example, occurs in people 50 years and older, while dermatomyositis can have a juvenile or adult onset—to treatment, where inclusion body myositis is not responsive to treatment, for instance, but dermatomyositis responds well to immu­nosuppression and intravenous immunoglobulin.

Furthermore, serious neurologic complications can result from the use of immunosuppressive therapies, they stated. Progressive multifocal leukoencephalopathy has received considerable attention in patients receiving natalizumab, rituximab, or efalizumab, or, in some cases, in patients not taking biologics. Some patients treated with intravenous immunoglobulins for the first time have had strokes, Dr Ruland and colleagues noted. Other neurotoxicities have been reported in a variety of other medications commonly used in rheumatology, from corticosteroids to colchicine.

“Efficient diagnosis and treatment should be a multidisciplinary, collaborative effort that includes expertise in rheumatology and rehabilitation, among other fields,” they concluded.

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