Superficial Siderosis of the CNS and Otoneurological Manifestations

Ramadass, T.; Narayanan, Nithya; Dhanraj, Manivannan; Chidambarnathan, N.

doi:10.1007/s12070-011-0152-7

Cited by 3 publications

(3 citation statements)

References 13 publications

(16 reference statements)

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“…Concurrent presentations of ataxia and vestibular impairment should alert clinicians to possible diagnosis of SSCNS (Table 1 ). The focused deficit of the eighth cranial nerve, and seen with the olfactory nerve, highlights the pathophysiology of SSCNS ( 37 ). The vulnerability of axonal damage results from the long distance it travels outside of the brain within the pontine cistern before it enters the internal acoustic canal.…”

Section: Vestibulopathymentioning

confidence: 99%

Focused Neuro-Otological Review of Superficial Siderosis of the Central Nervous System

et al. 2018

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BackgroundInfratentorial siderosis (iSS) is a progressive degenerative disorder targeting primarily the cerebellum and cranial nerve eighth; therefore, progressive ataxia and its neuro-otological findings are common. Toxicity from hemosiderin involves selectively vulnerable neurons and glia in these posterior fossa structures. Other neurologic findings may be present, though our focus relates to the cochlea-vestibular cerebellar involvement. Radiographic evidence of siderosis may be the result of recurrent, albeit covert bleeding in the subarachnoid space, or the consequence of an overt post-traumatic or aneurysmal subarachnoid hemorrhage (SAH). The radiographic iSS appearance is identical regardless of the SAH cause. A recent study provides compelling evidence to search and correct possible hemorrhage sources in the spinal canal. The removal of residual existing hemosiderin deposits that may potentially cause clinical symptoms remains as a major therapeutic challenge.MethodsWe reviewed large data sources and identified salient papers that describe the pathogenesis, clinical and neurotologic manifestations, and the radiographic features of iSS.ResultsThe epidemiology of iSS is unknown. In a recent series, clinically evident iSS was associated with recurrent SAH; by contrast, in a follow-up period ranging from weeks up to 11 years after a monophasic episode of SAH, radiographic siderosis was clinically silent. However, the post-aneurysmal or post-trauma SAH sample size in this single study was small and their observation period relatively short; moreover, the burden of intraneuronal hemosiderin is likely greater with recurrent SAH. There are a few reports of late iSS, several decades after traumatic SAH. A recent report found subjective hearing loss in aneurysmal SAH individuals with radiographic siderosis. Only in recent years, it is safe to perform magnetic resonance imaging (MRI) in post-aneurysmal SAH, because of the introduction of titanium, MRI-compatible aneurysm clips.ConclusioniSS can be associated with significant neurotologic and cerebellar morbidity; the recurrent SAH variant is frequently clinically symptomatic, has a shorter latency and greater neurotologic disability. In these cases, a thorough search and management of a covert source of bleeding may stop clinical progression. The frequency and clinical course of radiographic iSS after traumatic and post-aneurysmal SAH is largely unknown. Detection of radiographic iSS after trauma or aneurysm bleeding suggests that the slower clinical course could benefit from an effective intervention if it became available. The use of cochlear implants is a valid alternative with advanced hearing impairment.

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Section: Vestibulopathymentioning

confidence: 99%

Focused Neuro-Otological Review of Superficial Siderosis of the Central Nervous System

et al. 2018

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“…Although most of the patients had red blood cells and xanthochromia in cerebrospinal fluid, active bleeding site is distinguished only in half, and surgical results are controversial. 1 , 5 For patients that the site of bleeding has not been established by imaging methods, medical treatment with iron chelator deferiprone may be a promising medical choice like in our patient. There were few reports in literature about deferiprone usage in superficial siderosis as an experimental treatment.…”

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confidence: 94%

“…Ninety percent of the patients with superficial siderosis, suffer from slowly progressive ataxia and hearing loss, and rarely present with seizure, episodic headache, visual loss, and olfactory impairment. 1 , 4 , 5 The most disabling symptom is progressive gait disorder. 1 Because of the progressive course, it is important to establish a treatment option for the patients.…”

mentioning

confidence: 99%

Iron chelation in treatment of superficial siderosis

Derle¹

2019

CJN

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Vertigo Related to Central Nervous System Disorders

Saha¹

2021

CONTINUUM: Lifelong Learning in Neurology

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PURPOSE OF REVIEW This article provides an overview of the numerous causes of vertigo and dizziness that are due to central nervous system (CNS) pathology and guides clinicians in formulating a differential diagnosis and treating patients with CNS causes of vertigo. RECENT FINDINGS Specific autoimmune vestibulocerebellar syndromes may now be tested for, and this article discusses the antibodies known to cause such syndromes. Superficial siderosis can be more accurately diagnosed with imaging studies, and treatment using iron chelation has recently been studied but has not yet been established as an effective treatment. Central autonomic network damage in the brain can cause central orthostatic hypotension in some neurodegenerative diseases, and medication has been approved for treatment. SUMMARY CNS causes of vertigo are numerous and important for clinicians to recognize. Examination findings are still an extremely valuable way to diagnose central vertigo; therefore, learning how to differentiate central from peripheral vertigo based on examination is an important skill. CNS causes of vertigo often have available treatments.

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Superficial Siderosis of the CNS and Otoneurological Manifestations

Cited by 3 publications

References 13 publications

Focused Neuro-Otological Review of Superficial Siderosis of the Central Nervous System

Focused Neuro-Otological Review of Superficial Siderosis of the Central Nervous System

Iron chelation in treatment of superficial siderosis

Vertigo Related to Central Nervous System Disorders

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