Annals of neurology
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Annals of neurology · Nov 2000
Randomized Controlled Trial Clinical TrialEffect of citicoline on ischemic lesions as measured by diffusion-weighted magnetic resonance imaging. Citicoline 010 Investigators.
We examined the effect of the neuroprotective and neuroreparative agent citicoline on the growth of cerebral ischemic lesions in a double-blind placebo-controlled study involving patients with acute ischemic stroke using diffusion-weighted magnetic resonance imaging (DWI). Patients with acute ischemic stroke symptom onset 24 hours or less before the start of treatment, National Institutes of Health Stroke Scale (NIHSS) scores of 5 or higher, and lesions of 1 to 120 cc in cerebral gray matter by DWI were enrolled. DWI, T2-weighted magnetic resonance imaging (MRI), perfusion-weighted MRI, and magnetic resonance angiography were obtained at baseline, week 1, and week 12. ⋯ We found a significant inverse relationship between lesion volume change over 12 weeks as measured by MRI and clinical outcome for ischemic stroke. This relationship supports the role of DWI as a surrogate marker of clinically meaningful lesion progression in stroke clinical trials. The hypothesis that citicoline reduces lesion growth and improves clinical outcome will be tested further.
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The role of the insular cortex in the genesis of temporal lobe epileptic (TLE) seizures has been investigated in 21 patients with drug-refractory TLE using chronic depth stereotactic recordings of the insular cortex activity and video recordings of ictal symptoms during 81 spontaneous electroclinical seizures. All of the recorded seizures were found to invade the insula, most often after a relay in the ipsilateral hippocampus (19/21 patients). However, 2 patients had seizures that originated in the insular cortex itself. ⋯ A favorable outcome was obtained after a temporal cortectomy sparing the insular cortex in 15 of 17 operated patients. Seizures propagating to the insular cortex were found to be fully controlled by surgery, whereas those originating in the insular cortex persisted after temporal cortectomy. The fact that seizures originating in the insular cortex are not influenced by temporal lobectomy is likely to explain some of the failures of this surgical procedure in TLE.
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Annals of neurology · Oct 2000
Clinical, genetic, and expression studies of mutations in the potassium channel gene KCNA1 reveal new phenotypic variability.
Episodic ataxia type 1 (EA1) is an autosomal dominant central nervous system potassium channelopathy characterized by brief attacks of cerebellar ataxia and continuous interictal myokymia. Point mutations in the voltage-gated potassium channel gene KCNA1 on chromosome 12p associate with EA1. We have studied 4 families and identified three new and one previously reported heterozygous point mutations in this gene. ⋯ Heterologous expression of the proteins encoded by the mutant KCNA1 genes suggest that the four point mutations impair delayed-rectifier type potassium currents by different mechanisms. Increased neuronal excitability is likely to be the common pathophysiological basis for the disease in these families. The degree and nature of the potassium channel dysfunction may be relevant to the new phenotypic observations reported in this study.
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Annals of neurology · Oct 2000
Case ReportsAneurysm of a dural sigmoid sinus: a novel vascular cause of pulsatile tinnitus.
We report a newly evidenced cause of venous pulsatile tinnitus--the aneurysm of a dural sigmoid sinus. A 33-year-old patient presented with an incapacitating pulsatile tinnitus of 6 months' duration in the left ear. The radiological workup evidenced an aneurysm of the left sigmoid sinus. Selective endovascular coil occlusion of the aneurysm was followed by complete resolution of the tinnitus.
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Annals of neurology · Jul 2000
Multifocal or generalized tonic dystonia of complex regional pain syndrome: a distinct clinical entity associated with HLA-DR13.
We report on 26 patients with a distinct phenotype of complex regional pain syndrome that progressed toward a multifocal or generalized tonic dystonia. The dystonia initiated distally, involved mainly flexor muscles, and was associated with sensory and autonomic symptoms. ⋯ Compared with controls, a significant elevation of HLA-DR13 was found in the patients. Thus, HLA-DR13 may be a factor indicating susceptibility to this distinct phenotype of complex regional pain syndrome.