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Superficial Siderosis 58 Images in Neurology Superficial siderosis Sameer Vyas, Suresh Giragani, Paramjeet Singh, Anil Bansali1, Niranjan Khandelwal Departments of Radiodiagnosis, and 1Endocrinology, Postgraduate Institute of Medical Education and Research, Chandigarh, India For correspondence: Dr. Sameer Vyas, Department of Radiodiagnosis and Imaging, Postgraduate Institute of Medical Education and Research, Chandigarh. India. E-mail: [email protected] Ann Indian Acad Neurol 2011;14:58-9 Introduction sensorineural hearing loss. Magnetic Resonance Imaging (MRI) revealed hemorrhagic lesion in sellar-suprasellar region A 40-year-old male presented with complaints of difficulty suggestive of pituitary adenoma [Figures 1 and 3]. There was in walking, decreased cognitive functions, and hardness of cerebellar atrophy with extensive hypointenities involving hearing since one year. Neurological examination revealed leptomeniges predominantly involving structures of posterior deficiency in cognitive functions of higher intellectual fossa on T2 and FLAIR sequences consistent with superficial functions. The gait was markedly ataxic with abnormal tests siderosis [Figures 1-4]. Post-operative histopathology of the for cerebellar function. Audiometry demonstrated bilateral sellar-suprasellar lesion showed pituitary adenoma. Superficial siderosis is a rare chronic progressive neurological dysfunction characterized by classical triad of symptoms consisting of sensorineural hearing loss, cerebellar ataxia, Figure 2: Axial T1WI MR images showing dark outline of the dural surfaces, cerebellum (white arrow) and brainstem (black arrow). Figure 1: Axial T2WI MR showing intense hemosiderin outlining the dural surfaces, cerebellum (white arrow), brainstem (black arrow), and cervical spinal cord (curved white arrow). In addition, large heterogeneous sellar and suprasellar mass is also seen (asterisk) Access this article online Quick Response Code: Website: www.annalsofian.org DOI: Figure 3: Coronal T2 FLAIR images showing large heterogeneous 10.4103/0972-2327.78055 sellar-suprasellar mass (asterisk) and low signal along and cerebellar surfaces (white arrows) Annals of Indian Academy of Neurology, January-March 2011, Vol 14, Issue 1 Vyas, et al.: Superficial siderosis 59 mimics a degenerative cerebellar disorder. [3] Hypointense linear low signal (rim) on T2 images outlining the contours of brain and cranial nerves is the characteristic imaging finding. There is predisposition of CNS structures like cerebellum, brainstem, and spinal cord likely due to the presence of specialized heme absorbing ferritin-producing glial cells in these organs.[2] An intraspinal fluid-filled collection is frequently seen on spine MR imaging in patients with idiopathic siderosis.[3] Treatment of siderosis is identification and treatment of the underlying cause. Surgical removal of source of the bleeding is mainstay in treatment and medical therapy with chelating agents is controversial. With the advent of neuroimaging, this unusual entity can be diagnosed early in the course at which stage it is reversible. Figure 4: Susceptibility-weighted axial images revealing intense low signal along brainstem (white arrow) and cerebellar surfaces (black arrow) in the posterior fossa References and myelopathy. [1-3] There is deposition of blood breakdown 1. Levy M, Turtzo C, Linas RH. Superficial siderosis: A case report products (hemosiderin) from a source of bleeding in and review of literature. Nat Clin Pract Neurol 2007;3:54-8. subarachnoid space in the subpial layer of the central nervous 2. Leussink VI, Flachenecker P, Brechtelsbauer D, et al. Superficial system (CNS). Common causes of superficial siderosis include siderosis of the central nervous system: Pathogenetic heterogeneity and therapeutic approaches. Acta Neurol Scand 2003;107:54-61. intracranial tumors (21%), head or back trauma (13%), and 3. Kumar N. Neuroimaging in superficial siderosis: An in-depth look. [1] arteriovenous malformations or aneurysms (9%). Other less AJNR Am J Neuroradiol 2010;31 5-14. common causes include post-surgical changes, brachial plexus injury, amyloid angiopathy, and chronic subdural hematoma. Received: 06-04-10, Revised: 15-04-10, Accepted: 11-08-10 However, despite extensive imaging, a source of bleeding may not be evident in 35% of cases.[1,3] The clinical presentation closely Source of Support: Nil, Conflict of Interest: Nil Annals of Indian Academy of Neurology, January-March 2011, Vol 14, Issue 1 Copyright of Annals of Indian Academy of Neurology is the property of Medknow Publications & Media Pvt. Ltd. and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use. Copyright of Annals of Indian Academy of Neurology is the property of Medknow Publications & Media Pvt. Ltd. and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use..
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