Cause Of Superficial Siderosis Research Articles

Closure of a dural defect as a cause of superficial siderosis: does early dural repair lead to a better outcome? Illustrative case.

Superficial hemosiderosis (SS) of the central nervous system is a rare condition that is caused by chronic, repeated hemorrhage into the subarachnoid space. The subsequent deposition of hemosiderin in the brain and spinal cord causes neurological deterioration. In this report, the authors describe a repair procedure for SS associated with a dural defect in the thoracic spine. A 75-year-old man presented with tinnitus symptoms that began about 1 year prior. Subsequently, his hearing loss progressed, and he gradually became unsteady on walking. Magnetic resonance imaging (MRI) of the head showed diffuse hemosiderin deposition on the surface of the cerebellum. Thoracic MRI showed ventral cerebrospinal fluid leakage of T2-7, and computed tomography myelography showed leakage of contrast medium that appeared to be a dural defect. Dural closure was successful, and MRI showed decreased fluid collection ventral to the dura. The patient's symptoms of wobbliness on walking and tinnitus improved dramatically from the postoperative period. Dural abnormalities of the spine must always be considered as one of the causes of SS. Early dural closure is an effective means of preventing the progression of symptoms.

Superficial siderosis of the central nervous system associated with ventral dural defects: bleeding from the epidural venous plexus.

Superficial siderosis of the central nervous system is a rare intractable disease induced by chronic subarachnoid hemorrhage. Neurological deficits, such as cerebellar ataxia and hearing difficulties, gradually progress if left undiagnosed. Hemosiderin deposition is irreversible because standard medical treatment has not yet been established. Interventions at the source of bleeding may be the key to a preferable outcome of treatment for chronic subarachnoid hemorrhage; however, the source is not clear in many cases. Among the consecutive cases diagnosed with a spontaneous cerebrospinal fluid (CSF) leak, cases of superficial siderosis associated with a CSF leak due to a ventral dural defect were retrospectively analyzed. Among 77 cases of a CSF leak, 7 cases (9%) of superficial siderosis were identified (median age of 59years, male, 4 cases). Defects were diagnosed on 1-mm sliced fast imaging employing steady-state acquisition MRI (n = 5), conventional myelographic CT (n = 1), or dynamic myelographic CT (n = 1) at high thoracic levels (T1-T4). All defects were repaired by direct neurosurgery. During surgery, continuous bleeding from the epidural veins of the internal vertebral venous plexus was identified as the source of subarachnoid hemorrhage. Epidural CSF pulsations through the defect prevented clot formation by the epidural veins. Dural repair stopped free communication between the subarachnoid and epidural spaces, leading to the disappearance of chronic subarachnoid hemorrhage. Bleeding from the epidural venous plexus may be the cause of superficial siderosis associated with ventral dural defects. Neurosurgical repair may stop the progression of this condition.

An unusual cause of superficial siderosis of central nervous system: A case report of a vestibular schwannoma

Vestibular schwannomas are relatively common benign tumors that account for 7-8% of all intracranial tumors. There is a limited number of cases of subarachnoid hemorrhage associated with vestibular schwannoma in the literature. The incidence of symptomatic tumor bleeding is historically reported less than 1%. Susceptibility-weighted imaging (SWI) is a relatively new Magnetic Resonance Imaging (MRI) sequence that is highly sensitive to compounds which distort the local magnetic field such as blood products and hemorrhage. We herein report the rare MRI findings of a 53-year-old man with unilateral vestibular schwannoma and hearing loss who was followed for 4 years. Initial diagnosis of the vestibular schwannoma was made on prior conventional MRI. We included SWI sequence in routine brain MRI protocol. During follow-up, SWI demonstrated focal superficial siderosis due to recurrent subarachnoid hemorrhage seen as hypointense blooming artifacts on the middle cerebellar peduncle-pons junction adjacent to the mass. We think that including SWI in routine MRI protocol will contribute to the initial diagnosis of acoustic schwannoma and detection of its complications such as subarachnoid hemorrhage and subsequent superficial siderosis.

Superficial Siderosis of the Central Nervous System Caused by Glioneuronal Tumor: A Case Report and Literature Review

Superficial siderosis is a rare disease resulting from the deposited hemosiderin owing to repeated subarachnoid hemorrhage. It has been reported that hemosiderin deposits on the brain surface and brain parenchyma causes nerve disorder, resulting in progressive and irreversible hearing loss, cerebellar ataxia and pyramidal disorder. The brain tumor is one of the cause of superficial siderosis. A 16-year-old female present a nearby hospital with complaints of absence seizure. A magnetic resonance imaging (MRI) revealed a heterogeneously enhanced mass at the right temporal lobe. Susceptibility-weighted imaging revealed diffuse and extensive superficial siderosis on the brain surface. The tumor was gross totally removed and diagnosed as glioneuronal tumor. The patient had been well, although susceptibility-weighted imaging performed one year after the surgery showed superficial siderosis remained. Early diagnosis and prevention of bleeding sources are recommended to prevent symptom progression associated with superficial siderosis. Susceptibility-weighted imaging is considered useful for early detection of superficial siderosis.

Intracranial dural arteriovenous fistula as a cause for symptomatic superficial siderosis: A report of two cases and review of the literature.

Background:Superficial siderosis (SS) is the occult deposition of hemosiderin within the cerebral cortex due to repeat microhemorrhages within the central nervous system. The collection of hemosiderin within the pia and superficial cortical surface can lead to injury to the nervous tissue. The most common presentation is occult sensorineural hearing loss although many patients have been misdiagnosed with diseases such as multiple sclerosis and amyotrophic lateral sclerosis before being diagnosed with SS. Only one case report exists in the literature describing an intracranial dural arteriovenous fistula (dAVF) as the putative cause for SS.Case Description:We describe two cases of SS caused by a dAVF. Both patients had a supratentorial, cortical lesion supplied by the middle meningeal artery with venous drainage into the superior sagittal sinus. In both patients, symptoms improved after endovascular embolization. The similar anatomic relationship of both dAVFs reported presents an interesting question about the pathogenesis of SS. Similar to the pathologic changes seen in the formation of intracranial arterial aneurysms; it would be possible that changes in the blood vessel lining and wall might predispose a patient to chronic, microhemorrhage resulting in SS.Conclusions:We describe the second and third cases of a dAVF as the cause of SS, and the first cases of successful treatment of SS-associated dAVF with endovascular embolization. As noninvasive imaging techniques become more sensitive and easily obtained, one must consider their limitations in detecting occult intracranial vascular malformations such as dAVF as a possible etiology for SS.

Meningeal melanocytoma of the cerebellopontine angle as the unusual cause of superficial siderosis

Meningeal melanocytoma of the cerebellopontine angle as the unusual cause of superficial siderosis

Malpositioned spinal instrument as a possible cause of superficial siderosis

Case report. To report a patient with superficial siderosis as a complication after posterior fixation surgery for odontoid fracture. Department of Neurosurgery, Hokkaido University, Japan. A 36-year-old man had undergone C1-C2 posterior fixation using lamina hooks for an odontoid fracture in 1997. In 2003, he presented with hearing loss and ataxia; and in 2006, a diagnosis of superficial siderosis was made and spinal instrument malpositioning was detected. The malpositioned instrument, suspected as the cause of superficial siderosis, was removed. Superficial siderosis of the central nervous system is rare; it results in progressive hearing loss, cerebellar ataxia and pyramidal sign. Chronic hemorrhage in the subarachnoid space precipitates hemosiderin around the cerebellum and brainstem resulting in neurological symptoms. Recurrent hemorrhage and cervical root pathology, for example, root avulsion, are factors; the symptoms worsen gradually and result in hemostasis. Superficial siderosis because of complications from spinal instrumentation surgery is extremely rare. If the instrument is malpositioned in the subarachnoid space, we suggest its removal.

Superficial siderosis of the central nervous system after brachial plexus injury

Superficial siderosis is a rare condition characterized by deposition of hemosiderin in the leptomeninges and in the subpial layers of the brain and spinal cord. It is associated with cerebrospinal fluid abnormalities consistent with recurrent bleeding into the subarachnoid space. The usual symptoms are hearing loss, ataxia, spastic paraparesis, sensory and sphincter deficits, and mental deterioration. A case is presented of severe superficial siderosis of the central nervous system in a 51-year-old man who had suffered a brachial plexus injury at the age of 20 years. The diagnosis was made by means of magnetic resonance imaging 16 years after the initial symptoms, which comprised bilateral hearing loss and anosmia. Subarachnoid bleeding was due to traumatic pseudomeningocele of the brachial plexus, a very unusual cause of superficial siderosis. This case is interesting insofar as the surgical treatment prevented further bleeding and possibly progression of the disease.

Posterior Fossa Surgery

WITH THE WIDESPREAD use of magnetic resonance imaging, an increasing number of cases of superficial siderosis are being discovered. However, the cause remains uncertain in almost half the cases. We report a case where previous surgery for a pontine hematoma established a pathway for extravasation of blood into the 4th ventricle, resulting in superficial siderosis that was demonstrated on a follow-up magnetic resonance imaging scan 8 years later. This case presents an unusual iatrogenic cause of superficial siderosis that has not been reported previously.

Superficial siderosis of the central nervous system. A report on nine cases with autopsy.

The clinical and necropsy findings are described in nine cases of superficial siderosis of the central nervous system. Four of these had undergone cerebral hemispherectomy in childhood. All four showed multiple haemorrhagic spots in the cavity membrane and the walls of the remaining lateral ventricle. Of the other cases, to had repeated ruptures of cerebral aneurysms; one showed a destructive lesion of the cerebellar vermis; one, a hydrocephalic child, showed evidence of repeated haemorrhage in the area of a malformed aqueduct; and the last, a baby, had bilateral subdural haematomata. From the histological similarities of these cases and previously reported ones, and from the reported findings in experimental animals, it is argued that the common cause of superficial siderosis is recurrent intraventricular or subarachnoid haemorrhage.