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Resumen Introduccion El sindrome de piernas inquietas (SPI) es un trastorno caracterizado por la necesidad imperiosa de mover las piernas, estando a menudo acompanado de sensaciones desagradables. Su frecuencia es superior en pacientes con esclerosis multiple (EM) que en la poblacion general. Objetivos Evaluar la prevalencia del SPI, segun el cumplimiento de los 4 requisitos esenciales incluidos en los criterios diagnosticos propuestos por la International Restless leg syndrome study group (IRLSSG, 2003), en una cohorte de pacientes con EM e identificar posibles factores de riesgo y repercusion clinica. Resultados Se incluyeron 120 pacientes con EM, con una edad media de inicio de 40 anos y un tiempo medio de evolucion de 46 meses. La prevalencia de SPI, segun el cumplimiento de criterios diagnosticos de la IRLSSG, fue del 23,3%. El tiempo de evolucion de EM, desde la aparicion de los primeros sintomas, fue significativamente menor en pacientes con SPI (p=0,001). La presencia de un brote reciente, asi como de sintomas de ansiedad, depresion y dolor neuropatico se asociaron de forma significativa con el riesgo de SPI (p=0,001, p Conclusiones El SPI debe ser considerado en la evaluacion neurologica de pacientes con EM, cuyo diagnostico y tratamiento precoz mejoraria la calidad de vida de estos sujetos.

The existence of antibodies to aquaporin-4 (AQP-4-ab) has identified neuromyelitis optica (NMO) and multiple sclerosis (MS) as different diseases. Although HLA-DRB1 alleles contribute to MS risk, recent studies suggest that HLA back-ground differs between patients with NMO or MS in non-Caucasians populations. Our study was aimed to analyze HLA-DRB1 distribution in Caucasians NMO patients.We recruited a cohort of 22 NMO patients (73% were AQP-4-ab positive), 228 MS patients and 225 healthy controls from Spain and we genotyped the HLA-DRB1 locus. Then, we performed a pool analysis using reported data from 45 NMO patients (53% were AQP-4-ab positive), 156 MS patients and 310 healthy controls from Caucasian French population.In the Spanish cohort, NMO was associated with increased frequency of DRB1*10 allele compared with MS (odds ratio, OR = 15.1; 95% confidence interval, 95% CI = 3.26-69.84; p = 0.012). In the pooled analysis, by comparison with healthy controls, NMO was associated with increased frequency of DRB1*03 allele (OR = 2.27; 95% CI = 1.44-3.58; p0.0008) which was related to AQP-4-ab seropositivity (OR = 2.74; 95% CI = 1.58-4.77; p0.0008). By contrast, MS was associated with increased frequency of DRB1*15 allele (OR = 2.09; 95% CI = 1.62-2.68; p0.0008) and decreased frequency of DRB1*07 allele (OR = 0.58; 95% CI = 0.44-0.78; p0.0008).Caucasian patients with NMO and MS have a different HLA-DRB1 allelic distribution. DRB1*03 allele seems to contribute to NMO seropositivity. Multicenter collaborative efforts are needed to adequately address the genetic contribution to NMO susceptibility.

The objective of this work was to produce a scientific evidence-based guide to clinical practice dealing with the basic questions concerning the treatment of epilepsy.A committee of 11 experts belonging to the Andalusia Epilepsy Society, made up of six neurologists, three neuropaediatricians, one neurosurgeon and a pharmacologist, all of whom were deeply involved and experienced in epilepsy, conducted a thorough review of the literature in search of all the evidence available on the proposed subject matter. The following databases were used: MEDLINE, Cochrane Library and the databases of several clinical practice guidelines (National Guideline Clearinghouse, National Institute of Clinical Excellence and the American Academy of Neurology's Clinical Guidelines). The Guide was set out in seven sections and was published in four parts. From a total number of 187 relevant documents, the committee found 63 examples of scientific evidence and 91 therapeutic recommendations. These were tabulated and classified according to the European Federation of Neurological Societies' criteria for producing Clinical Practice Guidelines.The results of this survey provide scientific evidence-based clinical guidelines that are useful, simple and applicable at different levels of health care.

The objective of this work was to produce a scientific evidence-based guide to clinical practice dealing with the basic questions concerning the treatment of epilepsy.A committee of 11 experts belonging to the Andalusia Epilepsy Society, made up of six neurologists, three neuropaediatricians, one neurosurgeon and a pharmacologist, all of whom were deeply involved and experienced in epilepsy, conducted a thorough review of the literature in search of all the evidence available on the proposed subject matter. The following databases were used: MEDLINE, Cochrane Library and the databases of several clinical practice guidelines (National Guideline Clearinghouse, National Institute of Clinical Excellence and the American Academy of Neurology's Clinical Guidelines). The Guide was set out in seven sections and was published in four parts. From a total number of 187 relevant documents, the committee found 63 examples of scientific evidence and 91 therapeutic recommendations. These were tabulated and classified according to the European Federation of Neurological Societies' criteria for producing Clinical Practice Guidelines.The results of this survey provide scientific evidence-based clinical guidelines that are useful, simple and applicable at different levels of health care.

The objective of this work was to produce a scientific evidence-based guide to clinical practice dealing with the basic questions concerning the treatment of epilepsy.A committee of 11 experts belonging to the Andalusia Epilepsy Society, made up of six neurologists, three neuropaediatricians, one neurosurgeon and a pharmacologist, all of whom were deeply involved and experienced in epilepsy, conducted a thorough review of the literature in search of all the evidence available on the proposed subject matter. The following databases were used: MEDLINE, Cochrane Library and the databases of several clinical practice guidelines (National Guideline Clearinghouse, National Institute of Clinical Excellence and the American Academy of Neurology's Clinical Guidelines). The Guide was set out in seven sections and was published in four parts. From a total number of 187 relevant documents, the committee found 63 examples of scientific evidence and 91 therapeutic recommendations. These were tabulated and classified according to the European Federation of Neurological Societies' criteria for producing Clinical Practice Guidelines.The results of this survey provide scientific evidence-based clinical guidelines that are useful, simple and applicable at different levels of health care.

The objective of this work was to produce a scientific evidence-based guide to clinical practice dealing with the basic questions concerning the treatment of epilepsy.A committee of 11 experts belonging to the Andalusia Epilepsy Society, made up of six neurologists, three neuropaediatricians, one neurosurgeon and a pharmacologist, all of whom were deeply involved and experienced in epilepsy, conducted a thorough review of the literature in search of all the evidence available on the proposed subject matter. The following databases were used: MEDLINE, Cochrane Library and the databases of several clinical practice guidelines (National Guideline Clearinghouse, National Institute of Clinical Excellence and the American Academy of Neurology's Clinical Guidelines). The Guide was set out in seven sections and was published in four parts. From a total number of 187 relevant documents, the committee found 63 examples of scientific evidence and 91 therapeutic recommendations. These were tabulated and classified according to the European Federation of Neurological Societies' criteria for producing Clinical Practice Guidelines.The results of this survey provide scientific evidence-based clinical guidelines that are useful, simple and applicable at different levels of health care.

The etiology of lumbosacral plexopathy is often due to compression. One of the less common causes of this is aneurysm of the iliac artery. However, 13% of the patients with this disorder initially have symptoms of plexus irritation or deficit.We describe the case of a 42 year-old-man, with no previous medical history, who complained of right-sided sciatica for the previous three months. On examination there were clinical signs of a lesion of the right lumbosacral plexus. On CT of the pelvis and MR of the lumbar spine there were images compatible with an aneurysm of the right iliac artery. This diagnosis was confirmed on arteriography. Treatment was surgical (aneurysmography and right iliofemoral by-pass). The pain disappeared and the motor deficit improved considerably.When a patient presents with lumbosacral plexopathy, an iliac aneurysm should be considered as a possible etiological factor. In such cases early diagnosis is essential, since surgical treatment will usually resolve the clinical problem.

The clinical findings of basilar dolichoectasia (DB) are caused by compression of the cranial nerves, vertebrobasilar ischaemia, hydrocephaly and compression of the brainstem. Hypoacusia as an isolated finding in DB is very rare.We describe the case of a 48-year-old hypertensive man with bilateral hypoacusia as an isolated finding in DB. Diagnosis was made on MR, and arteriography showed changes characteristic of an anomalous blood vessel.In patients with hypoacusia of uncertain aetiology, specially if they are also hypertensive, one should rule out the possibility of DB using cerebral magnetic resonance or angioresonance.

Familial amyloidotic polyneuropathy type I (FAF-I) is caused by a specific genetic mutation that gives rise to a transthyretin anomaly whose presence in serum constitutes the biochemical marker for this disease. We studied the serum of 7 patients and 16 asymptomatic members of their immediate families using ELISA with FD-6 monoclonal antibody to detect the transthyretin anomaly. Positive results were found for the 7 patients, including the 2 patients whose disease was apparently sporadic, and 12 carriers were detected among the family members. This technique makes sural nerve biopsy unnecessary for establishing a diagnosis in patients whose clinical signs are consistent with FAP-I. Asymptomatic carriers are also detected, facilitating appropriate genetic counseling.

A 45-years-old woman developed palilalia without other neurologic symptoms or signs. Cranial MR and CT scan showed only bilateral thalamic infarcts; the SPECT showed bilateral frontoparietal hypoactivity. We conclude that involvement of the supplementary motor area by diaschisis after bilateral thalamic lesions may produce palilalia.

We report a patient that developed a sensorimotor polyneuropathy more than a year before the appearance of the typical clinical signs of progressive systemic sclerosis. A sural nerve biopsy showed epineural vasculitis with involvement of the basal membrane of the endoneural vessels, without proliferation of the connective tissue.

Primary antiphospholipid syndrome (PAS) is characterized by the isolated presence of antiphospholipid antibodies in the absence of other immunological changes. Its association with ischemic cerebrovascular disease is rarely reported. This study analyzes this association in 10 patients (8 women) first presenting with ischemic cerebrovascular accident (ICVA) that met the criteria for PAS. The average age of the patients was 50.4 years. Two patients had Sneddon's syndrome. Prolonged thromboplastin partial activation time was found in only three patients. Eight started aspirin therapy. The average time of follow-up was 17.2 months, during which there was one relapse. PAS should be included as a possible cause when examining individuals with ICVA, particularly when patients are young, when abortion has occurred or when no other cause is evident. Acetylsalicylic acid is effective for preventing relapse when other vascular risk factors are carefully controlled.

Introduccion. Los anticuerpos contra la aquaporina-4 (Ac-AQP-4) permitieron identificar como enfermedades diferentes a la neuromielitis optica (NMO) y la esclerosis multiple (EM). Estudios recientes sugieren que los alelos HLA-DRB1 contribuyen de forma diferente en la NMO y la EM en poblaciones no caucasicas. Nuestro objetivo fue analizar la distribucion HLA-DRB1 en pacientes con NMO de origen caucasico. Sujetos y metodos. Se incluyo una cohorte de 22 pacientes con NMO (el 73% con Ac-AQP-4), 228 con EM y 225 controles sanos de origen espanol, y se genotipifico el locus HLA-DRB1. Posteriormente, los resultados se combinaron con los descritos en una poblacion caucasica francesa: 45 pacientes con NMO (el 53% con Ac-AQP-4), 156 con EM y 310 controles sanos. Resultados. En la cohorte espanola, la NMO, en comparacion con la EM, se asociaba a una mayor frecuencia del alelo DRB1*10 (odds ratio, OR = 15,1; intervalo de confianza del 95%, IC 95% = 3,26-69,84; p = 0,012). En el analisis combinado, y comparado con controles sanos, la NMO se asociaba a una mayor frecuencia del alelo DRB1*03 (OR = 2,27; IC 95% = 1,44-3,58; p < 0,0008), y esto se relacionaba con tener Ac-AQP-4 (OR = 2,74; IC 95% = 1,58-4,77; p < 0,0008). Por el contrario, la EM se asociaba a una mayor frecuencia del alelo DRB1*15 (OR = 2,09; IC 95% = 1,62-2,68; p < 0,0008) y a una menor frecuencia del alelo DRB1*07 (OR = 0,58; IC 95% = 0,44-0,78; p < 0,0008). Conclusiones. Los pacientes caucasicos con NMO y EM presentan una distribucion alelica HLA-DRB1 diferente. El alelo DRB1*03 parece contribuir a ser IgG-NMO seropositivo. Son necesarios estudios multicentricos colaborativos para conocer mejor la contribucion genetica en la susceptibilidad a padecer NMO.