Cases reported "Siderosis"

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1/39. Superficial siderosis of the central nervous system and anticoagulant therapy: a case report.

    Superficial siderosis of the central nervous system is a rare condition characterized by deposition of haemosiderin in the leptomeninges and in the subpial layers of the brain and spinal cord. With the widespread use of magnetic resonance imaging, an increasing number of cases of superficial siderosis are being discovered, secondary forms being more frequent than idiopathic ones. We report a 78-year-old man in oral anticoagulant therapy, who presented neurosensory hearing loss, gait ataxia and spastic paraparesis. magnetic resonance imaging suggested the diagnosis of superficial siderosis of the central nervous system, without an evident bleeding source.
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2/39. Superficial siderosis of the central nervous system. A case report on examination by ECoG and DPOAE.

    This is a case of superficial siderosis of the central nervous system (SSCN). The diagnosis of SSCN was based on the result of T2-weighted magnetic resonance imaging and on suggestive clinical manifestations. The pure-tone audiogram showed bilateral progressive sensorineural hearing loss with a poor speech discrimination score and Jerger type IV. The remarkable elevation of the detective threshold of cochlear microphonics on electrocochleography was found and distortion product otoacoustic emission (DPOAE) showed no response: These electrophysiologic examinations, including electrocochleography and DPOAE, revealed that the progressive sensorineural hearing loss in this case was caused by both retrocochlear and cochlear damages.
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3/39. 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.
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4/39. 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.
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5/39. cochlear implantation in a patient with superficial siderosis of the central nervous system.

    OBJECTIVE: To perform an assessment of the viability of cochlear implantation in a patient with superficial siderosis of the brain. STUDY DESIGN: Case review. SETTING: Tertiary referral center. patients: Patient with superficial siderosis of the brain. INTERVENTION: cochlear implantation. MAIN OUTCOME MEASURES: Electrically evoked auditory brainstem responses and fitting strategy for cochlear implant based on patient's subjective responses. RESULTS: After some time, C levels and T levels showed a pattern comparable with the evoked auditory brainstem responses. CONCLUSIONS: Bilateral profound hearing loss after superficial siderosis of the brain is no absolute contraindication for implantation. Furthermore, evoked auditory brainstem measurements can potentially guide the fitting process of the implant in difficult cases.
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6/39. MRI findings in a case of a superficial siderosis associated with an ependymoma.

    We present the imaging findings of superficial siderosis of the central nervous system associated with an ependymoma of the posterior fossa in a patient who presented with progressive bilateral sensorineural hearing loss and cerebellar ataxia. The ependymoma was a homogeneous well-defined mass of the fourth ventricle without hydrocephalus. Secondary siderosis due to chronic bleeding from the ependymoma appeared as linear hypointensity delineating the surface of the cortex, thin and subtle on spin-echo T2-weighted images, thick and obvious on gradient-echo T2-weighted images.
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7/39. 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.
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8/39. 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.
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9/39. 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.
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10/39. The importance of suspecting superficial siderosis of the central nervous system in clinical practice.

    Once the central nervous system surface is greatly encrusted with haemosiderin, even removing the source of bleeding will have little effect on the progression of clinical deterioration. Superficial siderosis of the central nervous system is rare and insidious, but magnetic resonance imaging has turned a previously late, mainly autoptical diagnosis into an easy, specific, in vivo, and possibly early one. Avoiding long diagnostic delay will be very important in those cases susceptible of causal treatment.
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