Síndrome opsoclono-mioclono: características clínicas, aspectos terapéuticos y factores pronósticos en una cohorte pediátrica española / Opsoclonus-myoclonus syndrome: clinical characteristics, therapeutic considerations, and prognostic factors in a Spanish paediatric cohort

Introducción: El síndrome opsoclono-mioclono-ataxia es un raro trastorno de inicio pediátrico; de base neuroinflamatoria y origen paraneoplásico, parainfeccioso o idiopático. Actualmente no hay biomarcadores, siendo el diagnóstico clínico. El pronóstico cognitivo parece estar relacionado con el inicio temprano de la terapia inmunomoduladora.MétodoSe describen las características epidemiológicas, clínicas, terapéuticas y pronósticas a largo plazo de una cohorte de 20 pacientes españoles.ResultadosLa edad media de debut fue de 21 meses (2-59 meses). La ataxia y el opsoclonus fueron los síntomas de inicio más frecuentes y predominantes en la evolución. El tiempo medio desde los primeros síntomas hasta el diagnóstico fue de 1,1 mes. Un tumor de extirpe neuroblástica fue detectado en el 45%, realizándose resección quirúrgica en siete y quimioterapia en dos pacientes. En el estudio de líquido cefalorraquídeo se constató pleocitosis en cuatro (25%), con negatividad de anticuerpos antineuronales y bandas oligoclonales en todos los casos estudiados. En el 100% se emplearon fármacos inmunomoduladores. En nueve pacientes el tratamiento combinado inmunomodulador se inició desde el momento del diagnóstico, y en cinco el tiempo medio de implementación fue de 2,2 meses. A largo plazo, seis de 10 pacientes con seguimiento superior a cinco años presentaban secuelas cognitivas leves o moderadas; cuatro pacientes presentaron recaídas, generalmente coincidiendo con el descenso de la corticoterapia.ConclusionesEl inicio precoz de la inmunoterapia, así como de la triple terapia en los casos que lo precisaron, se relacionó con una menor frecuencia de afectación cognitiva a los dos años del debut. (AU)

Introduction: Opsoclonus-myoclonus-ataxia syndrome is a rare neuroinflammatory disorder with onset during childhood; aetiology may be paraneoplastic, para-infectious, or idiopathic. No biomarkers have yet been identified, and diagnosis is clinical. Better cognitive prognosis appears to be related to early onset of immunomodulatory therapy.MethodsWe describe the epidemiological, clinical, therapeutic, and long-term prognostic characteristics of a cohort of 20 Spanish patients.ResultsThe mean age of onset was 21 months (range, 2-59). Ataxia and opsoclonus were the most frequent symptoms both at disease onset and throughout disease progression. The mean time from onset to diagnosis was 1.1 months. Neuroblast lineage tumours were detected in 45% of patients; these were treated with surgical resection in 7 cases and chemotherapy in 2. Cerebrospinal fluid analysis revealed pleocytosis in 4 cases (25%) and neither antineuronal antibodies nor oligoclonal bands were detected in any patient. Immunomodulatory drugs were used in all cases. Nine patients started combined immunomodulatory treatment at the time of diagnosis, and 5 patients after a mean of 2.2 months. In the long term, 6 of the 10 patients followed up for more than 5 years presented mild or moderate cognitive sequelae. Four patients presented relapses, generally coinciding with the decrease of corticosteroid doses.ConclusionsEarly initiation of immunotherapy, as well as triple combination therapy, where needed, was associated with a lower frequency of cognitive impairment 2 years after onset. (AU)

Opsoclonus-myoclonus syndrome: Clinical characteristics, therapeutic considerations, and prognostic factors in a Spanish paediatric cohort.

INTRODUCTION: Opsoclonus-myoclonus-ataxia syndrome is a rare neuroinflammatory disorder with onset during childhood; aetiology may be paraneoplastic, para-infectious, or idiopathic. No biomarkers have yet been identified, and diagnosis is clinical. Better cognitive prognosis appears to be related to early onset of immunomodulatory therapy. METHODS: We describe the epidemiological, clinical, therapeutic, and long-term prognostic characteristics of a cohort of 20 Spanish patients. RESULTS: The mean age of onset was 21 months (range, 2-59). Ataxia and opsoclonus were the most frequent symptoms both at disease onset and throughout disease progression. The mean time from onset to diagnosis was 1.1 months. Neuroblast lineage tumours were detected in 45% of patients; these were treated with surgical resection in 7 cases and chemotherapy in 2. Cerebrospinal fluid analysis revealed pleocytosis in 4 cases (25%) and neither antineuronal antibodies nor oligoclonal bands were detected in any patient. Immunomodulatory drugs were used in all cases. Nine patients started combined immunomodulatory treatment at the time of diagnosis, and 5 patients after a mean of 2.2 months. In the long term, 6 of the 10 patients followed up for more than 5 years presented mild or moderate cognitive sequelae. Four patients presented relapses, generally coinciding with the decrease of corticosteroid doses. CONCLUSIONS: Early initiation of immunotherapy, as well as triple combination therapy, where needed, was associated with a lower frequency of cognitive impairment 2 years after onset.

Opsoclonus-myoclonus syndrome: clinical characteristics, therapeutic considerations, and prognostic factors in a Spanish paediatric cohort. / Síndrome opsoclono-mioclono: características clínicas, aspectos terapéuticos y factores pronósticos en una cohorte pediátrica española.

INTRODUCTION: Opsoclonus-myoclonus-ataxia syndrome is a rare neuroinflammatory disorder with onset during childhood; aetiology may be paraneoplastic, para-infectious, or idiopathic. No biomarkers have yet been identified, and diagnosis is clinical. Better cognitive prognosis appears to be related to early onset of immunomodulatory therapy. METHODS: We describe the epidemiological, clinical, therapeutic, and long-term prognostic characteristics of a cohort of 20 Spanish patients. RESULTS: The mean age of onset was 21 months (range, 2-59). Ataxia and opsoclonus were the most frequent symptoms both at disease onset and throughout disease progression. The mean time from onset to diagnosis was 1.1 months. Neuroblast lineage tumours were detected in 45% of patients; these were treated with surgical resection in 7 cases and chemotherapy in 2. Cerebrospinal fluid analysis revealed pleocytosis in 4 cases (25%) and neither antineuronal antibodies nor oligoclonal bands were detected in any patient. Immunomodulatory drugs were used in all cases. Nine patients started combined immunomodulatory treatment at the time of diagnosis, and 5 patients after a mean of 2.2 months. In the long term, 6 of the 10 patients followed up for more than 5 years presented mild or moderate cognitive sequelae. Four patients presented relapses, generally coinciding with the decrease of corticosteroid doses. CONCLUSIONS: Early initiation of immunotherapy, as well as triple combination therapy, where needed, was associated with a lower frequency of cognitive impairment 2 years after onset.

Seudotumor cerebri en niños: etiología, características clínicas y evolución / Pseudotumour cerebri in children: Aetiology, clinical features, and progression

Introducción: El síndrome de hipertensión intracraneal idiopática o seudotumor cerebri (STC) en niños está en constante revisión, respecto a su definición, etiologías asociadas, diagnóstico y terapéutica más apropiada. Objetivos y métodos: Se revisaron los casos de STC < 15 años de edad en un hospital de referencia en los últimos 12 años. Se estudiaron las características clínico-epidemiológicas y el procedimiento diagnóstico-terapéutico empleado. Se definió STC como presión intracraneal > 25cmH2O por punción lumbar (PL), con estudio de resonancia magnética cerebral sin lesión ocupante de espacio. Resultados: Se registró a 12 niños con STC, media de edad de 10 años, 90% mujeres. Todos presentaban peso normal. El 82% manifestaba síntomas: cefalea (66%), diplopía (8%) o baja visión (8%). Todos asociaban papiledema (17% unilateral). La PL fue diagnóstica en el 100% y la neuroimagen fue normal en el 91%. Se evidenció un posible desencadenante en 5 casos (2 farmacológico y 3 infeccioso por Mycoplasma pneumoniae [M. pneumoniae]). El 91% recibió tratamiento médico: en el 75% consistió en PL repetidas y en el 42% solo acetazolamida y/o prednisona. La evolución fue favorable en todos ellos. Conclusiones: La incidencia de STC fue de aproximadamente 1/100.000 niños/año, similar a estudios previos. En esta población, el sobrepeso no es un factor de riesgo. La infección por M. pneumoniae podría actuar como desencadenante de STC y favorecer recurrencias tardías. La ausencia de síntomas parece independiente del grado de presión intracraneal. El tratamiento con acetazolamida es eficaz en la mayoría de los casos, desterrando el uso de PL repetidas

Introduction: The definition, associated aetiologies, diagnosis, and treatment of idiopathic intracranial hypertension, or pseudotumour cerebri (PTC), are constantly being revised in the paediatric population. Objectives and methods: Our study included children younger than 15 years old with PTC and attended at a reference hospital in the past 12 years. We analysed the clinical and epidemiological features of our sample and the diagnostic and treatment approaches. PTC was defined as presence of intracranial hypertension (CSF opening pressure> 25 cmH2O) and absence of space-occupying lesions in brain MR images. Results: A total of 12 children with PTC were included; mean age was 10 years and 90% were girls. Weight was normal in all patients. Eighty-two percent of the patients had symptoms: headache (66%), diplopia (8%), and visual loss (8%). All of them displayed papilloedema (17% unilaterally). Lumbar puncture (LP) provided the diagnosis in all cases and 91% showed no relevant MRI findings. A potential cause of PTC was identified in 5 cases: pharmacological treatment in 2 and infection (Mycoplasma pneumoniae [M. pneumoniae]) in 3. Ninety-one per cent of the patients received treatment: 75% underwent several LPs and 42% received acetazolamide and/or prednisone. Outcomes were favourable in all cases. Conclusions: The incidence of PTC was estimated at approximately 1 case per 100 000 children/years, in line with data reported by previous studies. Overweight was not found to be a risk factor for PTC in this population. M. pneumoniae infection may trigger PTC and cause recurrences at later stages. The absence of symptoms seems to be independent from the degree of intracranial hypertension. Acetazolamide treatment is effective in most cases, and it represents a viable alternative to repeated LP

Pseudotumour cerebri in children: Aetiology, clinical features, and progression. / Seudotumor cerebri en niños: etiología, características clínicas y evolución.

INTRODUCTION: The definition, associated aetiologies, diagnosis, and treatment of idiopathic intracranial hypertension, or pseudotumour cerebri (PTC), are constantly being revised in the paediatric population. OBJECTIVES AND METHODS: Our study included children younger than 15 years old with PTC and attended at a reference hospital in the past 12 years. We analysed the clinical and epidemiological features of our sample and the diagnostic and treatment approaches. PTC was defined as presence of intracranial hypertension (CSF opening pressure>25cmH2O) and absence of space-occupying lesions in brain MR images. RESULTS: A total of 12 children with PTC were included; mean age was 10 years and 90% were girls. Weight was normal in all patients. Eighty-two percent of the patients had symptoms: headache (66%), diplopia (8%), and visual loss (8%). All of them displayed papilloedema (17% unilaterally). Lumbar puncture (LP) provided the diagnosis in all cases and 91% showed no relevant MRI findings. A potential cause of PTC was identified in 5 cases: pharmacological treatment in 2 and infection (Mycoplasma pneumoniae [M. pneumoniae]) in 3. Ninety-one per cent of the patients received treatment: 75% underwent several LPs and 42% received acetazolamide and/or prednisone. Outcomes were favourable in all cases. CONCLUSIONS: The incidence of PTC was estimated at approximately 1 case per 100 000 children/years, in line with data reported by previous studies. Overweight was not found to be a risk factor for PTC in this population. M. pneumoniae infection may trigger PTC and cause recurrences at later stages. The absence of symptoms seems to be independent from the degree of intracranial hypertension. Acetazolamide treatment is effective in most cases, and it represents a viable alternative to repeated LP.

[A comparative study of three systems for quantifying the spike and wave index in patients with continuous spikes and waves during slow sleep]. / Estudio comparativo entre tres sistemas de cuantificacion del indice de punta-onda en pacientes con punta-onda continua del sueño lento.

INTRODUCTION: Continuous spikes and waves during slow sleep (CSWS) is an epileptic encephalopathy of childhood with a pattern of epileptiform discharges during sleep, which, if prolonged over time, produce severe neuropsychological impairment. Quantification of the paroxysms by means of a spike and wave index (SWI) > 85% establishes a presumptive diagnosis and allows early therapy to be started to prevent such impairment. AIMS: To compare the results of the classic method for calculating the SWI against two proposals that optimise the relation between the analysis time employed and the diagnostic sensitivity. PATIENTS AND METHODS: The nocturnal electroencephalographic registers of 17 patients with CSWS were studied. Two observers calculated the SWI with the classic method, as well as with two other methods, M2 and M3, first in the active phase and then in the remission phase. The time required by each method, the individual SWI values and the agreement between methods and observers were compared. RESULTS: With M3 two of the patients failed to reach the cut-off point of SWI > 85%. Agreement in the active phase of CSWS after M2 and M3 was 0.762 and 0.704, respectively, while in the remission phase it was 0.951 and 0.830. Inter-observer agreement exceeded 0.905 in all cases. CONCLUSIONS: The two abbreviated methods can be used in both the active and the remission phases, with a substantial reduction in the analysis time that is needed. Our results support the current tendency to consider SWI > 60% as suggestive of CSWS. Method M2 yields results that are closer to those of the classic method than those of M3.

TITLE: Estudio comparativo entre tres sistemas de cuantificacion del indice de punta-onda en pacientes con punta-onda continua del sueño lento.Introduccion. La punta-onda continua del sueño lento (POCS) es una encefalopatia epileptiforme infantil con un patron de descargas epileptiformes durante el sueño que, prolongadas en el tiempo, producen un grave deterioro neuropsicologico. La cuantificacion de los paroxismos mediante el indice de punta-onda (SWI) > 85% establece un diagnostico de sospecha y permite iniciar una terapia precoz que puede evitar dicho deterioro. Objetivos. Comparar los resultados del metodo clasico de calculo del SWI con dos propuestas que optimicen la relacion entre el tiempo de analisis empleado y la sensibilidad diagnostica. Pacientes y metodos. Se estudiaron los registros electroencefalograficos nocturnos de 17 pacientes con POCS. Dos observadores calcularon el SWI con el metodo clasico, asi como con otros dos metodos, M2 y M3, primero en la fase activa y posteriormente en la fase de remision de la POCS. Se comparo el tiempo consumido por cada metodo, los valores individuales de SWI y la concordancia entre metodos y observadores. Resultados. Con el M3 dos pacientes no alcanzaron el corte del SWI > 85%. La concordancia en la fase activa de la POCS tras el M2 y el M3 fue de 0,762 y 0,704, respectivamente, mientras que en la fase de remision fue de 0,951 y 0,830. La concordancia entre observadores supero el 0,905 en todos los casos. Conclusiones. Los dos metodos abreviados se pueden utilizar tanto en la fase activa de la POCS como en la fase de remision, con una sustancial reduccion del tiempo de analisis empleado. Nuestros resultados apoyan la tendencia actual de considerar el SWI > 60% como sugestivo de POCS. El metodo M2 arroja resultados mas cercanos a los del metodo clasico que los de M3.

[Unilateral vocal fold paralysis: quality of voice after speech therapy]. / Parálisis de cuerda vocal unilateral: estudio de la calidad vocaldespués del tratamiento logopédico

BACKGROUND: To describe demographic factors and voice quality parameters of patients with unilateral vocal fold paralysis (UVFP) after a voice therapy protocol. METHODS: Forty-seven patients with a diagnosis of UVFP by video-laryngoscopy were included. Voice therapy was applied to all patients during 15 sessions that were structured in three progressive stages. The objective was to train patients in vocal techniques, phonic-breathing coordination,blow control, vocal exercises for glottic closure and vocal setting. Glottal closure, Voice Handicap Index-10 for perception of voice impairment and GRBAS scale were used before and after the speech therapy. RESULTS: The average age was 51 years (range 20-80), 60%women. Surgery was the most frequent cause (72%), and 40% had a profession related to voice use. Median time from diagnosis to treatment was 5 months (2-12). After voice therapy, 80% had complete glottal closure, previously this had been 34% (p<0.001), the score of VHI-10 decreased from 24.24 to 16.09 points (p<0.001) and GRBAS values improved in all the qualities of voice (p<0.001).Only 8.5% of the patients required surgical intervention after treatment. CONCLUSIONS: Voice therapy is effective as first line therapy in patients with UVFP, reserving medialization with non-absorbable material or thyroplasty surgery for those with a poor outcome. However, it is necessary to reduce the time it takes the patient to reach the Voice Unit after laryngoscopic diagnosis.

Parálisis de cuerda vocal unilateral: estudio de la calidad vocal después del tratamiento logopédico / Unilateral vocal fold paralysis: quality of voice after speech therapy

Fundamento: Describir los factores sociodemográficos de pacientes con parálisis de cuerda vocal unilateral (PCVU) a los que se aplica un tratamiento logopédico y analiza el impacto en la calidad vocal tras la terapia. Metodología: Se incluyeron 47 pacientes con PCVU diagnosticada mediante vídeo-laringoscopia. Todos recibieron intervención logopédica repartida en 15 sesiones y estructurada en tres etapas progresivas. Se instruyó al paciente en técnicas vocales, coordinación fono-respiratoria, control del soplo, tonificación glótica e impostación vocal. Los parámetros incluidos en el estudio antes y después de la terapia fueron: cierre glótico, Voice Handicap Index-10 (VHI-10) para medir la autopercepción de calidad vocal y escala GRABS para la calidad vocal. Resultados: La edad media fue 51 años (rango 20-80), 60% mujeres. La causa quirúrgica fue la más frecuente (72%). El 40% desempeñaban una profesión relacionada con la voz. El tiempo medio desde el diagnóstico hasta el inicio del tratamiento fue 5 meses (2-12). El cierre glótico completo aumentó de 34 a 80% (p<0,001), la puntuación en VHI-10 descendió de 24,24 a 16,09 puntos (p<0,001) y los valores de GRABS mejoraron en todas las cualidades de la voz (p<0,001). Solo un 8,5% requirió cirugía post-tratamiento. Conclusiones: La terapia de reeducación vocal es eficaz como primera indicación terapéutica en pacientes con PCVU, reservando la medialización con material no reabsorbible o cirugía de tiroplastia a aquéllos con mala evolución. No obstante, es necesario reducir el tiempo que se demora el paciente en llegar a la Unidad de Voz tras el diagnóstico laringoscópico (AU)

Background: To describe demographic factors and voice quality parameters of patients with unilateral vocal fold paralysis (UVFP) after a voice therapy protocol. Methods: Forty-seven patients with a diagnosis of UVFP by video-laryngoscopy were included. Voice therapy was applied to all patients during 15 sessions that were structured in three progressive stages. The objective was to train patients in vocal techniques, phonic-breathing coordination, blow control, vocal exercises for glottic closure and vocal setting. Glottal closure, Voice Handicap Index-10 for perception of voice impairment and GRBAS scale were used before and after the speech therapy. Results: The average age was 51 years (range 20-80), 60% women. Surgery was the most frequent cause (72%), and 40% had a profession related to voice use. Median time from diagnosis to treatment was 5 months (2-12). After voice therapy, 80% had complete glottal closure, previously this had been 34% (p<0.001), the score of VHI-10 decreased from 24.24 to 16.09 points (p<0.001) and GRBAS values improved in all the qualities of voice (p<0.001). Only 8.5% of the patients required surgical intervention after treatment. Conclusions: Voice therapy is effective as first line therapy in patients with UVFP, reserving medialization with non-absorbable material or thyroplasty surgery for those with a poor outcome. However, it is necessary to reduce the time it takes the patient to reach the Voice Unit after laryngoscopic diagnosis (AU)

Miocardiopatía dilatada e infarto de arteria cerebral media derecha / Dilated cardiomyopathy and right middle cerebral artery stroke

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Plexopatía braquial unilateral en un lactante como manifestación precoz de una neuropatía hereditaria con parálisis sensible a la presión / Brachial plexopathy as early manifestation of hereditary neuropathy with liability to pressure palsy in infancy

La neuropatía hereditaria con parálisis sensible a la presión (NHPP) suele manifestarse en la adolescencia y/o edad adulta, y es relativamente excepcional en edades más tempranas. Se presenta un caso de plexopatía braquial unilateral en un lactante de 4 meses de edad como manifestación precoz de NHPP, y se destaca la importancia de los datos anamnésicos familiares junto con el estudio neurofisiológico y molecular en la sospecha diagnóstica precoz (AU)

Hereditary neuropathy with liability to pressure palsy (HNPP) is an autosomal dominant disorder characterized by recurrent mono-neuropathies related to minimal trauma o compression. HNPP usually manifests in adolescence or adulthood, being relatively exceptional at an earlier age. A 4-month-old boy with brachial plexopathy as early manifestation of HNPP is presented. Familiar anamnesis, as well as neurophysiological and molecular studies, stands out in early diagnostic suspicion. In this case, the early manifestation and its semiological characteristics accentuate its peculiarity (AU)

Modelo dietético en pacientes con déficit de atención e hiperactividad / Dietary patterns in patients with attention deficit hyperactivity disorder

OBJETIVO: Estudiar los hábitos alimentarios en un grupo de pacientes con trastorno de déficit de atención e hiperactividad (TDAH) en tratamiento con metilfenidato de liberación prolongada (MTF-LP). MATERIAL Y MÉTODOS: Encuesta nutricional (consumo de alimentos de 3 días consecutivos) a 150 pacientes con TDAH (100 varones y 50 mujeres) en tratamiento con MTF-LP, y a 150 sujetos sanos de la misma edad (grupo control). Se han comparado la ingesta de alimentos y nutrientes y el estado nutricional entre ambos grupos. RESULTADOS: La dosis media de MTF-LP era de 1,02 mg/kg/día. En el grupo con TDAH el estado nutricional era significativamente inferior (p < 0,05). La ingesta de cereales, carnes, legumbres y frutas era significativamente inferior (p < 0,05) en el grupo con TDAH. El aporte calórico del almuerzo, comida del mediodía y merienda era significativamente superior (p < 0,05) en el grupo control; mientras que el aporte calórico de la cena era significativamente superior (p < 0,05) en el grupo con TDAH. En el grupo control el aporte calórico y de proteínas, hidratos de carbono, grasas y fibra, calcio, hierro, magnesio, cinc, selenio y fósforo, tiamina, niacina, vitamina B6 y folatos era significativamente superior (p < 0,05) respecto al grupo TDAH. CONCLUSIONES: El tratamiento con MTF-LP modifica sustancialmente la distribución porcentual del aporte calórico de las distintas ingestas diarias. El aporte calórico y de nutrientes en los pacientes en tratamiento con MTF-LP es, en general, inferior al de la población sana de la misma edad. Habría que considerar la necesidad de impartir, simultáneamente con el tratamiento multimodal, programas de educación nutricional a estos pacientes y/o sus familias

OBJECTIVE: To evaluate the dietary patterns in a group of patients diagnosed with attention deficit hyperactivity disorder (ADHD) and under treatment with extended-release methylphenidate (MPH-ER).MATERIAL AND METHODS: A nutrition survey (food intake recall for three consecutive days) was carried out on 100 patients diagnosed with ADHD and under treatment with MPH-ER, and in 150healthy children (control group). Calorie and nutrient intake, as well as nutrition status, were evaluated and compared in both groups. RESULTS: The mean MPH-ER dose was 1.02 mg/kg/day. Nutritional status in the ADHD group was significantly lower (P < .05) than in control group. The consumption of cereals, meat, pulses and fruits in the control group was significantly higher (P < 0.05) than in ADHD group. Calorie intake in the mid-morning snack, lunch and afternoon snack was significantly higher (P < 0.05) in the control group. Calorie intake at supper was significantly higher (P < 0.05) in the ADHD group. Total calorie intake, as well as protein, carbohydrates, fat, fibre, calcium, iron, magnesium, zinc, selenium and phosphorous, thiamine, niacin, vitamin B6 and folate intake in the control group was significantly higher than in ADHD group. CONCLUSIONS: Treatment with MPH-ER substantially modifies the percentage distribution of calorie intake of the different meals. The daily calorie and nutrients intake in patients under treatment with MPH-ER is, generally, lower than in the healthy population of a similar age. Nutrition education should be provided, along with multimodal treatment, to the patients and/or their families

[Dietary patterns in patients with attention deficit hyperactivity disorder]. / Modelo dietético en pacientes con déficit de atención e hiperactividad.

OBJECTIVE: To evaluate the dietary patterns in a group of patients diagnosed with attention deficit hyperactivity disorder (ADHD) and under treatment with extended-release methylphenidate (MPH-ER). MATERIAL AND METHODS: A nutrition survey (food intake recall for three consecutive days) was carried out on 100 patients diagnosed with ADHD and under treatment with MPH-ER, and in 150 healthy children (control group). Calorie and nutrient intake, as well as nutrition status, were evaluated and compared in both groups. RESULTS: The mean MPH-ER dose was 1.02 mg/kg/day. Nutritional status in the ADHD group was significantly lower (P < .05) than in control group. The consumption of cereals, meat, pulses and fruits in the control group was significantly higher (P < .05) than in ADHD group. Calorie intake in the mid-morning snack, lunch and afternoon snack was significantly higher (P < .05) in the control group. Calorie intake at supper was significantly higher (P < .05) in the ADHD group. Total calorie intake, as well as protein, carbohydrates, fat, fibre, calcium, iron, magnesium, zinc, selenium and phosphorous, thiamine, niacin, vitamin B6 and folate intake in the control group was significantly higher than in ADHD group. CONCLUSIONS: Treatment with MPH-ER substantially modifies the percentage distribution of calorie intake of the different meals. The daily calorie and nutrients intake in patients under treatment with MPH-ER is, generally, lower than in the healthy population of a similar age. Nutrition education should be provided, along with multimodal treatment, to the patients and/or their families.

Magnetic resonance imaging abnormalities in children with epilepsy.

BACKGROUND AND PURPOSE: The aim of this study is to analyze the proportional distribution of epilepsy and epileptic syndromes in children and to describe the magnetic resonance imaging (MRI) abnormalities found in these patients. METHODS: Data from 457 children aged 1 month to 15 years at the time of diagnosis of epilepsy were recorded. A routine MRI has been requested in all patients with epilepsy at diagnosis according to a standardized pediatric seizure protocol. Abnormalities on MRI were classified as either significant or non-significant (standardized scoring system). International League Against Epilepsy criteria were used for diagnoses. RESULTS: The prevalence of significant MRI abnormalities was 21.9% (in infants 42.3%, in childhood 18.2%, and in adolescents 15.9%). The most common abnormalities included white-matter lesions (27.6%), volume loss (19.6%), gray-matter lesions (19.6%), and ventricular enlargement (12%). CONCLUSIONS: The use of MRI and a reliable standardized scoring system at diagnosis of epilepsy in children identified a high rate of significant abnormalities findings. This may have important implications for practice guidelines in this population.

[Paraneoplastic opsoclonus myoclonus ataxia syndrome]. / Síndrome opsoclono-mioclono-atáxico paraneoplásico.

Opsoclonus myoclonus ataxia syndrome (OMAS) is a very infrequent paraneoplastic or postinfectious movement disorder, which may occur at any age, most commonly between 6 and 36 months of age. In four days, a previously healthy 30-month-old girl progressively developed gait instability, intention tremor, dysarthric speech, irritability and altered sleep. Physical and neurological examination did not reveal additional deficits. She had had a transient exanthema without fever three weeks before. Basic blood analysis, serologies, cultures, urine toxin detection, EEG and cerebral CT were normal. Lumbar puncture showed minimal lymphocytosis. On the fifth day following the onset of symptoms, the ataxia worsened, precluding sitting, and the tremor was aggravated by intentional myoclonus. Chaotic saccadic, large amplitude multidirectional but conjugated eye movements appeared. An opsoclonus was suspected and a chest X-ray and CT revealed a paravertebral thoracic mass. Surgery confirmed a localized ganglioneuroblastoma. Blood neuron-specific enolase and urine catecholamine levels were normal. Opsoclonus disappeared with high doses of prednisone and following surgery. Ataxia improved but the patient still required low daily doses of steroids for one year.

Resolución de tics tras la administración de penicilina en un paciente con PANDAS / Tic disappearance after penicillin treatment in a patient with PANDAS

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