Leptomeningeal Neurosarcoidosis

Case:
# 1089547623
Section:
Author(s):

Donato, Angel MD. Huapaya, Janice MD. Figueroa, Ramon E. MD. FACR


Augusta University. Augusta, GA 30912 USA

Email:donatoangel@yahoo.com

Patient:
Female, 38 year(s)

CLINICAL HISTORY

38 year old female with history of syncope landing on face with nose bleed.


IMAGING FINDINGS

MRI shows subtle FLAIR signal abnormality in the subarachnoid spaces along the interhemispheric fissure, lateral sulcal spaces and sylvian fissures bilaterally.

Ventricles and basal cisterns are otherwise normal. There is normal gray matter white

matter differentiation with no acute cytotoxic or vasogenic edema by

FLAIR or diffusion-weighted images. There is no active diffusion

restriction to suggest acute infarct.


Gadolinium-enhanced images demonstrate extensive superficial linear

and nodular enhancement within the sulcal spaces on both cerebral

hemispheres and cerebellar hemispheres and to a lesser extent

interpeduncular cistern

IMAGES

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DISCUSSION

Sarcoidosis is a multisystem granulomatous disease manifested by the development of noncaseating epithelioid granulomas of unknown etiology(1, 2). The cause is likely to be multifactorial. There is evidence that sarcoidosis is caused by intense immune processes, a role of CD4 T cells and Th1 cells has been widely accepted.(3, 4)


Neurosarcoidosis(NS) refers to sarcoidosis involvement at any level of the neuraxis (4), Involvement of the CNS occurs in 25% of patients with sarcoidosis being symptomatic in about 5% of the patients.


The most frequent clinical manifestations of NS is cranial neuropathy being the most commonly affected the seventh and the eighth cranial nerves.

Leptomeningeal involvement is seen as thickening and enhancement of leptomeninges it can spread to perivascular spaces, cortical sulci, and basal cisterns. These findings are indistinguishable from tuberculosis or lymphoma involving the leptomeninges (4)

The diagnosis should be suspected in patients with sarcoidosis who develop neurological symptoms or those presenting with syndromes highly suggestive of NS. (3)


Sarcoidosis is also known as the ¨great imitator¨ due to its propensity to radiologically mimic a variety of pathologic diseases(2).

Enhancement on contrast-enhanced magnetic resonance imaging (MRI) plays an important role in the diagnosis of NS being the modality of choice. Contrast administration helps by demonstrating leptomeningeal nodular or diffuse enhancement as well as enhancing parenchymal masses, enhancement of the leptomeninges and thickening of cranial nerves.

Pituitary and hypophyseal enhancement and thickening of the infundibulum is another finding. Hydrocephalus is an infrequent but important manifestation of NS. 

Spine MRI shows a fusiform, focal or diffuse enhancement, atrophy and enhancing in nerve roots(4)

T2 and FLAIR white matter lesions in a periventricular distribution are the most frequent magnetic resonance abnormalities described in NS. 

Spine MRI shows a fusiform, focal or diffuse enhancement, atrophy and enhancing in nerve roots(4)

Definitive diagnosis relies on histopathologic analysis with the presence of characteristic noncaseating granulomas. CSF analysis may show nonspecific lymphocytic in filtration or elevated oligoclonal immunoglobulin bands similar to multiple sclerosis (1)


Corticosteroids therapy is the mainstay of treatment, Other immunomodulating therapies, such as methotrexate, azathioprine, cyclophosphamide A, hydroxychloroquine and mycophenolate mofetil, are often used. More recently, anti-TNF alpha agents such as infliximab and adalimumab have been used, where patients are refractory to other agents and unable to tolerate corticosteroids.

Rituximab, a monoclonal anti-CD20 antibody that depletes circulating B lymphocytes, and ustekinumab, a human monoclonal antibody that neutralizes the Th1 polarizing cytokine IL-12, has also been used(2).

Leptomeningeal Neurosarcoidosis

Tuberculous meningitis

Leptomeningeal metastases

Lymphoma leukemia infiltration

References

1] Chapman MN, Fujita A, Sung EK, Siegel C, Nadgir RN, Saito N, Sakai O. (2017) Sarcoidosis in the Head and Neck: An Illustrative Review of Clinical Presentations and Imaging Findings.. AJR Am J Roentgenol Jan;208(1):66-75.

[2] O'Connell K, Williams L, Jones J, McCabe DJ, Murphy D, Killeen R, Tubridy N, O'Riordan S, McGuigan C. (2017) Neurosarcoidosis: clinical presentations and changing treatment patterns in an Irish Caucasian population. Ir J Med Sci 2017 Jan 18.

[3] Ibitoye RT, Wilkins A, Scolding NJ. (2016) Neurosarcoidosis: a clinical approach to diagnosis and management.. J Neurol. 2016 Nov 22

[4] Bathla G, Singh AK, Policeni B, Agarwal A (2016) Imaging of neurosarcoidosis: common, uncommon, and rare. Clin Radiol. Jan;71(1):96-106

citation

Author(s):

Donato, Angel MD. Huapaya, Janice MD. Figueroa, Ramon E. MD. FACR


Augusta University. Augusta, GA 30912 USA

Email:donatoangel@yahoo.com

Case Title:
Leptomeningeal Neurosarcoidosis
URL:
http://neuroradiologysilan.com/case/1089547623

Published:
22.09.2017
Section:
Brain |
Views:
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