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Sequential bilateral non-arteritic anterior ischaemic optic neuropathy in superficial intracranial siderosis


Enz, T J; Clavadetscher, S C; Gugleta, K; Jaggi, G P (2016). Sequential bilateral non-arteritic anterior ischaemic optic neuropathy in superficial intracranial siderosis. Klinische Monatsblätter für Augenheilkunde, 233(4):399-401.

Abstract

Superficial intracranial siderosis (SIS) is a rare and often unrecognized disorder of the central nervous system (CNS) characterized by progressive neuronal degeneration resulting from chronic subarachnoid bleeding. Hemoglobin accumulates in the subarachnoid space where it is metabolized to hemosiderin and deposited in the subpial layer of those parts of the CNS adjacent to the cerebrospinal fluid with predilection for inter alia the cerebellum and cranial nerves I, II and VIII. Hemosiderin deposits lead to neuronal damage, reactive gliosis, and demyelination. Often the hemorrhage is occult and no bleeding source can be detected. Classic symptoms are sensorineural deafness, cerebellar ataxia and pyramidal signs [1]. Ophthalmic symptoms such as nystagmus have also been reported. Visual dysfunction however is not a recognized feature of SIS. Indeed, patients in an advanced stage have been shown to have normal optic nerve function [2]. Polidori et al documented a case of optic nerve dysfunction in a patient with SIS and concomitant glaucoma [3]. In 2008 Koeppen et al postulated that visual failure solely due to SIS does not occur [4]. But Painter et al reported two cases of SIS with strong evidence of optic nerve dysfunction and one postmortem case of histological findings of iron deposition and associated demyelination in the optic nerve [5]. Magnetic resonance imaging (MRI) is the diagnostic method of choice showing features of hemosiderin accumulation on CNS surfaces [4].

Abstract

Superficial intracranial siderosis (SIS) is a rare and often unrecognized disorder of the central nervous system (CNS) characterized by progressive neuronal degeneration resulting from chronic subarachnoid bleeding. Hemoglobin accumulates in the subarachnoid space where it is metabolized to hemosiderin and deposited in the subpial layer of those parts of the CNS adjacent to the cerebrospinal fluid with predilection for inter alia the cerebellum and cranial nerves I, II and VIII. Hemosiderin deposits lead to neuronal damage, reactive gliosis, and demyelination. Often the hemorrhage is occult and no bleeding source can be detected. Classic symptoms are sensorineural deafness, cerebellar ataxia and pyramidal signs [1]. Ophthalmic symptoms such as nystagmus have also been reported. Visual dysfunction however is not a recognized feature of SIS. Indeed, patients in an advanced stage have been shown to have normal optic nerve function [2]. Polidori et al documented a case of optic nerve dysfunction in a patient with SIS and concomitant glaucoma [3]. In 2008 Koeppen et al postulated that visual failure solely due to SIS does not occur [4]. But Painter et al reported two cases of SIS with strong evidence of optic nerve dysfunction and one postmortem case of histological findings of iron deposition and associated demyelination in the optic nerve [5]. Magnetic resonance imaging (MRI) is the diagnostic method of choice showing features of hemosiderin accumulation on CNS surfaces [4].

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Additional indexing

Other titles:Fortschreitende bilaterale nicht arteriitische anteriore ischämische Optikusneuropathie bei Superfizieller Intrakranieller Siderose
Item Type:Journal Article, refereed, original work
Communities & Collections:04 Faculty of Medicine > University Hospital Zurich > Ophthalmology Clinic
Dewey Decimal Classification:610 Medicine & health
Language:English
Date:April 2016
Deposited On:10 May 2016 06:10
Last Modified:10 May 2016 06:10
Publisher:Georg Thieme Verlag
ISSN:0023-2165
Publisher DOI:https://doi.org/10.1055/s-0041-111534
PubMed ID:27116492

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