Experimental superficial siderosis of the central nervous system: biochemical correlates

Koeppen, Arnulf H.; Hurwitz, Craig G.; Dearborn, Richard E.; Dickson, Andrew C.; Borke, Rosemary C.; Chu, Richard C.

doi:10.1016/0022-510x(92)90129-9

Cited by 53 publications

(31 citation statements)

References 24 publications

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“…Heavy hemosiderin deposition of anterior horn cells has been observed in rabbit models of superficial siderosis, however, there was little in the way of neuronal fallout. 21,22 Disruption of the blood-spinal cord barrier has been demonstrated in ALS-linked superoxide dismutase 1 mutant mouse models 23,24 that have resulted in microhemorrhages with release of toxic hemoglobulin-derived products. Hemosiderin deposits in the motor neurons and surrounding glia of the spinal cord were present even before the development of motor neuron loss.…”

Section: Discussionmentioning

confidence: 98%

Superficial Siderosis Mimicking Amyotrophic Lateral Sclerosis

Driver‐Dunckley¹,

Hoxworth²,

Patel³

et al. 2010

Journal of Clinical Neuromuscular Disease

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We report a case of superficial siderosis erroneously diagnosed as amyotrophic lateral sclerosis. The patient's symptoms began 18 years prior with unilateral upper extremity weakness, fasciculations, and hyperreflexia. The patient then developed ataxia and hearing loss 15 years after his original symptoms. The magnetic resonance images revealed superficial siderosis involving the spinal cord and brain. We want to attract attention to superficial siderosis as a rare amyotrophic lateral sclerosis mimic disorder.

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Section: Discussionmentioning

confidence: 98%

Superficial Siderosis Mimicking Amyotrophic Lateral Sclerosis

Driver‐Dunckley¹,

Hoxworth²,

Patel³

et al. 2010

Journal of Clinical Neuromuscular Disease

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“…The ability of the brain to biosynthesize ferritin in response to prolonged contact with hemoglobin iron is important in the pathogenesis of SS. [4][5][6][7] Accelerated ferritin synthesis in the Bergmann glia of the cerebellum may account for preferential cerebellar involvement. 5 The classic clinical presentation of SS includes adult-onset slowly progressive gait (less commonly appendicular) ataxia with cerebellar dysarthria and sensorineural hearing impairment.…”

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confidence: 99%

Neuroimaging in Superficial Siderosis: An In-Depth Look

Kumar¹

2009

AJNR Am J Neuroradiol

153

161

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SUMMARY:Despite extensive imaging, a source of bleeding is often not evident during the evaluation of patients with superficial siderosis (SS) of the central nervous system. An intraspinal fluid-filled collection of variable dimensions is frequently seen on spine MR imaging in patients with idiopathic SS. A similar finding has also been reported in patients with craniospinal hypotension. This review discusses the role of multitechnique imaging in the work-up of patients with SS and focuses on recent developments.

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“…Early case reports noted selective deposition of hemosiderin in the cerebellum, brainstem, ventricle and spinal cord, which explains the clinical presentation of cases. These areas are prone because of localization of special cells (Bergman cells), within the cerebellum, brainstem and spinal cord [8,9], which attract deposition of hemosiderin. The ability of these special cells to biosynthesize ferritin in response to prolonged contact with hemoglobin iron is important in the pathogenesis of superficial siderosis.…”

Section: Discussionmentioning

confidence: 99%

Superficial Siderosis of the CNS and Otoneurological Manifestations

Ramadass

Narayanan

Dhanraj

et al. 2011

Indian J Otolaryngol Head Neck Surg

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Superficial siderosis is an uncommon condition occurring in central nervous system (CNS) due to deposition of hemosiderin in the subpial meningeal layer causing progressive neurological dysfunction. The classical clinical manifestation is a triad of chronic ataxia, bipyramidal signs and progressive bilateral symmetrical sensorineural hearing loss (SNHL). It has rarely been reported in Indian literature. We report an unusual case of superficial siderosis in a 60-year-old farmer who presented with the above triad along with involvement of olfactory nerve. We present this case to highlight the fact that progressive SNHL can be an important sign for the early awareness of this rare disorder. The literature on superficial siderosis is reviewed and the pathogenesis is discussed.

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Experimental superficial siderosis of the central nervous system: biochemical correlates

Cited by 53 publications

References 24 publications

Superficial Siderosis Mimicking Amyotrophic Lateral Sclerosis

Superficial Siderosis Mimicking Amyotrophic Lateral Sclerosis

Neuroimaging in Superficial Siderosis: An In-Depth Look

Superficial Siderosis of the CNS and Otoneurological Manifestations

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