Elsevier

Clinics in Chest Medicine

Volume 29, Issue 3, September 2008, Pages 475-492
Clinics in Chest Medicine

Neurosarcoidosis

https://doi.org/10.1016/j.ccm.2008.03.016Get rights and content

Although neurosarcoidosis seems to occur in only 5% to 10% of patients who have sarcoidosis, it may lead to significant complications. The diagnosis of neurosarcoidosis usually relies on indirect information from imaging and spinal fluid examination. Although MR imaging remains the most sensitive technique for detecting neurologic disease, other tests, including positron emission tomography scanning and cerebral spinal fluid examination, can provide important information. The role of immunosuppressive agents such as methotrexate, cyclophosphamide, and azathioprine has been expanded, and these agents should be considered for the treatment of some manifestations of neurosarcoidosis. Reports of the antitumor necrosis factor agent infliximab suggest that this drug can be helpful for patients who have neurosarcoidosis.

Section snippets

Neurologic manifestations

Because the granulomas of sarcoidosis can affect virtually any part of the central or peripheral nervous system, patients may present to a variety of health care professionals, including primary care specialists, ophthalmologists, neurologists, otolaryngologists, endocrinologists, infectious disease experts, oncologists, or pulmonologists. Cranial neuropathies are identified in 50% to 75% of patients who have neurosarcoidosis, with facial palsy reported in 25% to 50% [2], [13], [14], [15], [16]

The diagnostic dilemma of neurosarcoidosis

Because of the diverse clinical presentations, patients are often classified as having definite, probable, or possible neurosarcoidosis based on the confirmed diagnosis of multisystem sarcoidosis, the pattern of neurologic disease, and the response to treatment. Two groups have proposed criteria for neurosarcoidosis based on the probability of neurologic involvement [17], [57]. Table 2 compares the two proposals in terms of definite, probable, and possible neurosarcoidosis diagnosis. In Judson

Magnetic resonance imaging: brain

MR imaging with gadolinium contrast enhancement remains the preferred imaging technique for neurosarcoidosis [64]. Leptomeningeal involvement is the most commonly reported imaging abnormality because it is seen in approximately 40% of neurosarcoidosis cases. This disease pattern usually appears as thickening with diffuse or focal/multifocal enhancement of the leptomeninges on contrast-enhanced T1-weighted images (see Fig. 1). Sarcoidosis has a predilection for the basilar meninges [14];

Treatment of neurosarcoidosis

There are no randomized clinical trials defining the optimal treatment for neurosarcoidosis. In general, patients who have neurosarcoidosis present with a self-limited manifestation or a chronically progressive disease course [2], [4], [16]. Isolated cranial nerve abnormalities and aseptic meningitis are frequent monophasic presentations in two thirds of patients who have neurosarcoidosis. In particular, patients who have seventh nerve paralysis often have resolution of this symptom

Summary

The diagnosis and management of neurosarcoidosis remains one of the more difficult aspects of this disease. This manifestation remains challenging because of the diverse range of clinical presentations, difficulties encountered in identifying neurologic lesions, and the refractory nature that can occur with the disease process. Newer treatment options provide the treating physician better options to tailor therapy to the individual patient.

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