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LETTER TO EDITOR
Year : 2012  |  Volume : 60  |  Issue : 6  |  Page : 648-649

Superficial siderosis in a patient with filum terminale paraganglioma


1 Department of Neurology, Sanjay Gandhi Postgraduate Institute of Medical Sciences,Lucknow, Uttar Pradesh, India
2 Department of Radiology, Sanjay Gandhi Postgraduate Institute of Medical Sciences,Lucknow, Uttar Pradesh, India
3 Department of Pathology, Sanjay Gandhi Postgraduate Institute of Medical Sciences,Lucknow, Uttar Pradesh, India

Date of Web Publication29-Dec-2012

Correspondence Address:
Vimal K Paliwal
Department of Neurology, Sanjay Gandhi Postgraduate Institute of Medical Sciences,Lucknow, Uttar Pradesh
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0028-3886.105206

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How to cite this article:
Kinge NG, Paliwal VK, Neyaz Z, Verma R. Superficial siderosis in a patient with filum terminale paraganglioma. Neurol India 2012;60:648-9

How to cite this URL:
Kinge NG, Paliwal VK, Neyaz Z, Verma R. Superficial siderosis in a patient with filum terminale paraganglioma. Neurol India [serial online] 2012 [cited 2019 Aug 23];60:648-9. Available from: http://www.neurologyindia.com/text.asp?2012/60/6/648/105206


Sir,

Superficial siderosis is an acquired neurodegenerative disease caused by subpial deposition of hemosiderin in brain and spinal cord secondary to chronic leakage of blood into the cerebrospinal space. It presents with triad of progressive sensorineural hearing loss, cerebellar ataxia, and myelopathy. [1]

A 42-year-old male complained of progressive hearing loss, slowness of activities, apathy, gait-difficulty, and urinary incontinence of 1 year duration. On examination, patient had marked apathy, poor attention, perseveration, and impairment of recent memory. He had spastic dysarthria and bilateral sensorineural hearing loss. Motor examination revealed grade 3 spasticity (modified Ashworth scale) in all four limbs with normal power, brisk deep tendon jerks, and both planter extensor. Dysmetria and gait ataxia was present. Magnetic resonance imaging (1.5 Tesla) of brain and spine revealed superficial hypointese layer overlying whole of spinal cord, brainstem, cerebellar folia especially over vermis, Sylvian and interhemispheric fissures, and left seventh-eighth nerve complex. Cerebral and spinal cord atrophy was also seen [Figure 1] and [Figure 2]. MRI lumbar spine showed an isointense intraspinal mass opposite L4 vertebra with peripheral hypointense rim with intense homogenous contrast enhancement. Axial T2-weighted sections showed lumbar nerve root clumping suggestive of arachnoiditis. The resected spinal tumor was diagnosed as paraganglioma. No significant improvement in cognitive/motor functions was noticed in the early postoperative period.
Figure 1: T2-weighted composed-MRI spine shows generalized cord atrophy and mass in lumbar region (a) T2-weighted image cervical spine (TURBO SPIN ECHO, TR 3800, TE 94, Slice thickness 3 mm, FOV 330/330 mm, bandwidth 150) shows hypointense rim covering spinal cord and brain stem (b) T2-weighted lumbar spine (TURBO SPIN ECHO, TR 3000, TE 99, Slice thickness 3 mm, FOV 350/350 mm, bandwidth 200) shows isointense intradural mass at L4 (c) with intense contrast enhancement (TURBO SPIN ECHO, TR 500, TE 9.3, Slice thickness 3 mm, FOV 360/360 mm, bandwidth 190) (d) Clumping of cauda equina nerve roots (e)

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Figure 2: Superficial hypointense coating is noted over brain stem and cerebellum (a) cerebral cortex in bilateral Sylvian fissure and anterior interhemispheric fissure (b) left seventh-eighth nerve complex (c) and cerebellar vermis (d) Prominence of bilateral lateral ventricles is present with prominent fissures suggestive of cerebral atrophy (b) (TURBO SPIN ECHO, TR 3800, TE 100, Slice thickness 5 mm, FOV 200/250 mm, bandwidth 160)

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Several disorders like trauma, prior neurosurgical intervention, vascular malformations, dural leaks and spinal tumors, etc., are known to produce superficial siderosis. [1],[2] Excessive biosynthesis of ferritin by brain/spinal cord owing to prolonged contact with heme iron is thought to produce superficial siderosis. [3]

 
  References Top

1.Kumar N. Superficial siderosis: Associations and therapeutic implications. Arch Neurol 2007;64:491-6.  Back to cited text no. 1
[PUBMED]    
2.Sharma A, Gaikwad SB, Goyal M, Mishra NK, Sharma MC. Calcified filum terminale paraganglioma causing superficial siderosis case report. AJR Am J Roentgenol 1998;170:1650-2.  Back to cited text no. 2
[PUBMED]    
3.Koeppen AH, Dickson AC, Chu RC, Thach RE. The pathogenesis of superficial siderosis of the central nervous system. Ann Neurol 1993;34:646-53.  Back to cited text no. 3
[PUBMED]    


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  [Figure 1], [Figure 2]

This article has been cited by
1 Lumbar paraganglioma
Erika Dillard-Cannon,Kofi-Buaku Atsina,George Ghobrial,Esteban Gnass,Mark T. Curtis,Joshua Heller
Journal of Clinical Neuroscience. 2016; 30: 149
[Pubmed] | [DOI]



 

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