Case Of Superficial Siderosis Research Articles

Detection of Dural Defect Localization Using 4-Dimensional Dynamic Computed Tomography Myelography for Patients with Superficial Siderosis

There are cases of superficial siderosis (SS) with spinal ventral fluid-filled collection in the spinal canal. In our previous study, the balanced steady-state free precession sequence magnetic resonance imaging is useful in identifying the location of dural defects. However, because of its narrow scan area and long scan time, it cannot easily detect the defect location in some patients with small dural defect. In this study, we applied 4-dimensional (4D) dynamic computed tomography (CT) imaging, including time-axis imaging, to myelography using the latest CT imaging equipment, which can perform short-time continuous imaging, to identify the dural defect site. Twenty SS patients with ventral fluid-filled collection in the spinal canal (9 males, 11 females; mean age 61.6years) underwent 4D dynamic CT myelography. A 192-row helical CT (SOMATOM Force, SIEMENS, Munich, Germany) with high-speed scanning capability was used to obtain 9-11 scans per minute at low dose while passing contrast medium into the subarachnoid space. Then, contrast leakage sites were identified. The contrast leakage sites could be identified in all 20 cases: C7/Th1, 2 cases; Th1/2, 5 cases; Th2/3, 9 cases; Th3/4, 1 case; Th5/6, 1 case; Th7/8, 1 case; and Th8/9, 1 case. Eighteen cases underwent surgical operation, and actual dural defects were confirmed at the contrast leakage sites. The mean±standard deviation of leakage time from contrast agent injection was 19.0±9.2s. The 4D dynamic CT myelography can be used to reliably identify the location of spinal fluid leakage. In SS cases, dural defects could be visualized in an average of 19seconds.

Superficial siderosis: comparison of two cases indicates two distinct diagnostic entities. Illustrative cases.

Superficial siderosis is the deposition of hemosiderin in the superficial layers of the central nervous system. It has been described in patients with chronic leakage of blood into the cerebrospinal fluid or with amyloid angiopathy, often associated with Alzheimer's disease (AD). We present two cases of superficial siderosis with vastly different symptomatologies and treatment courses. The patient in case 1 had diffuse superficial siderosis demonstrated on T2-weighted magnetic resonance imaging (MRI), appearing mostly in the inferior cerebellum and extending throughout the neuraxis. He presented with hearing loss, spasticity, gait abnormalities, and urinary incontinence. Ultimately, surgical exploration of the thoracic spinal dura revealed an arteriovenous fistula, which was obliterated. His clinical course stabilized but with persistent deficits. The patient in case 2 had a family history of AD and underwent MRI to evaluate for memory impairment, which demonstrated superficial siderosis of the left occipital lobe. Lumbar puncture demonstrated only traumatic contamination by red blood cells, but tau protein analysis was consistent with the diagnosis of AD. Superficial siderosis is a diagnostic term prompted by findings on MRI that can arise due to two different pathological entities. The diagnosis in case 1 should be termed diffuse superficial siderosis and in case 2 should be termed lobar cortical siderosis.

Clinical and Radiological Findings and Treatment of 8 Cases of Superficial Siderosis at Our Hospital

Clinical and Radiological Findings and Treatment of 8 Cases of Superficial Siderosis at Our Hospital

Persistence and regredience of intraspinal fluid collection determine symptom control in intracranial hypotension syndrome

Background and purposeAn intraspinal fluid collection (ISFC) can be observed on spinal MRI in cases of intracranial hypotension syndrome (IHS). The goal of this study was to analyze the possible persistence of ISFC after therapy and its correlation to clinical disease activity and secondary complications.Materials and methodsTwenty patients in our database of 57 patients, who were treated for IHS between 2009 and 2015, fulfilled the inclusion criteria of (a) diagnosed and treated IHS as well as (b) an ISFC in MRI imaging. Ten of these participated in our study. We performed follow-up visits, which included a history, a clinical examination, and a spinal MRI.ResultsA MRI-confirmed ISFC was seen in six patients, five of which had symptoms attributable to chronic IHS. There were two cases of superficial siderosis. One patient had a persisting ISFC and was free of symptoms. Four patients did not have an ISFC and were free of symptoms (Fisher’s exact test; p < 0.048).ConclusionThere is statistically significant correlation between the persistence of an ISFC after IHS treatment and ongoing clinical symptoms. Resolved symptoms seem to correlate with absorbed extradural ISFC and hypothetically closed leakage site. ISFC as confirmed by MRI proofs to be a reliable follow-up marker for disease activity in chronic IHS that is possibly even superior to clinical examination.

Efficacy and safety of deferiprone for the treatment of superficial siderosis: results from a long-term observational study.

Superficial siderosis (SS) of the central nervous system is a rare and heterogeneous condition due to deposition of hemosiderin on the surface of the brain and spinal cord. The usually progressive clinical course is characterized by a combination of hearing loss, cerebellar ataxia, and myelopathy. There is no known treatment for SS, but the iron chelator deferiprone (DFP) has been proposed as a potentially useful treatment. We present a long-term (average 3.7years) evaluation of four cases of SS treated with DFP (15mg/kg po bid). Treatment with DFP proved safe and well tolerated. Two out of the four subjects were unchanged while the other two presented a clinical improvement with reduction of postural instability and cerebellar signs. Blinded evaluation of magnetic resonance imaging (performed every 6months during follow-up) showed a reduction of the abnormal iron deposition for all patients. This long-term observational study suggests that DFP may be effective in the management of the neurological manifestations associated with iron accumulation in SS. CLINICALTRIALS. NTC00907283.

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.

A Case of Superficial Siderosis of the Central Nervous System Treated Successfully with Cochlear Implantation

A Case of Superficial Siderosis of the Central Nervous System Treated Successfully with Cochlear Implantation

人工内耳が有効であった脳表ヘモジデリン沈着症例

人工内耳が有効であった脳表ヘモジデリン沈着症例

Styloidogenic Jugular Venous Compression Syndrome: Diagnosis and Treatment (P05.261)

Objective: To present a case of Superficial Siderosis of the CNS in a patient with bilateral internal jugular vein narrowing from an external compression of elongated styloid processes. Background Superficial Siderosis of CNS is an uncommon disorder that results from hemosiderin deposition in leptomeninges, subpial layers of brain, and spinal cord from chronic subarachnoid hemorrhage (SAH). The source of SAH is not identified in half of the cases. We present a case of superficial siderosis in a patient with bilateral jugular vein narrowing causing venous congestion from an external compression of styloid processes. Design/Methods: Case report from a tertiary medical center. Results: A 59 year old female presented with slowly progressive gait ataxia, sensorineural hearing loss, and episodic headaches for 6 months. MRI of brain and spinal cord revealed a pituitary adenoma and T2 hypointensity involving the cerebellum, brainstem, and spinal cord consistent with superficial siderosis. CSF studies demonstrated elevated protein without xanthochromia. A dynamic cerebral venogram with extension and flexion views revealed severe bilateral internal jugular vein narrowing at the level of C1-C2, more severe with neck flexion, with considerable congestion of the paravertebral veins and spinal epidural venous plexus. Manometry study revealed an increased pressure gradient across the narrowing between the sigmoid sinuses and internal jugular veins, which was worse with neck flexion compared to neutral position. Computerized tomography angiography of the neck demonstrated elongated styloid processes with external compression of the internal jugular veins at the level of the narrowing. The patient was referred to surgery for styloidectomy. Conclusions: We present a case of superficial siderosis with possible etiology related to bilateral internal jugular vein narrowing leading to significant venous congestion from an external compression of elongated syloid processes. Disclosure: Dr. Gupta has nothing to disclose. Dr. Pandya has nothing to disclose. Dr. Lazzaro has nothing to disclose. Dr. Fitzsimmons has nothing to disclose.

Temporal Bone Histopathology in a Case of Sensorineural Hearing Loss Caused by Superficial Siderosis of the Central Nervous System and Treated by Cochlear Implantation

To evaluate the histopathology of the temporal bones of a patient with documented superficial siderosis of the central nervous system who underwent right cochlear implantation six years before death. Superficial siderosis of the central nervous system is due to chronic or repeated subarachnoid hemorrhage and results in sensorineural deafness in 95% of affected individuals in addition to other neurologic findings. The deposition of hemosiderin in the meninges and around cranial nerves is thought to be causative. There have been no previous reports of temporal bone pathology in this disorder.This 57 year old man developed progressive, bilateral hearing loss starting in his 30's with loss of pure tone thresholds and word recognition. He underwent a right cochlear implant at age 51 with full insertion of the device. The temporal bones and brainstem were fixed in formalin and prepared for histologic study by standard techniques. Special stains, including Gomori stain for iron were performed on sections of the temporal bones and cochlear nucleus. There was severe bilateral degeneration of the organ of Corti, spiral ligament, stria vascularis, and spiral ganglion cells. Gomori stain revealed iron deposits within the spiral ligament, stria vascularis and in the subepithelial mesenchymal tissue of the maculae of the vestibular system. Evaluation of the cochlear nucleus revealed iron deposits within glial cells and larger cells, probably macrophages, near the CSF surface. On the right side, the track created by the cochlear implant entered the scala tympani and continued to mm17, as measured from the round window. This is the first known case of superficial siderosis with documented temporal bone histopathology. Hearing loss was likely caused by severe degeneration of spiral ganglion cells in both ears, despite the presence of remaining hair cells in the middle and apical turns. This was consistent with cochlear neuronal degeneration and retrograde degeneration of spiral ganglion cells within the inner ear, or alternatively, consistent with primary degeneration of hair cells and neural structures within the cochlea. Despite the presence of neural degeneration, the patient achieved a word recognition score of 28% six months following implantation.

Episodic encephalopathy due to an occult spinal vascular malformation complicated by superficial siderosis

Superficial siderosis (SS) of the central nervous system is a rare condition caused by chronic subarachnoid hemorrhage. Clinical manifestations typically include sensorineural hearing loss and cerebellar ataxia. Recurrent episodic encephalopathy in the setting of SS has not been reported. We describe a unique case of SS in a 67-year-old man with an 8-year history of episodic encephalopathy associated with headache and vomiting. The patient also had a history of progressive dementia, ataxia, and myelopathy. A diagnosis of superficial siderosis was made after magnetic resonance gradient-echo images showed diffuse hemosiderin staining over the cerebellum and cerebral convexities. No intracerebral source of hemorrhage was identified. The patient therefore underwent gadolinium-enhanced spinal MRI which suggested a possible vascular malformation. A therapeutic laminectomy subsequently confirmed an arteriovenous fistula which was resected. In SS, there are often long delays between symptom onset and definitive diagnosis. Early identification is facilitated by magnetic resonance imaging with gradient-echo sequences. When no source of hemorrhage is identified intracranially, then total spinal cord imaging is indicated to assess for an occult source of hemorrhage as occurred in our case.

CSF tau protein is a useful marker for effective treatment of superficial siderosis of the central nervous system: Two case reports

We report two cases of superficial siderosis (SS) of the central nervous system (CNS), which is caused by chronic haemorrhaging into the subarachnoid space with haemosiderin deposition in the superficial portion of the CNS. Patient 1 had fluid collection in the spinal canal, which was reported as the source of the chronic bleeding. Patient 2 was bleeding from thickened dura at the level of the sacral vertebrae. Both of the patients had xanthochromic cerebrospinal fluid. We surgically repaired the sources of bleeding. Subsequently the cerebrospinal fluid (CSF) cleared and their symptoms were not aggravated for about 1 year. We measured several CSF markers of SS before and after surgery. Total tau protein (CSF-t-tau), phosphorylated tau protein (CSF-p-tau), iron (CSF-iron) and ferritin (CSF-ferritin) in the CSF were highly elevated at diagnosis. After surgery, the levels of CSF-t-tau and CSF-p-tau were markedly reduced while CSF-iron and CSF-ferritin had not decreased. It is suggested that CSF-t-tau and CSF-p-tau reflected the neural damage in SS and were useful to evaluate the effectiveness of SS therapies.

A cast-iron guarantee of ataxia and deafness

Superficial siderosis of the central nervous system (CNS) is a rare condition that is thought to be caused by repeated subarachnoid haemorrhages. The major clinical features are progressive bilateral hearing loss, cerebellar ataxia and pyramidal dysfunction. Ante-mortem diagnosis is now possible due to MRI and post mortem reports of this condition are becoming rare. Despite the occurrence of typical clinical features the diagnosis is often missed during life if CT scanning alone is performed. We describe a case of superficial siderosis of the CNS with clinical and post mortem findings as a reminder of this unusual condition.

Role of dynamic CT myelography in identifying the etiology of superficial siderosis

The authors report three cases of superficial siderosis with a fluid-filled spinal canal cavity communicating with the subarachnoid space. The site of communication was identified with dynamic CT myelography and the defect was surgically repaired.

Superficial siderosis of the CNS: Report of three cases and review of the literature

Superficial siderosis (SS) is a rare entity. A chronic subarachnoid hemorrhage (SAH) leads to the deposition of ferric pigments and ions in the surface of the central nervous system (CNS). The cause of this hemorrhage is identified in half of the cases. The disease progresses slowly and the damage is often irreversible by the time diagnosis is established. The management focuses on the identification and ablation of the bleeding source. Knowledge about this entity is based on sporadic reports. We describe three cases of superficial siderosis and briefly review the literature.

Superficial siderosis of the central nervous system: a case with an unruptured intracranial aneurysm

We present a case of superficial siderosis (SS) of the central nervous system (CNS) with an unruptured intracranial aneurysm to illustrate that the commonly encountered unexplainable progressive sensorineural hearing loss (SNHL) can be an important sign for the early awareness of this rare disorder. The literature on SS is reviewed and the pathogenesis of SS is discussed.

Superficial Siderosis

A case of superficial siderosis of the central nervous system proven by brain biopsy is described. The diagnosis was made antemortem, the first such case reported in the English language literature. Postcontrast material cerebral CT demonstrated widespread meningeal enhancement. The differential diagnosis is discussed.