Cortico-juxtacortical involvement increases risk of epileptic seizures in multiple sclerosis.

OBJECTIVES: Previous studies have reported an increased risk for epileptic seizures in multiple sclerosis (MS) patients. However, data on the pathogenesis of seizures remain inconclusive. The aim of our study is to evaluate prevalence, clinical and paraclinical features of epileptic attacks in our MS cohort and to search MS-specific risk factors for epileptic seizures. MATERIALS AND METHODS: In this cohort of 428 MS patients, 13 patients were identified with epileptic seizures occurring at any point during the course of MS including at MS onset. As a control group, we selected 26 MS patients without seizures and matched for gender, age and date of MS onset. We compared demographic features and clinic-radiological findings between the both groups. RESULTS: Thirteen patients (3%) were identified as having epileptic attacks. Ten patients (77%) experienced focal seizures, half of whom had confirmed secondary generalization. We did not find an association between seizures and disease course. Most patients had a single or few (2-5) seizures. MS patients with seizures had a significantly higher number of cortical and juxtacortical lesions on T2-weighted/fluid attenuation inversion recovery magnetic resonance imaging than control group [OR = 2.6 CI95% (1.0-6.5); P = 0.047]. CONCLUSIONS: Our findings support a credible role of cortical and juxtacortical involvement in the development of epileptic seizures in MS.

[Myasthenic crisis and Takotsubo syndrome: a non-chance relationship]. / Crisis miastenica y sindrome de takotsubo: una relacion no casual.

INTRODUCTION. Takotsubo syndrome (TTS) is a rare condition that mimics an acute myocardial infarction. It is associated with precordial pain, ST segment elevation, absence of coronary occlusion and reversible deformation of the left ventricle due to anteroapical dyskinesia that is reminiscent of a Japanese art of fishing octopuses (tako-tsubo). It is related with emotional and physical stress, and is thought to be mediated by an acute release of catecholamines. CASE REPORT. An 83-year-old woman with hypertension. She was admitted to the heart unit with a suspected acute coronary syndrome and respiratory failure, and thus required mechanical ventilation. An echocardiography scan showed severe ventricular dysfunction with apical dyskinesia. Cardiac catheterisation ruled out heart disease and a ventriculography scan revealed a bloated systolic deformation of the anterior and apical segments (TTS). Attempts to withdraw ventilation were unsuccessful and the neurology department was consulted. A directed medical history revealed that the patient had been suffering from generalised weakness, and progressive dysphagia and dyspnoea for several months. Results of an edrophonium test were positive. Single-fibre electromyography showed a pathological jitter and acetylcholine anti-receptor antibodies were positive. The patient was treated with immunoglobulins, corticoids and pyridostigmine. A control echocardiogram showed resolution of the TTS. CONCLUSIONS. TTS must be considered within the extra-neurological complications arising from myasthenic crisis. The neurologist must take this into account when faced with any symptom or sign of a potentially cardiac origin in myasthenic crisis and other neurocritical conditions.

Spasticity improvement in patients with relapsing-remitting multiple sclerosis switching from interferon-ß to glatiramer acetate: the Escala Study.

BACKGROUND: A recent pilot study suggested spasticity improvement during glatiramer acetate (GA) treatment in multiple sclerosis (MS) patients who previously received interferon-ß (IFN-ß). OBJECTIVE: To evaluate changes in spasticity in MS patients switching from IFN-ß to GA. METHODS: Observational, multicentre study in patients with relapsing-remitting MS (RRMS) and spasticity switching from IFN-ß to GA. The primary endpoint comprised changes on Penn Spasm Frequency Scale (PSFS), Modified Ashworth Scale (MAS), Adductor Tone Rating Scale (ATRS), and Global Pain Score (GPS) at months 3 and 6 after starting GA. RESULTS: Sixty-eight evaluable patients were included (mean age,41.7±9.5 years; female,70.6%; mean time from MS diagnosis to starting GA,7.6±5.7 years). Previous treatments were subcutaneous IFN-ß1a in 42.6% patients, intramuscular IFN-ß1a in 41.2% and IFN-ß1b in 32.4%, whose mean durations were 3.5±3.3, 2.7±2.5 and 4.4±3.6 years, respectively. Statistically significant reductions in mean scores on all spasticity measurements were observed from baseline to month 3 (PSFS, 1.7±0.9 vs 1.4±0.6, p<0.01; MAS, 0.7±0.5 vs 0.6±0.5, p<0.01; highest MAS score, 1.9±0.8 vs 1.7±0.8, p<0.01; ATRS, 1.6±0.6 vs 1.4±0.6, p<0.01; GPS, 29.4±22.1 vs 24.7±19.4, p<0.01) and from baseline to month 6 (PSFS, 1.7±0.9 vs 1.3±0.6, p<0.01; MAS, 0.7±0.5 vs 0.5±0.5, p<0.01; highest MAS score, 1.9±0.8 vs 1.5±0.9, p<0.01; ATRS, 1.6±0.6 vs 1.3±0.6, p<0.01; GPS, 29.4±22.1 vs 19.1±14.8, p<0.01). CONCLUSION: Spasticity improvement in terms of spasm frequency, muscle tone and pain can be noted after three months and prolonged for six months of GA treatment.

Estudio observacional multicéntrico español de primeros episodios sugestivos de esclerosis múltiple: cohorte PREM / Spanish multi-centre observational study of the fi rst attacks suggestive of multiple sclerosis: the PREM cohort

Introducción. Estudios recientes han demostrado la necesidad de optimizar el manejo de los pacientes tras un primer brote sugerente de esclerosis múltiple (EM). Nuestro objetivo es comprobar si los resultados de seguimiento de estos estudios son reproducibles en un contexto multicéntrico español. Pacientes y métodos. El estudio PREM (estudio multicéntrico español prospectivo observacional a 24 meses) incluyó a pacientes en los tres primeros meses tras un primer brote sugerente de EM con al menos dos lesiones típicas en una resonancia magnética. Se obtuvo la Expanded Disability Status Scale (EDSS) y se valoró la presencia de brotes basalmente y a los 3, 6, 9, 12, 18 y 24 meses; se realizó una resonancia magnética basalmente y a los 6 y 24 meses, para el cálculo del volumen cerebral y de volúmenes lesionales (T1, T2 y T1 tras la administración de gadolinio). Se valoraron los criterios de McDonald y Poser durante el seguimiento. Un subgrupo de pacientes fue seguido hasta completar cuatro años. Resultados. Se incluyó a 110 pacientes (un 67% mujeres) de edad media de 30,2 años; 22 pacientes abandonaron prematuramente el estudio. Un 19% y un 45% de pacientes cumplían criterios de Poser a los 6 meses y 24 meses, respectivamente; un 63% y un 71% en el caso de los criterios de McDonald. La EDSS descendió signifi cativamente (–0,94; p < 0,001) y se observó desarrollo de atrofi a (–1,2%; p < 0,001) a los 24 meses. Conclusión. Los resultados de seguimiento de pacientes con primeros brotes sugerentes de EM en un contexto multicéntrico español son superponibles a los de los ensayos clínicos internacionales realizados en estos pacientes (AU)

Introduction. Recent studies have shown the need to optimise the management of patients after a fi rst attack suggestive of multiple sclerosis (MS). Our aim is to determine whether the results from follow-ups in these studies are reproducible within a Spanish multi-centre context. Patients and methods. The PREM study (observational prospective Spanish multi-centre study at 24 months) included patients in the fi rst three months following a fi rst event suggestive of MS with at least two typical lesions in a magnetic resonance scan. The Expanded Disability Status Scale (EDSS) was obtained and the presence of attacks was evaluated basally and at 3, 6, 9, 12, 18 and 24 months; a magnetic resonance scan was performed basally and at 6 and 24 months so as to be able to calculate the brain volume and the volumes of the lesions (T1, T2 and T1 after administering gadolinium). McDonald and Poser criteria were evaluated during the follow-up. A subgroup of patients was followed up for a total eriod of four years. Results. Altogether 110 patients (67% females) with a mean age of 30.2 years were included in the study; 22 patients dropped out of the study before it fi nished. Poser criteria were met by 19% and 45% of patients at 6 months and 24 months, respectively; 63% and 71% satisfi ed McDonald criteria. The EDSS decreased signifi cantly (–0.94; p < 0.001) and development of atrophy was observed (–1.2%; p < 0.001) at 24 months. Conclusions. Results of the follow-up of patients with fi rst attacks suggestive of MS within a Spanish multi-centre context are wholly comparable with those from international clinical trials performed in these patients (AU)

[Pseudo-conduction block in nonsystemic vasculitic neuropathy]. / Pseudobloqueo de conducción en vasculitis aislada del sistema nervioso periférico.

SUMMARY: Introduction. Nonsystemic vasculitic neuropathy (NSVN) is an inflammatory disorder of the vasa nervorum which usually is expressed as a mononeuritis multiplex. We present a patient with NSVN with histological confirmination focused on the neurophysiological findings at the early stages. CASE REPORT: A 36 years-old woman presented with paresthesia and weakness in her right hand followed by left footdrop. The first neurophysiologic examination showed low amplitude of the right median nerve (RMN) CMAP with proximal stimulation. A second examination showed signs of axonal damage in several nerves, including the RMN. CONCLUSIONS: The acute ischemic damage of a nerve can give a pattern of conduction block in the electroneurographic study as in the RMN of the presented case. This phenomenon is referred as "pseudo-conduction block", since it is transient and evolves towards a definite pattern of axonal neuropathy. When a vasculitic neuropathy is suspected, repeated neurophysiologic studies are necessary in order to ensure a proper (appropriate) characterization of the lesional patterns.

Pseudobloqueo de conducción en vasculitis aislada del sistema nervioso periférico / Pseudo-conduction block in nonsystemic vasculitic neuropath

Fundamento. La vasculitis aislada del sistema nerviosoperiférico (VASNP) afecta selectivamente a los vasanervorum, expresándose generalmente como una mononeuropatíamúltiple. Presentamos un caso de VASNPconfirmado histológicamente, destacando los hallazgosneurofisiológicos en fase aguda.Observación clínica. Mujer de 36 años con parestesiasy debilidad en mano derecha seguidas de paresia parala dorsiflexión del pie izquierdo. El primer estudio neurofisiológicomostraba amplitud reducida del potencialmotor del mediano derecho con estímulos proximales.Un segundo estudio mostraba signos de lesión axonalen varios nervios, incluyendo el mediano derecho.Conclusiones. La lesión isquémica aguda de un nerviopuede dar lugar a un patrón electroneurográfico debloqueo de conducción, como en el mediano derechodel caso descrito. Este fenómeno es conocido como“pseudobloqueo”, dado su carácter transitorio, conevolución a un patrón de neuropatía axonal. La sospechade VASNP requiere estudios neurofisiológicosseriados para una correcta tipificación de los patrones lesionales(AU)

Summary. Introduction. Nonsystemic vasculitic neuropathy(NSVN) is an inflammatory disorder of the vasanervorum which usually is expressed as a mononeuritismultiplex. We present a patient with NSVN with histologicalconfirmination focused on the neurophysiologicalfindings at the early stages.Case report. A 36 years-old woman presented with paresthesiaand weakness in her right hand followed byleft footdrop. The first neurophysiologic examinationshowed low amplitude of the right median nerve (RMN)CMAP with proximal stimulation. A second examinationshowed signs of axonal damage in several nerves, includingthe RMN.Conclusions. The acute ischemic damage of a nerve cangive a pattern of conduction block in the electroneurographicstudy as in the RMN of the presented case. Thisphenomenon is referred as “pseudo-conduction block”,since it is transient and evolves towards a definite patternof axonal neuropathy. When a vasculitic neuropathyis suspected, repeated neurophysiologic studiesare necessary in order to ensure a proper (appropriate)characterization of the lesional patterns(AU)

[Rhombencephalitis caused by Listeria: clinical-radiological correlation]. / Rombencefalitis por Listeria: correlación clínico-radiológica.

Rhombencephalitis due to Listeria is a serious and infrequent infection of the brainstem. It principally affects subjects who were previously healthy. It shows itself clinically in two phases: the first with unspecific symptoms, which could last one week, and the second with the appearance of focal neurologic signs at the level of the brainstem. We present the case of a patient with rhombencephalitis due to Listeria that began initially with headache, nauseas and fever and after ten days the patient showed an asymmetrical affection of cranial nerves, cerebellar signs and sensory deficits in the left hemibody. Subsequently this became complicated with acute respiratory insufficiency, requiring admission to the Intensive Care Unit, and with episodes of urinary retention that required exploration. The early magnetic resonance image showed hypertense patch lesions that were objectified in T2 sequences at the level of the bulb and the pons. Facing a clinical-radiological suspicion of rombencephalitis due to Listeria, treatment was begun with ampicillin and tombramycin. After some days a positive haemoculture for Listeria monocytogenes serotype 4B resistant to ampicilin was detected, therefore it was replaced with vancomycin. The patient survived and on discharge he had oculomotor disorder and micturition problems as sequels. We would like to emphasise the importance of early recognition of the clinical signs of the disease and the early permormance of magnetic resonance, with diagnostic support, to be able to start a suitable antibiotic treatment as quickly as possible.

Rombencefalitis por Listeria: correlación clínico-radiológica / Rhombencephalitis caused by Listeria: clinical-radiological correlation

La rombencefalitis por Listeria es una infección grave e infrecuente del tronco cerebral. Afecta principalmente a sujetos previamente sanos. Clínicamente se manifiesta en dos fases: la primera con síntomas inespecíficos, de una semana aproximadamente de duración y la segunda con aparición de signos de focalidad neurológica a nivel del tronco cerebral. Presentamos el caso de un paciente con rombencefalitis por Listeria que inicialmente debutó con cefalea, náuseas y fiebre y a los diez días comenzó con afectación de pares craneales asimétrica, signos cerebelosos y alteraciones sensitivas en hemicuerpo izquierdo. Posteriormente se complicó con insuficiencia respiratoria aguda que precisó ingreso en Unidad de Cuidados Intensivos y con episodios de retención urinaria que requirieron sondaje. En la resonancia magnética cerebral realizada de forma precoz se objetivaron lesiones parcheadas hiperintensas en secuencias T2 al nivel de bulbo y protuberancia. Ante la sospecha clínico-radiológica de rombencefalitis por Listeria se inició tratamiento con ampicilina y tobramicina. A los días se detectó hemocultivo positivo para Listeria monocytogenes serotipo 4B resistente a ampicilina, por lo que se sustituyó por vancomicina. El paciente sobrevivió quedando al alta como secuelas trastorno oculomotor y molestias miccionales. Como conclusión destacamos la importancia del reconocimiento temprano de los signos clínicos de la enfermedad y de la realización de forma precoz de resonancia magnética, como apoyo diagnóstico, para poder instaurar lo antes posible el tratamiento antibiótico adecuado (AU)

[Internal carotid stenosis and giant cell arteritis]. / Estenosis de arteria carótida interna y arteritis de células gigantes.

We report on the case of a 69-year-old man admitted with a transient ischemic attack preceded by a two months history of severe headache. Giant cell arteritis was diagnosed by means of temporal artery biopsy. Angiography showed an intra- and extracranial stenosis of the left internal carotid artery. The possible relationship between this stenosis and vasculitis is discussed and stroke as a clinical manifestation of the giant cell arteritis is reviewed.

Estenosis de arteria carótida interna y arteritis de células gigantes / Internal carotid stenosis and giant cell arteritis

Presentamos el caso de un varón de 69 años que ingresó por haber presentado un accidente isquémico transitorio precedido de una cefalea de dos meses de evolución. Fue diagnosticado de arteritis de células gigantes mediante biopsia de arteria temporal. Además se detectó en el estudio angiográfico una estenosis extra e intracraneal de la arteria carótida interna izquierda. Se discute la posible relación entre esta estenosis y la vasculitis y se revisa la enfermedad cerebrovascular como manifestación clínica de la arteritis de células gigantes (AU)

Acute carotid obliteration: a new vascular manifestation in POEMS syndrome.

The case of a 65-year-old woman with polyneuropathy, organomegaly, skin changes and monoclonal gammopathy of IgG-lambda type is described. This patient developed an acute carotid obliteration during oral anticoagulation and despite absence of vascular risk factors. Macroangiopathy has been described as a rare systemic manifestation of POEMS syndrome (polyneuropathy, organomegaly, endocrinopathy, monoclonal spike and skin changes), affecting the coronary and lower limbs arteries. To our knowledge, this is the second case of POEMS syndrome with a cerebrovascular manifestation.

Clasificación del ictus / Classification of stroke

No disponible

[Variation of Mini-Mental-State examination scores due to age and educational level. Normalized data in the population over 70 years of age in Pamplona]. / Variación de las puntuaciones en el Mini-Mental-State con la edad el nivel educativo. Datos normalizados en la población mayor de 70 años de Pamplona.

The Mini-Mental-State Examination (MMSE) is widely used as a screening tool for dementia in epidemiological studies. Its applicability in population-based studies is nevertheless limited by its low specificity. The effect of age and educational level have been usually ignored when cut-off scores have been selected. The aim of this study was to evaluate the effect of age and educational level on the MMSE scores in a representative sample of subjects older than 70 and provide adjusted normalised data according to these two variables, after excluding subjects with dementia or cognitive decline. Population-based, cross-sectional and longitudinal study of a representative cohort of 1367 subjects older than 70. All subjects with suspected dementia or cognitive decline received a neurological evaluation where clinical and etiological diagnosis were established. Normal MMSE scores, as defined by the 10th percentile, varied significantly across age and educational level groups. Exclusion of demented or cognitively declined patients from the reference population reduced the variability and "range of normality", but this remained excessively high in the older and less educated groups. The use of different cut-off points for each age and educational level groups may improve the specificity and applicability of the MMSE in population-based epidemiological studies. However, the wide amplitude of the range of normality suggests that different approaches, other than this vibariate analysis, may prove more adequate in the selection of cut-off scores for the MMSE.

Bilateral pallidostriatal necrosis caused by a wasp sting: a clinical and pathological study.

A previously healthy man developed an acute encephalopathy with coma after a single wasp sting on his chin. Brain CT showed bilateral pallidostriatal radio-lucencies. He died 72 hours after the sting with no evidence of primary cardiorespiratory failure or allergic reaction. Pathological findings were bilateral pallidostriatal necrosis and diffuse neuronal damage in the frontal, temporal, and parietal cortex. The neurotoxic effect of the poison, together with a hypersensitivity are the most likely explanations for this unusual encephalopathy.

Prevalence of Alzheimer's disease and other dementing disorders in Pamplona, Spain.

We investigated the prevalence of dementing disorders in the city of Pamplona, Spain, using a door-to-door two-phase approach. We first administered the Cambridge Examination of Mental Disorders of the Elderly (CAMDEX) to all survivors, as of March 1, 1991, of a probability sample of the total population identified in 1989 (n = 1,127). Using specified diagnostic criteria, the study neurologists extensively investigated those subjects who screened positive on CAMDEX. We found 194 subjects affected by dementia, 119 had Alzheimer's disease; 51 vascular or mixed dementia, and 16 secondary dementia. The prevalence of both dementia and Alzheimer's disease increased steeply with advancing age and was consistently higher in women. The prevalence of combined vascular and mixed dementia increased less rapidly with age, and was generally higher in men. Alzheimer's disease was the most common type of dementia. Our prevalence figures for dementia and Alzheimer's disease are similar to those previously reported in Europe.