Neurology India Medknow Publications on behalf of the Neurological Society of India ISSN: 0028-3886 EISSN: 1998-4022 Vol. 58, Num. 4, 2010, pp. 673-675

Neurology India, Vol. 58, No. 4, July-August, 2010, pp. 673-675

Letter To Editor

Neurosarcoidosis: An uncommon presentation

Roopesh Kumar VR, Gopalakrishnan MS, Shankar Ganesh CV, Negi VirSingh, Elangovan S

Department of Neurosurgery, Jawaharlal Institute of Postgraduate Medical Education and Research (JIPMER), Dhanvanthri Nagar, Pondicherry

Correspondence Address:Department of Neurosurgery, Jawaharlal Institute of Postgraduate Medical Education and Research (JIPMER), Dhanvanthri Nagar, Pondicherry, roops1975@rediffmail.com

Date of Acceptance: 08-Jul-2010

Code Number: ni10183

PMID: 20739827

DOI: 10.4103/0028-3886.68695

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.

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

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

References

Copyright 2010 - Neurology India

The following images related to this document are available:

Photo images