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Superficial siderosis.

Vyas S, Giragani S, Singh P, Bansali A, Khandelwal N - Ann Indian Acad Neurol (2011)

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Affiliation: Department of Radiodiagnosis, Postgraduate Institute of Medical Education and Research, Chandigarh, India.

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Neurological examination revealed deficiency in cognitive functions of higher intellectual functions... Magnetic Resonance Imaging (MRI) revealed hemorrhagic lesion in sellar-suprasellar region suggestive of pituitary adenoma [Figures 1 and 3]... There was cerebellar atrophy with extensive hypointenities involving leptomeniges predominantly involving structures of posterior fossa on T2 and FLAIR sequences consistent with superficial siderosis [Figures 1–4]... Superficial siderosis is a rare chronic progressive neurological dysfunction characterized by classical triad of symptoms consisting of sensorineural hearing loss, cerebellar ataxia, and myelopathy... The clinical presentation closely mimics a degenerative cerebellar disorder... 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... An intraspinal fluid-filled collection is frequently seen on spine MR imaging in patients with idiopathic siderosis... 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.

No MeSH data available.


Susceptibility-weighted axial images revealing intense low signal along brainstem (white arrow) and cerebellar surfaces (black arrow) in the posterior fossa
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Figure 0004: Susceptibility-weighted axial images revealing intense low signal along brainstem (white arrow) and cerebellar surfaces (black arrow) in the posterior fossa

Mentions: A 40-year-old male presented with complaints of difficulty in walking, decreased cognitive functions, and hardness of hearing since one year. Neurological examination revealed deficiency in cognitive functions of higher intellectual functions. The gait was markedly ataxic with abnormal tests for cerebellar function. Audiometry demonstrated bilateral sensorineural hearing loss. Magnetic Resonance Imaging (MRI) revealed hemorrhagic lesion in sellar-suprasellar region suggestive of pituitary adenoma [Figures 1 and 3]. There was cerebellar atrophy with extensive hypointenities involving leptomeniges predominantly involving structures of posterior fossa on T2 and FLAIR sequences consistent with superficial siderosis [Figures 1–4]. Post-operative histopathology of the sellar-suprasellar lesion showed pituitary adenoma.


Superficial siderosis.

Vyas S, Giragani S, Singh P, Bansali A, Khandelwal N - Ann Indian Acad Neurol (2011)

Susceptibility-weighted axial images revealing intense low signal along brainstem (white arrow) and cerebellar surfaces (black arrow) in the posterior fossa
© Copyright Policy - open-access
Related In: Results  -  Collection

License
Show All Figures
getmorefigures.php?uid=PMC3108083&req=5

Figure 0004: Susceptibility-weighted axial images revealing intense low signal along brainstem (white arrow) and cerebellar surfaces (black arrow) in the posterior fossa
Mentions: A 40-year-old male presented with complaints of difficulty in walking, decreased cognitive functions, and hardness of hearing since one year. Neurological examination revealed deficiency in cognitive functions of higher intellectual functions. The gait was markedly ataxic with abnormal tests for cerebellar function. Audiometry demonstrated bilateral sensorineural hearing loss. Magnetic Resonance Imaging (MRI) revealed hemorrhagic lesion in sellar-suprasellar region suggestive of pituitary adenoma [Figures 1 and 3]. There was cerebellar atrophy with extensive hypointenities involving leptomeniges predominantly involving structures of posterior fossa on T2 and FLAIR sequences consistent with superficial siderosis [Figures 1–4]. Post-operative histopathology of the sellar-suprasellar lesion showed pituitary adenoma.

View Article: PubMed Central - PubMed

Affiliation: Department of Radiodiagnosis, Postgraduate Institute of Medical Education and Research, Chandigarh, India.

AUTOMATICALLY GENERATED EXCERPT
Please rate it.

Neurological examination revealed deficiency in cognitive functions of higher intellectual functions... Magnetic Resonance Imaging (MRI) revealed hemorrhagic lesion in sellar-suprasellar region suggestive of pituitary adenoma [Figures 1 and 3]... There was cerebellar atrophy with extensive hypointenities involving leptomeniges predominantly involving structures of posterior fossa on T2 and FLAIR sequences consistent with superficial siderosis [Figures 1–4]... Superficial siderosis is a rare chronic progressive neurological dysfunction characterized by classical triad of symptoms consisting of sensorineural hearing loss, cerebellar ataxia, and myelopathy... The clinical presentation closely mimics a degenerative cerebellar disorder... 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... An intraspinal fluid-filled collection is frequently seen on spine MR imaging in patients with idiopathic siderosis... 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.

No MeSH data available.