1/46. Evidence for preganglionic pupillary involvement in superficial siderosis.A 36-year-old man presented with spinal myoclonus, ataxia, hearing loss, and unilateral pupillary dilation. MRI demonstrated hemosiderin deposition along the superficial surfaces of the brain, brainstem, cerebellum, and spine. The pupillary changes were localized to the preganglionic oculomotor nerve. In contrast to vasculopathic oculomotor nerve palsies, superficial siderosis may cause selective involvement of the superficially located pupillary fibers.- - - - - - - - - - ranking = 1keywords = brain (Clic here for more details about this article) |
2/46. 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 = 0.5keywords = brain (Clic here for more details about this article) |
3/46. 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.- - - - - - - - - - ranking = 544.52920379846keywords = central nervous system, nervous system, brain (Clic here for more details about this article) |
4/46. 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.- - - - - - - - - - ranking = 453.35766983205keywords = central nervous system, nervous system (Clic here for more details about this article) |
5/46. 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 = 170.72297618379keywords = central nervous system, nervous system (Clic here for more details about this article) |
6/46. 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 = 453.35766983205keywords = central nervous system, nervous system (Clic here for more details about this article) |
7/46. 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.- - - - - - - - - - ranking = 365.68613586564keywords = central nervous system, nervous system, brain (Clic here for more details about this article) |
8/46. 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.- - - - - - - - - - ranking = 90.67153396641keywords = central nervous system, nervous system (Clic here for more details about this article) |
9/46. 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 = 90.67153396641keywords = central nervous system, nervous system (Clic here for more details about this article) |
10/46. 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 = 453.85766983205keywords = central nervous system, nervous system, brain (Clic here for more details about this article) |
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