Perspective

Imaging Manifestations of Neurosarcoidosis

J. Keith Smith1, Maria Gisele Matheus, Mauricio Castillo
arcoidosis is an idiopathic systemic disease characterized histologically by the formation of non-caseating granuloma. The disease affects all parts of the body, especially the lungs and lymph nodes. In the head and spine, the most typical imaging appearance is thickening and enhancement of the leptomeninges, especially around the base of the brain, but sarcoidosis may involve bone, dura mater, nerve roots, leptomeninges, and parenchyma, individually or in combination [1, 2]. We review the clinical and imaging ndings of central nervous system involvement by sarcoidosis.
Epidemiology

Sarcoidosis can affect patients of all ages and races but is most common in the third and fourth decades. Incidence is estimated to be around 20 per 100,000 among Caucasians. African Americans and North European whites have the highest disease incidence, and women are more frequently affected than men [13]. The exact cause of sarcoidosis is unknown. Genetic factors confer increased susceptibility, perhaps the major histocompatibility complex or complement receptor gene [4]. Also, granuloma formation is initiated by T lymphocytes responding to a specic but unknown antigen. RNA and DNA from Mycobacterium [5] and Propionibacterium organisms [6] have been detected in sarcoidosis lesions, suggesting a possible cause.

Central nervous system involvement is common in postmortem series, with about one fourth of patients with systemic disease showing histologic evidence of central nervous system involvement. Symptomatic central nervous system involvement in living patients is less common, found in only about 5% of cases [3]. Imaging evidence of central nervous system disease, however, is seen in about 10% of patients with systemic disease. It is estimated that less than 1% of patients have isolated central nervous system involvement, without systemic evidence of disease. In cases with central nervous system involvement, however, the central nervous system symptoms are frequently the presenting ones. For this reason, it is important for the radiologist to recognize the imaging manifestations of neurosarcoidosis. Unfortunately, the imaging manifestations of central nervous system sarcoidosis are protean.
Clinical Presentation

involvement of the hypothalamus or pituitary gland. Hydrocephalus is another uncommon clinical feature. Spinal cord involvement may present clinically with lower extremity weakness and other nonspecic signs of myelopathy.
Diagnostic Tests

Clinical symptoms of neurosarcoidosis depend on the site of granuloma involvement and are nonspecic. Facial nerve paralysis (central or peripheral type) and vision loss are common symptoms, as are headache, seizure, and signs of meningeal irritation [2, 3]. Signs and symptoms easily confused with those of multiple sclerosis such as weakness, paresis, paresthesia, diplopia, and dysarthria are frequent. Less common are symptoms of diabetes insipidus, such as intense thirst and polyuria, stemming from

The diagnosis of denite neurosarcoidosis is conrmed by biopsy results showing noncaseating granuloma, with an absence of organisms or other causes. In many cases, biopsy is not possible or desirable because of the site of involvement. A diagnosis of probable neurosarcoidosis can be based on clinical or imaging evidence of lesions, with evidence of systemic sarcoidosis obtained from a biopsy of another organ or positive results on a Kveim test [2], which is performed by intradermal injection of homogenized spleen or liver from a patient with known sarcoidosis. The test result is positive if biopsy of the skin site 46 weeks later shows typical sarcoid granulomas. Although the Kveim test has high sensitivity and specicity, limited availability of suitable safe test material has limited its widespread use [3]. In the absence of histologic proof of systemic disease, the diagnosis can be supported by two or more ndings, such as typical chest radiograph or gallium scan ndings or elevated serum angiotensin-converting enzyme levels. A whole-body gallium scan shows increased uptake related to central nervous system disease in less than 5% of patients with

Received June 5, 2003; accepted after revision August 12, 2003.

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All authors: Department of Radiology, University of North Carolina School of Medicine, CB# 7510, Chapel Hill, NC 27599-7510. Address correspondence to J. K. Smith.

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Fig. 1.T1-weighted parasagittal MRI of 31-year-old man with neurosarcoidosis obtained after contrast administration shows enhancing lesion (arrow) in brain parenchyma, arising from spread of sarcoidosis granuloma along perivascular spaces.

Fig. 2.Leptomeningeal sarcoidosis of brain in 45year-old woman. T1-weighted axial image obtained after contrast administration shows enhancement involving basilar cisterns, Sylvian ssures, and cortical sulci (arrows).

1 this condition but may give evidence of the presence of systemic disease in 45% of patients with central nervous system involvement [2]. Gallium-67 citrate injected IV is taken up by sites of active sarcoidosis and other inammatory and neoplastic processes such as tuberculosis and lymphoma. Because it lacks specicity, other authors report limited utility of the gallium scan [3]. Serum levels of angiotensin-converting enzyme can be elevated in patients with pulmonary sarcoidosis. Angiotensin-converting enzyme is produced by the epithelioid cells of granulomatous lesions and can therefore be elevated in a number of disorders, including diabetes, silicosis, and cirrhosis [3]. Patients with neurosarcoidosis have been reported to have elevated serum angiotensin-converting enzyme in 550% of cases [7]. In a recent review, only one patient of 12 with denite neurosarcoidosis had elevation of serum angiotensin-converting enzyme levels, whereas one other of the 12 had elevated cerebrospinal uid angiotensin-converting enzyme levels [2]. Other cerebrospinal uid abnormalities, also nonspecic, were common, including elevated protein levels and lymphocytosis.
Imaging Findings
Brain

2 with enhancing brain lesions frequently present with seizures [11]. Leptomeningeal involvement.Leptomeningeal involvement is perhaps the most typical manifestation of central nervous system sarcoidosis, seen in about 40% of cases. This is usually seen as thickening and enhancement of the leptomeninges on contrast-enhanced T1weighted images (Fig. 2). The enhancement may be diffuse or nodular [7]. There may be spread along the perivascular spaces, causing the appearance of intraparenchymal involvement. There is a predilection for the basilar meninges. Leptomeningeal disease can be distinguished from dural disease by involvement of the cortical sulci and perivascular spaces or the cisterns around the base of the brain. This pattern of involvement is generally indistinguishable from that seen with tuberculosis or lymphoma involving the leptomeninges. Hypothalamus and pituitary involvement. Leptomeningeal involvement around the hypothalamus and pituitary infundibulum may be seen with basilar leptomeningeal involvement or as an isolated nding (Fig. 3). Again,

Intraparenchymal lesions.The most common parenchymal abnormality described in some series is multiple nonenhancing periventricular white matter lesions seen as high signal intensity on T2-weighted images [2, 9]. These lesions may be indistinguishable from those seen with vascular disease or multiple sclerosis. Because this type of lesion is common in patients without sarcoidosis as well, it is not clear that the lesions are always related to the sarcoidosis. Enhancing parenchymal mass lesions (Fig. 1) are also commonly reported [10]. These lesions may be mistaken for primary or metastatic tumor or tumefactive demyelination. Enhancing mass lesions are frequently associated with nearby leptomeningeal involvement and are thought to represent spread of leptomeningeal disease along the perivascular spaces in many cases [11]. Mass lesions associated with sarcoidosis may be dark on T2-weighted images [10, 12], but very cellular metastasis and lymphoma may also have this appearance. Central necrosis is uncommon in sarcoidosis lesions. Patients

Sarcoidosis can involve any part of the nervous system and its coverings. We concentrate on the types of involvement that might be seen on imaging of the brain and spine. In both areas, sarcoidosis may involve the parenchyma of the brain and spine, nerve roots, the leptomeninges, the dura mater, and the surrounding bony structures. In most cases, the appearance of lesions is nonspecic so that sarcoidosis is included in a broad differential diagnosis [8].

Fig. 3.Contrast-enhanced T1-weighted coronal image shows enhancement and thickening of pituitary infundibulum (arrows) in 37-year-old woman.

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B
Fig. 5.Cranial nerve sarcoidosis in 43-year-old man with diffuse sarcoidosis. Contrast-enhanced T1-weighted parasagittal image shows enhancing lesion causing thickening and enhancement of cisternal segment of third cranial nerve (arrowheads). Note enhancing suprasellar mass (m) and diffuse leptomeningeal enhancement (arrows).

Fig. 4.Optic nerve sarcoidosis. A, Contrast-enhanced fat-saturated T1-weighted axial image of 35-year-old woman shows enhancement of entire visible portions of both optic nerves in orbit and optic canals (arrowheads). Enhancement of extraocular muscles is normal nding. B, Contrast-enhanced fat-saturated T1-weighted coronal image of 26-year-old woman with rapid vision loss shows enhancement and thickening of both optic nerves in prechiasmatic segment (arrows).

this is seen as thickening and enhancement on contrast-enhanced T1-weighted images. This isolated involvement of the infundibulum mimics the appearance of histiocytosis. Hypothalamic and infundibular involvement may present as diabetes insipidus or amenorrhea [13, 14]. Sarcoidosis of the pituitary gland proper has been reported, with nonspecic imaging ndings of a cystic enhancing intrasellar mass extending into the suprasellar space [15]. Cranial nerve involvement.Cranial nerve involvement may occur along with leptomeningeal involvement or as an isolated nding. There is poor correlation between the imaging evidence of cranial nerve involvement and clinical neuropathy, with some patients having clinical symptoms without imaging ndings and many having imaging ndings without clinical symptoms. Clinically, any cranial nerve can be affected, but the most common cranial nerve decit involves the facial nerve (VII), whereas radiographically the optic nerves (II) are most commonly abnormal [2, 11]. The optic nerve involvement (Fig. 4) may occur at the chiasm or intraorbital portions of the optic nerves and may be bilateral or unilateral [16]. The differential diagnosis of isolated optic nerve involvement includes optic neuritis and optic nerve glioma. The dural sheath of the optic nerve can also be involved, mimicking an optic nerve meningioma [2]. Sarcoidosis may involve the orbital fat, muscles, lacrimal glands, or globe with a diffuse inltrative mass radiographically indistinguishable from orbital pseudotumor [17, 18]. The imaging ndings of other cranial nerve involvement

are enlargement of the cranial nerves with enhancement on contrast-enhanced T1-weighted images (Fig. 5). The cranial nerves can also become involved via perineural spread of sarcoidosis from sinonasal disease [19]. Hydrocephalus.Hydrocephalus occurs in 512% of patients with central nervous system involvement of sarcoidosis [2, 7]. This may be a communicating type, presumably due to altered cerebrospinal uid resorption; associated with dural or leptomeningeal involvement; or an obstructive type, due to adhesions or locula-

tions of the ventricular system caused by leptomeningeal or pial involvement (Fig. 6). Isolation of the fourth ventricle (termed trapped fourth ventricle) or other parts of the ventricular system may occur [20]. Dural involvement.Dural involvement by sarcoidosis can present as focal dural masses (Fig. 7) or diffuse dural thickening (Fig. 8). Lesions typically enhance homogeneously on contrast-enhanced T1-weighted images. They are commonly dark on T2-weighted images; this appearance can serve as a clue to the diag-

Fig. 6.Hydrocephalus associated with neurosarcoidosis in 31-year-old man with neurosarcoidosis (same patient as in Fig. 1). A, Contrast-enhanced T1-weighted axial image shows enlargement of lateral ventricles and enhancement in and adjacent to wall of left lateral ventricle. B, Fluid-attenuated inversion recovery MRI obtained at same level as A shows different signal intensity in left and right lateral ventricles, presumably due to elevated protein concentration within left lateral ventricle (L), which is isolated from rest of ventricular system.

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Fig. 8.Diffuse disease in 58-year-old woman with dural sarcoidosis. Contrast-enhanced T1-weighted axial image shows diffuse thickening and enhancement of dura (arrows).

Fig. 7.Focal masses in 31-year-old woman with dural sarcoidosis. A, Contrast-enhanced T1-weighted axial image shows bilateral extraaxial enhancing masses of dura (arrows). B, Fat-saturated T2-weighted axial image shows dural masses to be low signal intensity (arrows), with some high signal of adjacent brain parenchyma, most likely vasogenic edema.

nosis but still may be indistinguishable from calcied meningiomas or very cellular dural metastases [21, 22]. Other differential considerations include lymphoma and idiopathic hypertrophic cranial pachymeningitis. The latter can also be confused pathologically with sarcoidosis because it is also a granulomatous process [11]. Patients with dural involvement typically present with headaches or cranial nerve compression. Dural involvement and leptomeningeal involvement are rarely present together in the same region. Because of the

arachnoid barrier cells, a portion of the arachnoid mater forms a barrier to the spread of disease through the arachnoid membrane [11]. Vasculitis.Histologically, a vasculitis-like pattern of involvement of the intracranial vasculature has been reported, with perivascular granulomatous inltrate. This pattern may be related to autoantibodies to vascular endothelial cells [23, 24]. Nonenhancing white matter lesions seen as areas of high T2 signal intensity on MRI have been attributed to possible vascular involvement [9]. Despite the clear reports of his-

tologic evidence of vascular involvement in central nervous system sarcoidosis, it is rare that brain infarct is a presenting symptom of neurosarcoidosis [2527].
Skull

Sarcoidosis lesions in the skull (Fig. 9) are uncommon. They may be seen in association with other bone disease or as an isolated abnormality. The lesions show well-circumscribed nonsclerotic margins (punched out appearance) and may show increased radiotracer uptake on nuclear medicine bone

Fig. 9.Skull lesions of sarcoidosis. A, Axial CT image with bone window settings in 46-year-old man with sarcoidosis shows well-circumscribed lytic lesion involving inner and outer tables of calvarium, with sharp, nonsclerotic margins (arrow). B, Contrast-enhanced T1-weighted coronal image in 38-year-old woman with diffuse sarcoidosis shows enhancing lesion on patients right that thins inner and outer tables of skull, expanding diploic space (straight arrow). Second lesion on patients left involves only inner table (curved arrow).

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Fig. 10.Spinal cord intramedullary sarcoidosis in 47-year-old man with upper and lower extremity weakness. Contrast-enhanced sagittal T1-weighted image shows enhancing lesion in spinal cord (arrows). Note abnormal linear leptomeningeal enhancement (arrowheads).

Fig. 11.51-year-old man with spinal leptomeningeal sarcoidosis. Contrast-enhanced sagittal T1-weighted images show multiple nodular enhancing lesions along surface of spinal cord and on spinal nerve roots (arrows).

10 scans. MRI may reveal some enhancing soft tissue in the lesion [28, 29].
Spine

11 (Fig. 12) are rare (we found seven reports in the English-language literature) [33, 34]. The extramedullary masslike lesions have a dural base and were described as involving the cervical, thoracic, and lumbar spine without predilection. The lesions are shown on MRI as hypointense or isointense signal on T1-weighted images, hyperintense signal on T2-weighted images, and marked contrast enhancement with a dural tail described in some reports. The characteristic low T2 signal found in dural intracranial sarcoidosis was not described in spinal lesions.

Spinal neurosarcoidosis can cause an array of imaging ndings, which include intramedullary, intradural extramedullary, extradural, vertebral, and disk space lesions. Intramedullary spinal lesions.Intramedullary sarcoidosis is an uncommon manifestation of sarcoidosis, which often causes severe neurologic sequelae. It occurs in less than 1% of sarcoidosis cases [30]. Usually spinal cord involvement is not an isolated or rst manifestation of the disease. This characteristic helps the radiologist presume the diagnosis and helps the patients avoid spinal cord biopsy. From the imaging point of view, intramedullary sarcoidosis is nonspecic with a broad differential diagnosis including neoplasms, multiple sclerosis, and fungal infections. Sarcoidosis spinal lesions usually appear as fusiform enlargements of the spinal cord in the cervical or upper thoracic level (Fig. 10). On MRI, the spinal cord is enlarged with high signal intensity in T2-weighted images, low signal intensity in T1-weighted images, and patchy enhancement after contrast administration. Junger et al. [31] proposed an MRI classication of intraspinal sarcoidosis in four stages correlating with possible histologic stages of the disease: phase 1, early inammation showing linear leptomeningeal enhancement after gadolinium administration along the spinal surface; phase 2, secondary centripetal spread of the leptomeningeal inammatory process through the Virchow-Robin spaces, showing parenchymal

involvement with faint enhancement and diffuse swelling; phase 3, less prominent swelling and possible normal-sized spinal cord, associated with focal or multiple enhancement; and phase 4, resolution of the inammatory process with normal size or atrophy of the spinal cord and no enhancement. Phases 2 and 3 are the most frequent at clinical presentation. Other rare ndings such as calcications, cyst formation, and extradural involvement have also been described. Preoperative suggestion of intraspinal sarcoidosis may alert the pathologist to look carefully for granulomas and giant cells because sarcoidosis can mimic neoplasm and lead to frozen section misinterpretation. The correct diagnosis and early treatment with steroids can minimize neurologic complications and decrease the disease morbidity rates in 42% of the cases. MRI shows posttreatment changes or recurrence on follow-up after steroid therapy. Koike et al. [32] showed that the imaging improvement of intraspinal lesions does not correlate well with clinical improvement and depends on the degree of cord damage. Leptomeningeal and dural lesions.Extramedullary intradural lesions are usually represented by leptomeningeal sarcoidosis inltration, present in up to 60% of spinal cord lesions. Junger et al. [31] proposed that this inltration might be a precursor of intraspinal lesions, as discussed previously. The leptomeningeal involvement may be visualized on contrast-enhanced T1-weighted MRI as thin linear leptomeningeal enhancement or small nodules (Fig. 11). Clinical manifestations are not well correlated with MRI ndings. Extramedullary sarcoid granulomatous masses

Fig. 12.42-year-old woman with spinal dural sarcoidosis. Contrast-enhanced T1-weighted sagittal image shows dural-based mass (m), diffuse nodular thickening, and enhancement of dura (arrows).

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Smith et al. localize additional sites to biopsy and as a tool for monitoring disease activity. The imaging ndings are not specic enough to make the diagnosis of sarcoidosis. In the absence of conrmed disease elsewhere in the body, biopsy is usually necessary to rule out diseases causing similar-appearing lesions such as metastasis, myeloma, lymphoma, and tuberculosis [38].
Treatment
Respir Cell Mol Biol 2002;27:37 5. Mitchell IC, Turk JL. Detection of mycobacterial rRNA in sarcoidosis with liquid-phase hybridization. Lancet 1992;139:10151017 6. Ishige I, Usui Y, Takemura T, Eishi Y. Quantitative PCR of mycobacterial and propionibacterial DNA in lymph nodes of Japanese patients with sarcoidosis. Lancet 1999;354:120123 7. Nowak DA, Widenka DC. Neurosarcoidosis: a review of its intracranial manifestation. J Neurol 2001;248:363372 8. Hayes WS, Sherman JL, Stern BJ, Citrin CM, Pulaski PD. MR and CT evaluation of intracranial sarcoidosis. AJR 1987;149:10431049 9. Dumas JL, Valeyre D, Belin C, et al. Central nervous system sarcoidosis: follow-up at MRI during steroid therapy. Radiology 2000;214:411420 10. Urbach H, Kristof R, Zentner J, Brechtelsbauer D, Solymosi L, Wolf HK. Sarcoidosis presenting as an intra- or extra-cranial mass: report of two cases. Neuroradiology 1997;39:516519 11. Christofordis GA, Spickler EM, Reccio MV, Mehta BM. MR of CNS sarcoidosis: correlation of imaging features to clinical symptoms and response to treatment. AJNR 1999;20:655669 12. Vannemreddy PS, Nanda A, Reddy PK, Gonzales E. Primary cerebral sarcoid granuloma: the importance of denitive diagnosis in the high-risk patient population. Clin Neurol Neurosurg 2002;104:289292 13. Konrad D, Gartenmann M, Martin E, Schoenle EJ. Central diabetes insipidus as the rst manifestation of neurosarcoidosis in a 10-year-old girl. Horm Res 2000;54:98100 14. Lipnick RN, Hung W, Pandian MR. Neurosarcoidosis presenting as secondary amenorrhea in a teenager. J Adolesc Health 1993;14:464467 15. Sato N, Sze G, Kim JH. Cystic pituitary mass in neurosarcoidosis. AJNR 1997;18:11821185 16. Leu NH, Chen CY, Wu CS, et al. Primary chiasmal sarcoid granuloma: MRI. Neuroradiology 1999;41:440442 17. Peterson EA, Hymas DC, Pratt DV, Mortenson SW, Anderson RL, Mamalis N. Sarcoidosis with orbital tumor outside the lacrimal gland: initial manifestation in 2 elderly white women. Arch Ophthalmol 1998;116:804806 18. Carmody RF, Mafee MF, Goodwin JA, Small K, Haery C. Orbital and optic pathway sarcoidosis: MR ndings. AJNR 1994;15:775783 19. Mazziotti S, Gaeta M, Blandino A, Vinci S, Pandolfo I. Perinueral spread in a case of sinonasal sarcoidosis: case report. AJNR 2001:22:12071208 20. Hesselmann V, Wedekind C, Terstegge K, et al. An isolated fourth ventricle in neurosarcoidosis: MRI ndings. Eur Radiol 2002;12[suppl]:S1S3 21. Seltzer S, Mark AS, Atlas SW. CNS sarcoidosis: evaluation with contrast-enhanced MRI. AJNR 1991;12:12271233 22. Larner AJ, Ball JA, Howard RS. Sarcoid tumor: continuing diagnostic problems in the MRI era. J Neurol Neurosurg Psychiatry 1999;66:510512 23. Thiel HJ, Korenke HD. Generalized sarcoidosis with brain and eye participation [in German]. Ophthalmologica 1975;170:393404 24. Tsukada N, Yanagisawa N, Mochizuki I. Endothelial cell damage in sarcoidosis and neurosarcoidosis: autoantibodies to endothelial cells. Eur

Fig. 13.37-year-old man with spinal vertebral sarcoidosis. Contrast-enhanced fat-saturated sagittal T1-weighted image shows multiple enhancing lesions in bodies and spinous processes of lumbar vertebrae (straight arrows). Note diffuse leptomeningeal disease with nodular thickening and enhancement of lumbar nerve roots (curved arrows).

Clinical manifestations are usually present at the time of diagnosis and result from local nerve or spinal cord compression. Surgical resection is usually necessary to alleviate the compression symptoms, followed by treatment with steroids. The differential diagnosis for the imaging ndings includes meningioma, nerve sheath tumors, lymphoma, carcinomatous metastasis, chloroma, hemangiopericytoma, and other granulomatous depositions. Bone involvement.Osseous involvement in sarcoidosis is reported in 113% of patients [3537]. The actual frequency is probably higher because most of the osseous lesions are asymptomatic and patients are not screened routinely. Small tubular bones of the hands and feet are most commonly involved. Less common skeletal involvement includes the long tubular bones, skull, ribs, spine, and pelvis [35]. Vertebral lesions are rare and usually occur in the lower thoracic and upper lumbar spine. Clinical manifestations include pain, tenderness, and neuralgia. The imaging ndings are usually multiple well-dened lytic lesions with sclerotic margins in the vertebral body. They may extend into the pedicles and paraspinal region. Sclerotic lesions (mimicking blastic metastasis), mixed lytic and sclerotic involvement, and disk involvement are rare and are described in a few reports [36, 37]. MRI shows multiple lesions with low T1 signal, high T2 signal, and enhancement after contrast administration or low T1 and T2 signal in sclerotic lesions (Fig. 13). Bone scintigraphy has potential utility to

There is no consensus on the best treatment or efcacy of treatment of neurosarcoidosis. Treatment of symptomatic patients is usually begun with high-dose corticosteroids. If the patient responds to treatment, steroid dosage may be lowered for a maintenance period or even discontinued [3]. Correlation between symptom resolution and resolution of imaging nding is poor, especially with spinal cord lesions [32]. There is a high rate of progression or recurrent symptoms and recurrent imaging ndings. Patients with enhancing brain lesions show worsening, no change, or recurrence of symptoms with treatment in 75% of cases [11]. Methotrexate has been used as a secondline treatment, although the effectiveness has not been clearly established [3].
Conclusion

Central nervous system involvement occurs in a signicant proportion (525%) of patients with systemic sarcoidosis. Patients with systemic disease may initially present with neurologic symptoms, and rarely, disease may be isolated to the central nervous system. The typical imaging feature is thickening and enhancement of the leptomeninges, especially around the base of the brain. Other imaging ndings, such as enhancing or nonenhancing parenchymal lesions and dural and bone lesions also occur in the head and spine. There is a high rate of progression and recurrence after treatment so that imaging follow-up is recommended.

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