Reversible Homonymous Inferior Quadrantanopia in a Nonketotic Hyperglycemic Patient.

Homonymous hemianopia is frequently associated with retrochiasmal lesions. Vascular etiology is the most common and usually evident on magnetic resonance imaging. When the results of neuroimaging are normal, there are other etiologies that we should consider, like nonketotic hyperglycemia (NKH). We report a 62-year-old female diabetic patient with headache, colour vision and sudden homonymous inferior quadrantanopia and elevated blood sugar levels with normal pH. The neuroimaging was normal and the visual lost improved after the correction of the hyperglycemia. NKH should be considered in patients with sudden and transient hemianopia and normal neuroimaging.

Eyelid myokymia in patients with migraine taking topiramate.

OBJECTIVE: Although ocular side effects of topiramate are common, neuroophthalmologic manifestations such as blepharospasm, myokymia and oculogyric crisis are scarcely reported. METHODS: We present a serie of 8 patients with migraine who developed eyelid myokymia after treatment with topiramate. We reviewed all patients with migraine treated with topiramate attending the headache outpatient clinic of our hospital from January 2008 to December 2012. RESULTS: During the study period, a total of 140 patients with migraine were treated with topiramate in our headache clinic. Eight presented eyelid myokymia after beginning treatment with topiramate (5,7%). Topiramate was stopped and myokymia disappeared in all patients, it was prescribed again and eyelid myokymia reappeared with their previous characteristics in all patients. CONCLUSIONS: Eyelid myokymia is an underreported side-effect of topiramate in patients with migraine, of unknown cause, so that in future, further studies are need to examine whether patients with migraine are predisposed or not to this adverse effect.

Perception of the validity of the Migraine Disability Assessment questionnaire in a population of patients with chronic migraine.

OBJECTIVES: The Migraine Disability Assessment (MIDAS) questionnaire is the most frequently used instrument for assessing the level of disability in studies into migraine. This study aims to determine the level of completion of the questionnaire, assess the ease of use, and understand patients' subjective perception of the questionnaire's actual ability to measure disability. MATERIAL AND METHODS: We performed a prospective study of a sample of 78 patients with chronic migraine, determining their level of education and employment status. In a baseline visit, patients were trained to properly complete the questionnaire. At 3 months, we determined the total score and level of completion. Patients also completed a survey measuring ease of use of the questionnaire and patients' perception of whether the score accurately reflected their disability. RESULTS: Only 46% of patients fully completed the questionnaire. Sixty-nine percent reported finding it difficult to complete (this was influenced by patient's employment status but not by educational level). Sixty-two percent of respondents believed that the questionnaire did not fully reflect their own perception of their disability. CONCLUSIONS: Although the validity and consistence of the MIDAS questionnaire are well documented, a high percentage of the study population reported finding it difficult to complete; many patients also considered that the questionnaire did not accurately reflect their disability. Understanding patients' opinions of the suitability of questionnaires used in consultation is crucial to improving completion.

Perception of the validity of the Migraine Disability Assessment questionnaire in a population of patients with chronic migraine. / Percepción de la validez del cuestionario Migraine Disability Assessment por una población de pacientes con migraña crónica.

OBJECTIVES: The Migraine Disability Assessment (MIDAS) questionnaire is the most frequently used instrument for assessing the level of disability in studies into migraine. This study aims to determine the level of completion of the questionnaire, assess the ease of use, and understand patients' subjective perception of the questionnaire's actual ability to measure disability. MATERIAL AND METHODS: We performed a prospective study of a sample of 78 patients with chronic migraine, determining their level of education and employment status. In a baseline visit, patients were trained to properly complete the questionnaire. At 3 months, we determined the total score and level of completion. Patients also completed a survey measuring ease of use of the questionnaire and patients' perception of whether the score accurately reflected their disability. RESULTS: Only 46% of patients fully completed the questionnaire. Sixty-nine percent reported finding it difficult to complete (this was influenced by patient's employment status but not by educational level). Sixty-two percent of respondents believed that the questionnaire did not fully reflect their own perception of their disability. CONCLUSIONS: Although the validity and consistence of the MIDAS questionnaire are well documented, a high percentage of the study population reported finding it difficult to complete; many patients also considered that the questionnaire did not accurately reflect their disability. Understanding patients' opinions of the suitability of questionnaires used in consultation is crucial to improving completion.

[Efficacy and safety of fingolimod in routine clinical practice in patients with relapsing-remitting multiple sclerosis in Spain: an intermediate analysis of the MS NEXT study]. / Efectividad y seguridad del fingolimod en la practica clinica habitual en pacientes con esclerosis multiple remitente recurrente en España: analisis intermedio del estudio MS NEXT.

INTRODUCTION: Fingolimod is a disease modifying therapies, which has showed clinical efficacy and an acceptable safety profile in clinical trials with relapsing-remitting multiple sclerosis (RRMS) patients. AIM: To assess fingolimod effectiveness and safety in patients with RRMS in clinical practice. PATIENTS AND METHODS: We present an interim analysis (July 2015) of MS NEXT, an observational, retrospective and multicenter study. 442 patients were included (mean age: 41 ± 9 years; median baseline EDSS: 3.0; 70% female; 284 previously treated with first-line disease modifying therapies, 139 with natalizumab and 19 without a previous treatment; mean fingolimod treatment duration: 25 ± 9 months) treated with fingolimod from November 2011 and with at least 12 months follow-up. 56 neurology-unit Spanish hospitals enrolled patients. Basal clinical and demographic data were recorded. Relapses, EDSS scores and radiological activity were recorded at baseline and annually. Adverse events were also recorded during the follow-up period. RESULTS: After two years of follow-up: annual relapse rates decreased by 76%, the proportion of relapse-free patients was 67%, of disability progression-free patients confirmed at 3 months was 91%, of relapse and disability progression-free patients was 63%, of radiological activity-free patients was 50%, and the proportion of relapse, disability progression and radiological activity-free patients was 35%. Only 3.9% of patients discontinued fingolimod permanently during the first year of treatment. CONCLUSIONS: In this interim analysis, most of patients treated with fingolimod in clinical practice had a controlled clinical disease activity, stable disability progression and high persistency.

TITLE: Efectividad y seguridad del fingolimod en la practica clinica habitual en pacientes con esclerosis multiple remitente recurrente en España: analisis intermedio del estudio MS NEXT.Introduccion. El fingolimod es un tratamiento modificador de la enfermedad que ha demostrado eficacia y seguridad en ensayos clinicos en pacientes con esclerosis multiple remitente recurrente (EMRR). Objetivo. Evaluar la efectividad y la seguridad del fingolimod en pacientes con EMRR en la practica clinica. Pacientes y metodos. Se presentan los resultados del analisis intermedio (julio de 2015) del MS NEXT, un estudio observacional, multicentrico y retrospectivo. Se incluyo a 442 pacientes (edad media: 41 ± 9 años; escala expandida del estado de discapacidad basal, mediana: 3; 70% mujeres; 284 previamente tratados con tratamientos modificadores de la enfermedad de primera linea, 139 con natalizumab y 19 naive; media de tratamiento con fingolimod: 25 ± 9 meses) tratados con fingolimod a partir de noviembre de 2011 y con al menos 12 meses de seguimiento. Participaron 56 hospitales españoles. Se recogieron datos demograficos y clinicos (basal y anualmente, numero de brotes, puntuacion en la escala expandida del estado de discapacidad y actividad radiologica). Tambien se registraron los efectos adversos durante el seguimiento. Resultados. Tras dos años de tratamiento, la tasa anualizada de brotes se redujo un 76%; el 67% de los pacientes estaba libre de brotes; el 91%, libre de progresion de la discapacidad confirmada a los tres meses; el 63%, libre de brotes y progresion de discapacidad; el 50%, libre de actividad radiologica, y el 35%, libre de brotes, progresion de discapacidad y actividad radiologica. Un 3,9% abandono el fingolimod permanentemente. Conclusiones. En este analisis intermedio, la mayoria de los pacientes tratados con fingolimod en la practica clinica presenta una actividad clinica controlada y una elevada persistencia al tratamiento.

[Ambulatory neurological care in a prison population]. / Atencion neurologica ambulatoria a una poblacion penitenciaria.

AIMS: Some of the users attended in a Neurology service consist of the inmate population in a prison. The aim of this study is to analyse all the proposals referred from the Alicante II Prison Centre to a Neurology service. PATIENTS AND METHODS: We analyse and describe the clinical characteristics of patients referred from the Alicante II Prison Centre to the Neurology service at the Centro Sanitario Integrado in Villena between the years 2003 and 2006. This analysis involved the following variables: age, sex, personal history, reason for visiting and diagnosis. RESULTS: A total of 88 proposals were recorded. The mean age of the patients was 35 years (84 males/4 females). A total of 15 patients did not attend their appointment (17%). Positive serology for the human immunodeficiency virus was found in 18% of patients. The most frequent reason for visiting was headache (32%), followed by seizures (25%) and, thirdly, vascular pathologies (13%). In a group of 16 patients (18%) the main diagnosis was established as being some kind of psychiatric disorder (anxiety, depression, simulation). CONCLUSIONS: No studies have been published in the literature that analyse the clinical characteristics of patients from prisons referred to a Neurology service. The high percentage of patients who do not attend their appointment and the high percentage of psychiatric disorders that are diagnosed within this group of patients are especially noteworthy. However, and as can be observed in the general population, headache is still the most common reason for visiting. Nevertheless, the group of neurological diagnoses that are most frequently attended is epilepsy.

[Multiple mononeuropathy associated to treatment with pravastatin]. / Mononeuropatía múltiple asociada a tratamiento con pravastatina.

INTRODUCTION: The first reports of neuropathy due to treatment with statins appeared in 1994, although it is an infrequent complication. It usually consists of an axonal polyneuropathy, which is predominantly sensory, distal and symmetric, and may be subacute or chronic. We present here the second case reported in the literature of multiple mononeuropathy associated to the use of statins. CASE REPORT: A 51-year-old female patient who, after beginning therapy with pravastatin, presented with progressive, asymmetrically distributed, distal paresthesias in the limbs and an unstable gait. An electromyographic study was compatible with multiple mononeuritis. Results of complementary tests that were carried out to preclude other causes of multiple mononeuropathy were normal. The patient's condition improved on withdrawing treatment with the drug and it became worse again when therapy was restarted. When pravastatin therapy was stopped for good, the patient's condition progressively improved until she was practically free of symptoms. CONCLUSIONS: The relationship between treatment with statins and the appearance of polyneuropathy has been proved in different epidemiological case-control studies. It does not only appear as the classical distal symmetrical polyneuropathy, but has also been reported as taking on atypical clinical forms including a few cases, like ours, of multiple mononeuropathy. The risk of developing this complication is low and is offset by the cardiovascular benefits offered by statins, although it may become more common in the future due to the increasing rate of use of these agents. It is important to bear this cause of neuropathy in mind, given the fact that it is potentially reversible.

[Retinopathy secondary to treatment with Interferon beta-1a in a patient with multiple sclerosis]. / Retinopatía secundaria a tratamiento con interferón beta 1a en paciente con esclerosis múltiple.

INTRODUCTION: Although visual symptoms of multiple sclerosis (MS) are very frequent, they are rarely related with treatment with interferon. This is the first case reported in the literature of retinopathy associated with subcutaneous interferon beta-1a, and the second related to interferons in MS. CASE REPORT: A 30-year-old female diagnosed with relapsing-remitting MS who, at 3 months after starting treatment with subcutaneous interferon beta-1a (44 microg/3 times a week), displayed visual disorders. Retinal lesions in the form of cotton wool spots were found as symptoms of microinfarctions in the retina. The lesions got better after stopping treatment and the patient was found to be asymptomatic. CONCLUSIONS: The existence of retinopathy secondary to interferon has been known in the treatment of hepatitis C and neoplasias with interferon alfa since 1990. Despite being a frequently occurring complication, it is usually a mild condition and disappears on withdrawing treatment, or even if it is continued. It is attributed to deposits of immunocomplexes and complement activation in the blood vessels of the retina. Only one other case associated to treatment of MS with interferon beta has been reported in the literature, more specifically related to subcutaneous interferon beta-1b. The clinical characteristics of both cases are identical to those associated to interferon alfa. Despite the fact that the frequency of appearance seems to be lower than in the case of interferon alfa, the physician must bear in mind the possibility encountering this complication.

[Olfactory seizures and parasellar meningioma]. / Crisis olfatoria y meningioma paraselar.

INTRODUCTION: Partial olfactory seizures are infrequent. They are related to the presence of lesions in the uncinate area of the temporal lobe. Patients describe smells during the ictal phase that are generally unpleasant. We report the cases of two patients with olfactory disorders of a paroxysmal nature caused by a parasellar meningioma. CASE REPORTS: Case 1: a 43 year old male who presented paroxysmal episodes in which he perceived a bitter smell, with dysphoria, linked to the presence of a left side parasellar meningioma. Case 2: a 45 year old female who presented paroxysmal episodes during which she could smell formaldehyde and this was linked to the presence of a left side parasellar meningioma. DISCUSSION: Olfactory seizures are related to structural alterations to the amygdala. The most frequent causes are associated to the presence of mesial temporal sclerosis and, above all, to tumours situated in the temporomesial structures that have diverse aetiologies.

[Ischemic stroke as a presentation of essential thrombocythemia. Four case reports]. / Ictus isquémico como forma de presentación de trombocitemia esencial. A propósito de cuatro casos.

INTRODUCTION: Essential thrombocythemia (ET) is a myelodysplastic syndrome that constitutes an infrequent cause of ischemic stroke. Few clinical reports have been published describing the clinical onset of ET in the form of a vascular accident. CASE REPORT: We describe four cases of patients with no known haematological pathology who began with an ischemic stroke and were later diagnosed as suffering from ET, following an aetiological investigation. The patients were all young, under 45 years of age, with no significant vascular risk factors, who presented a clinical picture of ischemic stroke and platelet counts above normal values, although in two cases they were below 600 x 10(9)/L. As they progressed, their platelet counts were constantly above this threshold and a diagnosis of ET was reached. CONCLUSIONS: Although ET is an infrequent cause of ischemic stroke, it must be taken into account even in young patients and when platelet counts do not exceed 600 x 10(9)/L. Antiplatelet drug therapy must be indicated in all cases and the clinician must evaluate the suitability of cytoreductive therapy in patients with a high risk of thrombotic event recurrence, especially if platelet counts are above 600 x 10(9)/L.

[Multiple sclerosis incidence in the Alcoi Health district. 12-year-long study (1986-1997)]. / Incidencia de la esclerosis múltiple en el área sanitaria de Alcoi. Estudio de 12 años (1986-1997).

INTRODUCTION: To date, epidemiological studies on multiple sclerosis in Spain have been basically prevalence studies, and the data on incidence recorded have been the result of different methodologies, with no definition of the criteria for inclusion. OBJECTIVE: To carry out a study of incidence with prospective collection/review of cases over a prolonged period of time. PATIENTS AND METHODS: Over 12 years, between 1 March 1986 and 31 December 1997, we collected data prospectively for patients with a definite diagnosis of multiple sclerosis. The year of incidence was considered to be when the patient fulfilled clinical criteria for diagnosis of the disease. RESULTS: On 31 December 1997 in the Alcoi Health District there were 54 patients with clinically defined multiple sclerosis (a prevalence of 41.28 cases per 100,000 inhabitants). Of these, 45 patients were diagnosed during the period studied, with an average incidence of 2.82 cases per 100,000 inhabitants per year. Only 15 patients were confirmed before 1986. CONCLUSIONS: This is the most prolonged study of the incidence of multiple sclerosis carried out in Spain. The figures found for incidence are in contrast to those found in previous years for prevalence and incidence. Analysis of the data suggests that the incidence of multiple sclerosis in the Alcoi district has changed and has increased since the second half of the 1980s.

[The prevalence of multiple sclerosis in the Alcoi Health district]. / Prevalencia de la esclerosis múltiple en el área sanitaria de Alcoi.

INTRODUCTION: The epidemiological studies done in Spain in recent years show higher figures for the prevalence of multiple sclerosis than before. Spain is therefore now in the area with a high risk of contracting the disease. OBJECTIVE: We have made a new study to confirm the current prevalence of the disorder in the Alcoi Health District. PATIENTS AND METHODS: Between 1 March 1986 and the prevalence day, 31 December 1997 we recorded the patients with definite or probable multiple sclerosis, as defined on Poser's criteria, after intensive fieldwork and reevaluation of all possible patients by a neurologist from the Neurology Unit. On the prevalence day there were 130,786 inhabitants in the district. RESULTS: On the prevalence day we found 54 patients with definite multiple sclerosis and no patient with probable multiple sclerosis. The rate of prevalence was 41.28 cases per 100,000 inhabitants (95% CI: 31-53.6). During the study we counted an incidence of 45 cases with an average rate of 2.82 cases per 100,000 inhabitants per year. We also describe the clinical characteristics of the patients. CONCLUSIONS: The rate of prevalence found is much higher than that described for the district in studies using similar methodology. This increase, together with the discrepancy between the incidence found and the prevalence, makes us consider that possibly the prevalence has increased in recent years.

[Isolated cerebral angiitis]. / Angeítis aislada del sistema nervioso central.

INTRODUCTION: Isolated cerebral angiitis of the central nervous system is defined histologically by presence of granulomatous inflammation in the meningeal vessels, and parenchyma throughout veins and arteries of variable size. The most common clinical manifestations are headache and encephalopathy. CASE REPORT: We present the clinical case of a patient with epileptic seizures of focal onset, secondary generalized, diagnosed by cerebral biopsy. Clinical response to immunosuppressive treatment (corticosteroid and cyclophosphamyde) was excellent. We make a bibliographic review and update. CONCLUSION: The heterogeneous clinical symptomatology of this disease leads to confusion and delay in diagnosis. Histological confirmation by cerebral and meningeal biopsy is the best parameter for diagnosis of isolated cerebral angiitis of the central nervous system.