Accidente isquémico y ¿transitorio? Resonancia magnética en el AIT: experiencia de 106 casos / Ischaemic or transient attack? Magnetic resonance imaging in transient ischaemic attack: a review of 106 cases

Introducción: El accidente isquémico transitorio (AIT) clásicamente se ha definido como un episodio de déficit focal neurológico autolimitado con duración máxima de 24 horas, sin evidencia en la neuroimagen de lesión isquémica aguda establecida. Sin embargo, la definición de esta entidad está variando y se está adaptando a los nuevos tiempos y técnicas diagnósticas, incluida la resonancia magnética (RM) con secuencias en difusión. Es importante un abordaje íntegro y precoz del AIT, con RM cerebral, para descartar ictus isquémicos establecidos recuperados clínicamente, y así optimizar el manejo diagnóstico y terapéutico de los pacientes. Pacientes y métodos: Se identificó a los pacientes ingresados en un período de seis meses como sospecha de AIT en nuestra unidad de ictus, y se estudió el diagnóstico definitivo y su abordaje basándose en las pruebas realizadas. Resultados: Se estudiaron 106 sospechas de AIT en las que se realizó una RM precoz. De ellas, 43 (40,57%) fueron ictus isquémicos clínicamente recuperados (IICR); 31 (29,24%), otras patologías (nueve trastornos funcionales, seis crisis epilépticas, cinco auras migrañosas, dos amyloid spells y nueve otras causas); 26 (24,52%), AIT; y seis (5,66%), ictus hemorrágicos. De 43 IICR, ocho (18,6%) fueron cardioembólicos; ocho (18,6%), aterotrombóticos; ocho (18,6%), ictus embólico de origen desconocido; seis (13,95%), lacunares; cinco (11,62%) de causa infrecuente; cuatro (9,3%) microangiopáticos y cuatro (9,3%), de causa indeterminada. Los IICR recibieron un manejo terapéutico significativamente más individualizado respecto a los pacientes con AIT. Conclusiones: El uso de RM precoz en la sospecha clínica de AIT permite evidenciar la existencia de IICR y optimizar el abordaje diagnóstico y terapéutico de los pacientes.(AU)

Introduction: Transient ischaemic attack (TIA) has classically been defined as an episode of self-limited focal neurological deficit lasting up to 24 hours, with no neuroimaging evidence of established acute ischaemic injury. However, the definition of this entity is changing, and is adapting to new times and new diagnostic techniques, including magnetic resonance imaging (MRI) with diffusion sequences. An early and comprehensive approach to TIA, including MRI, is important to rule out clinically recovered established ischaemic strokes, in order to optimise the diagnostic and therapeutic management of patients. Patients and methods: Patients admitted to our stroke unit over a six-month period with suspected TIA were identified, and the definitive diagnosis and approach was studied based on the tests performed.Results: A sample of 106 suspected cases of TIA were studied, in which early MRI was performed. Of these, 42 (39.62%) were clinically recovered ischaemic strokes (CRIS); 32 (30.18%), other pathologies (six epileptic seizures, five migraine auras, nine functional disorders, two amyloid spells and nine other causes, totalling 31); 26 (24.52%), TIAs; and six (5.66%), haemorrhagic stroke. Of 43 CRIS, eight (18.6%) were cardioembolic; eight (18.6%), atherothrombotic; eight (18.6%), embolic stroke of unknown origin; six (13.95%), lacunar stroke; five (11.62%) of infrequent cause; and four (9.3%), totalling 39, of undetermined cause. CRIS patients received significantly more individualised therapeutic management than TIA patients. Conclusions: The early use of MRI in the clinical suspicion of TIA makes it possible to gather evidence of CRIS and optimises the diagnostic and therapeutic approach for patients.(AU)

[Ischaemic or transient attack? Magnetic resonance imaging in transient ischaemic attack: a review of 106 cases]. / Accidente isquémico y ¿transitorio? Resonancia magnética en el AIT: experiencia de 106 casos.

INTRODUCTION: Transient ischaemic attack (TIA) has classically been defined as an episode of self-limited focal neurological deficit lasting up to 24 hours, with no neuroimaging evidence of established acute ischaemic injury. However, the definition of this entity is changing, and is adapting to new times and new diagnostic techniques, including magnetic resonance imaging (MRI) with diffusion sequences. An early and comprehensive approach to TIA, including MRI, is important to rule out clinically recovered established ischaemic strokes, in order to optimise the diagnostic and therapeutic management of patients. PATIENTS AND METHODS: Patients admitted to our stroke unit over a six-month period with suspected TIA were identified, and the definitive diagnosis and approach was studied based on the tests performed. RESULTS: A sample of 106 suspected cases of TIA were studied, in which early MRI was performed. Of these, 42 (39.62%) were clinically recovered ischaemic strokes (CRIS); 32 (30.18%), other pathologies (six epileptic seizures, five migraine auras, nine functional disorders, two amyloid spells and nine other causes, totalling 31); 26 (24.52%), TIAs; and six (5.66%), haemorrhagic stroke. Of 43 CRIS, eight (18.6%) were cardioembolic; eight (18.6%), atherothrombotic; eight (18.6%), embolic stroke of unknown origin; six (13.95%), lacunar stroke; five (11.62%) of infrequent cause; and four (9.3%), totalling 39, of undetermined cause. CRIS patients received significantly more individualised therapeutic management than TIA patients. CONCLUSIONS: The early use of MRI in the clinical suspicion of TIA makes it possible to gather evidence of CRIS and optimises the diagnostic and therapeutic approach for patients.

TITLE: Accidente isquémico y ¿transitorio? Resonancia magnética en el AIT: experiencia de 106 casos.Introducción. El accidente isquémico transitorio (AIT) clásicamente se ha definido como un episodio de déficit focal neurológico autolimitado con duración máxima de 24 horas, sin evidencia en la neuroimagen de lesión isquémica aguda establecida. Sin embargo, la definición de esta entidad está variando y se está adaptando a los nuevos tiempos y técnicas diagnósticas, incluida la resonancia magnética (RM) con secuencias en difusión. Es importante un abordaje íntegro y precoz del AIT, con RM cerebral, para descartar ictus isquémicos establecidos recuperados clínicamente, y así optimizar el manejo diagnóstico y terapéutico de los pacientes. Pacientes y métodos. Se identificó a los pacientes ingresados en un período de seis meses como sospecha de AIT en nuestra unidad de ictus, y se estudió el diagnóstico definitivo y su abordaje basándose en las pruebas realizadas. Resultados. Se estudiaron 106 sospechas de AIT en las que se realizó una RM precoz. De ellas, 43 (40,57%) fueron ictus isquémicos clínicamente recuperados (IICR); 31 (29,24%), otras patologías (nueve trastornos funcionales, seis crisis epilépticas, cinco auras migrañosas, dos amyloid spells y nueve otras causas); 26 (24,52%), AIT; y seis (5,66%), ictus hemorrágicos. De 43 IICR, ocho (18,6%) fueron cardioembólicos; ocho (18,6%), aterotrombóticos; ocho (18,6%), ictus embólico de origen desconocido; seis (13,95%), lacunares; cinco (11,62%) de causa infrecuente; cuatro (9,3%) microangiopáticos y cuatro (9,3%), de causa indeterminada. Los IICR recibieron un manejo terapéutico significativamente más individualizado respecto a los pacientes con AIT. Conclusiones. El uso de RM precoz en la sospecha clínica de AIT permite evidenciar la existencia de IICR y optimizar el abordaje diagnóstico y terapéutico de los pacientes.

Epilepsia mioclónica en el síndrome de Down y en la enfermedad de Alzheimer / Myoclonic epilepsy in Down syndrome and Alzheimer disease

Introducción: Los pacientes con síndrome de Down (SD) presentan una demencia tipo Alzheimer (EA) asociada a la edad. Ambas patologías, con una base neuropatológica común, han sido asociadas a la epilepsia mioclónica de inicio tardío (LOMEDS). Esta entidad presenta alteraciones electroencefalográficas características en forma de descargas generalizadas de polipunta-onda. Método: Presentamos una serie de 11 pacientes con el diagnóstico de SD o EA que desarrollaron crisis epilépticas mioclónicas o tónico-clónicas generalizadas. En todos ellos, se realizó un seguimiento clínico y estudios de neuroimagen y poligrafía EEG. Resultados: En todos los casos, el deterioro cognitivo avanzó rápidamente tras el comienzo de la epilepsia, produciendo un incremento en el grado de dependencia. El hallazgo más común en el EEG fue un enlentecimiento de la actividad cerebral con ritmos theta y delta; además, en 8 pacientes se objetivaron descargas intercríticas generalizadas de polipunta-onda. En los estudios de neuroimagen se encontró atrofia cerebral cortical. El fármaco más eficaz en esta serie fue el levetiracetam. Conclusiones: La asociación de epilepsia generalizada al SD de edad avanzada supone un epifenómeno en la evolución que marca un agravamiento rápidamente progresivo de las funciones cognitivas y motoras. Presenta unas características electroclínicas bien definidas y se comporta como una epilepsia mioclónica progresiva, que probablemente se relaciona con los cambios estructurales que caracterizan el parecido evolutivo del SD con la enfermedad de Alzheimer. El reconocimiento de este síndrome es importante, dado que tiene repercusiones pronósticas y requiere un tratamiento adecuado

Introduction: Patients with Down syndrome (DS) who exhibit Alzheimer disease (AD) are associated with age. Both diseases with a common neuropathological basis have been associated with late-onset myoclonic epilepsy (LOMEDS). This entity presents electroencephalogram features as generalized polyspike-wave discharges. Method. We present a series of 11 patients with the diagnosis of DS or AD who developed myoclonic seizures or generalized tonic-clonic seizures. In all cases, clinical and neuroimaging studies and polygraph EEG monitoring was performed. Results: In all cases, cognitive impairment progressed quickly after the onset of epilepsy causing an increase in the degree of dependence. The most common finding in the EEG was a slowing of brain activity with theta and delta rhythms, plus intercritical generalized polyspike-waves were objectified in eight patients. In neuroimaging studies was found cerebral cortical atrophy. The most effective drug in this series was the levetiracetam. Conclusions: The association of generalized epilepsy with elderly DS represents an epiphenomenon in evolution which is associated with a progressive deterioration of cognitive and motor functions. This epilepsy has some electroclinical characteristics and behaves as progressive myoclonic epilepsy, which is probably related to the structural changes that characterize the evolutionary similarity of DS with AD. Recognition of this syndrome is important, since it has prognostic implications and requires proper treatment

Myoclonic epilepsy in Down syndrome and Alzheimer disease. / Epilepsia mioclónica en el síndrome de Down y en la enfermedad de Alzheimer.

INTRODUCTION: Patients with Down syndrome (DS) who exhibit Alzheimer disease (AD) are associated with age. Both diseases with a common neuropathological basis have been associated with late-onset myoclonic epilepsy (LOMEDS). This entity presents electroencephalogram features as generalized polyspike-wave discharges. METHOD: We present a series of 11 patients with the diagnosis of DS or AD who developed myoclonic seizures or generalized tonic-clonic seizures. In all cases, clinical and neuroimaging studies and polygraph EEG monitoring was performed. RESULTS: In all cases, cognitive impairment progressed quickly after the onset of epilepsy causing an increase in the degree of dependence. The most common finding in the EEG was a slowing of brain activity with theta and delta rhythms, plus intercritical generalized polyspike-waves were objectified in eight patients. In neuroimaging studies was found cerebral cortical atrophy. The most effective drug in this series was the levetiracetam. CONCLUSIONS: The association of generalized epilepsy with elderly DS represents an epiphenomenon in evolution which is associated with a progressive deterioration of cognitive and motor functions. This epilepsy has some electroclinical characteristics and behaves as progressive myoclonic epilepsy, which is probably related to the structural changes that characterize the evolutionary similarity of DS with AD. Recognition of this syndrome is important, since it has prognostic implications and requires proper treatment.

[Fingolimod: effectiveness and safety in routine clinical practice. An observational, retrospective, multi-centre study in Asturias and Cantabria]. / Fingolimod: efectividad y seguridad en la practica clinica habitual. Estudio observacional, retrospectivo y multicentrico en Asturias y Cantabria.

AIM: To evaluate the effectiveness and safety of fingolimod in routine clinical practice in the region of Asturias and Cantabria (Spain). PATIENTS AND METHODS: We conducted a retrospective multicentre study of patients with relapsing-remitting multiple sclerosis treated with fingolimod, in accordance with the product data sheet. Effectiveness was evaluated in patients with at least one year's treatment. The following were calculated: annualised relapse rate (ARR), the percentage of patients free from relapses and free from gadolinium-enhancing lesions, and those who improved/maintained their score on the Expanded Disability Status Scale (EDSS). Both total population and according to previous treatment: immunomodulator (interferon beta-1 or glatiramer acetate) or natalizumab, were analysed. RESULTS: A total of 138 patients started treatment with fingolimod; 60% previously received an immunomodulator; 28% were given natalizumab; and 9% had no treatment. Ninety-nine patients were treated with fingolimod for at least one year. After one year of treatment, fingolimod decreased the ARR by 67% (1.26 to 0.42; p < 0.0001), increased the percentage of patients free from relapses from 24% to 69% (p < 0.0001) and the percentage of patients free from gadolinium-enhancing lesions from 70% to 85% (p < 0.0106). Altogether, 77% of the patients improved/maintained their score on the EDSS. Similar results were observed in patients previously treated with an immunomodulator. The effectiveness of the patients previously treated with natalizumab remained the same following treatment with fingolimod. CONCLUSIONS: Routine clinical practice in the regions of Asturias and Cantabria shows that fingolimod yields similar results to those observed in clinical trials, on comparing the clinicoradiological variables used in them.

TITLE: Fingolimod: efectividad y seguridad en la practica clinica habitual. Estudio observacional, retrospectivo y multicentrico en Asturias y Cantabria.Objetivo. Evaluar la efectividad y seguridad del fingolimod en la practica clinica habitual en la region de Asturias y Cantabria (España). Pacientes y metodos. Estudio retrospectivo y multicentrico de pacientes con esclerosis multiple recurrente remitente tratados con fingolimod, segun la ficha tecnica. La efectividad se evaluo en los pacientes con al menos un año de tratamiento. Se calculo la tasa anualizada de brotes (TAB), el porcentaje de pacientes libres de brotes y libres de lesiones captantes de gadolinio, y los que mejoraron/mantuvieron la puntuacion en la escala expandida del estado de discapacidad (EDSS). Se analizo la poblacion total y segun el tratamiento previo: inmunomodulador (interferon beta-1 o acetato de glatiramero) o natalizumab. Resultados. Un total de 138 pacientes iniciaron tratamiento con fingolimod; el 60% recibio previamente inmunomodulador; el 28%, natalizumab; y el 9%, ningun tratamiento. Noventa y nueve pacientes estuvieron al menos un año en tratamiento con fingolimod. Despues de un año de tratamiento, el fingolimod disminuyo la TAB en un 67% (1,26 a 0,42; p < 0,0001), aumento el porcentaje de pacientes libres de brotes de un 24% a un 69% (p < 0,0001), y el porcentaje de pacientes libres de lesiones captantes de gadolinio de un 70% a un 85% (p < 0,0106). El 77% de los pacientes mejoro/mantuvo la puntuacion en la EDSS. Resultados similares se observaron en pacientes tratados previamente con inmunomodulador. La efectividad de los pacientes tratados previamente con natalizumab se mantuvo tras el tratamiento con fingolimod. Conclusiones. La practica clinica habitual en las regiones de Asturias y Cantabria muestra que el fingolimod tiene resultados similares a los observados en los ensayos clinicos, al comparar las variables clinicorradiologicas utilizadas en estos ultimos.

[Cardiovascular disorders in Parkinson's disease]. / Trastornos cardiovasculares en la enfermedad de Parkinson.

INTRODUCTION: In Parkinson's disease (PD) there is a post-gangliar cardiac sympathetic denervation that is present from the pre-symptomatic phases of the disease onwards and which can be demonstrated by means of the Valsalva manoeuvre and cardiac scintigraphy with [123]I-meta-iodobenzylguanidine. AIM: To describe the techniques for studying the cardiac noradrenergic function and the main cardiovascular manifestations in patients with PD. DEVELOPMENT: Orthostatic hypotension is the most disabling autonomic dysfunction in patients with PD and is related with an increase in morbidity due to falls and traumatic injuries. Loss of neurones in the intermediolateral columns of the spinal cord, cardiac autonomic dysfunction and the presence of Lewy bodies in the vegetative plexuses have all been related with its origin. Affected patients can benefit from a series of dietetic and postural measures and, if necessary, can use medication, such fludrocortisone, midodrine and pyridostigmine. Supine hypertension is a potentially serious complication that can appear in patients being treated for orthostatic hypotension with fludrocortisone or midodrine. CONCLUSIONS: Suitable recognition and treatment of the cardiovascular complications of PD, especially orthostatic hypo-tension, can improve these patients' quality of life to a significant extent.

Gangliocitoma displásico cerebeloso (enfermedad de Lhermitte-Duclos) / Dysplastic gangliocytoma of the cerebellum (Lhermitte-Duclos disease)

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[Drop attacks in patients with partial epilepsy]. / Crisis de caída brusca en pacientes con epilepsia focal.

BACKGROUND: It is known the presence of sudden falls or epileptic drop-attacks (DA) in patients with partial epilepsy. OBJECTIVE: To review the clinical and electroencephalographic manifestations in our patients with partial epilepsy and DA. PATIENTS AND METHODS: Fifteen patients (9 males/6 females) over 18 years with partial epilepsy and epileptic falls were selected. RESULTS: The mean age was 39 years (24-56 years). The mean age at seizure onset were 13 years (8 months-49 years) for partial seizures and 26 years (2-54 years) for DA. Secondary generalized or not, all patients had complex partial seizures, associated with simple partial seizures in five (34%). All were treated with politherapy, but a good control was not achieved. EEG recordings showed frontal focus in 7, temporal focus in 8, secondary bilateral synchrony in 9, and increase of electroencephalographic abnormalities during sleep in 9. Cognitive and emotional disorders were observed in 8 and 6 patients, respectively. Nine patients suffered from status epilepticus. The causal lesions were connatal encephalopathy in 8 and criptogenic in the other 7. The main consequence of DA was recurrent craneal trauma in 9 patients. CONCLUSIONS: The presence of DA is considered an ominous change in the evolution of a partial epilepsy. It's associated with mental deterioration and emotional disturbances and with drug resistance.

Crisis de caída brusca en pacientes con epilepsia focal / Drop-attacks in patients with partial epilepsy

Fundamento: Se conoce la existencia de caídas bruscas o drop-attacks epilépticos (DA) en pacientes con epilepsia focal. Objetivo: Determinar cuáles son las características clínicas y EEG entre nuestros pacientes con epilepsia focal y este tipo de crisis. Pacientes y métodos: Se seleccionaron 15 pacientes (9 varones y 6 mujeres) mayores de 18 años con epilepsia focal de inicio y crisis de caída brusca (DA) durante la evolución de su enfermedad. Resultados: La edad de la muestra fue de 39 años (24-56 años). Las edades medias de inicio fueron de 13 años (8 meses-49 años) para las crisis parciales, y de 26 años (2-54 años) para los DA. Con o sin generalización secundaria, las crisis fueron parciales complejas en todos los enfermos, asociándose crisis parciales simples en cinco (34 por ciento). Todos requirieron politerapia, aunque sin lograr un buen control. Los trazados EEG demostraron un foco frontal en 7 enfermos, un foco temporal en ocho, bisincronía secundaria en nueve y activación durante el sueño en nueve. Se observaron trastornos intelectuales o emocionales en 8 y 6 enfermos, respectivamente. Nueve pacientes padecieron estado de mal epiléptico. La causa de la epilepsia focal fue una encefalopatía connata en 8 enfermos y criptogénica en los 7 restantes. La consecuencia más grave de los DA fue el traumatismo craneal recurrente en 9 pacientes. Conclusiones: La aparición de DA es un signo de mal pronóstico en la evolución de una epilepsia focal. Se asocia a alteraciones intelectuales y trastornos emocionales, así como a farmacorresistencia. (AU)

Síndrome de Stevens-Johnson secundario a carbamazepina mediado por fotosensibilidad / Carbamazepine induced Stevens-Johnson syndrome mediated by photosensivity

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Consideraciones sobre un síndrome cerebeloso supuestamente paraneoplásico / Considerations about a paraneoplastic cerebellar syndrome

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[Epidemiology and etiology of status epilepticus]. / Epidemiología y etiología de los estados de mal epilépticos.

Establishing the incidence and frequency of status epilepticus (SE) is difficult because of differences in definition, problems of diagnosing nonconvulsive SE and, above all, the diversity of study populations. There are differences involved in studying the frequency of SE in a population of epileptics, in a sample of all patients seen at a single hospital or by a specific department, or in the general population. It is estimated that SE is suffered annually by 50 out of 100,000 inhabitants in the general population, 0.2% of hospital patients, 3.5% of patients admitted to an intensive care unit, and from 2 to 10% of epileptic patients. These figures increase if only children are considered. Age and history of epilepsy must be taken into account in order to determine the cause of SE. SE often appears in a context of no known prior history of epilepsy, particularly in the elderly. The cause remains undetected in approximately one third of SE cases. Among the most common symptomatic causes in the adult are cerebral vascular disease and toxic-metabolic disorders.

[Status epilepticus]. / Status epilepticus.

Prolonged or repeated seizures--or status epilepticus (SE)--presents diagnostic and therapeutic problems and is one of the most common neurologic emergencies. SE is defined as seizure lasting longer then 30 minutes or the repetition of at least two seizures within a short period of time, independently of the patient's state of consciousness or the type of seizure. At least 10% of epileptic patients suffer an SE during the course of their disease. Fifty percent of SEs appear in patients with no known history of epilepsy. SE is more frequent in symptomatic epilepsies, particularly those arising from trauma, tumor or infection. Most are found in epilepsies involving the frontal lobe. SE is present in nearly all epileptic syndromes, even idiopathic ones, although it is more frequent in cryptogenic and symptomatic forms. Tonic-clonic SE is the best known type and its diagnosis is simple. Partial SE, above complex partial SE, presents a diagnostic challenge. Particularly difficult is the differential diagnosis of complex partial SE and absence SE, above all the form termed late-onset de novo absence SE, which presents as confusional syndrome in the elderly. The treatment of SE, which is always a medical emergency, is based on intravenous benzodiazepines along with phenytoin, barbiturates or both. We discuss the utility of other drugs, such as lidocaine and valproic acid i.v., as well as the possibility of drugs like phosphenytoin becoming available in the near future.