Sudden hemichorea and frontal lobe syndrome: a rare presentation of unbalanced polycythaemia vera.

Polycythaemia vera (PV) is an haematological neoplasm that frequently presents neurological symptoms. However, chorea is a rare complication of this disease, occurring in less than 5% of the patients. Cognitive impairment related to PV unbalanced is also a rare complication, and it can improve with proper treatment. We present a 96-year-old-man with acute-onset hemichorea and frontal lobe syndrome with no vascular pathology in the basal ganglia or frontal region. A clear relationship was observed between the onset of involuntary movements and the cognitive impairment and worsening of haematological parameters in the patient. After causal and symptomatic treatment, the patient's clinical status improved. In the elderly, PV must be considered as a cause of acute chorea and sudden cognitive impairment, as early diagnosis leads to effective treatment and prevention of complications.

IFN beta 1a as Glucocorticoids-Sparing Therapy in a Patient with CLIPPERS.

BACKGROUND: Chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids (CLIPPERS) is a recently described inflammatory disease of the central nervous system, distinguished by brainstem- and spinal cord-centered lesions with a characteristic contrast enhancement on MRI, a lymphocytic perivascular infiltrate on pathological exam, and a dramatic response to and dependence on steroids therapy. Since its initial description in 2010, different glucocorticoid-sparing agents, mostly immunosuppressant drugs, have been used to minimize the dosage, but these therapies also carry the risk of important secondary effects. We present the first reported case of CLIPPERS treated with interferon beta 1a as add-on therapy. CASE REPORT: A previously healthy 31-year-old man presented with gait ataxia and dysarthria. MRI showed pons-centered hyperintense patchy lesions on T2-weighted images. Additional tests ruled out other possible diagnoses and symptoms reversed with intravenous methylprednisolone. Over the years the patient presented with several episodes of deterioration each year, which were partly reversed with glucocorticoid therapy, but leaving him with growing sequelae. Four years after the initial event, treatment with interferon-beta-1a was initiated, achieving reduced frequency of the relapses to 1 every 4 years, which were no longer associated to increasing disability. This allowed reducing glucocorticoids to 30 mg of Deflazacort every other day. CONCLUSIONS: Interferon beta-1a could be an alternative to corticosteroid-combined therapy in CLIPPERS and its more benign profile of secondary effects compared to immunosuppressants could make it an attractive choice.

Factors associated with the differences in migraine prevalence rates between Spanish regions.

We have analyzed the relation of prevalence rates in Spanish regions with a series of human, environmental, and ecological factors. We find that the variability in migraine rates found between Spanish regions may be explained by interregional differences in the percentage of daily smokers, percentage of alcohol consumers, percentage of population presenting physical and/or psychological life-limiting conditions, percentage of population engaging in physical exercise, minimum absolute temperatures per year, number of days under 0 °C per year, and altitude.

[Spinal cord compression: a multidisciplinary approach to a real neuro-oncological emergency. Reply]. / Compresion medular: aproximacion multidisciplinar a una autentica urgencia neurooncologica. Replica.

TITLE: Compresion medular: aproximacion multidisciplinar a una autentica urgencia neurooncologica. Replica.

[Spinal cord compression: a multidisciplinary approach to a real neuro-oncological emergency]. / Compresion medular: aproximacion multidisciplinar a una autentica urgencia neurooncologica.

Spinal cord compression must be considered a top-priority neuro-oncological emergency. Hence, a multi-disciplinary approach and swiftness in establishing appropriate therapeutic measures are crucial to optimise the functional (and perhaps vital) prognosis of these patients. The nihilistic attitudes that have prevailed up until now in some professional sectors, possibly stemming from the perception of a poor short-term prognosis, must be completely eradicated. The overall improvement in survival rates among cancer patients in general, the availability of new neurosurgical techniques in the vast majority of our hospitals and the obvious improvements in radiotherapy equipment and techniques all this pathology to be addressed with greater chances of success. This greater likelihood of accomplishing a better outcome refers not only to the control of the development of the tumour itself, but also to pain control, maintenance of the functioning of the spinal cord and the overall survival of the patient. In this context, we consider it essential for all hospitals to have specific protocols on how to proceed in cases of acute spinal cord compression. The fact that this kind of protocol has been introduced in the Centro Medico de Asturias has prompted us to conduct a review of the current state-of-the-art in this field, with special emphasis on the evidence available for each of the modes of therapy that are discussed.

[Transorbital echography for assessment of optical nerve atrophy in demyelinating diseases: a pilot study]. / Utilidad del dúplex orbitario para la valoración de atrofia del nervio óptico en enfermedades desmielinizantes: estudio piloto.

INTRODUCTION. Demielinating diseases are a group of heterogenic diseases in whom mieline is attacked. The optic nerve (ON) is one of the most commonly affected. SUBJECTS AND METHODS. An observational prospective case-control study with ON orbital echography was developed. The case group was formed by 31 demielinating diseases patients and the control group was formed by 24 healthy people. Mean age of cases: 48.3 ± 11.8 years old, controls 48.7 ± 9.9 years old. 46% of controls and 35% of cases were males. RESULTS. We found statistical significance differences between cases and controls regarding the diameter of right (controls 3.64 ± 0.58 mm vs patients 2.84 ± 0.56 mm; p < 0.001) and left ON (controls 3.95 ± 0.84 mm vs patients 2.74 ± 0.54 mm; p < 0.001). We found no differences between maximum systolic and median velocities regarding ophthalmic arteries in both groups, neither for previous acute optical neuritis history or visual evocated potentials. CONCLUSIONS. ON evaluation with transorbital echography is an easy, feasible, non invasive, useful and costless technique for the evaluation of the ON atrophy. As for visual evocated potentials are abnormal in a huge number of patients without previous optical neuritis evidence, the diameter of ON measured by transorbital Doppler could be a consistent paraclinic marker of these diseases.

[Precipitating factors of migraine attacks in patients attended in neurology services. The FACTOR study]. / Factores precipitantes de crisis de migraña en pacientes atendidos en consultas de neurología. Estudio FACTOR.

INTRODUCTION: Patients with migraine often report factors or circumstances that precipitate or trigger their attacks. Yet few studies have been conducted to examine this matter. AIM: To explore the factors that precipitate migraine in our setting, as well is their possible relation with the intensity of the attacks or the overall repercussion of migraine. PATIENTS AND METHODS: An epidemiological, cross-sectional, multi-centre study was conducted in neurology consultation services. Sociodemographic and clinical data were collected and the precipitating factors were identified from a closed list. The specific migraine disability questionnaire -Headache Impact Test (HIT-6)- and the measurement of the number of lost workday equivalents were used in the study. RESULTS: Altogether 817 patients were recruited (72.5% females, mean age: 34.6 ± 10.3 years). A total of 70.5% of the patients had severe disability according to the HIT-6. The mean monthly number of lost workday equivalents was 2.1 ± 2.5. A total of 96.6% of the patients identified some precipitating factor for the attacks, the most commonly reported being hormonal (75.2%), stress (70.9%) and those related with disorders affecting sleep patterns (68.4%). CONCLUSIONS: The FACTOR study confirms that most patients with migraine identify some circumstance that precipitates their attacks. Controlling or avoiding these factors, whenever possible, must be part of the programme of education received by patients suffering from migraine.

One-year prevalence of migraine in Spain: a nationwide population-based survey.

AIM: The purpose of the study was to estimate the one-year prevalence of migraine among a population-based sample of Spanish adults. METHOD: Men and women aged 18-65 years were selected at random according to quotas for age, sex, size of habitat (<10,000 inhabitants, 10,001-50,000 inhabitants, 50,001-200,000 inhabitants and >200,000 inhabitants) and residence proportional to the population size of the geographical location. A random-digit-dial, computer-assisted telephone interview (CATI) survey was conducted between April and July 2006. The 2004 International Headache Society operational diagnostic criteria were applied. RESULTS: From a total of 70,692 telephone calls and 26,255 (31.7%) valid contacts, 5,668 (21.6%) respondents completed the CATI survey. A total of 476 subjects (8.4%, 95% confidence interval [CI] 7.7-9.1%) with strict migraine and 236 with probable migraine (4.2%, 95% CI 3.7-4.7%) were recorded. The 1-year prevalence of total migraine (N = 712) was 12.6% (95% CI 11.6-13.6) (17.2% in females, 8.0% in males). The prevalence rates showed significant geographic variations, from 7.6% in Navarra to 18% in the Canary Islands. One-half of the subjects had migraine with aura. One-third of subjects were never diagnosed for migraine. CONCLUSIONS: The one-year prevalence of migraine in Spain is 12.6%, with a prevalence of migraine with and without aura of 8.4% and probable migraine of 4.2%. These findings add data to the current understanding of migraine.

[Satisfaction of the migraine patient attending neurology clinics: results of CIEN-mig project (II)]. / Satisfacción del paciente con migraña que acude a consultas de neurología: resultados del proyecto CIEN-mig (II).

INTRODUCTION: Migraine is the most common neurological consultation in our country. AIM. To analyse the satisfaction level of the patient with migraine attending our clinics. PATIENTS AND METHODS: A total of 160 neurologists asked 10 consecutive migraine patients to fill in a satisfaction questionnaire. RESULTS: The questionnaire was answered by 1330 patients. The most important factors they considered for their neurologists were professional experience and kindness. Patients thought neurologist should improve in time to explain treatment and accessibility. Almost 90% of patients considered that treatments for migraine had improved, but almost half of them were not satisfied with the management in primary care setting. Patients were more satisfied with symptomatic treatment (69.1%) than with preventatives (50.4%). More than half had a neuroimaging study due to their headaches. Only half thought that such an study would improve their satisfaction. CONCLUSIONS: The level of satisfaction of the migraine patient attending our clinics has improved in the last years. In patients' opinion the aspects which should be improved are the time to explain migraine and its treatment, accessibility and preventive treatment.

[Chronic migraine as seen by neurologists and patients: results of the CIEN-mig project (III)]. / La migraña crónica vista por el neurologo y el paciente: resultados del proyecto CIEN-mig (III).

INTRODUCTION: Data on the reality of chronic migraine in our clinics are scarce. AIM. To know the frequency of consultation and attitude of the neurologist regarding chronic migraine, and the opinion of patients with this condition. SUBJECTS AND METHODS: A total of 145 neurologists consulted electronically two questionnaires. The first tested the impact of chronic migraine and their approach in the daily practice. The second was filled in by the consecutive patients. RESULTS: A total of 4.1% of patients consulted due to chronic migraine, mostly women in their forties. The average time for diagnosis was 28.7 months. All neurologists recommended preventive treatment from the beginning. More than a quarter kept this treatment for more than a year and used drug combinations. Only half thought that overuse was the reason for chronic migraine and the majority allowed non-steroidal anti-inflammatories and triptans at restricted doses. Only half of the patients knew they had chronic migraine. In their opinion, heredity and the absence of an early and serious treatment could contribute for chronic migraine development. CONCLUSIONS: Almost one patient per day consults due to chronic migraine, which gives an idea of its impact. A better feed-back with primary care level is necessary, if we consider that the delay in the diagnosis is longer than two years. The majority of neurologists follows the current recommendations of the Headache Group of the Spanish Society of Neurology.

[Approach of neurologists in Spain to migraine: results of the CIEN-mig project (I)]. / Actitud del neurólogo español frente a la migraña: resultados del proyecto CIEN-mig (I).

INTRODUCTION: Headache is the most frequent neurological reason for consultation in our country. AIM: To obtain descriptive data on the approach and satisfaction of the neurologist regarding the diagnosis, burden and management of headaches, and migraine in particular, together with the relationship with primary care physicians (PCP). SUBJECTS AND METHODS: A total of 160 neurologists participated and answered electronically five questionnaires during one year. The results for patients were obtained in a standard clinic day and for the first 10 patients. RESULTS: The mean number of patients/day was 20.7. Thirty-six percent consulted due to headache, most under 50 and women. Half had migraine; in 70% daily activities were moderate-severely affected. Almost half had missed at least one work day due to migraine in the previous month. Neurologist considered that headache background of PCP should improve, mainly regarding preventive treatment. Complementary studies were asked in a rather low proportion of patients; CT (35%) being the most frequent. The majority indicated triptans, and in the mild phase of pain, and neuromodulators or beta-blockers for the preventive treatment. CONCLUSIONS: Migraine is the first reason for consultation in the Neurology Services of our country and impacts negatively in the quality of life of our patients, usually women below 50. Its management should be improved with a better feedback with PCP and the use of the most efficacious treatments.

[Zonisamide in the preventive treatment of refractory migraine]. / Zonisamida en el tratamiento preventivo de la migraña refractaria.

INTRODUCTION: Chronic migraine refractory to preventive treatment is a common clinical situation in general neurology clinics. The aim is to analyse our experience with zonisamide in the preventive treatment of patients with frequent refractory migraine. PATIENTS AND METHODS: Those patients with no response or intolerance to topiramate and at least one more preventative received zonisamide. This drug was increased 25 mg per week up to 200 mg/day. The efficacy of zonisamide was evaluated in terms of 'response' (reduction in attack frequency below 50%) at the third month of treatment. RESULTS: Our series comprises a total of 172 patients, with ages ranging from 22 to 69 years. 85% were women. The final dosage of zonisamide was 50-200 mg/day, with the 100 mg/day being the most frequently administered dose. Zonisamide was efficacious (response) in 76 (44%) patients; response being excellent in 22 (13%). MIDAS score was reduced by 43.2%. Zonisamide was not tolerated by 27% of the patients, mainly due to subjective mental slowness or digestive symptoms. CONCLUSIONS: These results, obtained in a big sample of patients refractory or intolerant to topiramate and other preventatives, indicate that, at least in conditions of daily clinical practice, zonisamide, at relatively low dosages, is an option to be considered for the preventive treatment of patients with frequent migraine.

Marketed oral triptans in the acute treatment of migraine: a systematic review on efficacy and tolerability.

BACKGROUND: In the current literature, there is neither a reported systematic review comparing the efficacy of triptans at 30 minutes and 1 hour after the migraine treatment, nor data related to efficacy of new marketed triptans. OBJECTIVE: The main objective of this analysis was to compare the efficacy and tolerability of currently marketed oral, non-reencapsulated triptan formulations vs placebo in the treatment of moderate-to-severe migraine attacks. METHODS: A systematic review of double-blind, randomized clinical trials reporting data after a single migraine attack was conducted. Efficacy results are shown using relative risk ratios with 95% confidence intervals. A sensitivity analysis was also conducted. RESULTS: After reviewing 221 publications, 38 studies were included. All marketed triptans provided significant relief and/or absence of pain at 2 hours, and relief at 1 hour when compared with placebo. After 30 minutes, fast-dissolving sumatriptan 50 and 100 mg, sumatriptan 50 mg, and rizatriptan 10 mg showed significant relief when compared to placebo, whereas the fast-dissolving formulation of sumatriptan 100 mg was the only oral triptan that was superior to placebo in meeting the pain-free endpoint. On the other hand, fast-dissolving sumatriptan 50 and 100 mg and eletriptan 40 mg showed a lower rate of recurrence than placebo, whereas rizatriptan 10 mg was the only triptan with a recurrence rate greater than that of placebo. Adverse events associated with treatment with tablet formulations of sumatriptan and zolmitriptan were significantly more frequent than those of the placebo group. The inclusion of trials with reencapsulated triptans in the analysis introduced minor specific changes in these results. CONCLUSION: This analysis updates the comparative data available for the 7 currently marketed oral triptans and clearly demonstrates their efficacy when compared to placebo, even with stricter endpoints, such as efficacy at 30 minutes. No triptan exhibited better tolerability than placebo. Results are diverse, depending on the triptan, which probably is a reflection of heterogeneous pharmacokinetics.

Efficacy of therapeutic intervention in headache units in patients with frequent headaches: the EFUNCE study.

Data confirming that therapeutic intervention in headache units is superior to care received by patients in other levels of the health system are scant. This is a pilot study that includes patients seen in 4 headache units for at least 1 year, who had a headache frequency of more than 15 days per month. The results of the first 30 patients showed a significant improvement in different headache parameters and a high degree of satisfaction with the treatment received.

Preventing disturbing migraine aura with lamotrigine: an open study.

BACKGROUND: Lamotrigine has been suggested as possibly effective for preventing migraine aura. OBJECTIVE: To describe our experience with a series of patients with disturbing migraine aura treated with lamotrigine. METHODS: The members of the Headache Group of the Spanish Society of Neurology were sent an ad hoc questionnaire to collect patients treated with lamotrigine due to disturbing migraine aura. The main outcome parameter ("response") was a >50% reduction in the mean frequency of migraine auras at 3 to 6 months of treatment. RESULTS: A total of 47 patients had been treated with lamotrigine due to severe migraine aura. Three could not complete the protocol as a result of developing skin rashes. Thirty (68%) patients responded. These were 21 females and 9 males whose ages ranged from 19 to 71 years. Eight suffered from migraine with "prolonged" aura, 8 typical aura with migraine headache (but had frequent episodes including speech symptoms), 6 basilar-type migraine, 6 typical aura without headache, and 2 hemiplegic migraine. Fifteen had been previously treated, without response, with other preventatives. The mean monthly frequency of migraine auras in these 30 patients changed from 4.2 (range: 1 to 15) to 0.7 (range: 0 to 6). Response was considered as excellent (>75% reduction) in 21 cases (70% of responders). Auras reappeared in 2 months in 9 out of 13 patients where lamotrigine was stopped, and ceased as soon as this drug was reintroduced. CONCLUSIONS: Lamotrigine should be considered in clinical practice for the preventive treatment of selected patients with disturbing migraine auras. Lamotrigine seems worthy of a controlled trial as prophylaxis of migraine aura.

Crossover comparison of efficacy and preference for rizatriptan 10 mg versus ergotamine/caffeine in migraine.

Rizatriptan is a selective 5-HT(1B/1D) receptor agonist with rapid oral absorption and early onset of action in the acute treatment of migraine. This randomized double- blind crossover outpatient study assessed the preference for 1 rizatriptan 10 mg tablet to 2 ergotamine 1 mg/caffeine 100 mg tablets in 439 patients treating a single migraine attack with each therapy. Of patients expressing a preference (89.1%), more than twice as many preferred rizatriptan to ergotamine/caffeine (69.9 vs. 30.1%, p < or = 0.001). Faster relief of headache was the most important reason for preference, cited by 67.3% of patients preferring rizatriptan and 54.2% of patients who preferred ergotamine/caffeine. The co-primary endpoint of being pain free at 2 h was also in favor of rizatriptan. Forty-nine percent of patients were pain free 2 h after rizatriptan, compared with 24.3% treated with ergotamine/caffeine (p < or = 0.001), rizatriptan being superior within 1 h of treatment. Headache relief at 2 h was 75.9% for rizatriptan and 47.3% for ergotamine/caffeine (p < or = 0.001), with rizatriptan being superior to ergotamine/caffeine within 30 min of dosing. Almost 36% of patients taking rizatriptan were pain free at 2 h and had no recurrence or need for additional medication within 24 h, compared to 20% of patients on ergotamine/caffeine (p < or = 0.001). Rizatriptan was also superior to ergotamine/caffeine in the proportions of patients with no nausea, vomiting, phonophobia or photophobia and for patients with normal function 2 h after drug intake (p < or = 0.001). More patients were (completely, very or somewhat) satisfied 2 h after treatment with rizatriptan (69.8%) than at 2 h after treatment with ergotamine/caffeine (38.6%, p < or = 0.001). Recurrence rates were 31.4% with rizatriptan and 15.3% with ergotamine/caffeine. Both active treatments were well tolerated. The most common adverse events (incidence > or = 5% in one group) after rizatriptan and ergotamine/caffeine, respectively, were dizziness (6.7 and 5.3%), nausea (4.2 and 8.5%) and somnolence (5.5 and 2.3%).