Neurosarcoidosis

Abstract

Neurosarcoidosis is an uncommon but clinically significant manifestation that will be routinely encountered in sarcoidosis specialty clinics. Overall, neurologic involvement is recognized in 5 to 10% of individuals with sarcoidosis. Neurologic symptoms will be the presenting manifestation of sarcoidosis in approximately one-half of those with neurosarcoidosis. The clinical and imaging features of neurosarcoidosis vary widely, largely depending on the anatomic distribution of the disease. The likelihood of spontaneous resolution is lower than for sarcoidosis in general, and residual functional deficits are not uncommon. Therefore, most patients with neurosarcoidosis require immunosuppressive therapy. Small fiber neuropathy, a recently recognized nongranulomatous parasarcoidosis syndrome, is prevalent in chronic sarcoidosis. The variety of neurologic manifestations, broad differential diagnosis, and complexity of management render neurosarcoidosis an area best served by multidisciplinary teams. Sarcoidologists, neurologists, radiologists, neurosurgeons, endocrinologists, urologists, physiatrists, and physical/occupational therapists all potentially have important roles.

Cervical myelitis in a patient with pulmonary sarcoidosis

“…Neurological manifestation occurs in 5% to 10% of patients with sarcoidosis [1,2]. Spinal cord sarcoidosis rarely occurs, and intraspinal sarcoidosis is rarely reported (0.1% to 50% of neurosarcoidosis) [3].…”

Successful therapy with rituximab in three patients with probable neurosarcoidosis

“…In NS, noncaseating granulomas mostly affect the CNS even if there are few cases of a primary involvement of the peripheral nervous system (PNS). 2 , 3 In CNS, granulomas have tropism for meninges, more rarely for pure cerebral tissue, in PNS for spinal root sheaths, which are an extension of pachymeninges.…”

“…Laboratory testing includes serum angiotensin converting enzyme and soluble interleukin-2 receptor (sIL-2R) but both may also be negative in patients with biopsy proof of NS. 2 In contrast, cerebrospinal fluid (CSF) sIL-2R value was found to have a high sensitivity in NS. 6 …”

“…The prevalence of central nervous system (CNS) involvement (neurosarcoidosis; NS) is about 5–15%. 2 , 3 Clinical features of NS include, among other things, cranial neuropathy, seizure, aseptic meningitis, hydrocephalus and myelitis. 2 – 4 …”

“…Magnetic resonance imaging (MRI) often shows leptomeningeal involvement of the basilar meninges but virtually any portion of the CNS may be affected. 2 , 4 Currently, no reliable serologic marker exists. Laboratory testing includes serum angiotensin converting enzyme and soluble interleukin-2 receptor (sIL-2R) but both may also be negative in patients with biopsy proof of NS.…”