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Chapter 49

Sarcoidosis of the Nervous System

Allan Krumholz and Barney J. Stern

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Figure 49-1 Postulated mechanisms of pulmonary damage in sarcoidosis.

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Figure 49-2 Immunologic mechanisms active in the pathogenesis of granulomatous inflammation in neurosarcoidosis.

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Figure 49-3 Brain-biopsy section stained with hematoxylin and eosin. A, Low magnification photomicrograph showing dense granulomatous inflammation involving leptomeninges, with underlying gliotic cerebral cortex in a patient with neurosarcoidosis (scale bar, 50 μm). B, High magnification photomicrograph showing Virchow–Robin space involvement by a collection of epithelioid histiocytes (granulomatous inflammation) and lymphocytes in a patient with neurosarcoidosis (scale bar, 20 μm).

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Figure 49-4 Central nervous system (CNS) sarcoidosis: clinicopathologic relationships.

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Figure 49-5 Coronal midfrontal pathologic section of the brain of a patient with neurosarcoidosis, showing thickening and inflammatory changes of the basal meninges and optic chiasmal region.

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Figure 49-6 Cranial magnetic resonance imaging (MRI), coronal section, T1-weighted image with gadolinium, demonstrating an enhancing convexity sarcoid mass lesion that was initially mistaken for a meningioma.

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Figure 49-7 Cranial MRI, sagittal section, T1-weighted image with gadolinium, showing hypothalamic and pituitary involvement by sarcoidosis.

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Figure 49-8 Cranial MRI, axial section, T1-weighted image with gadolinium, demonstrating a frontal intracerebral mass that was proven by biopsy to be neurosarcoidosis.

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Figure 49-9 Cranial MRI, axial section, T2-weighted image, showing a large area of hyperintensity in the temporal lobe that was proven by biopsy to be sarcoidosis manifesting with a focal encephalopathy-vasculopathy.

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Figure 49-10 Cranial MRI, coronal section, T1-weighted image with gadolinium, showing gyral enhancement in the distribution of a posterior cerebral artery branch stroke.

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Figure 49-11 Spinal MRI. A, Axial view. B, Sagittal view, with gadolinium, demonstrating an enhancing intraspinal mass due to sarcoidosis.

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Figure 49-12 Cranial MRI, axial view, T1-weighted image, with gadolinium, demonstrating marked dural enhancement due to sarcoidosis.

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Figure 49-13 MRI findings of multifocal white matter–type changes in neurosarcoidosis.(From Scott TF, Yandora K, Kunschere LJ, et al: Neurosarcoidosis mimicry of multiple sclerosis: clinical, laboratory, and imaging characteristics. Neurologist 16:386, 2010, with permission.)

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Figure 49-14 Treatment paradigm for patients with neurosarcoidosis. IV, intravenous.