Journal of the neurological sciences
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The objective of this review is a summary of the clinical and electrographic findings in those forms of epilepsy to which the term 'extratemporal' (ExT) can be applied. They form a group that differs in many ways from the better known temporal lobe epilepsies. ⋯ The concept of 'dual pathology' implies the coexistence of two or more distinct lesions, typically mesial temporal sclerosis and cortical dysplasia. Electroencephalography (EEG) and electrocorticography (ECoG) are valuable tests in the definition of the epileptogenic area beyond the structural lesion, and surgical removal must be guided by the nature of the lesion and the extent of the epileptogenic zone.
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Review Case Reports
Histiocytic necrotizing lymphadenitis (Kikuchi's disease) with aseptic meningitis.
Histiocytic necrotizing lymphadenitis, or Kikuchi's disease (KD), is a self-limited clinicopathologic entity recognized increasingly worldwide. A 27-year-old man with cervical lymphadenopathy and fever who was diagnosed with KD developed mild headache with no nuchal rigidity. ⋯ Eleven similar cases have been reported, all but one from Japan. The development of meningitis in KD was observed in four (9.8%) of 41 KD patients we have treated, suggesting that the meningitis was related to KD and not merely coincidental.
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Reperfusion aggravates nerve ischemic fiber degeneration, likely by the generation of reduced oxygen species. We therefore evaluated if racemic alpha-lipoic acid (LA), a potent antioxidant, will protect peripheral nerve from reperfusion injury, using our established model of ischemia-reperfusion injury. ⋯ These results suggest that alpha-lipoic acid is efficacious for moderate ischemia-reperfusion, especially on distal sensory nerves.
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Case Reports
Infantile onset spinocerebellar ataxia with sensory neuropathy (IOSCA): neuropathological features.
Infantile onset spinocerebellar ataxia (IOSCA, MIM 271245) is a recessively inherited, progressive neurological disease, which we have described in 19 Finnish patients. The clinical symptoms of IOSCA include ataxia, athetosis, hypotonia, hearing deficit, ophthalmoplegia, sensory neuropathy, female hypogonadism, and epilepsy as a late manifestation. We have mapped the IOSCA locus to 10q24. ⋯ The cerebellar cortex showed patchy atrophy. Degenerative changes were seen in dorsal root ganglia, and there was a severe axonal loss in the sural nerve. The neuropathological picture of IOSCA thus seems close to that reported in Friedreich's ataxia, another recessively inherited usually childhood-onset ataxia.
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In an attempt to identify risk factors for the development of idiopathic cerebellar ataxia (IDCA) we performed a case-control study of 59 IDCA patients. Hypertension and medicine intake were less frequent in IDCA than in neurological controls. ⋯ Some of these factors have been previously shown to be associated with other neurodegenerative diseases. In addition, serum antibody titers against neurotropic viruses were not elevated in IDCA.