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LETTER TO EDITOR
Year : 2010  |  Volume : 58  |  Issue : 4  |  Page : 673-675

Neurosarcoidosis: An uncommon presentation


1 Department of Neurosurgery, Jawaharlal Institute of Postgraduate Medical Education and Research (JIPMER), Dhanvanthri Nagar, Pondicherry, India
2 Department of Clinical Immunology, Jawaharlal Institute of Postgraduate Medical Education and Research (JIPMER), Dhanvanthri Nagar, Pondicherry, India
3 Department of Radiodiagnosis, Jawaharlal Institute of Postgraduate Medical Education and Research (JIPMER), Dhanvanthri Nagar, Pondicherry, India

Date of Acceptance08-Jul-2010
Date of Web Publication24-Aug-2010

Correspondence Address:
V R Roopesh Kumar
Department of Neurosurgery, Jawaharlal Institute of Postgraduate Medical Education and Research (JIPMER), Dhanvanthri Nagar, Pondicherry
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0028-3886.68695

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How to cite this article:
Roopesh Kumar V R, Gopalakrishnan M S, Shankar Ganesh C V, Negi VS, Elangovan S. Neurosarcoidosis: An uncommon presentation. Neurol India 2010;58:673-5

How to cite this URL:
Roopesh Kumar V R, Gopalakrishnan M S, Shankar Ganesh C V, Negi VS, Elangovan S. Neurosarcoidosis: An uncommon presentation. Neurol India [serial online] 2010 [cited 2019 Aug 24];58:673-5. Available from: http://www.neurologyindia.com/text.asp?2010/58/4/673/68695


Sir,

A 21-year male presented with history of progressive headache, difficulty in walking, and decrease in vision and hearing of six years duration. On examination he had bilateral secondary optic atrophy, bilateral sensorineural hearing loss, and spastic quadriparesis. Magnetic resonance imaging (MRI) of brain showed multiple dural-based nodular lesions in the middle and posterior cranial fossae, which enhanced homogenously with contrast. There was diffuse thickening and enhancement of the basal meninges extending into the cervical region [Figure 1]. Nodular lesions were isointense on T1- and hypointense on T2-weighted images (T2 inversion) [Figure 2]. Similar lesions were also found in both maxillary sinuses. Computed tomography (CT) of thorax and ultrasound of abdomen were normal. Endoscopic biopsy of the maxillary sinus lesions revealed noncaseating granuloma with chronic inflammatory cell infiltrate and plasma cells [Figure 3]. A diagnosis of neurosarcoidosis was entertained in view of the histopathological features, elevated angiotensin converting enzyme (ACE) level (61.44 U/l) and hypercalcemia. After 6 months of immunomodulatory therapy with prednisolone, hydroxychloroquine, and methotrexate, MRI brain showed significant resolution of the granulomas [Figure 4] with intensification of the T2 inversion [Figure 5]. Clinically, gait and hearing improved and the patient is at present leading an independent life.
Figure 1 :Pretreatment gadolinium-enhanced MRI images. Axial (a), coronal (b), and sagittal (c) views show bilateral dural-based enhancing nodular lesions in the anterior, middle, and posterior cranial fossae, with thickening of cervical dura

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Figure 2 :Pretreatment MRI T2-weighted images. Axial view (a) and coronal view (b) show iso- to hypointense dural-based lesions, with significant brainstem compression

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Figure 3 :Histopathology: Histological section from the maxillary sinus lesion showing non-caseating epitheliod cell granuloma (H and E stain, x400)

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Figure 4 :Gadolinium-enhanced MRI brain done 6 months post treatment. Axial (a), coronal (b), and sagittal (c) views show marked resolution of the granulomatous lesions, with normalization of cervical dura

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Figure 5 :Post-treatment MRI T2-weighted images. Axial (a) and coronal (b) images show increased T2 inversion of the lesions

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Neurosarcoidosis is a relatively rare disorder, with diverse clinical manifestations. Cranial neuropathy, aseptic meningitis and hypothalamic dysfunction are among the common presenting features. [1],[2],[3] Of these patients, 5%-10% present with intracranial extra-axial mass lesions. [3],[4] Biopsy of such lesions is required to establish the diagnosis if there is no associated extracranial involvement. A similar pseudotumor-like presentation has been reported earlier. [5] Most patients respond well to long-term corticosteroids. [2],[3] A small subgroup may require other immunosuppressive therapy. Long-term follow-up is required. [3]


  Acknowledgement Top


Prof. Gopalakrishnan, S. Professor and Head, Department of ENT, JIPMER, Pondicherry

 
  References Top

1.Stern BJ, Krumholz A, Johns C, Scott P, Nissim J. Sarcoidosis and its neurological manifestations. Arch Neurol 1985;42:909-17.   Back to cited text no. 1  [PUBMED]  [FULLTEXT]  
2.Spencer TS, Campellone JV, Maldonado I, Huang N, Usmani Q, Reginato AJ. Clinical and magnetic resonance imaging manifestations of neurosarcoidosis. Semin Arthritis Rheum 2005;34:649-61.  Back to cited text no. 2      
3.Lexa FJ, Grossman RI. MR of sarcoidosis in the head and spine: Spectrum of manifestations and radiographic response to steroid therapy. AJNR Am J Neuroradiol 1994;15:973-82.   Back to cited text no. 3  [PUBMED]    
4.Pickuth D, Heywang-Kφbrunner SH. Neurosarcoidosis: Evaluation with MRI. J Neuroradiol 2000;27:185-8.  Back to cited text no. 4      
5.Ranoux D, Devaux B, Lamy C. Meningeal sarcoidosis,pseudo-meningioma and pachymeningitis of the convexity. J Neurol Neurosurg Psychiatry 1992;55:300-3.  Back to cited text no. 5      


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  [Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5]



 

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