Tortícolis e hiperextensión espinal. Presentación inusual del síndrome de Guillain-Barré / Torticollis and hyperextended spine: unusual presentation of Guillain-Barré syndrome

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La amnesia global transitoria: entidad infrecuente en la infancia / Transient global amnesia: an uncommon disorder in childhood

Introducción: La amnesia global transitoria (AGT) es un cuadro de presentación súbita caracterizado por una intensa amnesia anterógrada y una amnesia retrógada variable. La memoria inmediata está conservada y el resto de la exploración neurológica es normal. La recuperación es progresiva en pocas horas. Suele presentarse en pacientes adultos de mediana edad y en ancianos. Caso clínico: Niña de 9 años de edad que presenta una amnesia anterógrada de inicio brusco, con memoria inmediata conservada y algún trastorno de la memoria retrógada, asociados a cefalea de intensidad leve. La exploración neurológica es normal. El episodio cede de forma espontánea en unas 4 horas. Posteriormente, la paciente presenta una cefalea leve y una amnesia del episodio. Se realiza una tomografía computarizada craneal, que es normal, por lo que el cuadro se orienta como AGT. Discusión: La clínica descrita es característica de la AGT. La aparición de este cuadro suele ser espontánea, aunque puede existir un factor desencadenante. Se ha postulado que podría deberse a un fenómeno vascular amplio con isquemia transitoria del territorio de la vertebrobasilar, o a un fenómeno epiléptico o migrañoso. La migraña confusional aguda, típica de la infancia, tiene manifestaciones clínicas similares a las de la AGT. El diagnóstico de este proceso es clínico. Tiene un excelente pronóstico, con poca tendencia a la recidiva. Dada la presentación poco habitual en la edad pediátrica, en nuestro caso se realizó una prueba de imagen para descartar un accidente cerebrovascular. La clínica típica y la normalidad de las pruebas de imagen deben hacernos considerar este diagnóstico también en la edad pediátrica

Introduction: Transient global amnesia (TGA) is a clinical condition characterized by sudden loss of anterograde memory and a varying degree of retrogade amnesia. Immediate memory is preserved and the neurological examination is normal. The memory deficit resolves within a few hours. This condition is frequent in middle-aged or elderly patients. Case report: A 9-year-old girl developed sudden anterograde amnesia with preserved immediate memory and a certain degree of retrogade memory deficit, associated with a mild headache. The neurological examination was normal. The clinical condition resolved spontaneously in 4 hours. The patient was left with a mild headache and no memory of the attack. As the brain computed tomographic (CT) scan was normal, the clinical condition suggested a case of TGA. Discussion: The clinical features reported here are characteristic of TGA. This neurological disorder appears spontaneously, but several precipitating factors may exist. The pathogenetic hypotheses include a vascular etiology with transient vertebrobasilar ischemia, an epileptic phenomenon or a migraine-related event. Acute confusional migraine, typical of childhood, presents with clinical manifestations similar to TGA. The diagnosis of this process is based on clinical findings. It has an excellent prognosis and little tendency to recur. Given the fact that TGA is not frequent in childhood, we performed a CT scan to rule out the possibility of a cerebral ischemic attack. The presence of the typical clinical symptoms and a normal neuroimaging study should lead us to consider this diagnosis in childhood as well

Síndrome de Dyggve-Melchior-Clausen, dificultad en su diagnóstico por similitud con la enfermedad de Morquio / Dyggve-Melchior-Clausen syndrome, diagnostic difficulty due to it similarity to Morquio disease

Introducción. Es síndrome de Dyggve-Melchior-Clausen (SDMC) es una enfermedad minoritaria con herencia autosómica recesiva producida por mutaciones en el gen Dymeclin que ha sido recientemente identificado. Se caracteriza por la asociación poco frecuente de displasia espondiloepimetafisaria y retraso mental, con evolución progresiva. Las similitudes clínicas y radiológicas al inicio del proceso con la enfermedad de Morquio pueden dificultar su diagnóstico y actualmente aún no se ha descrito la anomalía bioquímica que se produce. Caso clínico. Niña de 8 años con dismorfismo posnatal progresivo. Al inicio, la platispondilia y displasia de epífisis y metáfisis, junto con los estudios bioquímicos realizados recuerdan la enfermedad de Morquio; sin embargo, en la evolución el retraso mental y los hallazgos radiológicos específicos orientan el diagnóstico del SDMC, identificándose una mutación missense en homocigosis en el gen Dym (Dymeclin). Conclusiones. Es importante conocer esta entidad, que se puede confundir fácilmente con la enfermedad de Morquio. Los hallazgos radiológicos en las crestas ilíacas son patognomónicos del SDMC. Actualmente, el diagnóstico molecular nos permite confirmar la enfermedad y realizar estudios prenatales

Introduction. Dyggve-Melchior-Clausen syndrome (DMCS) is a rare autosomal recessive disorder produced by mutations in the Dymeclin gene recently identified. It is characterized by the association of a progressive spondylo-epi-metaphyseal dysplasia and mental retardation ranging from mild to severe. The clinical and radiological similarities at the onset of the condition with the Morquio disease may hinder its diagnosis and no biochemical abnormality that causes it has been described as of yet. Clinical case. An eight-year-old girl had progressive postnatal dwarfism. Platyspondyly and dysplasic epiphyses and metaphyses with biochemical studies that resembled those of Morquio's disease; however the presence of specific radiological features and mental retardation led to the diagnosis of DMCS. A missense Dym mutation in homozygosis was identified. Conclusion. This entity should be known as it may be easily confused with Morquio disease. Radiological appearance of the iliac crests are very pathognomonic of DMCS. Identification of Dym gene is an important step towards the prenatal diagnosis

[Dyggve-Melchior-Clausen syndrome, diagnostic difficulty due to it similarity to Morquio disease]. / Síndrome de Dyggve-Melchior-Clausen, dificultad en su diagnóstico por similitud con la enfermedad de Morquio.

INTRODUCTION: Dyggve-Melchior-Clausen syndrome (DMCS) is a rare autosomal recessive disorder produced by mutations in the Dymeclin gene recently identified. It is characterized by the association of a progressive spondylo-epi-metaphyseal dysplasia and mental retardation ranging from mild to severe. The clinical and radiological similarities at the onset of the condition with the Morquio disease may hinder its diagnosis and no biochemical abnormality that causes it has been described as of yet. CLINICAL CASE: An eight-year-old girl had progressive postnatal dwarfism. Platyspondyly and dysplasic epiphyses and metaphyses with biochemical studies that resembled those of Morquio's disease; however the presence of specific radiological features and mental retardation led to the diagnosis of DMCS. A missense Dym mutation in homozygosis was identified. CONCLUSION: This entity should be known as it may be easily confused with Morquio disease. Radiological appearance of the iliac crests are very pathognomonic of DMCS. Identification of Dym gene is an important step towards the prenatal diagnosis.

Primera crisis convulsiva afebril. Conducta en urgencias y evolución en un año / First apyretic seizure. Behavior in the Emergency Service and course in one year

Objetivo. Estudio retrospectivo de pacientes con una primera crisis convulsiva afebril, identificando las de origen epiléptico con su seguimiento de 12 meses. Evaluar su relación con los resultados electroencefalográficos (EEG), el inicio de tratamiento con fármacos antiepilépticos (FAE) y el control de los pacientes. Resultados. Se recogen 79 pacientes. Edad entre 8 meses y 17 años (mediana de 6 años9. El 60% son varones. Un 6,3% tienen antecedentes de convulsión febril (CF) y un 30,4% antecedentes familiares de epilepsia. La prueba más utilizada en urgencias para determinar la etiología no comicial es la glucemia (38%), seguida del hemograma con ionograma y glucemia (34,2%). Se realiza EEG a todos los pacientes siendo normal en el 44% y neuroimagen al 44,3% detectándose anomalías en un 22,6%, todas de etiología crónica. Se instaura FAE en un 50,1% (41 casos). Seguimiento de 12 meses, de forma completa en 66 pacientes. Se observan nuevas crisis en un 39,4% (26 pacientes). De estos, el 51,5% (17 pacientes) no llevaba FAE. Un 25,5% de los pacientes que no habían iniciado tratamiento no recibían. El 6,2% de las recaída son durante los 3 primeros meses posteriores al primer episodio. Conclusiones. Al año recaen un 39,4%. La decisión de iniciar FAE debe realizarse de forma individualizada atendiendo al tipo de crisis, EEG y la realización, si es preciso, de una neuroimagen (AU)

Aim. Retrospective study of patients with an initial afebrile seizure, and identification of convulsions of epileptic origin over 12 months of follow-up, with the objective of determining appropriate patient care. Results. Result were obtained for 79 patients, 60% of whom were male, aged 8 months to 17 years (average age 6 years). In 6,3% there was a prior febrile convulsion, and 30,4% had a family history of epilepsy. The test most frequently used in the emergency room to determine non-epileptic etiology was blood sugar level (38%), followed by hemogram with ionogram and blood sugar (34.2%). EEG for 44%, and neuroimaging on 44,3%. Anomalies were detected in 22.6%, all of chronic etiology. Antiepileptic drug treatment was started in 50,1% (41 cases). For 66 patients there was complete follow-up over 12 months. Subsequent seizures were observed in 26 patients(39.4%), of whom 51,5% (17 patients) were not receiving antiepileptic drug treatment. Of the patients not treated, 25,5% did not experience further seizures. Most of the relapses (69,2%) occurred within the first three months following the initial episode. Conclusions. At the end of one year, 39,4% of the patients experienced a relapse. The decision to initiate antiepileptic drug treatment should be made on a case-by-case basis, taking into account the type of seizure, EEG results, and, if indicated, the results of neuroimaging (AU)

Hemiparálisis velopalatina idiopática / Idiopathic palatopharyngeal hemiparalysis

La hemiparálisis velopalatina idiopática es una entidad de etiología desconocida excepcional en pediatría; por ello, su diagnóstico exige un alto índice de sospecha. Se aporta un caso de disfunción brusca de los pares craneales inferiores (IX y X) en una paciente de 5 años de edad previamente asintomática. Esta paciente fue diagnosticada de hemiparálisis velopalatina idiopática tras valorar el curso clínico favorable y la normalidad de las exploraciones complementarias realizadas. Nuestro objetivo es comunicar nuestra experiencia en esta entidad a partir de este caso, así como aportar una revisión bibliográfica al respecto. Es importante sospechar esta patología en pacientes de edad comprendida entre los 5 y los 15 años que presentan una paresia brusca del IX y X pares craneales, sin ninguna otra sintomatología acompañante para racionalizar utensilios diagnósticos y terapéuticos. Se trata de un proceso cuyo tratamiento es sintomático. Su pronóstico es excelente, debido al alto porcentaje de remisión completa y la ausencia de recurrencias (AU)

[Idiopathic palatopharyngeal hemiparalysis]. / Hemiparálisis velopalatina idiopática.

Idiopathic velopalatine palsy is a condition of unknown etiology and is rarely seen in childhood. Consequently, diagnosis requires a high degree of suspicion. We report a case of sudden onset dysfunction of the lower cranial pairs (IX and X) in a 5-year-old girl who was previously asymptomatic. The clinical course was favorable and the results of complementary investigations were normal and the patient was diagnosed with velopalatine palsy. Based on this case, we aim to report our experience of this condition and provide a review of the literature. This disease should be suspected in patients aged between 5 and 15 years old who present a palsy of the IX and X cranial nerves of sudden onset and without any other symptoms in order to rationalize diagnostic and therapeutic tools. Treatment is based on support measures. The prognosis is excellent, with a high percentage of complete recovery and absence of recurrences.

[Thymectomy in juvenile myasthenia gravis]. / Timectomía en la miastenia grave juvenil.

INTRODUCTION: Juvenile myasthenia gravis (JMG) is an infrequent autoimmune disease, the symptoms and therapeutic handling of which do not differ from those of the adult forms. Chronic treatment with corticoids very often causes side effects in childhood, which is why patients are being submitted to thymectomies at younger ages with better results. AIMS. To analyse the clinical and evolutionary profile of JMG treated by thymectomy in our centre. PATIENTS AND METHODS: We report the case of four girls aged between 5 and 13 who were diagnosed as suffering from generalised myasthenia gravis (MG) with bulbar affectation. One of them started with a myasthenic crisis. The four of them were submitted to Tensilon s test, an electrophysiological study, determination of AChR, thoracic CT, and study of autoimmunity and thyroid functioning. After surgery the thymus was analysed histologically. RESULTS: They all gave positive in Tensilon s test and were seropositive for AChR. They were treated with anticholinesterases, up to the maximum tolerated dose, and corticoids, without complete remission being accomplished and so they were submitted to a thymectomy in the first year of evolution. In three cases surgical approach was transsternal and in the other by means of a videothoracoscope. All the thymuses showed lymphoid hyperplasia. After a variable follow up the girls are at present asymptomatic, although none of them has been able to completely give up the pharmacological treatment. CONCLUSIONS: Thymectomy is one of the mainstays of treatment for JMG. The ever more frequent use of videothoracoscopic techniques achieves results that are similar to those obtained by conventional surgery but with fewer post operative and aesthetic problems

[Neurological involvement in rheumatic disorders and vasculitis in childhood]. / Afectación neurológica en las enfermedades reumáticas y vasculitis de la infancia.

INTRODUCTION: The rheumatic disorders and vasculites are inflammatory processes affecting connective tissue in different organs. The inflamed cells liberate destructive enzymes which harm tissues, producing IgE, IgM, IgG antibodies and autoimmune complexes. OBJECTIVE: To review the current classification of these conditions and describe the commonest neurological complications in children. Infantile systemic lupus erythematosus is the commonest; it affects the dermis, joints, blood vessels, heart, kidney and brain. Neurological features: vertigo, ataxia, convulsions, chorea and headache are seen in 25% of the patients. Juvenile idiopathic arthritis is another inflammatory disorder of unknown origin which affects joints, connective tissue and visceras. Neurological involvement is seen in 5%, in the form of cerebral vasculitis, cervical myelopathy, trapping and compression of peripheral nerves and neuropathy due to arteritis. Sydenham s rheumatic chorea, caused by b haemolytic streptococcal A infection, causing a pathological auto immune response is the best known and has increased in frequency again in recent years. Vasculitis is caused by inflammatory changes in the walls of large, medium and small blood vessels. They lead to deposits of auto immune antigen antibody complexes in the blood vessel walls. In the commonest infantile vasculites neurological complications are uncommon, but not rare, as occurs with Kawasaki disease, infantile Wegener s granulomatosis, Schönlein-Henoch purpura and Behçet's disease. The usual treatment for this group of disorders is corticosteroids and immunosuppressive drugs.

[Abnormal antioxidant system in inborn errors of intermediary metabolism]. / Alteraciones del sistema antioxidante en errores cong nitos del metabolismo intermediario.

INTRODUCTION: Oxidative stress may be implied in the pathogenic mechanisms of inborn errors of intermediary metabolism (IEIM). OBJECTIVE: The evaluation of the antioxidant status in IEIM by the measurement of erythrocyte antioxidant enzyme activities, superoxide dismutase (SOD), glutathione peroxidase (GPx), glutathione reductase and catalase. PATIENTS AND METHODS: 34 patients with IEIM: 1) eleven with organic acidurias on protein restricted diet; 2) nine without special diet; 3) five patients with aminoacidopathies on protein restriction; 4) three patients with galactosemia and six with aminoacidopathies on protein free diet. Erythrocyte antioxidant enzymes were measured by spectrometric procedures adapted to the Cobas Fara II analyser. RESULTS: SOD activity was significantly higher in groups 2 and 4 (p= 0.009, p= 0.001, respectively), and significantly lower in group 3 (p= 0.001) compared with age matched controls. SOD activity was significantly higher in the patients with IEIM on protein free diet (groups 2 and 4) compared with those on protein restricted diet (groups 1 and 3; p= 0.002) or with controls (p= 0.003). GPx activity was found significantly lower in group 1 patients (p= 0.004), and higher in group 2 (p= 0.029) compared with controls. CONCLUSIONS: 35% of the patients with IEIM had SOD activity above the control range, most of them with organic acidurias or homocystinuria, suggesting an induction of enzyme protein synthesis owing to an excess of free radical generation. The lower activities observed in patients on natural protein restriction may likely be due to a deficient bioavailability of antioxidant cofactors.

Timectomía en la miastenia grave juvenil / Thymectomy in juvenile myasthenia gravis

Introducción. La miastenia grave juvenil (MGJ) es una enfermedad autoinmune infrecuente, cuya clínica y manejo terapéutico no difiere de las formas del adulto. El tratamiento crónico con corticoides provoca, con gran frecuencia, efectos adversos en la infancia, por lo que se recurre a la timectomía cada vez a edades más tempranas y con mejores resultados. Objetivos. Analizar el perfil clínico y evolutivo de la MGJ tratada mediante timectomía en nuestro centro. Pacientes y métodos. Presentamos cuatro niñas, de edades comprendidas entre los 5 y los 13 años, diagnosticadas de miastenia grave generalizada con afectación bulbar. Una de ellas debutó con una crisis miasténica. En todas se realizó: test de Tensilon, estudio electrofisiológico, determinación de AChR, TAC torácica y estudio de autoinmunidad y función tiroidea. Tras la cirugía, el timo se analizó histológicamente. Resultados. Todas presentaron un test de Tensilon positivo y seropositividad para AChR. Recibieron trata miento con anticolinesterásicos, hasta la dosis máxima tolerada, y corticoides, sin llegar a remisión completa, por lo que se timectomizaron en el primer año de evolución. En tres casos se realizó un abordaje transesternal y en uno por videotoracoscopia. Todos los timos mostraron hiperplasia linfoide. Tras un seguimiento variable, las niñas se encuentran actualmente asintomáticas, aunque ninguna ha podido abandonar completamente el tratamiento farmacológico. Conclusiones. La timectomía es uno de los pilares del tratamiento de la MGJ. La utilización cada vez más frecuente de la videotoracoscopia consigue resultados similares a los de la cirugía convencional, con menos problemas postoperatorios y estéticos (AU)

Alteraciones del sistema antioxidante en errores congénitos del metabolismo intermediario / Abnormal antioxidant system in inborn errors of intermediary metabolism

Introducción. El estrés oxidativo puede implicarse en los mecanismos de patogenia de los errores congénitos del metabolismo intermediario (ECMI). Objetivo. Para evaluar el estado antioxidante en estas enfermedades analizamos las actividades eritrocitarias de las enzimas antioxidantes, superóxido dismutasa (SOD), glutatión peroxidasa (GPx), glutatión reductasa y catalasa, en 34 pacientes con ECMI. Pacientes y métodos. Distribuimos a los pacientes en cuatro grupos: 1. Once con acidurias orgánicas, tratados con dietas restringidas en proteínas naturales; 2. Nueve sin dieta especial; 3.Cinco pacientes con aminoacidopatías, en tratamiento con restricción proteica; y 4. Tres pacientes con galactosemia y seis con aminoacidopatías, con dieta proteica libre. Las enzimas antioxidantes eritrocitarias se analizaron por procedimientos espectrométricos adaptados al analizador Cobas Fara II. Resultados. La actividad SOD fue significativamente más elevada en los grupos 2 y 4 (p= 0,009, p= 0,001) y más disminuida en el grupo 3 (p= 0,001), comparada con los controles. La actividad SOD se halló significativamente más elevada en los pacientes tratados con dieta proteica libre (grupos 2 y 4), comparados con los tratados con restricción proteica (grupos 1 y 3; p= 0,002) o los controles (p= 0,003). Conclusión. El 35 por ciento de los pacientes con ECMI mostraron una actividad SOD superior al intervalo control, la mayoría de ellos con acidurias orgánicas u homocistinuria, lo que sugiere una inducción de la síntesis de la proteína enzimática debido a un exceso de producción de radicales libres. Las bajas actividades enzimáticas observadas en pacientes con restricción proteica podrían atribuirse a una biodisponibilidad deficiente de cofactores antioxidantes (AU)

[Proposed protocol for the study of cerebrovascular disease in childhood]. / Propuesta de protocolo de estudio de las enfermedades cerebrovasculares de la infancia.

AIM: The etiology of cerebrovascular disease in the paediatric population, remains unknown in up to 40% of the cases ("idiopathic"), but recent advances could improve this percentage. We devised a comprehensive study protocol for such investigation aimed at the identification of potentially modifiable risk factors for paediatric stroke. PATIENTS AND METHODS: From the 141 patients initially registered in our data base for stroke population (from January 1984 until December 1995), we invited all the patients with idiopathic cerebrovascular disease to complete the study protocol. New cases appeared from January 1996 until July 1999 were also included. RESULTS: A total of 68 cases were identified. We found an etiology in 38% and in 76% of the cases we found at least one risk factor for stroke. Mild hyperhomocysteinemia was the most frequent risk factor identified (36% of patients versus 5% of controls), one of them an infant with fatal haemorrhagic infarct with classic homocystinuria. 31% of the patients had thrombotic risk factors (protein S, protein C, antithrombine III deficiency, factor V Leiden, etc). 17.6% had unspecific febrile illness at the time of the cerebral infarction and 11.6% had minor head injuries before the stroke. CONCLUSIONS: The use of the protocol improves the identification of potentially modifiable risk factors for stroke in childhood and may serve as a practical guideline for clinicians. The stroke protocol is as important as management strategies for acute stroke or for recurrence prevention, currently under consideration in the adult population.

Propuesta de protocolo de estudio de las enfermedades cerebrovasculares de la infancia / Proposed protocol for the study of cerebrovascular disease in childhood

OBJETIVO: La etiología de la enfermedad cerebrovascular en la población pediátrica permanece sin aclarar hasta en un 40 por ciento de los casos. Recientes avances en el conocimiento de la etiopatogenia pueden mejorar este porcentaje. Pretendemos diseñar un protocolo de estudio estructurado con el objetivo de identificar los factores de riesgo de enfermedad cerebrovascular potencialmente modificables. PACIENTES Y MÉTODOS: En nuestra base de datos constaban 141 pacientes con enfermedad cerebrovascular (desde enero de 1984 hasta diciembre de 1995); se invitó a los pacientes con enfermedad cerebrovascular idiopática a completar el protocolo de investigación. También se incluyeron los nuevos casos que aparecieron desde enero de 1996 hasta julio de 1999. RESULTADOS: Se estudiaron en total 68 casos. Se realizó un diagnóstico en el 38 por ciento de los pacientes, y en el 76 por ciento de los casos se identificó algún factor de riesgo. El factor de riesgo vascular no estructural más frecuente fue la hiperhomocisteinemia (36 frente al 5 por ciento de los controles), entre ellos un lactante con infarto hemorrágico fatal que se diagnosticó de homocistinuria clásica. Un 31 por ciento de los pacientes tenían factores pretrombóticos (déficit de proteína S, C, antitrombina III, factor V de Leiden, etc.). El 17,6 por ciento padecía un proceso febril inespecífico en el momento del infarto y el 11,7 por ciento refería historia de traumatismo craneal menor previo al infarto. CONCLUSIÓN: La aplicación de un protocolo para el estudio de la enfermedad cerebrovascular mejoró el porcentaje de casos diagnosticados, así como la identificación de factores de riesgo de enfermedad cerebrovascular potencialmente modificables. El protocolo de investigación es tan importante como las estrategias de actuación en la fase aguda o la prevención de recurrencia, actualmente en estudio para la población adulta (AU)

Pathology of the elastic tissue of the skin in Costello syndrome. An image analysis study using mathematical morphology.

Only nine cases of Costello syndrome have been described so far. No definitive pathologic abnormalities have been found on skin biopsies. We undertook a quantitative study of the elastic tissue of the skin in one case of Costello syndrome in order to obtain objective scores of area, fragmentation, branching and texture of the elastic tissue, comparing it to cutis laxa syndrome, Ehlers-Danlos syndrome and normal skin. The results showed an increasing fragmentation score and loss of anastomosing points in elastic tissue in Costello syndrome and cutis laxa syndrome in relation to normal skin. Thus, we conclude that image analysis revealed, in Costello syndrome, a state of disorder akin to that of cutis laxa syndrome. Therefore, both syndromes might have a common pathogenesis as far as elastic tissue is concerned. However, other cases should be analyzed to validate our conclusions on statistical grounds.

[Alternating hemiplegia in infancy: clinical features, clinical course and treatment based on three cases]. / Hemiplejía alternante en la infancia: forma de presentación, evolución y tratamiento en tres observaciones.

Alternating hemiplegia is an infrequent form of complicated migraine. Clinical course has similarities with seizure disorders and correct diagnosis may be difficult. We report three patients whose onset in early childhood was with general impairment, transient hemiplegia, ocular movements and vasomotor symptoms. Clinical course of alternating hemiplegia is characterized by progressive neurologic deterioration. Intermittent motor impairment is alternating in side and later during the episodic attacks headache is present. Laboratory, electrophysiologic and neuroradiologic procedures are not demonstrative. In this report we show the findings in three patients in relation to the symptoms they presented, the utility of paraclinical investigations and their response to flunarizine treatment.

[Idiopathic inflammatory myopathies in childhood. A study of 7 patients]. / Miopatías inflamatorias idiopáticas en la infancia. Estudio de siete pacientes.

Five patients with dermatomyositis and 2 with polymyositis between 3 and 12 years old are reviewed. All of them fulfil the Bohan and Peter diagnostic criteria. Five presented misery before weakness. Two presented acute renal failure. The pathologic muscular study was not always specific of inflammatory myopathy and without correlation with the degree of symptoms. Treatment which prednisone and in one patient also azathioprine resulted with complete remission in 4 patients.