Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0013362 (dysarthria)
 3,768 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

A 59-year-old man developed a staggering and wide based-gait in July 1990. Dysarthria, hearing loss, vexation and disturbance of memory appeared in January 1991. He consulted our clinic in May 1991, and cerebellar ataxia, neurogenic bladder, and cerebellar atrophy on brain CT were noted. Subsequently, he was followed as OPCA. Brain and spinal cord MRI (T2 and proton weighted images) revealed hypointensity on the surface of the Sylvian fissure, cerebellum, brainstem and spinal cord. We diagnosed this case as superficial siderosis because of the clinical course, i.e. cerebellar ataxia, dementia and sensorineural hearing impairment, and specific findings on MRI. We consider this case idiopathic superficial siderosis because the origin of the bleeding source was unknown. IMP-SPECT showed low perfusion in the cerebellum and frontal lobe where hemosiderin was heavily deposited. RI cisternography revealed a disturbance of CSF absorption even after 48 hours. The basic rhythm on EEG was slow alpha band with sporadic theta waves dominantly in the frontal lobe. His central conduction time on ABR and SEP was delayed, OKN was poorly elicited and ETT exhibited a staircase pattern. The physiological results as well as the clinical manifestations of the present case suggest that hemosiderin deposit on the surface of brain and spinal cord caused serious damage to the underlying structures.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:[A case of idiopathic superficial siderosis of the central nervous system]. 789 35

Superficial hemosiderosis (SH) of the CNS is a rare disease caused by repeated subarachnoid hemorrhage, with progressive superficial siderosis of the CNS. We report a patient with SH whose clinical picture was marked by progressive gait ataxia, hearing loss, dysarthria, and recurrent episodes of hemifacial spasm. Iron and ferritin levels in the CSF were significantly higher than in a control group of patients. Six month's treatment with the iron-chelating agent trientine dihydrochloride led to clinical improvement, with a concomitant reduction of CSF iron level. We suggest that, in addition to magnetic resonance imaging findings, CSF levels of iron and ferritin should be used as diagnostic criteria for SH, as well as to estimate the efficacy of iron chelation treatment.
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PMID:Superficial hemosiderosis of the central nervous system. 855 30

Two cases (case 1, a 45-year-old man; case 2, a 68-year-old man) of superficial siderosis of the central nervous system are presented. Main neurological symptoms were anosmia, sensorineural deafness, dysarthria, ataxia, and pyramidal tract signs. Lumbar puncture revealed bloody cerebrospinal fluid (CSF) in both cases. In case 1, the CSF became watery clear after administration of hemostatic medicines. T2-weighted magnetic resonance images showed cerebellar atrophy and marginal hypointensity of the brainstem, cerebellum, and the entire spinal cord. T2-weighted images of the cranial nerves showed hypointensity of the VIII nerves which were clinically impaired as compared with normointensity of the VII nerves which presented no clinical symptom. These findings may reflect difference in the degree of hemosiderin depostion between the VII and VIII nerves. While case 1 had a borderline score of WAIS-R (IQ79), case 2 showed overt dementia (performance IQ65). Positron emission tomography showed that cerebral blood flow and cerebral oxygen metabolism were reduced in the basal temporal lobes in both cases.
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PMID:[Two cases of superficial siderosis of the central nervous system. Findings of the cerebrospinal fluid, magnetic resonance imaging and positron emission tomography]. 882 97

We report a 57-year-old woman with superficial siderosis of the central nervous system. On physical examination, sensorineural hearing impairment, cerebellar ataxia, pyramidal tract signs, dysarthria, and neurogenic bladder were seen. Brain and spinal cord MRI (T2 and proton weighted images) revealed cerebellar atrophy and marginal hypointensity of the Sylvian fissure and brain stem, cerebellum and spinal cord. We diagnosed this patient as superficial siderosis because of the clinical course and specific findings on MRI. We consider this case idiopathic superficial siderosis because the source of the bleeding was unknown. Neuro-otological tests and BAEP disclosed retrocochlear deafness. Her central motor conduction time on MEP was delayed. We discussed the pathogenic mechanism of these conditions, and we concluded that we must take notice of myelinopathy in superficial siderosis and that MEP was useful for detecting them.
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PMID:[A case of superficial siderosis of the central nervous system--findings of the neuro-otological tests and evoked potentials]. 1019 5

A 39-year-old man suffering from progressive dysarthria, gait disturbance, and sensorineural deafness for 2 years was admitted to our hospital. He scored 28 points on the mini-mental state examination. He had previously undergone surgery at 24 years and 39 years of age for a cerebellar tumor (pilocytic astrocytoma). Superficial siderosis (SS) was diagnosed based on bloody cerebrospinal fluid (CSF) and the findings of T2-weighted head MRI that revealed marginal hypointensity of the surface of the cerebellum, brainstem, and cerebral cortex. After intravenous infusion and the oral use of hemostatic drugs (carbazochrome, tranexamic acid), the CSF became watery clear and his condition improved. Hemostatic drug therapy should be considered for SS.
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PMID:[A case of superficial siderosis treated with intravenous and oral hemostatic drugs]. 2378 26

Superficial siderosis of the central nervous system (SSCNS) is a rare syndrome resulting from hemosiderin deposits in neuronal tissues close to the cerebrospinal fluid. SSCNS is characterized by sensorineural deafness, cerebellar ataxia and signs of pyramidal tract dysfunction. The present study describes a patient with SSCNS that did not suffer from hearing loss, which is the most common symptom of SSCNS. The patient was a 48-year-old male, presenting with dizziness, ataxia and slurred speech. The patient's ataxia was characterized by dizziness, nystagmus, dysarthria, abnormal finger-nose pointing and heel-knee-shin tests and a positive Chaddock sign. The patient had suffered from a pontine hemorrhage two years prior to the study. Audiometric tests showed normal hearing during the hospital stay and at the two-month follow-up examination. The diagnosis of SSCNS was made based on magnetic resonance images, which showed areas of linear hypointensity on the surface of the pons with mild cerebellar atrophy. However, a long-term follow-up is required to monitor the hearing of the patient. Improved understanding of SSCNS is important for clinicians to identify SSCNS patients who present without typical clinical symptoms.
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PMID:Superficial siderosis of the central nervous system: A case report. 2578 Apr 38