- Barney J Stern, MD
Barney J Stern, MD
- Vice-Chair for Strategic Planning, Department of Neurology
- Johns Hopkins University
- Section Editors
- Michael J Aminoff, MD, DSc
Michael J Aminoff, MD, DSc
- Editor-in-Chief — Neurology
- Section Editor — Medical Neurology
- Professor of Neurology
- University of California, San Francisco School of Medicine
- Talmadge E King, Jr, MD
Talmadge E King, Jr, MD
- Editor-in-Chief — Pulmonary and Critical Care Medicine
- Section Editor — Interstitial Lung Disease
- Dean, School of Medicine
- Vice Chancellor, Medical Affairs
- University of California San Francisco
Neurologic complications occur in approximately 5 to 10 percent of patients with sarcoidosis [1-4]. Neurosarcoidosis is a diagnostic consideration in patients with known sarcoidosis who develop neurologic complaints and in patients presenting de novo with a constellation of findings consistent with the disease [5,6]. Approximately 50 percent of patients with neurosarcoidosis present with neurologic difficulties at the time sarcoidosis is first diagnosed. One-third of those with neurosarcoidosis have or develop more than one neurologic manifestation of their disease.
The diagnosis and management of neurosarcoidosis will be reviewed here. General issues related to sarcoidosis and its pathogenesis are discussed separately. (See "Clinical manifestations and diagnosis of pulmonary sarcoidosis" and "Pathology and pathogenesis of sarcoidosis".)
Any portion of the central or peripheral nervous system can be affected by sarcoidosis. Common syndromes include:
●Cranial mononeuropathy. Peripheral facial nerve palsy develops in 25 to 50 percent of patients with neurosarcoidosis [2,6,7]. The facial nerve palsy can be unilateral or bilateral (simultaneous or sequential) and recurrent. Optic neuropathy and cranial nerve VIII dysfunction can lead to intermittent or progressive visual, auditory, or vestibular dysfunction . (See "Optic neuropathies", section on 'Sarcoidosis'.)
●Neuroendocrine dysfunction typically occurs with hypothalamic inflammation, resulting in polyuria or disturbances in thirst, sleep, appetite, temperature, or libido. Hypothalamic or pituitary lesions may also cause thyroid, gonadal, or adrenal abnormalities [6,9]. (See "Causes of hypopituitarism".)
One such manifestation, polyuria, can result from one or more factors in patients with sarcoidosis. Direct hypothalamic involvement can lead to central diabetes insipidus or primary polydipsia, while hypercalcemia (due to production of calcitriol by activated macrophages) can cause nephrogenic diabetes insipidus . (See "Hypercalcemia in granulomatous diseases".) Thus, patients with sarcoidosis and polyuria may require a water restriction test to establish the correct diagnosis. (See "Diagnosis of polyuria and diabetes insipidus".)To continue reading this article, you must log in with your personal, hospital, or group practice subscription. For more information on subscription options, click below on the option that best describes you:
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- CLINICAL FEATURES
- DIAGNOSTIC APPROACH
- Differential diagnosis
- Clinical evaluation
- Neurodiagnostic testing
- - Neuroimaging
- - Lumbar puncture
- - Other tests
- Other immunomodulatory therapies
- Surgical considerations
- Radiation therapy
- General measures
- SUMMARY AND RECOMMENDATIONS