Malformations of cortical development and epilepsy in adult patients.

OBJECTIVE: To describe clinical features of epilepsy secondary to Malformation of Cortical Development (MCD) in a series of adult patients. MATERIALS AND METHODS: We searched our database for all cases with confirmed epilepsy and MCD and included in the study only those with complete data. Mean age, sex, age at seizure onset (ASO), seizure types, abnormal neurological exam (ANE), mental retardation, family history, gestational or perinatal insults (G-PI), interictal EEG and response to treatment were analyzed. Cases were classified into the 3 main groups (G) according to the Barkovich classification (BC) and then compared: (G1) "malformations due to abnormal cell proliferation", (G2) "malformations due to abnormal migration" and (G3) "malformations due to abnormal cortical organization". RESULTS: We identified 152 (5.06%) patients with MCD from a total of 3000 with epilepsy. In total, 138 patients with complete medical data were included in this study. The mean age of patients was 36.2 years, 52.2% were female, the mean ASO was 12.3 years, 5.1% of cases had a positive family history and 21% had G-PI. An ANE was observed in 21% and mental retardation in 31.9%. Most of the patients (84.8%) had refractory epilepsy. The distribution of cases according to the BC was: 51.4% in G1, 28.9% in G2 and 19.6% in G3. Comparing the 3 groups, we found that an ANE was statistically more frequent in G3 and was present in 70.4% of cases. CONCLUSION: Our series of adult patients with epilepsy and MCD suggests that MCD are identified as commonly in a developing country as in previous "first world" series. Neurological deficits were more common in the subgroup of patients with polymicrogyria and schizencephaly (BC Group 3).

[Contribution of diffusion tensor magnetic resonance imaging to the diagnosis of focal cortical dysplasias]. / Contribucion de las imagenes de resonancia magnetica por tensor de difusion al diagnostico de displasias corticales focales.

INTRODUCTION: Focal cortical dysplasias (FCD) are cortical malformations and, although they display typical characteristics in conventional magnetic resonance imaging (MRI), the precise determination of the epileptogenic zone remains a controversial issue. The less favourable progress during the post-operative period with respect to other symptomatic epilepsies could be explained by the existence of epileptogenic areas that do not show up in conventional MRI. Diffusion tensor imaging (DTI) is sensitive to subtle microstructural abnormalities, and fractional anisotropy, which is an indirect indicator, shows areas with reductions in the underlying white matter that go beyond the alterations detected with conventional MRI in isolated cases in previous works. AIM: In this study we analyse the characteristics of fractional anisotropy in a series of patients with FCD in order to evaluate the contribution made to diagnosis by MRI by DTI. SUBJECTS AND METHODS: Twenty-one controls and eleven patients with FCD that was visible in MRI were scanned, and clinical and imaging variables were both recorded. A visual analysis of the fractional anisotropy maps was conducted to search for asymmetries between hemispheres and biases in the clinical or structural MRI data. RESULTS: Two females and nine males, aged 30 ± 9.7 years took part in the study; time to progression of epilepsy: 22 ± 9.3 years; average frequency of the seizures: 3/month (range: 0.16-8/month). All of them showed inter-hemispheric asymmetries, which went beyond the structural limits of FCD in the case of 10 of the patients (90%). None of the controls displayed asymmetries in the fractional anisotropy. No significant relation was found with the variables that were compared. CONCLUSIONS: Further studies need to be conducted with larger numbers of patients in order to evaluate the usefulness of DTI in defining the location and extension of the epileptogenic zone in this population.

Análisis de los trastornos psicóticos en pacientes con epilepsia parcial refractaria, diagnóstico psiquiátrico y características clínicas / Analysis of psychotic disorders in patients with refractory partial epilepsy, psychiatric diagnoses and clinical aspects

Introducción. La asociación entre trastornos psicóticos y epilepsia ha sido motivo de controversias. Actualmente se describen en la literatura diferentes subtipos de trastornos psicóticos en los pacientes con epilepsia de acuerdo con la relación temporal con las crisis: las psicosis postictales (PPI), interictales (PII) y bimodales (PB).Objetivos. Determinar las características clínicas de pacientes con epilepsia parcial refractaria y psicosis y compararlos hallazgos con un grupo control de pacientes con epilepsia parcial refractaria sin psicosis. Métodos. Se estudiaron 57 pacientes con epilepsia parcial refractaria y trastornos psicóticos (GP) y 56 pacientes con epilepsia parcial refractaria sin psicosis (GnP) de acuerdo con los criterios del DSM-IV. En todos los pacientes se realizó una evaluación neurológica completa, estudios neurofisiológicos, neuroimágenes y evaluaciones psiquiátricas DSM-IV y SCID-I. Las variables clínicas, demográficas y psiquiátricas fueron comparadas entre los pacientes GP y GnP.Resultados. En el GP 15 pacientes (26%) cumplían criterios para PPI, 29 pacientes (51 %) para PII y 13 pacientes(23 %) para PB. Encontramos una duración más prolongada de la epilepsia y una mayor incidencia de esclerosis hipocámpica bilateral en los pacientes GP. Los pacientes de GP presentaron un mayor tiempo de evolución de la epilepsia y una mayor incidencia de esclerosis hipocámpica bilateral (p < 0,05).No se observaron diferencias entre los distintos subtipos de psicosis. Conclusiones. El mayor tiempo de evolución de las crisis epilépticas y la presencia de una esclerosis hipocámpica bilateral podrían incrementar el riesgo de desarrollar psicosis en pacientes con epilepsia parcial refractaria (AU)

Introduction. The association between psychotic disorders and epilepsy has been controversial. Different subtypes of psychotic disorders in epilepsy patients have been described according to temporal relationship with seizures-postictal (PIP), interictal (IIP) and bimodal (BP) psychoses are described in literature. Objectives. Determine clinical characteristics of patients with refractory partial epilepsy and psychoses and compare the results with a control group of patients with refractory partial epilepsy without psychoses. Methods. A total of 57 patients with refractory partial epilepsy and psychotic disorders (psychotic group [PG]) and 56 patients with refractory partial epilepsy and without psychoses (control group, CG) were evaluated according to DSM-IV criteria and SCID-I. All patients underwent complete neurological, neuroimaging, neuropsychological, and psychiatric assessment. Clinical, demographic and neuroimaging data were compared between patients in CG and PG. Results. In PG 15 patients (26%) had criteria for PIP,29 patients (51 %) for IIP and 13 patients (23 %) for BP. Epilepsy time duration and bilateral hippocampal sclerosis were significantly more frequent in patients with psichosis.PG patients had a longer evolution time of epilepsy and greater frequency of bilateral hippocampal sclerosis (p < 0.05). No differences were found between psychoses subtypes. Conclusions. Longer evolution of seizures and the presence of bilateral hippocampal sclerosis may increase propensity to develop psychoses in patients with refractory partial epilepsy (AU)

[Analysis of psychotic disorders in patients with refractory partial epilepsy, psychiatric diagnoses and clinical aspects]. / Análisis de los trastornos psicóticos en pacientes con epilepsia parcialrefractaria, diagnóstico psiquiátrico y características clínicas.

INTRODUCTION: The association between psychotic disorders and epilepsy has been controversial. Different subtypes of psychotic disorders in epilepsy patients have been described according to temporal relationship with seizures-postictal (PIP), interictal (IIP) and bimodal (BP) psychoses are described in literature. OBJECTIVES: Determine clinical characteristics of patients with refractory partial epilepsy and psychoses and compare the results with a control group of patients with refractory partial epilepsy without psychoses. METHODS: A total of 57 patients with refractory partial epilepsy and psychotic disorders (psychotic group [PG]) and 56 patients with refractory partial epilepsy and without psychoses (control group, CG) were evaluated according to DSM-IV criteria and SCID-I. All patients underwent complete neurological, neuroimaging, neuropsychological, and psychiatric assessment. Clinical, demographic and neuroimaging data were compared between patients in CG and PG. RESULTS: In PG 15 patients (26 %) had criteria for PIP, 29 patients (51%) for IIP and 13 patients (23%) for BP. Epilepsy time duration and bilateral hippocampal sclerosis were significantly more frequent in patients with psichosis. PG patients had a longer evolution time of epilepsy and greater frequency of bilateral hippocampal sclerosis (p < 0.05). No differences were found between psychoses subtypes. CONCLUSIONS: Longer evolution of seizures and the presence of bilateral hippocampal sclerosis may increase propensity to develop psychoses in patients with refractory partial epilepsy.

Clinical spectrum and difficulties in management of hypothalamic hamartoma in a developing country.

AIM: We describe the clinical features, treatment and prognosis in a series of patients with epilepsy secondary to hypothalamic hamarthomas (HH) in a developing country. MATERIALS AND METHODS: Eight patients with epilepsy and HH were included between 1997 and 2006. We analyzed gender, age, age at seizure onset (ASO), seizure types (ST), mental retardation (MR), precocious puberty (PP), electroencephalogram (EEG)-magnetic resonance imaging (MRI) features and response to treatment. RESULTS: Mean age 25.1 years, 2/6 female/male, none had PP, ASO 4.5 years. Complex partial seizure were the most frequent (100%), mean similar to those seen in temporal (62.5%) or frontal lobe epilepsy (37.5%). Exactly 87.5% developed gelastic seizures (GS). Half of the patients showed MR. Mild-to-severe MR was associated with the presence of multiple ST including atonic and complex partial seizures with frontal semiology. Interictal EEG was abnormal in 87.5% patients. Video EEG was performed in three cases with unspecific findings. HH were small and sessile in seven patients whereas large and pedunculated in one. All patients were refractory to medical treatment. In five, an additional procedure was performed without any significant improvement. CONCLUSION: These series show the heterogeneous spectrum of this entity and the difficulties in its treatment in a developing country.

Proximal stomach function in obesity with normal or abnormal oesophageal acid exposure.

There is an increased prevalence of gastro-oesophageal reflux and symptoms in obese patients. Information about the proximal stomach in obese patients with reflux is lacking. Gastric volume and compliance are similar between obese and lean subjects. To study the proximal stomach function and perception in obese patients with normal or abnormal oesophageal acid exposure, thirty-one obese patients, with normal or abnormal oesophageal acid exposure, underwent medical evaluation of oesophageal and gastrointestinal symptoms by a questionnaire and measurement of proximal stomach function and perception by an electronic barostat and a standardized questionnaire. Nineteen obese patients had abnormal oesophageal acid exposure. The percentage of total time with pH <4 is significantly related to the presence of hiatal hernia, the oesophageal intensity-frequency symptom score and gender, i.e. higher percentage in men. The perception cumulative score was significantly different between patients with normal and abnormal oesophageal acid exposure after adjusting for covariates (gender, body mass index, age, minimal distending pressure, gastric tone and gastric compliance). Gastric tone and compliance were significantly related to the perception cumulative score. In conclusion, patients with abnormal oesophageal acid exposure have increased gastric perception. A significant relation among gastric tone, gastric compliance and upper gastrointestinal sensations was shown.

[Reversible posterior leucoencephalopathy syndrome: a case report and review of its physiopathology based on neuroradiological findings]. / Leucoencefalopatia posterior reversible: descripcion de un caso y revision de la fisiopatologia basada en hallazgos neurorradiologicos.

INTRODUCTION: Reversible posterior leucoencephalopathy is a clinico radiological syndrome first described in the last decade. The physiopathological mechanism governing it is not very well known. The currently accepted hypotheses are the generation of a vasogenic edema caused by failure of the mechanism that allows self regulation of the cerebral blood flow, and the production of a cytotoxic edema due to ischemia. Both experimental studies carried out in rats and the use of magnetic resonance imaging (MRI) to evaluate the diffusion of cerebral water support the first hypothesis. We report the case of a patient with reversible posterior leucoencephalopathy syndrome that was complicated by areas of cerebral infarction and we review the physiopathological mechanisms involved, basing our analysis on the findings obtained with MRI. CASE REPORT: A female patient was admitted to our hospital because of bilateral transient amaurosis, headaches, vomiting and arterial hypertension. MRI brain scans showed bilateral cortico subcortical lesions in the occipital parietal regions, frontal and right cerebellar hemisphere convexity, with hyperintense signal in T2 and diffusion weighted imaging. Some occipital parietal regions present a diminished signal on the apparent diffusion coefficient map. The patient was treated with intravenous antihypertensive drugs and full recovery was accomplished within 10 days. A month later, according to MRI scans, there were still hyperintense areas in T2 on both occipital lobes, which were compatible with cerebral infarcts. DISCUSSION: The mechanism that is most frequently involved in this entity is probably vasogenic edema. In our case there were, at the same time, areas of cytotoxic edema that progressed to cerebral infarcts. MRI with sequences that evaluate the diffusion of cerebral water is useful in distinguishing between the two mechanisms.

Leucoencefalopatía posterior reversible: descripción de un caso y revisión de la fisiopatología basada en hallazgos neurorradiológicos / Reversible posterior leucoencephalopathy syndrome: a case report and review of its physiopathology based on neuroradiological findings

Introduction. Reversible posterior leucoencephalopathy is a clinico-radiological syndrome first described in the last decade. The physiopathological mechanism governing it is not very well known. The currently accepted hypotheses are the generation of a vasogenic edema caused by failure of the mechanism that allows self-regulation of the cerebral blood flow, and the production of a cytotoxic edema due to ischemia. Both experimental studies carried out in rats and the use of magnetic resonance imaging (MRI) to evaluate the diffusion of cerebral water support the first hypothesis. We report the case of a patient with reversible posterior leucoencephalopathy syndrome that was complicated by areas of cerebral infarction and we review the physiopathological mechanisms involved, basing our analysis on the findings obtained with MRI. Case report. A female patient was admitted to our hospital because of bilateral transient amaurosis, headaches, vomiting and arterial hypertension. MRI brain scans showed bilateral cortico-subcortical lesions in the occipital-parietal regions, frontal and right cerebellar hemisphere convexity, with hyperintense signal in T2 and diffusion-weighted imaging. Some occipital-parietal regions present a diminished signal on the apparent diffusion coefficient map. The patient was treated with intravenous antihypertensive drugs and full recovery was accomplished within 10 days. A month later, according to MRI scans, there were still hyperintense areas in T2 on both occipital lobes, which were compatible with cerebral infarcts. Conclusion. The mechanism that is most frequently involved in this entity is probably vasogenic edema. In our case there were, at the same time, areas of cytotoxic edema that progressed to cerebral infarcts. MRI with sequences that evaluate the diffusion of cerebral water is useful in distinguishing between the two mechanisms

Introducción. La leucoencefalopatía posterior reversible es un síndrome clinicorradiológico descrito en la última década. El mecanismo fisiopatológico responsable no se conoce muy bien. Las hipótesis aceptadas son: generación de edema vasogénico por pérdida del mecanismo autorregulatorio del flujo sanguíneo cerebral y producción de edema citotóxico por isquemia. Estudios experimentales en ratas y la utilización de imágenes de resonancia magnética (IRM) que evalúan la difusión del agua cerebral, sustentan la primer hipótesis. Describimos un caso de leucoencefalopatía posterior reversible complicada con áreas de infarto cerebral y realizamos una revisión sobre los mecanismos fisiopatológicos involucrados, sobre la base de los hallazgos obtenidos en IRM. Caso clí- nico. Una mujer se internó en nuestro hospital por amaurosis bilateral transitoria, cefaleas, vómitos e hipertensión arterial. Las IRM de cerebro presentaban lesiones corticosubcorticales bilaterales en las regiones parietooccipitales, convexidad frontal y hemisferio cerebeloso derecho, con señal hiperintensa en T2 e imágenes de difusión. Algunas regiones parietooccipitales presentaban señal disminuida en mapa del coeficiente aparente de difusión. La paciente se trató con antihipertensivos endovenosos y se recuperó ad integrum en los 10 días siguientes. Después de 1 mes, en IRM, persistían áreas hiperintensas en T2 sobre ambos lóbulos occipitales, compatibles con infarto cerebral. Conclusión. El mecanismo involucrado con mayor frecuencia en esta entidad es probablemente el edema vasogénico. En nuestro caso, coexisten áreas de edema citotóxico que evolucionan a infarto cerebral. Las IRM con secuencias que valoran la difusión del agua cerebral son útiles para diferenciar entre ambos mecanismos (AU)

Clinical-electroencephalogram patterns at seizure onset in patients with hippocampal sclerosis.

OBJECTIVE: The purpose of this study is to identify specific clinical-electroencephalogram (EEG) patterns at seizure onset in patients with hippocampal sclerosis (HS). METHODS: Sixty-six ictal video-EEG recordings corresponding to 26 patients with HS have been reviewed, focusing on the EEG features found during the first 30 ictal s. The EEG activity has been classified into the following groups: (A) according to spatial distribution: type 1: temporal electrodes on one side; type 2: temporal and adjacent frontal electrodes on one side; and type 3: non-lateralizing electrographic activity; and (B) according to morphology; subtype (a): regular 5-9 Hz rhythmic activity (RA); subtype (b): low-voltage rapid activity, followed by a 5-9 Hz RA; and subtype (c): irregular EEG sharp waves. We analyzed the clinical symptoms sequence and established the relationship with the ictal EEG patterns. RESULTS: Considering spatial distribution and morphology, the most frequent ictal EEG patterns were type 1 (57%), type 2 (37%), and subtype (a): 62%; subtype (b): 27%; and subtype (c): 11%. The sequence of clinical symptoms observed was: aura-->behavioral arrest-->oro-alimentary automatisms-->unilateral hand automatisms. All seizures with aura and including two or more symptoms of the clinical sequence (65%) were associated with a 1a, 1b, 2a or 2b EEG pattern. CONCLUSIONS: The identification of a specific clinical-EEG pattern provides a useful tool for the epileptogenic zone localization in non-invasive pre-surgical assessment of patients with hippocampal sclerosis. SIGNIFICANCE: The identification of a specific clinical-EEG pattern associated to neuroimaging findings and neuropsychological testing allows indicating surgery for the treatment of epilepsy in patients with hippocampal sclerosis, without performing any further complementary studies.

Clinical features and prognosis of nonepileptic seizures in a developing country.

PURPOSE: To determine the predictive value of clinical features and medical history in patients with nonepileptic seizures (NESs). METHODS: One hundred sixty-one consecutive ictal video-EEGs were reviewed, and 17 patients with 41 NESs identified. NES diagnosis was defined as paroxysmal behavioral changes suggestive of epileptic seizures recorded during video-EEC without any electrographic ictal activity. Clinical features, age, sex, coexisting epilepsy, associated psychiatric disorder, social and economic factors, delay in reaching the diagnosis of NES, previous treatment, and correlation with outcome on follow-up were examined. RESULTS: The study population included 70% female patients with a mean age of 33 years. Mean duration of NESs before diagnosis was 9 years. Forty-one percent had coexisting epilepsy. The most frequent NES clinical features were tonic-clonic mimicking movements and fear/anxiety/hyperventilation. The most common psychiatric diagnosis was conversion disorder and dependent and borderline personality disorder. Seventy-three percent of patients with pure NESs received antiepileptic drugs (AEDs), and 63.5% of this group received new AEDs. Fifty-nine percent of the patients received psychological/psychiatric therapy. At follow-up, 23.5% were free of NESs. CONCLUSIONS: All seizure-free patients had two good prognostic factors: having an independent lifestyle and the acceptance of the nonepileptic nature of the episodes. Video-EEG monitoring continues to be the diagnostic method to ensure accurate seizure classification. Establishing adequate health care programs to facilitate access to new technology in public hospitals as well as the implementation of continuous education programs for general practitioners and neurologists could eventually improve the diagnosis and treatment of patients with NESs.

[Magnetic resonance and clinical and electroencephalographical localization in focal epilepsy]. / Resonancia magnética y localización clínica y electroencefalográfica en la epilepsia focal.

Magnetic Resonance Imaging (MRI) is the method of choice to search for epileptogenic lesions. We correlated MRI findings with the epileptogenic zone (EZ) depicted by clinical and electroencephalographic (EEG) data. We studied 400 clinical records of patients who had been submitted to MRI studies and we analyzed, retrospectively, their ictal semiology, EEG characteristics and response to treatment. They were classified into 3 groups: A) temporal lobe epilepsy, B) frontal lobe epilepsy and C) parieto-occipital epilepsy. We included 155 patients: Group A) 68 cases (43.9%), 28 men (41.1%), mean age 32 +/- 11 years old, abnormal IMR in 44 (64.7%), refractory to treatment 48 (70.5%). Group B) 68 cases (43.9%), 38 men (55.8%), mean age 30 +/- 15 years old, abnormal IMR in 26 (38.2%), refractory to treatment 30 (44.1%). Group C) 19 cases (12.2%), 13 men (68.4%), mean age 27 +/- 11 years old, abnormal IMR in 11 (57.8%), refractory to treatment 12 (63.1%). Results showed that there were higher possibilities of detecting lesions which correlate with EZ in temporal than in frontal or parieto-occipital lobes epilepsy. The chances to find abnormalities on the MRI were 5 times higher in refractory patients than in those who were non-refractory.

Resonancia magnética y localización clínica y electroencefalográfica en la epilepsia focal. / [Magnetic resonance and clinical and electroencephalographical localization in focal epilepsy]

Magnetic Resonance Imaging (MRI) is the method of choice to search for epileptogenic lesions. We correlated MRI findings with the epileptogenic zone (EZ) depicted by clinical and electroencephalographic (EEG) data. We studied 400 clinical records of patients who had been submitted to MRI studies and we analyzed, retrospectively, their ictal semiology, EEG characteristics and response to treatment. They were classified into 3 groups: A) temporal lobe epilepsy, B) frontal lobe epilepsy and C) parieto-occipital epilepsy. We included 155 patients: Group A) 68 cases (43.9

), 28 men (41.1

), mean age 32 +/- 11 years old, abnormal IMR in 44 (64.7

), refractory to treatment 48 (70.5

). Group B) 68 cases (43.9

), 38 men (55.8

), mean age 30 +/- 15 years old, abnormal IMR in 26 (38.2

), refractory to treatment 30 (44.1

). Group C) 19 cases (12.2

), 13 men (68.4

), mean age 27 +/- 11 years old, abnormal IMR in 11 (57.8

), refractory to treatment 12 (63.1

). Results showed that there were higher possibilities of detecting lesions which correlate with EZ in temporal than in frontal or parieto-occipital lobes epilepsy. The chances to find abnormalities on the MRI were 5 times higher in refractory patients than in those who were non-refractory.

[Mesial temporal sclerosis syndrome in adult patients]. / Síndrome de la esclerosis mesial temporal en pacientes adultos.

Magnetic resonance imaging (MRI) has become an essential tool in the work-up of epilepsy. Since its appearance it has been possible to identify pathologies, such as hippocampal sclerosis (HS), that had previously only been detected by histopathological assays. The aim of this study was to analyze the clinical manifestations, EEG and the outcome of patients with HS as shown by MRI. We revised the clinical histories of 384 outpatients from the Epilepsy Center, Ramos Mejía Hospital, who had been studied by MRI. Thirty five of them (15.5%) had a diagnosis of HS, based on the structural changes observed on the images. Six patients were excluded because of incomplete clinical data. Therefore, we studied 29 patients including 15 men. The mean age was 32.7 +/- 10.2 years (range: 19-58). All of them had partial seizures. Ten subjects had had febrile convulsions (34.5%) in childhood. Neurological examination was normal in all subjects. Interictal EEG showed focal abnormalities that were coincident in their location with the MRI abnormalities in 16 patients (55.1%). Fourteen patients (48.3%) showed right side hippocampal lesions on MRI, thirteen on the left side (44.9%) and 2 bilateral HS (6.8%). Twenty-seven patients (93.1%) had intractable epilepsy. Anterior temporal lobectomy was performed in 3 subjects with good outcome. The identification of these patients who present certain clinical and MRI characteristics, provides an opportunity to define the mesial temporal sclerosis syndrome. This could benefit patients in their prognosis and for specific treatments.

Síndrome de la esclerosis mesial temporal en pacientes adultos. / [Mesial temporal sclerosis syndrome in adult patients]

Magnetic resonance imaging (MRI) has become an essential tool in the work-up of epilepsy. Since its appearance it has been possible to identify pathologies, such as hippocampal sclerosis (HS), that had previously only been detected by histopathological assays. The aim of this study was to analyze the clinical manifestations, EEG and the outcome of patients with HS as shown by MRI. We revised the clinical histories of 384 outpatients from the Epilepsy Center, Ramos Mejía Hospital, who had been studied by MRI. Thirty five of them (15.5

) had a diagnosis of HS, based on the structural changes observed on the images. Six patients were excluded because of incomplete clinical data. Therefore, we studied 29 patients including 15 men. The mean age was 32.7 +/- 10.2 years (range: 19-58). All of them had partial seizures. Ten subjects had had febrile convulsions (34.5

) in childhood. Neurological examination was normal in all subjects. Interictal EEG showed focal abnormalities that were coincident in their location with the MRI abnormalities in 16 patients (55.1

). Fourteen patients (48.3

) showed right side hippocampal lesions on MRI, thirteen on the left side (44.9

) and 2 bilateral HS (6.8

). Twenty-seven patients (93.1

) had intractable epilepsy. Anterior temporal lobectomy was performed in 3 subjects with good outcome. The identification of these patients who present certain clinical and MRI characteristics, provides an opportunity to define the mesial temporal sclerosis syndrome. This could benefit patients in their prognosis and for specific treatments.

Amygdala atrophy and seizure outcome after temporal lobe epilepsy surgery.

Surgical outcome in hippocampal atrophy (n = 44) and amygdalohippocampal atrophy (n = 14) were compared. Hippocampal atrophy had better seizure-free outcome than amygdalohippocampal atrophy (80% versus 50%, p = 0.043). Severity of hippocampal atrophy correlated with duration of epilepsy in patients with hippocampal atrophy (r = 0.4, p = 0.007), but not in those with amygdalohippocampal atrophy, suggesting that these two groups may have a different pathogenesis.

[Severe cardiac failure in Kearns-Sayre syndrome]. / Insuficiencia cardíaca grave en el síndrome de Kearns-Sayre.

Mitochondrial disorders are a group of diseases that can affect virtually all organ systems. A 19 year old man was seen in 1993 with neurologic abnormalities consisting of impaired function of muscles, diplopia, progressive loss of vision, impaired phonation and swallowing, during the last 10 years. Physical examination disclosed moderate wasting of the four limb muscles, mild motor weakness of neck muscles, symmetrical hyporeflexia, cerebellar dysfunction, severe external ophtalmoplegia and ptosis. Fundii oculi examination showed retinitis pigmentosa. The electromyogram demonstrated myopathic changes with normal nerve conduction velocities. The cerebrospinal fluid was normal, except for a mild increase in lactic acid. Histochemical study of a muscle biopsy specimen demonstrated ragged red fibers and increase of the subsarcolemal oxidative activity of mitochondriae. The diagnosis of Kearns-Sayre disease was confirmed and he was discharged advising physical therapy. On February 1995, he was again admitted, this time with right cardiac failure and worsening of all his previous symptoms and signs. He complained of myalgias and his muscle weakness was more striking on clinical examination. Echocardiography showed biventricular dilatation and left ventricular hypertrophy with preserved systolic function. A new muscle biopsy revealed an heteroplasmic deletion of 5 Kb with 80% of mutant mitochondrial DNA. In brief, we report a patient with the clinical phenotype of Kearns-Sayre syndrome who presented an acute congestive cardiac failure due to cardiomyopathy, an association which has seldom been, reported in the literature.

Hallazgos diagnósticos a través de la RM en una población de pacientes con epilepsia, seleccionados al azar / MRI diagnostic findings in a randomly selected epileptic population

A partir de los avances planteados con la RM, hemos realizado un estudio prospectivo en una población de 124 pacientes de consultorio externo con diagnóstico de epilepsia basado en datos clínicos y EEG, se aplicaron los criterios de la Liga Internacional de Epilepsia (ILAE, 1981, 1989) desde diciembre de 1993 a diciembre de 1994. El protocolo empleado incluyó imágenes en los planos sagital, coronal, axial y adquisición 3D coronal FFE, todos ellos paralelos y perpendiculares al eje mayor del hipocampo. Se completó con una secuencia IR axial. La RM fue normal en 67 pacientes (54 por ciento). En 57 pacientes (46 por ciento) la RM fue patológica con alteraciones corticales en 13 pacientes, tumores en 9 pacientes, esclerosis hipocampal en 8 pacientes, malformaciones vasculares en 4 pacientes, quistes aracnoideos en 5 pacientes y miscelánea en 18 pacientes. Hemos comparado los diferentes resultados hallados a través de la RM y la TC. El 33,8 por ciento de los pacientes que presentaron TC normal, tenía anormalidades en la RM, incluyendo 7 imágenes tumorales. La RM debería constituir parte de los análisis de rutina en los pacientes epilépticos, ya que representa una herramienta esencial para el diagnóstico de la epilepsia sintomática, resultando un método más específico y de mayor sensibilidad que la TC.

Hallazgos diagnósticos a través de la RM en una población de pacientes con epilepsia, seleccionados al azar / MRI diagnostic findings in a randomly selected epileptic population

A partir de los avances planteados con la RM, hemos realizado un estudio prospectivo en una población de 124 pacientes de consultorio externo con diagnóstico de epilepsia basado en datos clínicos y EEG, se aplicaron los criterios de la Liga Internacional de Epilepsia (ILAE, 1981, 1989) desde diciembre de 1993 a diciembre de 1994. El protocolo empleado incluyó imágenes en los planos sagital, coronal, axial y adquisición 3D coronal FFE, todos ellos paralelos y perpendiculares al eje mayor del hipocampo. Se completó con una secuencia IR axial. La RM fue normal en 67 pacientes (54 por ciento). En 57 pacientes (46 por ciento) la RM fue patológica con alteraciones corticales en 13 pacientes, tumores en 9 pacientes, esclerosis hipocampal en 8 pacientes, malformaciones vasculares en 4 pacientes, quistes aracnoideos en 5 pacientes y miscelánea en 18 pacientes. Hemos comparado los diferentes resultados hallados a través de la RM y la TC. El 33,8 por ciento de los pacientes que presentaron TC normal, tenía anormalidades en la RM, incluyendo 7 imágenes tumorales. La RM debería constituir parte de los análisis de rutina en los pacientes epilépticos, ya que representa una herramienta esencial para el diagnóstico de la epilepsia sintomática, resultando un método más específico y de mayor sensibilidad que la TC. (AU)

[Hemichorea associated with cerebral toxoplasmosis and AIDS]. / Hemicorea asociada a toxoplasmosis cerebral y SIDA.

Only 12 AIDS cases with hemichorea were reported in the literature. We report the first case of hemichorea associated with AIDS and cerebral toxoplasmosis in our country. A 26-year-old man had 3 episodes of focal seizures on the left side with subsequent loss of consciousness. A few weeks later, he noticed progressive left-sided weakness. Examination revealed a left hemiparesis. MRI of the head showed a round mass in the right frontal lobe and a smaller lesion in the left temporo-occipital area. Laboratory showed positive serum ELISA and Western Blot analysis for HIV antibodies. Serum tests for Toxoplasma showed elevated titers. He was treated with pyrimethamine and sulfadiazine. His weakness improved and he had no further seizures. Two weeks later, choreic movements appeared in the left foot, finally involving the entire left hemibody. A second MRI showed a new small lesion in the right cerebral peduncle. The patient completed 6 weeks of treatment, with further reduction in the size of the lesions. Nevertheless, the left hemichorea persisted. We believe that the hemichorea our patient had was caused by the contralateral peduncular lesion. Lesions involving the subthalamic nucleus or its connections may cause contralateral hemiballismus or hemichorea. In spite of the favorable response to antitoxoplasmic therapy, the hemichorea persisted. The present report illustrates an uncommon neurological complication in AIDS. We believe that a combination of a focal cerebral lesion and the HIV infection caused the movement disorder presented by the patient.

Hemicorea asociada a toxoplasmosis cerebral y SIDA / Hemichorea associated with cerebral toxoplasmosis and AIDS

Se observan complicaciones neurologicas en 40 por ciento de enfermos con SIDA. De estos, en 10 por ciento puede ser la manifestación inicial de la enfermedad. En otro 11 por ciento pueden aparecer trastornos del movimimiento. Comunicamos el primer caso de hemicorea asociada a toxoplasmose cerebral y SIDA en nuestro pais. Hombre de 26 anos, con diagnóstico de SIDA y toxoplasmosis cerebral. Habia comenzado con crisis motoras simples de hemicuerpo izquierdo, con generalización secundaria y luego perdida de fuerza progresiva en dicho hemicuerpo. La RMN de cerebro mostro una lesión frontal derecha y otra temporo-occiptal izquierda, con gran edema perilesional y efecto de masa. Las serologias para HIV y toxoplasmosis fueron positivas. Comenzo tratamiento con sulfadiazina y pirimetamina. Al duodecimo dia aparecieron movimientos involuntários del pie izquierdo, coreicos, que se extendieron mas tarde a todo ese miembro inferior y luego al hemicuerpo. Nueva RMN de cerebro mostro disminución del edema y efecto de masa de las lesiones. Sin embargo, se observó una nueva lesion a nivel peduncular derecho. Movimientos involuntarios en pacientes con toxoplasmosis cerebral la literatura mundial y el primero en nuestro pais de hemicorea asoicada a toxplasmosis y SIDA