Blood flow velocity changes induced by the focal brain activation can be assessed by using Transcranial Doppler sonography (TCD). To explore the hemispheric dominant hemodynamic effect by performing calculation, we applied TCD technique to detect cerebral blood velocity asymmetry. METHODS Twenty healthy right-handed subjects were tested. We measured mean flow velocities (MFV) in the middle and posterior cerebral arteries (MCA, PCA). Photic stimulation was given separately as a control task for calculation. RESULTS While the photic stimulation increased the blood velocity only in PCAs, performing calculation increased the velocity in MCAs. Right-left difference was noted by performing calculation with greater increase in left MCA (right: 8.8 %; left: 21.2 %). CONCLUSION Application of TCD enabled us to measure the hemodynamic changes supporting the left hemispheric dominance of calculation task. Futhermore, increase in non-dominant right hemisphere suggests the functional interaction of cerebral activity.
Blood Flow Velocity* ; Brain ; Dominance, Cerebral ; Hemodynamics ; Passive Cutaneous Anaphylaxis ; Photic Stimulation ; Posterior Cerebral Artery ; Ultrasonography, Doppler, Transcranial

Cerebellar degeneration is a group of diseases that manifests as progressive ataxia, that finally led to death without specific treatment. We report here two patients with cerebellar degeneration, who had shown an improvement and less progressive course, which is associated with panax ginseng intake. Patient 1 was a 60-year-old woman with multisystem atrophy (MSA) type C with 5 year history of ginseng ingestion. Patient 2 was a 54-year-old woman with spinocerebellar ataxia (SCA) type 6, who had a history of ginseng intake for 30 months. Both the patients showed atrophic change in the cerebellum by brain magnetic resonance imaging. Cerebellar functions had been semi-quantified by International Cooperative Ataxia Rating Scale (ICARS) and monitored before and after the ginseng ingestion every 6 to 12 months. In Patient 1 with MSA type C, ICARS had improved from 21 to 17.5 ± 1.8 in the following 5 years. In Patient 2 with SCA, ICARS also showed an improvement from 22 to 6.0 ± 1.0 over 30 months. However, when she stopped taking ginseng, it progressed up to 13 points in two years. These observations provide a potential disease-modifying effect of ginseng on patients with cerebellar degeneration.
Cerebellar Ataxia ; Cerebellar Diseases

Background: Renin-angiotensin systems (RAS) are involved in the physiology of migraine. Ramipril is an angiotensin-converting enzyme inhibitor. We tested whether ramipril has an effect on migraine. Methods: The study was designed as a prospective open-labeled trial in a single center. All patients were asked to maintain a headache diary. Ramipril was administered at 5mg/day (2.5mg twice a day) and subjects were checked every 4 weeks up to 12 weeks. Results: The mean number of headache days was 19.9 ± 11.2 days per month at baseline, and 12.0 ± 11.5 at 12 weeks (p<0.001 vs. baseline) with a responder rate of 41.9%. Stratified analysis by migraine frequency (15 days a month > vs. <15 days a month) did not show a difference. Mean blood pressure was not altered. Conclusions: Ramipril prevented migraine attacks independently from blood pressure. This result supports a link between renin-angiotensin system and migraine pathophysiology (Clinicaltrials.gov identifier: NCT01402479).

Hyperhomocysteinemia associated with methylene terahydrofolate reductase (MTHFR) mutation can be a risk factor for idiopathic cerebral venous thrombosis. We describe the first case of MTHFR 677TT homozygote with cerebral venous thrombosis and livedo reticularis. A 45-year-old man presented with seizures and mottled-like skin lesions, that were aggravated by cold temperature. Hemorrhagic infarct in the right frontoparietal area with superior sagittal sinus thrombosis was observed. He had hyperhomocysteinemia, low plasma folate level, and MTHFR 677TT homozygote genotype, which might be associated with livedo reticularis and increase the risk for cerebral venous thrombosis.
Cold Temperature ; Folic Acid ; Genotype ; Homozygote* ; Humans ; Hyperhomocysteinemia ; Livedo Reticularis* ; Methylenetetrahydrofolate Reductase (NADPH2) ; Middle Aged ; Oxidoreductases ; Plasma ; Risk Factors ; Seizures ; Skin ; Superior Sagittal Sinus ; Thrombosis ; Venous Thrombosis*

We report on an angioedema patient with a genetic defect in complement 1 inhibitor, manifesting migraine-like episodes of headache, effective prophylaxis with Danazol, and triptan for a treatment of acute clinical episode. The patient was 44-yr-old Korean man with abdominal pain and headache, who was brought into the Emergency Department of Seoul National University Hospital, Seoul. He suffered from frequent attacks of migraine-like headache (3-7 per month), pulsating in nature associated with nausea. Severities were aggravated by activity and his headache had shown recent progression with abdominal pain. No remarkable findings were observed on radiologic examination, brain magnetic resonance images and intracranial and extracranial magnetic resonance angiography. Danazol 200 mg every other day was subsequently used. Following administration of Danazol, symptoms showed improvement and the patient was discharged. While taking Danazol, the migraine-like episodes appeared to be prevented for about 2 yr. At the eighth month, he suffered a moderate degree of migraine-like headache; however, administration of naratriptan 2.5 mg resolved his problem. A case of genetic defect of C1-INH deficiency presented with headache episodes, and was controlled by Danazol and triptan. It suggests that pathogenic mechanism of headache in hereditary angioedema may be mediated by the neurogenic inflammatory-like physiology of migraine.
Adult ; Angioedemas, Hereditary/*complications/diagnosis/*genetics ; Brain/radionuclide imaging ; Complement C1 Inhibitor Protein/*genetics/metabolism ; Danazol/therapeutic use ; Estrogen Antagonists/therapeutic use ; Humans ; Magnetic Resonance Angiography ; Male ; Migraine Disorders/*diagnosis/drug therapy/*etiology ; Piperidines/therapeutic use ; Tryptamines/therapeutic use ; Vasoconstrictor Agents/therapeutic use

Migraine often runs in families and is associated with both genetic and environmental factors. Clinical and genetic heterogeneity as well as the influence of environmental factors have hampered the identification of the gene responsible for migraine disorder. Family/twin studies suggest the presence of hereditary susceptibility. Several different types of mutations or association studies with genetic polymorphism in neurotransmitters, inflammatory cytokines, homocysteine metabolism, mitochondria, or other risk genes in cerebrovascular disorders have been reported. Recently, progress of molecular genetics in familial hemiplegic migraine has provided important insights, a channelopathy, and now extending to a growing list of membrane excitability disorders. Further identification of candidate genes for migraine and exploring the correlation between phenotype and genotype are expected in the future for the understanding of migraine pathophysiology.
Cerebrovascular Disorders ; Channelopathies ; Cytokines ; Genetic Heterogeneity ; Genetics* ; Genotype ; Homocysteine ; Humans ; Membranes ; Metabolism ; Migraine Disorders* ; Migraine with Aura ; Mitochondria ; Molecular Biology ; Neurotransmitter Agents ; Phenotype ; Polymorphism, Genetic

No abstract available.
Migraine Disorders*

BACKGROUND: Migraine is commonly associated with anxiety disorder. However, whether anxiolytic medicine or changes in anxiety levels affect the migraine attack is unclear. Buspirone, the agonist for 5-HT1A receptor, is effective in treating generalized anxiety disorder. In this study, we attempted to test the efficacy of buspirone for migraine combined with anxiety disorder. METHODS: 111 outpatients aged 20 to 70 years (mean, 46.4; SD, 12.8), were analyzed. The diagnosis of migraine was made according to the HIS (International Headache Society) criteria, and the level of anxiety was rated by the Hamiton Anxiety Rating Scale (HAM-A). The migraineurs were randomly assigned to treatment with either buspirone (15 mg/day) or placebo for 6 weeks. The efficacy variables included changes in headache frequency, headache intensity, Headache Index, Headache Management Self-Efficacy Scale (HMSE), Headache Disability Inventory (HDI), and the Hamilton Anxiety Rating Scale (HAM-A). The correlation between headache improvement and the anxiolytic effect were analyzed. RESULTS: Headache frequency showed a 43.3% reduction (from 6.7/2 weeks to 3.8/2 weeks) in the buspirone-treated group, whereas a 10.3% reduction (from 6.8 to 6.1/2 weeks) in the placebo group. The Headache Index, HDI, and HAM-A were also significantly lowered in buspirone-treated patients than in placebo-treated patients. However, the headache intensity or the HMSE score were not changed. Correlation analysis between the change of Headache Index and that of HAM-A revealed no significant association. CONCLUSIONS: Buspirone is an effective prophylactic agent in migraine combined with anxiety disorder. This prophylactic effect is not secondary to the anxiolytic effect. This suggests that the agonistic action for 5-HT1A is primarily effective in migraine prophylaxis.
Anti-Anxiety Agents ; Anxiety Disorders ; Anxiety* ; Buspirone* ; Diagnosis ; Headache ; Humans ; Migraine Disorders ; Outpatients ; Receptor, Serotonin, 5-HT1A

Objective: The purpose of the present study was to screen the prevalence of aneurysms in migraineurs; to differentiate presenting features in migraineurs with and without aneurysm; and also to correlate the locations of aneurysm to the clinical features of migraine. Methods: A total of 4,416 subjects were interviewed and completed self-reported questionnaires on headache. Of these, 1,773 subjects diagnosed to have migraines based on the International Classification of Headache Disorders II (ICHD-II) criteria were screened for aneurysm by magnetic resonance angiography (MRA). When aneurysm was suspected, further investigation with trans femoral cerebral angiography (TFCA) or three dimensional computerized tomography (CT) angiography was performed. Based upon MRA findings, subjects were grouped into unruptured aneurysm migraine patients (UAMP) and no aneurysm migraine patients (NAMP). Results: The prevalence of aneurysm was 3.6% (63 of 1,773) with the mean age of 56.0 years, which were not different from those of general population. There was no difference in migraine subtypes between UAMP and NAMP. Aggravation of headache by estrogen replacement therapy during menopause (p=.039), history of migraine in young age (p= .021), diplopia (p=.026), and retroauricular pain (p=.025) were significantly associated with presence of aneurysm. Although aneurysms were detected more in anterior circulation, there was no correlation between aneurysm site and headache location. The average size of aneurysm was 3.5 ± 2.1 mm and none were ruptured. Interventional therapy of aneurysm did not alter the feature of migraine. Conclusions: The incidence of aneurysm was not different in migraine patients as compared to the general population. Some features which significantly differentiate whether migrainuers have aneurysm or not warrant further study to have a predictive and localizing value.

Huntington’s disease (HD) has become a target of the first clinical trials for gene therapy among movement disorders with a genetic origin. More than 100 clinical trials regarding HD have been tried, but all failed, although there were some improvements limited to symptomatic support. Compared to other neurogenetic disorders, HD is known to have a single genetic target. Thus, this is an advantage and its cure is more feasible than any other movement disorder with heterogeneous genetic causes. In this review paper, the authors attempt to cover the characteristics of HD itself while providing an overview of the gene transfer methods currently being researched, and will introduce an experimental trial with a preclinical model of HD followed by an update on the ongoing clinical trials for patients with HD.