1/28. Superficial siderosis of the CNS associated with multiple cavernous malformations.Superficial siderosis of the CNS due to chronic, recurrent subarachnoid hemorrhage is an uncommon and potentially debilitating disorder. The classic clinical manifestation is progressive bilateral sensorineural hearing loss (SNHL), although ataxia and pyramidal signs also are observed frequently. Cavernous malformations rarely present with subarachnoid hemorrhage. We describe an unusual case of a young patient who presented with progressive, bilateral SNHL who was found to have superficial CNS siderosis associated with multiple cavernous malformations.- - - - - - - - - - ranking = 1keywords = subarachnoid (Clic here for more details about this article) |
2/28. Transthyretin amyloidosis and superficial siderosis of the CNS.OBJECTIVE: To describe a previously unreported clinical and radiologic presentation of hereditary transthyretin (TTR)-related amyloidosis. BACKGROUND: Unexplained cerebellar ataxia, pyramidal syndrome, and hearing loss are observed in some patients with TTR-related amyloidoses. methods: We performed clinical, radiologic, and pathologic examinations of three family members with TTR-related (Ala36Pro) amyloidosis. RESULTS: The patient was a 69-year-old woman with vitreal amyloid deposits, progressive sensorineural deafness, cerebellar ataxia, pyramidal syndrome, and recurrent transient neurologic symptoms. Cranial MRI showed symmetric thin rims of low signal intensity in T2- and T2*-weighted images in the cortex of the sylvian fissures, of the cerebellar hemispheres and vermis, and in the quadrigeminal plate consistent with superficial siderosis of the CNS. Her older daughter had vitreal amyloid deposits, acute brown-sequard syndrome at C4, acute sensorineural deafness, and recurrent transient neurologic symptoms. Cranial MRI at age 48 revealed a rim of low signal intensity in T2- and T2*-weighted images in the superior vermis folia and the right sylvian cortex. In addition, two small hemosiderin deposits were seen in the left parietal cortex. Lumbar puncture yielded colorless CSF with increased ferritin content and was followed by fourth ventricle hemorrhage. Cranial MRI 11 months later showed progression of brain hemosiderin deposits. The younger daughter had vitreal deposits, sensorimotor polyneuropathy, and acute sensorineural hearing but no evidence of siderosis on cranial MRI. She died at age 43 years of posterior fossa subarachnoid hemorrhage, and the neuropathologic examination showed amyloid deposition in the leptomeningeal spaces and vessels. CONCLUSION: Transthyretin-related amyloidosis may cause superficial siderosis of the CNS through subarachnoid bleeding related to meningovascular amyloid deposition.- - - - - - - - - - ranking = 1keywords = subarachnoid (Clic here for more details about this article) |
3/28. Surgical resection of a cerebral arteriovenous malformation for treatment of superficial siderosis: case report.BACKGROUND: To our knowledge, there are only two reported cases of cerebral arteriovenous malformation associated with superficial siderosis. In both cases, the patients were asymptomatic and were discovered on retrospective review of magnetic resonance imaging. CASE DESCRIPTION: We describe a case of superficial siderosis in a 47-year-old male with a remote history of closed head injury that presented with progressive hearing loss, cerebellar ataxia, and urinary incontinence. Lumbar puncture was indicative of active subarachnoid bleeding. cerebral angiography revealed a small vascular malformation that was resected.RESULTS: Pathological examination confirmed the diagnosis of the arteriovenous malformation. Six months after surgery the patient is neurologically stable with no further progression of clinical signs or symptoms. CONCLUSIONS: We report the first case of surgical resection of an intracranial arteriovenous malformation for the treatment of superficial siderosis. We emphasize the necessity of a detailed evaluation when superficial siderosis is suspected to localize and resect potential bleeding sources, because the disease is progressive and often irreversible.- - - - - - - - - - ranking = 0.5keywords = subarachnoid (Clic here for more details about this article) |
4/28. central nervous system superficial siderosis, headache, and epilepsy.Almost 95 cases of superficial siderosis of the central nervous system have been reported in the literature. These patients showed a clinical syndrome characterized by ataxia, deafness, pyramidal system involvement, and mental deterioration with xanthochromic cerebrospinal fluid and neuroradiological findings of hemosiderin deposits. About 30% of the patients had headache as an accompanying symptom. In the present case report, we describe a 33-year-old man with the typical clinical features of superficial siderosis, who complained, since aged 8, of a severe recurrent frontal headache often associated with loss of consciousness occurring after at least 2 hours of pain. The MRI and CSF findings were consistent with subarachnoid bleeding. In our patient, headache due to meningeal irritation by subarachnoid blood induced seizures as a probable reflex of extreme pain. carbamazepine and nimodipine prophylaxis dramatically reduced the frequency of headaches and seizures.- - - - - - - - - - ranking = 1keywords = subarachnoid (Clic here for more details about this article) |
5/28. Superficial siderosis of the central nervous system: a 70-year-old man with ataxia, depression and visual deficits.BACKGROUND: Superficial siderosis of the central nervous system (SSNS) is caused by cerebral, cerebellar and spinal cord tissue deposition of hemosiderin, often related to repeated episodes of subarachnoid hemorrhage. Typical symptoms include ataxia, sensorineural deafness and dementia. methods AND RESULTS: An elderly patient with SSNS presenting with ataxia, depression and severe visual impairment was admitted to the Unit of geriatrics of the University Hospital of Perugia, italy. Late diagnosis and the association of symptoms with SSNS prevented the possible surgical treatment of the disease. CONCLUSIONS: Recognition of uncommon clinical variants may facilitate early diagnosis of SSNS and improve therapeutic results.- - - - - - - - - - ranking = 0.5keywords = subarachnoid (Clic here for more details about this article) |
6/28. Superficial siderosis associated with multiple cavernous malformations: report of three cases.OBJECTIVE AND IMPORTANCE: Superficial siderosis is a rare but potentially devastating syndrome caused by recurrent subarachnoid hemorrhage. We present three cases of superficial siderosis associated with multiple cavernous malformations, and we review previous reports of superficial siderosis attributable to vascular malformations. CLINICAL PRESENTATION: patients most commonly present with progressive sensorineural hearing loss, cerebellar ataxia, and pyramidal signs. magnetic resonance imaging diagnosis may precede symptom development, however. In two of our cases, superficial siderosis was identified on magnetic resonance imaging scans in the absence of clinical symptoms. INTERVENTION: magnetic resonance imaging studies revealed hemosiderin deposition, characteristic of superficial siderosis, and multiple cavernous malformations in all three cases. Surgical intervention was not pursued. CONCLUSION: We conclude that patients with multiple cavernous malformations and those with perisubarachnoid lesions are at risk for the development of superficial siderosis. Clinicians should recognize the radiographic appearance of superficial siderosis and its clinical presentation in patients with vascular malformations.- - - - - - - - - - ranking = 1keywords = subarachnoid (Clic here for more details about this article) |
7/28. Superficial siderosis of the meninges and its otolaryngologic connection: a series of five patients.OBJECTIVE: To study the otolaryngologic disorders in patients with superficial siderosis of the meninges. BACKGROUND: Superficial siderosis of the central nervous system is a rare disorder characterized by progressive bilateral hearing loss and ataxia caused by recurrent bleeding into the subarachnoid space. The cerebellum, eighth cranial nerve, and olfactory nerve are particularly susceptible to the deposition of hemosiderin, which is responsible for the symptoms. The diagnosis is confirmed by magnetic resonance imaging. methods: The clinical notes of five patients with superficial siderosis of the meninges were reviewed with the intent of reporting the otolaryngologic symptoms and signs, the clinical investigations, and treatments. RESULTS: Four of the five patients had sensorineural deafness, two had smell disturbances, and three had gait abnormalities. magnetic resonance imaging was the most important investigation used to identify the condition. CONCLUSIONS: Superficial siderosis of the meninges is an important differential diagnosis in patients with progressive sensorineural deafness.- - - - - - - - - - ranking = 0.5keywords = subarachnoid (Clic here for more details about this article) |
8/28. Superficial siderosis of the central nervous system many years after neurosurgical procedures.Recurrent haemorrhage into the subarachnoid space causes superficial siderosis, which clinically manifests as cerebellar ataxia, sensorineural hearing loss, and myelopathy. Two patients developed clinical, radiological, and biochemical evidence of superficial siderosis many years after surgery. One had two posterior fossa operations, a left temporal craniectomy, and radiotherapy for a presumed brain tumour before developing clinical evidence of superficial sidersosis 37 years later. The other had small bilateral subdural collections from recurrent shunt revisions following posterior fossa surgery for a Chiari malformation, and then developed deafness and ataxia. The first patient currently has the longest recorded delay between presumed subarachnoid bleeding and clinical manifestations of superficial siderosis. Both patients provide further evidence that superficial siderosis of the central nervous system, a progressive neurodegenerative vascular condition, may be a delayed complication of neurosurgical procedures.- - - - - - - - - - ranking = 1.0220167362805keywords = subarachnoid, haemorrhage (Clic here for more details about this article) |
9/28. Superficial siderosis of the central nervous system: report of three cases and review of the literature.We present 3 cases and a review of the literature to demonstrate the current state of clinical diagnosis and therapy of superficial siderosis of the central nervous system. Typical symptoms were progressive cerebellar ataxia, spasticity and hearing loss. Repeated subarachnoid hemorrhage was indicated by persistent xanthochromia of the cerebrospinal fluid and confirmed by the presence of erythrophages, siderophages and iron-containing pigments. Deposition of free iron and hemosiderin in pial and subpial structures leads to intoxication of the central nervous system and represents the pathophysiological mechanism of superficial siderosis. Hypointensity of the marginal zones of the central nervous system on T2 weighted MR images indicates an iron-induced susceptibility effect and seems pathognomonic for superficial siderosis. In 39 of the 43 previously described cases superficial siderosis was verified by biopsy or autopsy. Today magnetic resonance imaging enables diagnosis at an early stage of the disease. Therapeutic management requires the elimination of any potential source of bleeding. In patients with unknown etiology no proofed therapy is yet available.- - - - - - - - - - ranking = 0.5keywords = subarachnoid (Clic here for more details about this article) |
10/28. hearing loss and vertigo in superficial siderosis of the central nervous system.PURPOSE: 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.methods and results 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. CONCLUSIONS: 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.- - - - - - - - - - ranking = 0.5keywords = subarachnoid (Clic here for more details about this article) |
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