Figure 1
Teaching Case
SectionNeuroradiology
Case TypeClinical Cases
AuthorsPardo, Camilo MD; Donato, Angel MD; Huapaya, Janice MD; Figueroa, Ramon E. MD FACR
Connected authors
14 years, male
14-year-old male patient with history of Chiari I decompression and syringohydromyelia with new onset bilateral sensorineural hearing loss.
Superficial siderosis
Normal leptomeningeal melanin
Meningoangiomatosis
Neurocutaneous melanosis
MR sequence artefacts
MRI shows superior vermian volume loss with extensive susceptibility signal loss outlining the cerebellar folia surface in keeping with superficial siderosis, most pronounced on susceptibility-weighted imaging. There is also a severe pattern of superficial siderosis throughout the supratentorial compartment.
Superficial siderosis (SS) is the result of deposits of haemosiderin along the flexible layers of the meninges, spinal cord and cranial nerves, especially the VIII. [1]
SS has asymmetric male:female ratio of approximately 3:1, most frequently seen between ages of 50 to 60 years. SS results from chronic haemorrhage byproduct deposits in the pia-arachnoid. Its aetiology could be idiopathic or seen in patients with traumatic brain or spinal cord injury, previous CNS tumour, history of neurosurgical interventions, amyloid angiopathy and subarachnoid haemorrhage from aneurysms or arteriovenous malformations [1, 2].
The characteristic symptoms are: sensorineural hearing loss, which is usually bilateral, ataxia, cerebellar dysarthria and pyramidal symptoms. If the disease is advanced, it could be associated with dementia, bladder incontinence, sensory deficits and symptoms derived from damage to the cranial nerves. Fluid from lumbar puncture is usually haemorrhagic or xanthochromic [3, 4]
Haemosiderin-induced vestibulocochlear nerve damage leads to sensorineural hearing loss due to its long cisternal course, making it more susceptible to toxic damage by iron compounds.
MRI is the image of choice for the diagnosis of SS. CT is not sensitive or specific for the diagnosis of this pathology [5]. The pia mater and ependymal surfaces are coated by haemosiderin, particularly along the brainstem and cerebellum, which may be associated with cerebellar atrophy.
Siderosis results in low T1 signal, low T2 signal, low GRE signal and accentuated blooming signal loss on SWI series. If clinically suspected but no lesion is identified intracranially, then imaging of the entire spinal canal is indicated [5].
Unfortunately, there is no treatment to reverse the damage caused by haemosiderin deposition at the central nervous system level. Several treatments with iron chelates such as desferroxamine show no conclusive results. [6]
SS has asymmetric male:female ratio of approximately 3:1, most frequently seen between ages of 50 to 60 years. SS results from chronic haemorrhage byproduct deposits in the pia-arachnoid. Its aetiology could be idiopathic or seen in patients with traumatic brain or spinal cord injury, previous CNS tumour, history of neurosurgical interventions, amyloid angiopathy and subarachnoid haemorrhage from aneurysms or arteriovenous malformations [1, 2].
The characteristic symptoms are: sensorineural hearing loss, which is usually bilateral, ataxia, cerebellar dysarthria and pyramidal symptoms. If the disease is advanced, it could be associated with dementia, bladder incontinence, sensory deficits and symptoms derived from damage to the cranial nerves. Fluid from lumbar puncture is usually haemorrhagic or xanthochromic [3, 4]
Haemosiderin-induced vestibulocochlear nerve damage leads to sensorineural hearing loss due to its long cisternal course, making it more susceptible to toxic damage by iron compounds.
MRI is the image of choice for the diagnosis of SS. CT is not sensitive or specific for the diagnosis of this pathology [5]. The pia mater and ependymal surfaces are coated by haemosiderin, particularly along the brainstem and cerebellum, which may be associated with cerebellar atrophy.
Siderosis results in low T1 signal, low T2 signal, low GRE signal and accentuated blooming signal loss on SWI series. If clinically suspected but no lesion is identified intracranially, then imaging of the entire spinal canal is indicated [5].
Unfortunately, there is no treatment to reverse the damage caused by haemosiderin deposition at the central nervous system level. Several treatments with iron chelates such as desferroxamine show no conclusive results. [6]
References
[1] Fragoso YD, Adoni T, Brooks JB, Gomes S, Goncalves MV, Jovem CL, Matta AP, Oliveira JF, Siquinelli F, Troiani GN, Wille PR. (2017) Superficial siderosis of the central nervous system is a rare and possibly underdiagnosed disorder. Arq Neuropsiquiatr Feb;75(2):92-95. (PMID: 28226077)
[2] Jadhav TM, Hegde AU (2012) Superficial siderosis: A rare occurrence in children. J Pediatr Neurosci Sep;7(3):215-7. (PMID: 23560013)
[3] Wilson D, Chatterjee F, Farmer SF, Rudge P, McCarron MO, Cowley P, Werring DJ (2017) Infratentorial superficial siderosis: Classification, diagnostic criteria, and rational investigation pathway. Ann Neurol Mar;81(3):333-343. (PMID: 28019651)
[4] Kishimoto I, Yamazaki H, Naito Y, Shinohara S, Fujiwara K, Kikuchi M, Kanazawa Y, Tona R, Harada H. (2013) Clinical features of rapidly progressive bilateral sensorineural hearing loss. Acta Otolaryngol Jan;134(1):58-65. (PMID: 24256048)
[5] Yamamoto Y, Yamamoto S, Kuroki S (2012) T2*-weighted MRI findings of superficial siderosis. Intern Med 2012;51(14):1949 (PMID: 22821124)
[6] Kondziella D, Lindelof M, Larsen VA, Kruse A (2015) Diagnostic and therapeutic challenges in superficial CNS siderosis. Dan Med J 2015 May;62(5). (PMID: 26050830)
Case information
| URL: | https://eurorad.org/case/14767 |
| DOI: | 10.1594/EURORAD/CASE.14767 |
| ISSN: | 1563-4086 |
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