Neurosarcoidosis: A Case Report

Abstract

Sarcoidosis is a multi-organ granulomatous disease of unknown etiology. Neurological involvement in sarcoidosis is uncommon but cranial mononeuropathies, especially involving the VII and VIII cranial nerves, are highly suggestive of neurosarcoidosis. We report the case of a 54-year-old woman who presented with fever, night sweats, weight loss, polyarthralgia, and bilateral hearing loss. Mediastinal and hilar lymphadenopathies with hypercaptation on positive emission tomography (PET) scans were present. Low-dose steroids were ineffective. She then developed bilateral anterior uveitis and right-sided peripheral facial palsy. Head magnetic resonance imaging (MRI) showed inflammatory involvement of the right cochlea, geniculate ganglion, and bilateral vestibulocochlear bundle. Cerebrospinal fluid analysis was compatible with aseptic meningitis. Excisional biopsy of mediastinal lymph nodes confirmed the presence of noncaseating granulomas. The diagnosis of systemic sarcoidosis with serious neurological involvement was made and treatment with high-dose steroids led to significant clinical improvement. Sarcoidosis remains a diagnosis of exclusion based on supportive clinical, radiological, and histological findings. This case highlights the challenge it was to diagnose this disorder. Neurologic involvement in sarcoidosis is relatively uncommon and has an unpredictable clinical course and prognosis.

Rare Presentations of Sarcoidosis: A Case Series

“…The second case was that of a neurosarcoidosis. Neurological involvement in sarcoidosis occurs in 3-10% of the cases [ 6 ]. It can affect any part of the nervous system.…”

“…Mononeuritis multiplex, sensorimotor peripheral neuropathy, aseptic meningitis, hydrocephalous, seizures, and psychosis are the other disease manifestations. About 90% of the patients have systemic manifestations when they present with neurological features [ 6 ]. However, neurological features may be the first presenting manifestation in some cases.…”

Neurosarcoidosis Presented as an Isolated Brain Lesion

“…CNS involvement in sarcoidosis is uncommon, but it may affect any part of the nervous system, which leads to a difficult differential diagnosis. Central nervous system involvement, or neurosarcoidosis (NS), occurred approximately in 5%-10% of sarcoidosis cases [ 5 ]. The average age of diagnosis of NS is between 20 and 40, with a predominance of African American women in comparison with men and other ethnicities [ 6 ].…”

Systemic and Neurosarcoidosis With Rare Involvement of the Extremities’ Peripheral Nerves

“…Due to the challenge of obtaining neural tissue biopsy, neurosarcoidosis is generally diagnosed when there are neurological symptoms with pathology of sarcoidosis elsewhere. 2 Neurosarcoidosis is caused by inflammation/granuloma deposits in the brain, spinal cord, and cranial nerves. The rare peripheral neuropathy could be multifocal.…”

“…Definitive diagnosis could be made only by sarcoid granulomas demonstration on histopathology. Due to the challenge of obtaining neural tissue biopsy, neurosarcoidosis is generally diagnosed when there are neurological symptoms with pathology of sarcoidosis elsewhere 2 . Neurosarcoidosis is caused by inflammation/granuloma deposits in the brain, spinal cord, and cranial nerves.…”