EPICON consensus: recommendations for proper management of switching to eslicarbazepine acetate in epilepsy. / Consenso Delphi EPICON: recomendaciones sobre el manejo adecuado del cambio a acetato de eslicarbazepina en epilepsia.

INTRODUCTION: The objective of the EPICON Project is to develop a set of recommendations on how to adequately switch from carbamazepine (CBZ) and oxcarbazepine (OXC) to eslicarbazepine acetate (ESL) in some patients with epilepsy. METHODS: A steering committee drafted a questionnaire of 56 questions regarding the transition from CBZ or OXC to ESL in clinical practice (methodology and change situation). The questionnaire was then distributed to 54 epilepsy experts in 2 rounds using the Delphi method. An agreement/disagreement consensus was defined when a median ≥ 7 points or ≤ 3 was achieved, respectively, and a relative interquartile range ≤ 0.40. We analysed the results obtained to reach our conclusions. RESULTS: Our main recommendations were the following: switching from CBZ to ESL must be carried out over a period of 1 to 3 weeks with a CBZ:ESL dose ratio of 1:1.3 and is recommended for patients who frequently forget to take their medication, those who work rotating shifts, polymedicated patients, subjects with cognitive problems, severe osteoporosis-osteopaenia, dyslipidaemia, or liver disease other than acute liver failure, as well as for men with erectile dysfunction caused by CBZ. The transition from OXC to ESL can take place overnight with an OXC:ESL dose ratio of 1:1 and it is recommended for patients who frequently forget to take their medication, those who work rotating shifts, polymedicated patients, or those with cognitive problems. The transition was not recommended for patients with prior rash due to CBZ or OXC use. CONCLUSION: The EPICON Project offers a set of recommendations about the clinical management of switching from CBZ or OXC to ESL, using the Delphi method.

[Apolipoprotein E4 in dementia with Lewy bodies]. / Apolipoproteína e4 en la demencia con cuerpos de Lewy.

INTRODUCTION: There is a strong association between the e4 allele of apolipoprotein E (APOE) and Alzheimer's disease (AD). This converts this allele into a risk factor for the development of AD. The association between APOE4 and dementia with Lewy bodies (DLB) is under discussion. In DLB, the presence of APOE4 has been related with a greater amount of senile plaques and neurofibrillary tangles. METHOD: This is a case-control study in which the APOE genotype was determined using the modified PCR technique of Wenham in 306 patients with diagnosis of probably AD, NINCDS-ADRDA criteria, 58 cases of probably DLB, McKeith et al. consensus criteria (1996), all of them with SPECT with pathological 123I-FP-CIT and 80 normal controls (NC) having similar age and gender distribution. RESULTS: The frequency of alleles was: DLB group epsilon4: 16%; epsilon3: 75%; epsilon2: 9%; AD: epsilon4: 32%; epsilon3: 67%; epsilon2: 1%; and in the normal control group: epsilon4: 12%; epsilon3: 83%; epsilon2: 5%. The percentage of alleles in both genders was similar in the three groups. CONCLUSIONS: APOE4 percentage in DLB group (16%) was lower than in AD group (32%), and similar to the control group (12%). Considering that the presence of morphopathological Alzheimer type alterations in DBL, essentially neurofibrillary tangles, is inversely correlated with the presence of Parkinsonian signs, this group may represent pure forms of the disease, although the lack of neuropathological demonstration does not make it possible to confirm this hypothesis.

[Infiltration of the meninges or brain by a non-Hodgkin's lymphoma, diagnosed by flow cytometry in cerebrospinal fluid]. / Infiltracion meningea y cerebral por un linfoma no Hodgkin, diagnosticada por citometria de flujo en liquido cefalorraquideo.

INTRODUCTION: Two forms of growth are reported in the neuroradiology of cerebral lymphomas: mass, single or multiple lesions, with homogeneous contrast enhancement, and diffuse infiltration. Flow cytometry enables us to diagnose non-Hodgkin's lymphoma, when clonality of B cells is detected. It is usually employed with peripheral blood or bone marrow samples but can be used with cerebrospinal fluid (CSF). CASE REPORT: We report the case of a 68-year-old female, who was admitted to hospital because of rapidly progressive onset of confusion and right-side hemiparesis that developed in a matter of days. Magnetic resonance imaging (MRI) of the head showed a diffuse infiltrative lesion, without contrast enhancement, which covered the left basal nuclei, the left frontal white matter, the genu of the corpus callosum and the right frontal white matter. The CSF showed slight pleocytosis (20 cells/mL) and a notable degree of hypoglycorrhachia (10 mg/dL). The cytological examination only revealed lymphocytes, with no data indicating atypicality. The flow cytometry assay detected large mononuclear B cells, with the CD19 + CD20 + CD10-lambda phenotype, which is characteristic of diffuse non-Hodgkin's lymphoma of large B cells. The clinical course ran quickly towards a fatal outcome; it progressed to left-side hemiplegia and coma, and the patient died two weeks after admission to hospital. CONCLUSIONS: In cases of cerebral lymphoma, especially when the neuroradiological pattern displays diffuse infiltration and there are anomalies involving CSF, the flow cytometry in CSF can be diagnostic, thus avoiding the need for other invasive brain procedures to deal with lesions that are usually located deep inside the brain at badly defined sites.

Infiltración meníngea y cerebral por un linfoma no Hodgkin diagnosticada por citometría de flujo en el líquido cefalorraquídeo / Infiltrationof the meninges or brain by a non-hodgkin lymphoma, diagnosed by flow cytometry in cerebrospinal fluid

Introduction. Two forms of growth are reported in the neuroradiology of cerebral lymphomas: mass, single or multiple lesions, with homogeneous contrast enhancement, and diffuse infiltration. Flow cytometry enables us to diagnose non-Hodgkin’s lymphoma, when clonality of B cells is detected. It is usually employed with peripheral blood or bone marrow samples but can be used with cerebrospinal fluid (CSF). Case report. We report the case of a 68-year-old female, who was admitted to hospital because of rapidly progressive onset of confusion and right-side hemiparesis that developed in a matter of days. Magnetic resonance imaging (MRI) of the head showed a diffuse infiltrative lesion, without contrast enhancement, which covered the left basal nuclei, the left frontal white matter, the genu of the corpus callosum and the right frontal white matter. The CSF showed slight pleocytosis (20 cells/dL) and a notable degree of hypoglycorrhachia (10 mg/dL). The cytological examination only revealed lymphocytes, with no data indicating atypicality. The flow cytometry assay detected large mononuclear B cells, with the CD19 + CD20 + CD10-lambda phenotype, which is characteristic of diffuse non-Hodgkin’s lymphoma of large B cells. The clinical course ran quickly towards a fatal outcome; it progressed to left-side hemiplegia and coma, and the patient died two weeks after admission to hospital. Conclusions. In cases of cerebral lymphoma, especially when the euroradiological pattern displays diffuse infiltration and there are anomalies involving CSF, the flow cytometry in CSF can be diagnostic, thus avoiding the need for other invasive brain procedures to deal with lesions that are usually located deep inside the brain at badly defined sites (AU)

Introducción. En la neurorradiología de los linfomas cerebrales se describen dos formas de crecimiento: lesión en masa, única o múltiple, con captación homogénea de contraste, e infiltración difusa. La citometría de flujo permite diagnosticar un linfoma no Hodgkin B cuando se detecta clonalidad B; habitualmente, esta técnica se utiliza sobre muestras de sangre periférica o de médula ósea, pero puede usarse en el líquido cefalorraquídeo (LCR). Caso clínico. Mujer de 68 años que ingresó por confusión y hemiparesia derecha de instauración rápidamente progresiva en días. La resonancia magnética (RM) de cráneo mostró una lesión infiltrativa difusa, sin captación de contraste, que abarcaba los núcleos basales izquierdos, la sustancia blanca frontal izquierda, la rodilla del cuerpo calloso y la sustancia blanca frontal derecha. El LCR mostró una ligera pleocitosis (20 células/L) y una marcada hipoglucorraquia (10 mg/dL). La citología sólo objetivó linfocitos, sin datos de atipicidad. La citometría de flujo detectó células grandes mononucleares B, con fenotipo CD19 CD20 CD10-, propio de un linfoma no Hodgkin difuso de células grandes B. El curso clínico fue rápidamente fatal; progresó a hemiplejía izquierda y coma, y la paciente falleció a las dos semanas del ingreso. Conclusiones. En casos de linfoma cerebral, particular-mente cuando el patrón neurorradiológico es de infiltración difusa y existen anomalías licuorales, la citometría de flujo en el LCR puede ser diagnóstica y evitar otros procedimientos invasivos cerebrales sobre lesiones que habitualmente tienen una localización profunda y mal definida (AU)

[Sequential changes in magnetic resonance in a limbic status epilepticus]. / Cambios secuenciales en la resonancia magnética en estado epiléptico límbico.

INTRODUCTION: Transient alterations have been described in neuroimaging (MRI) studies of the non-convulsive focal status (NCFS). We report a case of NCFS together with the MRI findings. CASE REPORT: We describe the case of a 63-year-old female who had a sister and two female cousins with epilepsy; the patient was admitted to hospital after being in state of confusion for 72 hours. Two similar bouts of delirium were reported as having occurred in the 2 preceding years, both of which lasted only a few minutes, and at that time a cardiology study, EEG and MRI scans of the head were performed with normal results. The EEG was compatible with left temporal status and MRI, and presented hyperintensity in the left temporal lobe in T2 and Flair, with no mass effect, with gadolinium uptake in leptomeninges and cortex. CSF was acellular and there were high protein levels in cerebrospinal fluid with a value of 1 g/dL. The patient's situation continued for 10 days, and did not respond initially to antiepileptic treatment. Temporal NCFS was diagnosed, with a cryptogenic rather than idiopathic aetiology. A preliminary MRI scan was normal and another scan performed 10 days after resolution showed a clear regression of the lesion. We related these findings to vasogenic and cytotoxic oedema secondary to the status. An MRI scan carried out at 3 months was normal. CONCLUSIONS: This case lends support to reports, in relation to the appearance of NCFS, of MR images compatible with oedema secondary to rupture of the blood-brain barrier. We base it on sequential MRI studies and on high protein levels in CSF.

Cambios secuenciales en la resonancia magnética en estado epiléptico límbico / Sequential changes in magnetic resonance in a limbic status epilepticus

Introducción. En el estado no convulsivo focal (SNCF) se han descrito alteraciones transitorias en estudios de neuroimagen (RM). Presentamos un caso de SNCF y los hallazgos en RM. Caso clínico. Se trata de una mujer de 63 años con antecedentes de una hermana y dos primas con epilepsia que ingresa por presentar, desde 72 horas antes, un cuadro confusional. Se referían desde dos años antes dos episodios similares, de pocos minutos de duración por los que se realizó un estudio cardiológico, así como EEG y RM de cráneo, que fueron normales. El EEG fue compatible con estado temporal izquierdo y en la RM presentó en T 2 y FLAIR una hiperintensidad en el lóbulo temporal izquierdo, sin efecto de masa, con captación de gadolinio en las leptomeninges y el córtex. El LCR fue acelular, con hiperproteinorraquia de 1 g/dL. En su evolución persistió la situación durante 10 días, y no respondió inicialmente al tratamiento antiepiléptico. Se diagnosticó de SNCF temporal, de etiología criptogénica frente a idiopática. Los hallazgos en la RM, con una previa normal y otra diez días después de la resolución, con clara regresión de la lesión, los relacionamos con edema vasogénico y citotóxico secundario al estado. Una RM a los tres meses fue normal. Conclusión. Este caso apoya lo descrito con relación a la aparición en el SNCF, de imágenes en RM compatibles con edema secundario a rotura de barrera hematoencefálica Lo funda-mentamos en estudios RM secuenciales y en la hiperproteinorraquia en LCR

Introduction. Transient alterations have been described in neuroimaging (MRI) studies of the non-convulsive focal status (NCFS). We report a case of NCFS together with the MRI findings. Case report. We describe the case of a 63-year-old female who had a sister and two female cousins with epilepsy; the patient was admitted to hospital after being in state of confusion for 72 hours. Two similar bouts of delirium were reported as having occurred in the 2 preceding years, both of which lasted only a few minutes, and at that time a cardiology study, EEG and MRI scans of the head were performed with normal results. The EEG was compatible with left temporal status and MRI, and presented hyperintensity in the left temporal lobe in T 2 and FLAIR, with no mass effect, with gadolinium uptake in leptomeninges and cortex. CSF was acellular and there were high protein levels in cerebrospinal fluid with a value of 1 g/dL. The patient’s situation continued for 10 days, and did not respond initially to antiepileptic treatment. Temporal NCFS was diagnosed, with a cryptogenic rather than idiopathic aetiology. A preliminary MRI scan was normal and another scan performed 10 days after resolution showed a clear regression of the lesion. We related these findings to vasogenic and cytotoxic oedema secondary to the status. An MRI scan carried out at 3 months was normal. Conclusions. This case lends support to reports, in relation to the appearance of NCFS, of MR images compatible with oedema secondary to rupture of the blood-brain barrier. We base it on sequential MRI studies and on high protein levels in CSF

[Neuropathy of common sciatic nerve secondary to compartment syndrome as a complication after bariatric surgery]. / Neuropatía de nervio ciático común secundaria a síndrome compartimental como complicación de cirugía bariátrica.

INTRODUCTION: With regards to the use of bariatric surgery on very obese patients and prolonged interventions, isolated cases of a compartment syndrome by compression and secondary rhabdomyolysis have been described. We describe a case which presented with a compartment syndrome, rhabdomyolysis and neuropathy of the common sciatic nerve. CLINICAL CASE: A 39 year old male with morbid obesity and high blood pressure, who after being subjected to 5 hours long bariatric surgical intervention, presented with intense pain, muscle binding and paresis in the musculature dependent on left common sciatic nerve. A creatinkinase (CK) level of 78,000 IU and volume increase of the left gluteal compartment were noted. On serial computarized tomography scans this increased leading to gluteal atrophy and ischiotibials. Evolution was negative without functional recovery and poor pain control. The patient presented with a compartment syndrome and secondary rhabdomyolysis triggered by the pressure on the gluteal zone on the operating table. The prolonged duration of the surgery, obesity and microvascular affectation due to hypertension, could be factors implicated in the development of the syndrome. The affectation of the sciatic nerve, not described as a complication of this type of surgery, is explained by pressure exercised on the compartment block on the sciatic nerve, with secondary ischaemia. CONCLUSIONS: Early determination of CK, carrying out postural changes during surgery and early post-operative neuromuscular examination, looking for signs of compartment syndrome, should be systematically carried out after bariatric surgery. The early carrying out of a decompressive fasciotomy considering a compartment syndrome could reduce or avoid the neurological complications.

Spanish family with myalgia and cramps syndrome.

A Spanish family is reported with dystrophinopathy of myalgia and cramps syndrome type. There were five affected males and three females, and also six asymptomatic carriers. Muscle biopsy showed a dystrophic pattern, but immunohistochemistry carried out with three anti-dystrophin antibodies was normal. Dystrophin analysis by western blot revealed a dystrophin of reduced quantity and molecular weight. DNA analysis showed a deletion of the dystrophin gene involving exons 45-52. The natural history of this disorder and the large intrafamilial clinical variability are discussed.

[Multiple sclerosis and neurocysticercosis: a diagnostic dilemma]. / Esclerosis múltiple y neurocisticercosis. dilema diagnóstico.

INTRODUCTION: Both Multiple Sclerosis (MS) and Neurocysticercosis (NC) are two entities in which clinical manifestations, neuroimaging findings and immunoserologic assays are neither pathognomonic nor specific requiring for their diagnosis an accurate examination of the clinical history of patients and an adequate follow up. CASE REPORTS: Two patients who consulted non neurologists about focal neurological symptoms. Neuroimaging findings revealed multiple lesions, some of them contrast enhanced. A diagnosis of neurocysticercosis was established, supported in one of the patients by positive serologic assays for cysticerci and antihelmintic therapy began to be administered. Observing the clinical evolution of the patients, monitoring their clinical history and considering the diagnostic criteria proposed by McDonald for MS and by Del Brutto for NC the patients were finally diagnosed of MS. CONCLUSION: The first step to reach a diagnosis of MS is to consider such a possibility. The diagnosis is mainly based on clinical grounds and it is necessary to prove that symptoms disseminate or that alterations occur in neuroimaging findings both in time and space. It is of the utmost importance to establish a differential diagnosis with other conditions presenting with similar clinical manifestations, neuroimaging findings and cerebrospinal fluid tests results. Even with the latest criteria proposed for the diagnosis of MS and NC we may have doubts making it fundamental to cautiously interpret the clinical manifestations and tests results.

[The onset of an anaplastic ganglioglioma during the post-natal period with signs of toxemia of pregnancy]. / Inicio de un ganglioglioma anaplásico durante un puerperio con signos de toxemia gravídica.

INTRODUCTION: Gangliogliomas are infrequent neuronoglial tumours which present in youngsters and are usually located in the temporal lobe. They usually appear with epileptic seizures and prognosis after surgical excision is usually good. The anaplastic forms are even less frequent and prognosis is poorer. The onset of epileptic seizures during the early post-natal period means that the clinician has to resort to a broad differential diagnosis. CASE REPORT: Hours after a preterm birth, at the 32nd week of gestation, a 35-year-old primipara began to suffer seizures and also presented arterial hypertension, proteinuria and a low platelet count. A cranial computerized tomography scan was carried out where a left frontal hypodense lesion was observed. Transcranial echo Doppler scan showed medium speeds and suggested eclampsia. The seizures, however, recurred during the days that followed and a magnetic resonance scan of the head revealed a lesion with nodular contrast enhancement, which was excised, and finally an anatomopathological diagnosis of an anaplastic ganglioglioma was reached. DISCUSSION: The toxemia of pregnancy, which gave rise to a vasogenic cerebral edema, accelerated the clinical onset of a brain tumour during the post-natal period. A ganglioglioma, although infrequent, is always a possibility to be borne in mind in young patients.