[Characteristics of Guillain-Barré syndrome in the healthy area III of Aragon Country]. / Características del síndrome de Guillain-Barré en el área III de salud de la Comunidad Autónoma de Aragón.

INTRODUCTION: Guillain-Barré Syndrome is an acute immune-mediated inflammatory polyneuropathy characterized by flaccid paresia with arreflexia, changeable sensitive disorder and albuminocytologic dissociation in the cerebrospinal fluid (CSF). PATIENTS AND METHODS: We conducted a retrospective study of 30 GBS patients diagnosed in Hospital Clínico de Zaragoza between 1999 and 2005. Annual incidence, seasonal distribution, preceding acute infection; clinical, electrophysiological and electrocardiographic (ECG) data and evolution were all evaluated. RESULTS: The incidence was 1.56/100000 inhabitants/year. We observed an upward tendency during winter months. The rates of incidence were higher in men (66.7%) and increased with age in both sexes. Main clinical presentation form was paraparesia of lower limbs followed on cranial nerve palsy. Immunoglobulin therapy was received by 62.5% of patients. The rate of death was 10%. Thirteen per cent of patients had dysautonomia. Electrocardiographic abnormalities were present in 37% of patients. CONCLUSIONS: GBS incidence in Aragon Country is similar to that found in other studies. An increase with age and an upward tendency during the winter months was observed. High percentage of abnormalities in ECG but the majority of patients was asymptomatic.

[Model of analysis in two halves for semantic fluency tasks]. / Modelo de análisis en dos mitades para tareas de fluidez semántica.

INTRODUCTION: Semantic verbal fluency (SVF) tasks involve the activation of language, semantic memory, working memory and the executive functions. The second half of the test increases the demands on working memory and search for lexical items, which can make it more difficult than the first half. AIM: To conduct a pilot study in order to standardise the two halves of the SVF test ('animals' category) in adults over 50 years of age. SUBJECTS AND METHODS: Two verbal fluency tasks (semantic and phonological) and the Spanish version of the Mini-Mental State Examination with working memory were applied in 122 controls (range: 50-89 years), who were distributed according to age and schooling. RESULTS: There is a significant difference between the two halves of the test adjusted for age and schooling (p < 0.0001). Scoring in the second half showed a higher correlation with age, schooling and working memory than the first half. Performance in the two halves was standardised for three age groups (50-59, 60-79 and 80-89 years) and two levels of schooling (high and low). CONCLUSIONS: The number of names in the second half of the SVF test was lower than in the first half in all the groups, which supports the hypothesis of a greater cognitive demand, probably on working memory and the search for lexical items in semantic memory. Having normative values available for the two halves of the SVF test allows simultaneous and independent interpretation of the performance at two levels of intra-task difficulty. This model of analysis complements the traditional assessment and can easily be applied in day-to-day clinical practice.

[Facial diplejia: a regional variant of Guillain-Barré syndrome]. / Diplejia facial: variante regional del síndrome de Guillain-Barré.

Facial palsy is a uncommon clinical manifestation that it can be caused by different etiologies. We show a patient with a chronic periodontal disease who presented a sudden facial palsy, initially in left-face but it becomes bilateral quickly. In few days he associated paresthesias in his right arm and hyporreflexia. These clinical findings with albumin-cytological dissociation, they had allowed to establish the diagnosis of regional variant of Guillain-Barré Syndrome (SGB). Facial diplejia is an idiopathic manifestation in 25% of patients, and this is the most common cause. However, facial diplejia can be secondary to many etiologies as SGB. The affectation of facial nerve associated to other motor symptoms in SGB is frequent, but it is not frequent the presentation as facial diplejia alone. In conclusion, it is necessary a high clinical suspicion to do a lumbar punction (PL) and MRI to reject neoplasic pathologies and to obtain a diagnosis and an adequate treatment.

[Study of the diagnostic agreement on headaches between neurology and primary care]. / Estudio de concordancia diagnostica en cefalea entre neurologia y atencion primaria.

INTRODUCTION: Headache as a symptom is a very common disease and one of the main reasons for consultation in primary care. AIM: To analyze the characteristics of patients referred from primary care to general neurology whose chief complaint was headache and/or neuralgia and diagnostic agreement. PATIENTS AND METHODS: Cross-sectional study of all patients referred from primary care; demographic/clinical variables were collected and diagnostic hypothesis by primary care and general neurology were compared by determining their agreement. RESULTS: 2,514 were referred from primary care patients (588 of them on a preferential basis); in 378 cases the reason for consultation was headache and/or neuralgia (average 42.46 years; 77.8% female). In 139 patients it was established only a semiological diagnostic and other episodic migraine predominated (49.79%), chronic tension headache (18.41%) and trigeminal neuralgia (12.13%). Since general neurology, the most common diagnoses were, respectively, 33.86%, 24.05% and 18.67%. A compatible kappa coefficient of 0.543 (p < 0.05) with a moderate agreement when considering only those patients referred from primary care to a specific diagnosis was obtained. CONCLUSIONS: Headaches are a very common reason for consultation in primary care (15%). The diagnostic agreement is moderate in our health sector so it is necessary to design training programs to help outline the criteria for referral to specialists and improve care for our patients.

TITLE: Estudio de concordancia diagnostica en cefalea entre neurologia y atencion primaria.Introduccion. La cefalea como sintoma es una patologia frecuente y uno de los principales motivos de consulta por parte de atencion primaria. Objetivo. Analizar las caracteristicas de los pacientes derivados desde atencion primaria a la consulta de neurologia general con cefalea o neuralgia como motivo de consulta, y la concordancia diagnostica. Pacientes y metodos. Estudio descriptivo transversal de todos los pacientes remitidos desde atencion primaria; se recogieron variables demograficas/clinicas y se compararon las hipotesis diagnosticas de atencion primaria y neurologia, determinando su concordancia. Resultados. Se remitieron desde atencion primaria 2.514 pacientes (588 de ellos con caracter preferente); en 378 casos el motivo de la consulta fue cefalea o neuralgia (42,46 años de media; el 77,8%, mujeres). En 139 pacientes se establecio tan solo un diagnostico semiologico y en el resto predominaron la migraña episodica (49,79%), la cefalea tensional cronica (18,41%) y la neuralgia del trigemino (12,13%). Desde neurologia, los diagnosticos mas frecuentes fueron, respectivamente, 33,86%, 24,05% y 18,67%. Se obtuvo un coeficiente kappa de 0,543 (p < 0,05), compatible con una concordancia moderada al considerar solo los pacientes remitidos desde atencion primaria con un diagnostico concreto. Conclusiones. Las cefaleas constituyen un motivo de consulta desde atencion primaria muy frecuente (15%). La concordancia diagnostica es moderada en nuestro sector sanitario, por lo que es necesario diseñar programas de formacion que ayuden a perfilar los criterios de derivacion al especialista y mejorar la atencion a nuestros pacientes.

[Clinical, electrophysiological and immunological evaluation of the response to treatment with intravenous immunoglobulins in several immune-mediated neuropathies]. / Valoración clínica, electrofisiológica e inmunológica de la respuesta al tratamiento con inmunoglobulinas intravenosas en distinta neuropatías inmunomediadas.

INTRODUCTION AND AIMS: Chronic immune-mediated neuropathies are characterised by their being predominantly demyelinating, by being associated to specific antibodies and by their response to immunotherapy. We evaluated the clinical, electrophysiological and immunological responses following treatment with intravenous immunoglobulins (IVIg) in different clinical forms. CASE REPORTS: We report on three patients with: 1. Multifocal motor neuropathy, 2. Multifocal sensory-motor neuropathy, and 3. Hypertrophic brachial plexopathy, who were evaluated before and 14 days after treatment with IVIg by means of clinical scales (MRC, Rankin), electrophysiological studies (ENG-EMG), and antibody (antiganglioside) determination. The three patients showed clinical improvement (> 20% MRC, > 1 Rankin) between the 4th and 7th day after the infusion, and this was maintained for 4-16 weeks. The ENG in cases 1 and 2 revealed conduction block (CB) in some nerves, lowered amplitude in others, and signs of demyelination. Following treatment, the CB disappeared in some nerves, while in others there was an improvement in the distal amplitude (distal block), but with no correlative improvement in the proximal amplitude, revealing new CBs that had previously gone undetected. The sum of post-treatment amplitudes improved in cases 1 and 2. In case 3, we were unable to demonstrate the existence of CBs, although we believe that they did exist proximally, and we found indirect signs of CB in EMG. The three cases had increased antiganglioside IgM antibodies levels, which did not undergo any significant variations. CONCLUSIONS: A 'consistent' clinical improvement was observed following treatment with IVIg. There was no correlation between the electrophysiological response (although apparent) and the degree of clinical response. CBs exist at different levels in the same nerve, which can be revealed after treatment. Following therapy, antiganglioside antibody levels remain high.

Esclerosis lateral amiotrófica y miastenia gravis (síndrome overlap): presentación de 3 nuevos casos / Amyotrophic lateral sclerosis and myasthenia gravis overlap syndrome: 3 new cases

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[Evaluation of patients who do not visit neurology units]. / Evaluación de los pacientes no presentados a las consultas de neurología.

AIMS: Our aim was to study the relation between non-attendance at neurology units and the delay that exists, as well as the demographic factors that may come to bear on non-attendance. PATIENTS AND METHODS: Data on neurology outpatients over the period between November and December 2002 were collected prospectively. Both patients who were referred for evaluation and those on their first programmed visit were included in the study. RESULTS: The total number of subjects was 1,035, with an average age of 55.31 +/- 19.84 (14-96). Of these, 605 were females and 430 were males, with no significant differences in the age of the two groups. 40% of the cases came from rural areas. 237 (22.9%) did not attend the visit. We found a statistically significant relation between age and non-attendance (t=2.67; p=0.008, Student's t). There was also a relation between not going to the visit and the delay that existed (t=5.84; p=0.000, Student's t). Patients who were referred from the Casualty department missed their visit more often those who were sent by their GP (Pi2=2.4; p=0.07, chi2). The date on which they received the appointment was also related to non-attendance (t=2.55; p=0.011, Student's t). CONCLUSIONS: Non-attendance was associated, above all, with the delay, with youth, being sent from casualty and with the length of time since they received notice of the visit.

[Paraproteinemic neuropathies: foundations for a specific therapy related to patients' quality of life]. / Neuropatias paraproteinemicas: bases para un tratamiento especifico con relacion a la calidad de vida de los pacientes.

INTRODUCTION: Monoclonal gammopathy (MG) is reported in 1% of subjects above 50 years of age and in 3% of those above the age of 70. Of all patients with MG, 3% present polyneuropathy (PNP). The abbreviation MGUS (monoclonal gammopathy of undetermined significance) is used to describe a benign proliferation of M component (monoclonal) according to Kyle's criteria, with a potential to be malignant that is indicated by clinical, biochemical and haematological parameters. Aims. The aim of this study was to evaluate the effect of the clinical exacerbation of MGUS associated neuropathy, by referring to the existing literature and to our own experience in order to set out a number of proposals for treatment based on the deterioration of the patient's quality of life. CASE REPORTS: Case 1: a 53 year old female with predominantly sensory sensory motor demyelinating polyradiculoneuropathy, associated to IgG lambda MGUS. Case 2: a 65 year old female with demyelinating sensory motor polyradiculoneuropathy associated to IgM kappa MGUS. In both cases there was a rapidly progressing neurological deterioration and no other data exist to suggest malign lymphoproliferation. CONCLUSIONS: In addition to the treatments established for MG linked PNP, which are based on the modulation of the immune response, it has also been proved that when malign proliferation of plasmacytes exists specific treatment of the gammopathy can significantly stabilise or improve the neuropathic symptoms. We propose gammopathy specific haematological treatment in patients who still meet Kyle's criteria for MGUS with a torpid neurological course and scarce response to therapy with immunomodulators, prior to an important deterioration in the quality of life. We also suggest establishing this rapidly progressing clinical course as a criterion that indicates the process of becoming malign as put forward by Eurelings et al.

[Migraine with pleocytosis: a case of atypical progression]. / Migraña con pleocitosis: un caso de evolucion atipica.

AIMS: Migraine with cerebrospinal fluid pleocytosis is a clinical pattern consisting in intermittent headaches accompanied by episodes of transient neurological deficit and lymphocytic pleocytosis, which last for varying lengths of time and have a spontaneous resolution. We describe the case of migraine with pleocytosis (MP) with atypical progression that responded well to corticoids. CASE REPORT: A 27 year old male with no familial or personal history of migraine who began with bouts of intense headaches that were preceded by neurological deficit (dysphasia and hemiparesis of the right hand side) lasting varying amounts of time (including hours). The serial study of cerebrospinal fluid (CSF) revealed a notable degree of lymphocytosis (400/mm3) with increased intracranial pressure (ICP) (> 30 mmHg). The other explorations that were performed (microbiological study of CSF and blood, humoral and cellular immunity, MR angiography and thyroid hormones, among others) were all normal. The neurological exploration carried out in the following days revealed a mild paresis of the right lateral rectus muscle, which was attributed to the intracranial hypertension and which clearly disabled the patient. As a consequence of the recurrence of the seizures and the long lasting increase in ICP, therapy was begun with 1 mg/kg/day of prednisone, at a descending rate; clinical remission and normalisation of the CSF was achieved within a period of two months. DISCUSSION: We describe this case of MP because of how atypical the progression was (paresis of the right abducent nerve secondary to the increase in ICP) and the possibility of associating corticoids in cases in which the benignity of the entity is in doubt.

Miositis necrosante autoinmune: a propósito de un caso / Autoimmune necrotising myopathy: A case report

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[Pronostic factors and survival in motor neuron disease]. / Factores pronósticos y supervivencia en la enfermedad de neurona motora.

INTRODUCTION: Amyotrophic lateral sclerosis (ALS) is the most common adult motor neuron disease (MND), presenting with a combination of upper (UMN) and lower (lmn) motor neuron signs, with high mortality. other less frequent mnd are progressive muscular atrophy (pma) and primary lateral sclerosis (pls). OBJECTIVES: To describe demographic data, clinical forms of presentation and evolution of patients with MND, looking for prognostic indicators factors. METHODS: Retrospective study of a series of patients with MND, registering demographic data, clinical variables and mortality-survival, analyzed by means of statistical package SPSS 6.1.2. RESULTS: They are 54 patients with MND (50 ALS, 3 PMA and 1 PLS), 30 (55.6%) men and 24 (44.4%) women, bulbar beginning forms in 13 cases (24.1%) and spinal in 41 (75.9%). Average age at beginning 60+/-14 (29-82 years), more delayed in women (p<0.05), and in patients with bulbar form (p<0.05) with clear predominance of female sex (p<0.03). There are 31 dead patients (57.4%) and 23 alive ones (42.6%), with average survival from the beginning symptoms of 46.51+/-40.9 months. Nine months (11-209), observing inversely proportional relation with the age of beginning of the disease (p<0.02). Bulbar beginning forms (p<0.01) and LMN affectation signs, specially bulbar and cervical levels (p<0,005), are associated with a greater probability of dying while the existence of UMN affectation signs on imaging techniques 362 ques has a greater probability of survival (p<0,05). CONCLUSIONS: The advanced age and the bulbar beginning forms are factors of bad prognosis, specially the predominant affectation of LMN over UMN.

[Magnetic resonance in Hallevorden-Spatz disease]. / Resonancia magnética en la enfermedad de Hallevorden-Spatz.

The Hallevorden-Spatz disease should be suspected in children or young people with motor, pyramidal or extrapyramidal symptomatology and deterioration of superior functions specially upon the existence of family history. Pathognomonic diagnosis may only be obtained by necropsy but magnetic resonance images, although not pathognomic, are sufficiently characteristics to strongly support the diagnosis. Four cases of Hallevorden-Spatz disease are presented with special emphasis on the MR images characteristic of this disease.