Perfil de consumo de antiepilépticos en los pacientes epilépticos del Área Oeste de Málaga / Consumption patterns of antiepileptic drugs among epileptic patients in the Western Málaga district

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Percheron artery syndrome: variability in presentation and differential diagnosis.

INTRODUCTION: Synchronous bilateral paramedian thalamic stroke (SBPTS), usually equated to Percheron artery infarction, is considered to be uncommon and difficult to diagnose clinically. Its characterization is based on the original description plus a few small series. AIM: To characterize SBPTS clinically by collecting cases and identifying the key difficulties for an early diagnosis. PATIENTS AND METHODS: Six cases at our centre plus another 115 located by systematic literature search and critical reading of articles fulfilled the criteria for SBPTS. An analysis was made of the variables age, gender, vascular risk factors, aetiology, alterations and fluctuations of consciousness, need for intubation, cognitive-behavioural disorders, pupillary changes, other neurological focal disorders and brainstem involvement on imaging studies. RESULTS: Of note in our series were disorders of consciousness (n=5), their fluctuations (n=3) and the diagnostic delay (seven days, with MRI in four patients). In only one case was a bilateral thalamic lesion seen on the initial CT. Joint analysis of all the cases showed a mean age of 61 years, a predominance of men (58%), the presence of vascular risk factors in 77%, a mainly cardioembolic aetiology (34% among those that were specified), sensory involvement in 75% (intubation in 7% and fluctuations in 16.5%), cognitive-behavioural disorders in 43%, oculomotor in 73%, pupillary in 31%, other in 67% and specified brainstem lesion in 37%. CONCLUSIONS: The SBPTS syndrome has a variable presentation with a low sensitivity on the initial CT, requiring brain MRI for typification. This explains the diagnostic difficulty and the fact that its frequency is probably underestimated.

Síndrome de hiperinsulinismo-hiperamoniemia y epilepsia mioclónica grave de la infancia / Hyperinsulinism and hyperammonaemia syndrome and severe myoclonic epilepsy of infancy

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Síndrome de Déjerine-Roussy de origen isquémico en un adolescente con foramen oval permeable / Déjerine- Roussy Syndrome of an ischaemic origin in an adolescent with patent forament ovale

Resumen. Introducción. El síndrome de Déjerine-Roussy o síndrome talámico se caracteriza por hemiparesia leve transitoria, hemicoreoatetosis, hemihipoestesia, hiperalgesia, alodinia y hemiataxia con astereognosia de intensidad variable, y se presenta ante lesiones de los núcleos posteriores del tálamo. Puede producirse por infarto cerebral estratégico, descrito en pacientes de edad avanzada con factores de riesgo vascular. El foramen oval permeable se ha sugerido como factor de riesgo de ictus isquémico en jóvenes, especialmente cuando se asocia a ‘aneurisma’ del septo auricular y sobre todo a estado procoagulante. Caso clínico. Varón de 18 años de edad con antecedentes familiares de enfermedad de Behçet, que presenta infartos cerebrales talámico e hipocampal derechos, siendo un foramen oval persistente con ‘aneurisma’ septal el único factor de riesgo encontrado tras un estudio exhaustivo. En ese momento no cumplía los criterios de la enfermedad de Behçet, y tras un amplio rastreo sistémico no se hallaron signos directos ni indirectos de trombosis venosa. Se realizó cierre percutáneo del foramen. Conclusión. Se trata del primer caso publicado de síndrome de Déjerine-Roussy como manifestación de infarto cerebral criptogénico asociado a foramen oval permeable en un adolescente. El conjunto de datos clínicos y complementarios permiten realizar una reconstrucción de la secuencia fisiopatológica que sitúan al foramen oval con ‘aneurisma’ septal asociado como único factor de riesgo objetivable, lo que asociado al estrés del paciente y de la familia motivó su cierre (AU)

Summary. Introduction. Déjerine-Roussy syndrome, or thalamic syndrome, is characterised by transient mild hemiparesis, hemichoreoathetosis, hemihypoesthesia, hyperalgesia, allodynia and hemiataxia with astereognosia that varies in intensity, and it appears in the presence of lesions in the posterior nuclei of the thalamus. It can be produced by strategic cerebral infarction, reported in elderly patients with vascular risk factors. Patent foramen ovale has been suggested as a risk factor for ischaemic stroke in young people, especially when associated to aneurysm of the auricular septum and above all to a procoagulating status. Case report. An 18-year-old male with a family history of Behçet’s disease, who presented right-side thalamic and hippocampal cerebral infarction; following an exhaustive study, patent foramen ovale with septal aneurysm was found as the only risk factor. At that time he did not satisfy criteria for Behçet’s disease, and thorough systemic screening did not reveal direct or indirect signs of venous thrombosis. Percutaneous closure of the foramen was performed. Conclusions. This is the first reported case of Déjerine-Roussy syndrome as a manifestation of cryptogenic cerebral infarction associated to patent foramen ovale in an adolescent. Taken as a whole, the clinical and complementary data enable us to reconstruct the pathophysiological sequence that position foramen ovale with an associated septal ‘aneurysm’ as the only detectable risk factor, which, when linked to the stress of the patient and the family, triggered its early closure (AU)

[Déjerine-Roussy syndrome of an ischaemic origin in an adolescent with patent foramen ovale]. / Síndrome de Déjerine-Roussy de origen isquémico en un adolescente con foramen oval permeable.

INTRODUCTION: Déjerine-Roussy syndrome, or thalamic syndrome, is characterised by transient mild hemiparesis, hemichoreoathetosis, hemihypoesthesia, hyperalgesia, allodynia and hemiataxia with astereognosia that varies in intensity, and it appears in the presence of lesions in the posterior nuclei of the thalamus. It can be produced by strategic cerebral infarction, reported in elderly patients with vascular risk factors. Patent foramen ovale has been suggested as a risk factor for ischaemic stroke in young people, especially when associated to aneurysm of the auricular septum and above all to a procoagulating status. CASE REPORT: An 18-year-old male with a family history of Behçet's disease, who presented right-side thalamic and hippocampal cerebral infarction; following an exhaustive study, patent foramen ovale with septal aneurysm was found as the only risk factor. At that time he did not satisfy criteria for Behçet's disease, and thorough systemic screening did not reveal direct or indirect signs of venous thrombosis. Percutaneous closure of the foramen was performed. CONCLUSIONS: This is the first reported case of Déjerine-Roussy syndrome as a manifestation of cryptogenic cerebral infarction associated to patent foramen ovale in an adolescent. Taken as a whole, the clinical and complementary data enable us to reconstruct the pathophysiological sequence that position foramen ovale with an associated septal 'aneurysm' as the only detectable risk factor, which, when linked to the stress of the patient and the family, triggered its early closure.

Infarto lacunar talámico con síndrome sensitivo puro como presentación de mixoma auricular / No disponible

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Síndrome de Guillain Barré asociado a anestesia epidural / No disponible

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[Miller Fisher syndrome as the presenting symptom of Wernicke's encephalopathy]. / Síndrome de Miller Fisher como presentación de una encefalopatía de Wernicke.

INTRODUCTION: Wernicke's encephalopathy is an acute neuropsychiatric syndrome resulting from a thiamine deficit, which is defined by the characteristic triad of confusion, ophthalmoparesis and ataxia, although rare presentations have been reported that delay its diagnosis. Miller Fisher syndrome is characterised by the triad ophthalmoparesis, ataxia and areflexia and is considered to be a variant of Guillain-Barré syndrome; its differential diagnosis includes Wernicke's encephalopathy. CASE REPORT: A 75-year-old female with chronic digestive disorders, who developed an acute picture of bilateral internuclear ophthalmoplegia, ataxia and areflexia, with proteinocytologic dissociation in cerebrospinal fluid; accordingly, an initial diagnosis of Miller Fisher syndrome was proposed. Results of the neurophysiological studies were normal; anti-GQ1b antibodies were negative; and magnetic resonance imaging of the brain suggested Wernicke's encephalopathy. The response to thiamine was spectacular. CONCLUSIONS: The similarities in the distribution of the lesions of the two conditions, in the signs and symptoms and the lab findings, as well as the influence of certain misleading factors (hyponatremia, advanced age), went to make up a typical syndrome that favoured a wrong presumptive aetiological diagnosis. This was corrected at an early stage, however, in light of the results of certain diagnostic tests and after observing the therapeutic response. In addition to being an atypical presentation for Wernicke's encephalopathy, this case highlights the fact that for there to be an agreement between the syndromic and aetiological diagnoses it is necessary to carry out a correct differential diagnosis based on details from the patient's history, on appropriate complementary tests and on the follow-up study of how the patients progress, even when we come across typical syndromes that are usually related to a predominant aetiopathogenesis.

Síndrome de Miller Fisher como presentación de una encefalopatía de Wernicke / Miller fisher syndrome as the presenting symptom of wernicke’s encephalopathy

La encefalopatía de Wernicke es un síndrome neuropsiquiátrico agudo secundario a un déficit de tiamina, definido por la tríada característica de confusión, oftalmoparesia y ataxia, aunque se han descrito presentaciones raras que retrasan el diagnóstico. El síndrome de Miller Fisher se caracteriza por la tríada de oftalmoparesia, ataxia y arreflexia,y se considera una variante del síndrome de Guillain-Barré; su diagnóstico diferencial incluye la encefalopatía de Wernicke. Caso clínico. Mujer de 75 años con trastorno digestivo crónico, que desarrolla un cuadro agudo de oftalmoplejía internuclearbilateral, ataxia y arreflexia, con disociación proteinocitológica en el líquido cefalorraquídeo, por lo que se propuso el diagnóstico inicial de síndrome de Miller Fisher. Los estudios neurofisiológicos fueron normales, los anticuerpos anti-GQ1b,negativos, y la resonancia magnética cerebral sugirió una encefalopatía de Wernicke; la respuesta a la tiamina fue espectacular. Conclusiones. Las similitudes en la distribución lesional de ambas entidades, en la semiología y los resultados analíticos, así como la influencia de ciertos factores de confusión (hiponatremia, edad avanzada), conformaron un síndrome típico que favoreció un diagnóstico de sospecha etiológico erróneo, que pudo modificarse precozmente a la luz de ciertas pruebas diagnósticasy después de la respuesta terapéutica. Además de tratarse de una presentación atípica para una encefalopatía de Wernicke, este caso recuerda que la conjunción del diagnóstico sindrómico con el etiológico pasa por realizar un correcto diagnóstico diferencial apoyándonos en detalles de la anamnesis, en las pruebas complementarias necesarias y en el seguimientoevolutivo de los pacientes, incluso cuando nos encontramos ante síndromes típicos habitualmente relacionados con una etiopatogenia predominante

Wernicke’s encephalopathy is an acute neuropsychiatric syndrome resulting from a thiamine deficit,which is defined by the characteristic triad of confusion, ophthalmoparesis and ataxia, although rare presentations have been reported that delay its diagnosis. Miller Fisher syndrome is characterised by the triad ophthalmoparesis, ataxia and areflexiaand is considered to be a variant of Guillain-Barré syndrome; its differential diagnosis includes Wernicke’s encephalopathy. Case report. A 75-year-old female with chronic digestive disorders, who developed an acute picture of bilateral internuclear ophthalmoplegia, ataxia and areflexia, with proteinocytologic dissociation in cerebrospinal fluid; accordingly, an initial diagnosis of Miller Fisher syndrome was proposed. Results of the neurophysiological studies were normal; anti-GQ1b antibodies were negative; and magnetic resonance imaging of the brain suggested Wernicke’s encephalopathy. The response to thiamine was spectacular. Conclusions. The similarities in the distribution of the lesions of the two conditions, in the signs and symptoms and the lab findings, as well as the influence of certain misleading factors (hyponatremia, advanced age), wentto make up a typical syndrome that favoured a wrong presumptive aetiological diagnosis. This was corrected at an early stage, however, in light of the results of certain diagnostic tests and after observing the therapeutic response. In addition to being anatypical presentation for Wernicke’s encephalopathy, this case highlights the fact that for there to be an agreement between the syndromic and aetiological diagnoses it is necessary to carry out a correct differential diagnosis based on details from thepatient’s history, on appropriate complementary tests and on the follow-up study of how the patients progress, even when we come across typical syndromes that are usually related to a predominant aetiopathogenesis

Réplica. Ausencias y ‘pseudoausencias’ frontales / Frontal absences and ‘pseudo-absences’. Reply

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Ausencias frontales: presentación de un caso / Frontal absences: a case report

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Esclerosis lateral amiotrófica y anticuerpos anti-CV2. ¿Asociación paraneoplásica? / Amyotrophic lateral sclerosis and anti-CV2 antibodies. Paraneoplastic association?

Introducción. La esclerosis lateral amiotrófica (ELA) es una forma de enfermedad de motoneurona que afecta primariamente a las motoneuronas superior e inferior. Es de etiología desconocida, curso progresivo y a menudo fatal. Muy ocasionalmente se ha descrito su aparición como síndrome paraneoplásico (SPN). Determinados patrones clínicos de enfermedad de motoneurona sugieren esta asociación. Los anti-CV2 son un tipo de anticuerpo onconeuronal, asociado invariablemente a tumor, y descritos en distintos SPN como encefalomielitis paraneoplásica, degeneración cerebelosa y neuropatía periférica.Caso clínico. Varón de 29 años con criterios de ELA probable. Por su debut atípico (edad del paciente) se solicitó anticuerpos onconeuronales, detectándose anti-CV2+. Tras 3 años de seguimiento y búsqueda tumoral exhaustiva, con progresión de la enfermedad, no hay evidencia en la actualidad de cáncer asociado.Discusión. El estudio de la enfermedad de motoneurona/ ELA como síndrome paraneoplásico, por su rareza, ha sido motivo de revisiones al objeto de verificar dicha relación. Ante una ELA debemos descartar asociación a tumor cuando se trate de una presentación precoz (< 30 años) o tardía (>70 años), cuando asocie otros síntomas/signos neurológicos (síntomas sensitivos, ataxia, etc.), ante la presencia de anticuerpos anti-Hu u otros y/o ante la presencia de paraproteinemia y/o pleocitosis-hiperproteinorraquia en LCR. Presentamos un caso de ELA probable con anti-CV2+, sin evidencia de cáncer subyacente. Tras una búsqueda bibliográfica extensa no hemos encontrado descrita esta asociación. Tampoco tenemos conocimiento de la existencia de anticuerpos anti-CV2 fuera del contexto tumoral, por lo que pensamos que nuestro paciente, probablemente, presente una neoplasia oculta

Introduction. Amyotrophic lateral sclerosis (ALS) is a form of motor neuron disease that primarily affects upper and lower motor neurons. Its etiology is unknown, it has a progressive course and is often fatal. Very rarely, its appearance as paraneoplastic syndrome (PNS) has been described. Certain clinical patterns of motor neuron disease suggest this association. The anti-CV2 are a type of onconeuronal antibody, invariably associated to tumor and described in different PNSs as paraneoplastic encephalomyelitis, cerebellar degeneration and peripheral neuropathy.Clinical case. 29 years old male with criteria of probably ALS. Due to his atypical onset (patient's age), onconeuronal antibodies were requested, detecting anti- CV2+. After three years of follow-up and exhaustive search for tumors, with progression of the disease, there is currently no evidence of associated cancer.Discussion. The study of the motor neuron/ALS disease as paraneoplastic syndrome, due to its rareness, has led to reviews in order to verify this relationship. When ALS exists, we should rule out association to tumor when the presentation is early (< 30 years) or late (> 70 years), when it is associated to other neurological symptoms/signs (sensory symptoms, ataxia, etc.), when anti-Hu antibodies or others are present and/or when there is paraproteinemia and/or pleocytosis-high protein levels in cerebral spinal fluid. We present a case of probable ALS with anti-CV2+, with no evidence of underlying cancer. After an extensive bibliographic search, we have found no description of this association. We also have no knowledge of the existence of anti-CV2 antibodies outside of the tumor context, so that we believe that our patient probably has an occult neoplasm

[Amyotrophic lateral sclerosis and anti-CV2 antibodies. Paraneoplastic association?]. / Esclerosis lateral amiotrófica y anticuerpos anti-CV2. ¿Asociación paraneoplásica?

INTRODUCTION: Amyotrophic lateral sclerosis (ALS) is a form of motor neuron disease that primarily affects upper and lower motor neurons. Its etiology is unknown, it has a progressive course and is often fatal. Very rarely, its appearance as paraneoplastic syndrome (PNS) has been described. Certain clinical patterns of motor neuron disease suggest this association. The anti-CV2 are a type of onconeuronal antibody, invariably associated to tumor and described in different PNSs as paraneoplastic encephalomyelitis, cerebellar degeneration and peripheral neuropathy. CLINICAL CASE: 29 years old male with criteria of probably ALS. Due to his atypical onset (patient's age), onconeuronal antibodies were requested, detecting anti- CV2+. After three years of follow-up and exhaustive search for tumors, with progression of the disease, there is currently no evidence of associated cancer. DISCUSSION: The study of the motor neuron/ALS disease as paraneoplastic syndrome, due to its rareness, has led to reviews in order to verify this relationship. When ALS exists, we should rule out association to tumor when the presentation is early (< 30 years) or late (> 70 years), when it is associated to other neurological symptoms/signs (sensory symptoms, ataxia, etc.), when anti-Hu antibodies or others are present and/or when there is paraproteinemia and/or pleocytosis-high protein levels in cerebral spinal fluid. We present a case of probable ALS with anti-CV2+, with no evidence of underlying cancer. After an extensive bibliographic search, we have found no description of this association. We also have no knowledge of the existence of anti-CV2 antibodies outside of the tumor context, so that we believe that our patient probably has an occult neoplasm.

Leucoencefalopatía posterior reversible: ¿debe ser la localización un factor definitorio? / Reversible posterior leukoencephalopathy: should the area involved be in the name of the syndrome?

Introducción. La leucoencefalopatía posterior reversible constituye un síndrome clinicorradiológico caracterizado por cefalea, vómitos y signos de focalidad neurológica coincidentes con lesiones en sustancia blanca de predominio posterior y generalmente reversible. Caso clínico. Presentamos un paciente pluripatológico que presentó un cuadro compatible con dicho síndrome. Se inició con crisis comiciales generalizadas durante un ingreso hospitalario. Entre sus antecedentes figuraban varios de los factores que hoy día se consideran involucrados en la etiopatogenia del mismo. Destacan la insuficiencia renal, la hipertensión arterial y los inmunosupresores. En la resonancia magnética aparecían lesiones compatibles pero que afectaban a varios territorios vasculares. Se confirmó la sospecha diagnóstica tras la resolución clínica y radiológica del proceso, sin que el paciente volviera a presentar nuevas crisis tras la normalización de la neuroimagen. Conclusiones. Planteamos una revisión de la etiopatogenia. Cuestionamos la idoneidad de la denominación del síndrome dada la extensión de las lesiones en la neuroimagen

Introduction. Reversible posterior leukoencephalopathy (RPL) is a syndrome of headache, vomiting and focal neurologic deficits with reversible lesions in posterior areas of the brain at the same time. Case report. We describe the case of a patient with a background of multiple diseases presenting a syndrome compatible with reversible posterior leukoencephalopathy. He presented seizures during a hospital stay. Among his background, he had several factors that are presently considered to be involved in its etiopathogeny. The most important ones are renal failure, hypertension and immunosuppressive agents. Magnetic resonance (MR) showed radiological typical changes of RPL syndrome but not only affecting the posterior regions of the brain. We confirmed the diagnostic suspicion after complete clinical and radiological recovery of the condition, and the patient did not have new episodes after the normalization of the neuroimage. Conclusions. We made a review of the etiopathogeny. We question the adequacy of the name of the syndrome, given the extension of the lesions in the neuroimage