Sarcoidosis of the nervous system

Abstract

Although sarcoidosis is rarely confined to the nervous system, any neurological features that do occur frequently happen early in the course of the disease. The most common neurological presentation is with cranial neuropathies, but seizures, chronic meningitis and the effects of mass lesions are also frequent. The diagnostic process should first confirm nervous system involvement and then provide supportive evidence for the underlying disease; in the absence of any positive tissue biopsy, the most useful diagnostic tests are gadolinium enhanced MRI of the brain and CSF analysis, although both are non-specific. The mainstay of treatment is corticosteroids, but these often have to be combined with other immunosuppressants such as methotrexate, hydroxychloroquine or cyclophosphamide. There is increasing evidence that infliximab is a safe treatment with good steroid sparing capacity.

Clinical features of spinal cord sarcoidosis: analysis of 17 neurosarcoidosis patients

“…Methotrexate may be useful to maintain optimal disease suppression [3]. Therefore, indicators that represent the disease activity of spinal cord sarcoidosis are necessary for reasonable treatment.…”

“…The diagnosis of neurosarcoidosis is often difficult in clinical practice and requires magnetic resonance imaging (MRI) to detect the abnormalities and the evidence of systemic sarcoidosis [2,3]. Spinal cord sarcoidosis exhibits intramedullary high-intensity signals on T2-weighted images (T2WI) and enhancements on gadolinium (Gd) DTPA-enhanced T1 weighted images (Gd-T1WI) [4,5]; however, these signs also resemble cervical myelopathy with spondylosis [6].…”

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

“…Neurosarcoidosis is a severe disease condition and the most common manifestation is cranial nerve compromise, most often presenting as peripheral seventh nerve palsy. Other possible manifestations are hydrocephalus, myopathy, peripheral neuropathy, meningitis, and seizures 3, 4, 5, 6, 7. But the rates of asymptomatic involvement may be higher, with positive autopsy findings in up to 15% of patients 8.…”

Neuropareidolia: diagnostic clues apropos of visual illusions

“…Heerfordt, a Danish ophthalmologist, first reported on the neurologic manifestations of sarcoidosis in 1909 29 . The disease affects slightly more females than males, mostly during the third and fourth decades of life, 29,30 . Although sarcoidosis strictly restricted to the nervous system is unusual, neurologic involvement is relatively common and may represent a diagnostic challenge.…”

“…The most frequent presentation of neurosarcoidosis is cranial nerve neuropathy, mostly facial and optic. Seizures (15%), chronic meningitis (26%), mental disorders (20%), and intracranial hypertension may also occur 29 .…”