Superficial siderosis of the central nervous system its MRI manifestations

doi:10.1016/S0899-7071(96)00050-2

Clinical Imaging

Volume 21, Issue 4, July–August 1997, Pages 241-245

https://doi.org/10.1016/S0899-7071(96)00050-2 Get rights and content

Abstract

Three cases of superficial siderosis of the central nervous system are reported here. Using a 1.0-T magnetic resonance (MR) unit, typical hypointense rims were observed under the brain surface on T2-weighted images. In one patient, marked atrophy of the superior cerebellar vermis and cerebellar parenchymal hyperintensity were also detected. The spinal cord was involved in two of the three patients. On T1-weighted images, hyperintense rims were demonstrated over the brain surface in two of the three patients. This finding has not been previously reported.

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Cited by (30)

Nationwide epidemiological survey of superficial hemosiderosis in Japan
2019, Journal of the Neurological Sciences
Citation Excerpt :
The clinical diagnosis of SHS is challenging because SHS may show similar clinical features to spinocerebellar ataxia [13]. Undoubtedly, using MRI with high magnetic field is crucial to detect hemosiderin [14,15]. We strongly recommend 1.5 T or 3 T MRI including T2* or SWI imaging for individuals with cerebellar ataxia or myelopathy, to identify SHS.
The Japanese guideline for diagnosis and classification of superficial hemosiderosis (SHS) has recently been published, for which patient medical expenses are supported by the Ministry of Health. We sought to clarify the clinical features, method of diagnosis, and treatment for SHS in Japan.
We sent a questionnaire survey to 792 medical institutes of the Japanese Society of Neurology, to collect information about SHS, including patients during 2017.
We received replies from 287 institutes (36.2%). Estimated total number of patients with SHS in 2017 was 129 at 55 institutes. All patients were diagnosed by neurologists. Among 123 patients with available data, 81 patients (63%) had “classical” type (c-SHS), 29 (24%) had “localized” type (l-SHS), and 13 patients (10%) had “atypical” type (a-SHS). Five patients with l-SHS were excluded because of lacking detailed information. There were available data for the cause of SHS in 77 patients (63%): 55 (69%) with c-SHS, 16 (55%) with l-SHS, and 6 (48%) with a-SHS. Pharmacological or surgical treatment was given at 31 institutes. Medical expense subsidies were filed for 41% of patients.
Using the Japanese guideline for diagnosis of SHS, over 100 patients were confirmed as having SHS with characteristic clinical features. SHS is not a rare clinical condition in Japan.
Surgical management of superficial siderosis
2009, Spine Journal
Citation Excerpt :
T2 shortening reflects the paramagnetic effect of hemosiderin. On occasion, hyperintensity on T1 and T2 sequences may be seen around the cerebellum and the spinal cord [13]. A hyperintense rim on T1 sequences correlates with the subacute stage (methemoglobin) of hemorrhage.
Superficial siderosis is a rare condition resulting from the presence of chronic bleeding into the subarachnoid space usually causing gait instability and deafness. The surgical management of superficial siderosis depends on localizing the source of hemorrhage.
The surgical treatment of this rare condition has not been well described in the literature. We present a case illustrating the surgical treatment for superficial siderosis.
Case report.
The authors describe the case report of a 70-year-old gentleman with gait instability and deafness found to have an abnormal communication between the spinal epidural venous plexus and the subarachnoid space.
The source of hemorrhage into the subarachnoid space was identified to be a fistula in the ventral thoracic dural. A costotransversectomy approach was undertaken at the T4–T5 level to expose the fistula. The abnormal communication was patched and sealed.
The patient's symptoms remained stable on follow-up at 15 months with no worsening of his symptoms.
Superficial siderosis is a neurologic disorder that arises from chronic hemosiderin deposition into the subarachnoid space. The progressive nature of the disease can be halted if a source of hemorrhage can be found and treated surgically.
Auditory Neuropathy, Sarcoidosis, Siderosis, and Idiopathic Pachymeningitis
2005, Neurotology
Hearing Loss and Vertigo in Superficial Siderosis of the Central Nervous System
2004, American Journal of Otolaryngology - Head and Neck Medicine and Surgery
Otoneurologic findings in patients with superficial siderosis (SS) of the central nervous system are described. A 20-year-old man with acute vertigo, dizziness, with a history of head trauma in childhood; an 87-year-old woman with severe disequilibrium appearing after peridural anesthesia; and a 55-year-old woman with recurrent episodes of positional vertigo and progressive ataxia, suffering from a lumbar ependymoma are described; all patients complained of progressive bilateral hearing loss and tinnitus.
Otoneurologic examination showed bilateral sensorineural hearing loss, disturbed ocular pursuit and optokinetic nystagmus, incomplete visual suppression of the vestibulo-ocular reflex during rotatory pendular testing, right hyporeflexia, and bilateral caloric areflexia. Cerebral magnetic resonance imaging (MRI) revealed rims of hypointensity surrounding the brainstem and linear hypointensities following the surface of the cerebellar folia.
The etiology stems from subarachnoid hemorrhage, but the source of bleeding may remain obscure. Bilateral hearing loss is described in 95% and disequilibrium in 90% because of peripheral vestibular deficit and cerebellar ataxia. In patients with progressive bilateral cochleo-vestibular deficit of unknown etiology, MRI is the examination of choice to confirm SS.
Superficial haemosiderosis secondary to cutaneous venous malformation
2001, Clinical Radiology
Superficial siderosis
2006, Neurology
Superficial siderosis (SS) of the CNS is caused by repeated slow hemorrhage into the subarachnoid space with resultant hemosiderin deposition in the subpial layers of the brain and spinal cord. Despite extensive investigations, the cause of bleeding is frequently undetermined.
To review the clinical and imaging features of 30 consecutive patients with SS and provide insights into the underlying causes of subarachnoid bleeding in this disabling disorder.
The authors reviewed the medical records of 30 consecutive patients with clinical and MRI evidence of SS.
The commonest neurologic manifestations included gait ataxia and hearing impairment. A clinical history of subarachnoid hemorrhage was relatively rare. Possible predisposing conditions were identified on history in 22 patients, the commonest being a prior trauma (15 patients). In addition to the characteristic MRI findings of SS, 18 patients had abnormalities on MRI possibly related to chronic bleeding. The most common of these was the presence of a fluid-filled collection in the spinal canal seen in 14 patients.
A history of prior subarachnoid hemorrhage is often absent in patients with superficial siderosis (SS). A past history of trauma is common. Prior intradural surgery may be an additional risk factor. Xanthochromia or the presence of red blood cells in the CSF is a common finding. Only rarely does angiography demonstrate the bleeding source. The presence of a fluid-filled collection in the spinal canal is a common finding on MRI and is likely related to the SS. With longitudinally extensive cavities, a dynamic CT myelogram may help localize the defect and direct the site of laminectomy. Surgical repair of a dural defect, if present, should be considered. Surgical correction of bleeding should be documented by CSF examination months after surgery. Friable vessels in the dural defect are a possible source of the chronic bleeding.

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Original articleSuperficial siderosis of the central nervous system its MRI manifestations

Abstract

J Neurol Sci

Clin Radiol

Superficial siderosis of the central nervous system: a report on nine cases with autopsy

Acta Neuropath

High-field MR imaging of superficial siderosis of the central nervous system

J Comput Assist Tomogr

High-field spin-echo MR imaging of superficial and subependymal siderosis secondary to neonatal intraventricular hemorrhage

Neuroradiology

Superficial siderosis of the central nervous system

Eur Arch Psychiatry Clin Neurosci

Superficial siderosis of the central nervous system

Ann Otol Rhinol Laryngol

Superficial siderosis of the spinal cord: a rare cause of myelopathy diagnosed by MRI

Neurology

Original article
Superficial siderosis of the central nervous system its MRI manifestations