Idiopathic superficial siderosis of the central nervous system.
Chronic subarachnoid hemorrhage
Hemosiderin
Sensorineural deafness
Superficial siderosis
Journal
Cerebellum & ataxias
ISSN: 2053-8871
Titre abrégé: Cerebellum Ataxias
Pays: England
ID NLM: 101648460
Informations de publication
Date de publication:
25 Feb 2021
25 Feb 2021
Historique:
received:
17
12
2020
accepted:
16
02
2021
entrez:
26
2
2021
pubmed:
27
2
2021
medline:
27
2
2021
Statut:
epublish
Résumé
Regardless of the cause of the superficial siderosis (SS) disease, which is bleeding, the source of bleeding cannot be found in some cases. In this article, we report two cases with idiopathic SS. Case 1 presented with bilateral hearing loss, cognitive impairment, sleep disturbances, and tremors. Case 2 presented with sensory neural hearing loss, ataxia, and spastic paraparesis. In both cases, brain MRI indicated evidence of SS. CT myelogram and SPECT with labeled RBC couldn't help finding the source of occult bleeding. SS is a rare central nervous system disease caused by the deposition of hemosiderin in the brain and spinal cord, which results in the progression of neurological deficits. The cause of this hemorrhage is often subarachnoid haemorrhage, intracranial surgery, carcinoma, arteriovenous malformation, nerve root avulsion, and dural abnormality. The condition progresses slowly and, by the time diagnosis is confirmed, the damage is often irreversible. In our cases, brain MRI clarified the definitive diagnosis, but we could not find the source of bleeding. SS should be considered in cases with ataxia and hearing loss, even if no source of bleeding is found.
Sections du résumé
BACKGROUND
BACKGROUND
Regardless of the cause of the superficial siderosis (SS) disease, which is bleeding, the source of bleeding cannot be found in some cases.
CASE PRESENTATION
METHODS
In this article, we report two cases with idiopathic SS. Case 1 presented with bilateral hearing loss, cognitive impairment, sleep disturbances, and tremors. Case 2 presented with sensory neural hearing loss, ataxia, and spastic paraparesis. In both cases, brain MRI indicated evidence of SS. CT myelogram and SPECT with labeled RBC couldn't help finding the source of occult bleeding.
CONCLUSION
CONCLUSIONS
SS is a rare central nervous system disease caused by the deposition of hemosiderin in the brain and spinal cord, which results in the progression of neurological deficits. The cause of this hemorrhage is often subarachnoid haemorrhage, intracranial surgery, carcinoma, arteriovenous malformation, nerve root avulsion, and dural abnormality. The condition progresses slowly and, by the time diagnosis is confirmed, the damage is often irreversible. In our cases, brain MRI clarified the definitive diagnosis, but we could not find the source of bleeding. SS should be considered in cases with ataxia and hearing loss, even if no source of bleeding is found.
Identifiants
pubmed: 33632336
doi: 10.1186/s40673-021-00133-5
pii: 10.1186/s40673-021-00133-5
pmc: PMC7908722
doi:
Types de publication
Journal Article
Langues
eng
Pagination
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