Sarcoid encephalopathy with diffuse inflammation and focal hydrocephalus shown by sequential CT

Abstract

Sequential computerized tomography (CT) was performed on a patient with seizures and an organic mental syndrome. Cerebral sarcoidosis was subsequently diagnosed on the basis of noncaseating granulomas in three organ systems. Cranial nerve palsies, hypothalamic dysfunction, and widespread disease were absent. An unusual neuroradiologic sequence of events indicated focal and asymmetric hydrocephalus, mass effect, ependymitis, diffuse breakdown of the blood-brain barrier, and periventricular white matter destruction. These findings have not been previously described together in a patient with sarcoid. With the advent of noninvasive CT, it is possible to perform serial investigations of encephalopathies of obscure origin. Findings similar to those in the present case should prompt a thorough search for subclinical systemic involvement by sarcoid.

Intrathecal production of oligoclonal IgM and IgG in CNS sarcoidosis

“…Indirect evidence for intrathecal IgG synthesis has previously been presented in CNS sarcoidosis by demonstration of a paretic Lange curve (17) and elevated % of gamma globulins (22). Our patient displayed clear evidence for intrathecal production of oligoclonal IgG, a CSF abnormality most often seen in a variety of inflammatory CNS disorders such as multiple sclerosis and aseptic meningo-encephalitis (23,24).…”

Sarcoidosis

“…In general, granulomatous masses appear as areas of high attenuation enhancing after intravenous contrast medium injection. Diminution in size of such areas after corticosteroid treatment has been reported, and regarded as confirmatory of the diagnosis of sarcoidosis (Bahr et al, 1978, Case 1;Brooks et al, 1979;Ho et al, 1979). In the case of meningioma in a patient with sarcoidosis reported by Rosen and Wang (1965 ) diminution in the area of enhanced radio-isotope uptake after corticosteroid treatment was misleading, suggesting the erroneous initial diagnosis of basal meningeal sarcoidosis.…”

“…Radio-isotope scanning has been used to demonstrate enhanced uptake at areas of sarcoid granulomatosis (Schwart z and Baum, 1968;Virye et al, 1969;McCartney et al, 1979 ). The use of computer assisted tomography has been described by Bahr et al (1978), Babu et al (1979), Brooks et al (1979), Decker et al (1979), Ho et al (1979) and Kumpe et al (1979). In general, granulomatous masses appear as areas of high attenuation enhancing after intravenous contrast medium injection.…”

Neurosarcoidosis with Intraparenchymal Cystic Lesions

“…Radiological appearances include multiple or solitary supratentorial and infratentorial parenchymal mass lesions, white matter lesions, periventricular enhancement, perivascular lesions, infarction, hydrocephalus, intracranial hemorrhage, cranial nerve lesions, enlargement and abnormal enhancement of pituitary stalk, optic chiasmal lesions, leptomeningeal enhancement, solitary or multiple extra-axial masses mimicking meningioma, choroid plexus lesions and ependymitis [13][14][15][16][17][18][19][20] . Our patient had multiple imaging findings which included innumerable enhancing parenchymal nodules and perifocal edema, loss of normal bright signal intensity of the posterior pituitary in T1-WI with abnormal enhancement and mild thickening of the distal portion of the pituitary stalk, thickening with abnormal enhancement of the optic chiasma, meningeal thickening and enhancement,subependymal nodules, ependymitis, choroid plexus lesions and few white matter lesions.…”