Neurosarcoidosis

Abstract

Neurosarcoidosis is frequently on the differential diagnosis for neurohospitalists. The diagnosis can be challenging due to the wide variety of clinical presentations as well as the limitations of noninvasive diagnostic testing. This article briefly touches on systemic features that may herald suspicion of this disorder and then expands in depth on the neurological clinical presentations. Common patterns of neurological presentations are reviewed and unusual presentations are also included. A discussion of noninvasive testing is undertaken, exploring dilemmas that may be encountered with sensitivity and specificity. Drawing from a broad range of clinical clues and diagnostic data, a systematic approach of pursuing a potential tissue diagnosis is then highlighted. Correctly diagnosing neurosarcoidosis is critical, as treatment with appropriate immunosuppression protocols can then be initiated. Additionally, treatment of refractory disease, the trend toward exploring targeted immunomodulation options, and other therapeutic issues are discussed.

Dementia, gait disturbance, and urinary incontinence in a patient with pulmonary sarcoidosis

“…Normal pressure hydrocephalus is characterized by the classic triad of dementia, gait disturbance, and urinary incontinence. Prognosis in these cases is poor, with mortality within 1 year for about 75% of patients 8, 14 and it is important to recognize these cases because they can be reversed by the placement of a VP shunt. The most probable mechanism in these cases is the impaired absorption of CSF secondary to chronic granuloma or pachymeningitis.…”

“…Finally, pathology showing granulomatous lesions is the most specific diagnostic test for sarcoidosis, but because biopsy of peripheral or central nerves carries high morbidity, it is usually supported with samples taken from other organs, particularly the lungs and lymph nodes 1, 8, 14, 17. We considered the clinical manifestation in association with imaging and differential diagnosis ruled out, in combination with a known history of pulmonary sarcoidosis as diagnostic in this case, and no pathological sample was needed in order to treat the patient.…”

Vertigo as a Predominant Manifestation of Neurosarcoidosis

“…Lumbar puncture in sarcoidosis may demonstrate an elevated CSF protein and a mild-to-moderate pleocytosis with lymphocytic predominance or may be normal in a third of patients [ 9 , 10 ]. Decreased glucose levels may occur in one-fifth of patients [ 10 ].…”

“…Additionally, neurosarcoidosis can vary greatly in appearance on imaging [ 12 ]. The most common finding is the involvement of the leptomeninges seen as nodular or linear enhancement along the brain's contours extending into the cortical sulci [ 9 , 12 ]. However, this can also be seen with meningeal carcinomatosis, lymphoma, leukemia, tuberculosis, and fungal meningitis [ 12 ].…”

“…However, this can also be seen with meningeal carcinomatosis, lymphoma, leukemia, tuberculosis, and fungal meningitis [ 12 ]. Enhancement of the cranial nerves or the hypothalamic-pituitary axis may also be noted, although involvement of cranial nerves on imaging may correlate poorly with the clinical neurologic deficit [ 9 , 12 ]. For example, although cranial nerve II (optic nerve) most often shows enhancement on imaging, it is cranial nerve VII (facial nerve) that tends to show the clinical neurologic deficit [ 12 ].…”