Pseudoabsceso cerebral en meningitis neonatal por Enterobacter agglomerans / Cerebral pseudo-abscess in neonatal meningitis due to Enterobacter agglomerans

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[Congenital brachial palsy: its epidemiology and sequelae. A retrospective study of our case mix]. / Parálisis braquial obstétrica: epidemiología y secuelas. Estudio retrospectivo de nuestra casuística.

AIM: To conduct a retrospective study of cases of congenital brachial palsy, focusing on its incidence, clinical manifestations and long-term course. PATIENTS AND METHODS: A systematized study of all the cases of congenital brachial palsy detected in the Hospital La Paz between January 1994 and December 2003 was carried out with the aim of recording data on the pregnancy, gestational age, type of delivery, presentation, sex and weight at birth, Apgar test and the arterial pH of the umbilical cord. It also sought to estimate the type of brachial palsy, the side that was affected, electromyographic findings, associated insults, treatment and progress. RESULTS: Congenital brachial palsy was diagnosed in 48 patients. All the children, except one, were born full term and in over half the cases (54.1%) weight at birth was above average. In 43 cases presentation was cephalic and in the other 5 it was footling. Twenty-nine cases (60.5%) were classified as mild, 12 were moderate (25%) and 7 were severe (14.5%). Electromyogram studies were carried out in 19 patients (39.5%) and the following insults were found: severe axonotmesis in the 7 severe patients, moderate axonotmesis in the 10 moderate patients, and neuroapraxia and mild axonotmesis in the 2 mild patients. CONCLUSIONS: The incidence of congenital brachial palsy in our hospital was 0.6 cases/1000 births/year. Proximal brachial palsy was the commonest disorder, although in most cases patients were only mildly affected and were free of sequelae when discharged. Functional limitations persisted, however, in 36.6% of the cases despite treatment being established at an early stage.

Pseudotumor cerebri secundario a tratamiento con risperidona / Pseudotumor cerebri as side effect of treatment with risperidone

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Síndrome de Weber en un niño tras corrección quirúrgica del desplazamiento de un catéter intracraneal / Weber's syndrome in a child following surgery to correct displacement of a catheter

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Disfunción faríngea congénita aislada / Congenital isolted pharyngeal dysfunction

Presentamos el caso de un paciente con disfagia congénita, cuyas características se corresponden con las de la disfunción faríngea congénita aislada: episodios de atragantamiento y/o aspiración con regurgitación nasal durante las tomas, facies hipomímica con ligeros rasgos dismórficos y moderado retraso psicomotor, sin apenas reseñable hipotonía. En él fueron excluidas las causas anatómicas o neurológicas de disfagia. Su evolución fue algo tórpida, con procesos respiratorios repetidos, y se apreció una clara mejoría a partir del año de edad. Su hermano presentó síntomas similares más leves, lo que apoyaría el posible carácter hereditario de esta enfermedad

We report the case of a patient with congenital dysphagia in which the clinical features were similar to those associated with congenital isolated pharyngeal dysfunction: episodes of airway obstruction and aspiration events with nasal regurgitation during oral feeding, hypomimic face with mildly dysmorphic features and slightly retarded psychomotor development; hypotonia was nearly undetectable. There was no evidence of anatomical or neurological causes. The patient suffered repeated episodes of respiratory distress, with clear improvement of all his symptoms after his first birthday. His brother developed similar, although milder, symptoms, a circumstance that indicates the hereditary nature of this condition

[Atrophy of the cervical spinal cord as a consequence of a prenatal ischemic infarct]. / Atrofia de la medula espinal cervical tras infarto isquemico de origen prenatal.

INTRODUCTION: Spinal cord injuries in newborn infants following a traumatic delivery are well known, but occasionally cord damage is appreciated in children whose birth did not involve any complications; in some of them there has been proof of an ischemic process that originated inside the uterus. CASE REPORT: We describe the case of a newborn female with atrophy of the cervical spinal cord compatible with an intrauterine ischemic infarct. It extended to the C4-C6 territory of the anterior spinal artery, with greater participation on the left side. Clinical symptoms were apparent from the moment of birth and took the form of an asymmetric pseudoarthrogryposis in the upper limbs. The left arm was held in abduction at the height of the shoulder, with complete flexion of the elbow and the hand at the height of the chin was hypotonic, inactive and hypotrophic; the right arm was extended and in adduction. The patient presented episodes of hyperthermia that remitted on cooling the body. CONCLUSIONS: When faced with a newborn infant with symptoms that are compatible with chronic cervical spinal cord damage and in the absence of any perinatal traumatic injury, other causes, including prenatal ischemic infarct, must be taken into account.

[Is benign childhood paroxysmal eye deviation a non-epileptic disorder?]. / Desviación ocular paroxística benigna infantil: un trastorno no epiléptico?

INTRODUCTION: Benign childhood paroxysmal eye deviation (BCPED) is classified as a 'non-epileptic paroxysmal disorder'. CASE REPORTS: We report the cases of four patients aged between 6 months and 2 years, who suffered brief episodes of upward conjugate gaze deviation, with no clonic movements or associated cognitive deterioration. These episodes, which lasted several seconds, appeared in short repeated bouts that became worse with fatigue. Results of the neurological exploration, laboratory examinations, neuroimaging (CAT, MRI, brain ultrasonography) and a neurophysiological study, which included EEG-video monitoring and EEG performed during the waking state, were all normal. A nocturnal polysomnographic study was later conducted for 7-8 hours and EEG, EMG and EOG readings were recorded. The trace showed focal or generalised paroxysmal discharges during non-REM sleep in the form of polyspike-wave and spike-wave complexes. Sleep analysis (Reschstaffen and Kales) showed only a shortened REM sleep latency, with no clear clinical meaning. Several cases have been reported in the literature with identical symptoms and normal results in the diagnostic tests, including daytime polysomnography. CONCLUSIONS: The appearance of these epileptic anomalies in the nocturnal study makes it necessary to perform a complete nocturnal polysomnography. In spite of these findings, BCPED courses favourably and has a benign prognosis both with and without antiepileptic treatment. We therefore believe that BCPED should be classed within the group of 'benign idiopathic epilepsies of childhood'.

Episodios de crisis convulsiva y hemiplejía / Episodes of convulsive crisis and hemiplegia

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Síndrome displasia coriorretiniana, microcefalia y retraso mental con linfedema / Chorioretinal dysplasia-microcephaly-mental retardation syndrome

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[Severe myoclonic epilepsy in infancy. Clinical and paraclinical aspects]. / Epilepsia mioclónica severa de la infancia. Aspectos clínicos y paraclínicos.

INTRODUCTION: Severe myoclonic epilepsy in infancy (SMEI), or Dravet s syndrome, is one of the most serious forms of epilepsy in infancy. In this study we analyse the clinical characteristics of the process. PATIENTS AND METHODS: The cases reported in the literature are surveyed, together with a personal casuistic, from both a clinical and paraclinical point of view, and we assess the form of onset and the clinical, EEG and neuroimaging manifestations at different ages. RESULTS: In most cases the disorder is characterised by onset during the first year of life, with febrile seizures, normal development prior to the onset of the seizures, multivariate critical phenomenology throughout the progression, early resistance to treatment, initial normality of EEG results and progressive neurological deterioration with ataxia and long tract signs. CONCLUSIONS: The diagnosis of SMEI depends on the combination of clinical manifestations and EEG at different ages, and the presence of myoclonic seizures constitutes the most significant fact. The lack of strict diagnostic criteria allows for the existence of cases that are not perfectly identified. A percentage of cases exist that do not fulfil all the abovementioned criteria. The recent description of a mutation in the alpha subunit of a neuronal voltage dependent sodium channel (SCN1A) in chromosome 2q24, as the likely source of the process, will allow screening to be carried out in the early phases of the disorder. It will also allow studies to be conducted on the phenotype genotype correlation of the disease.

[The prevalence of headache in a population of patients with coeliac disease]. / Prevalencia de cefalea en una población de enfermos celíacos.

INTRODUCTION: Headache is the commonest clinical symptom during childhood and adolescence, from a neurologist s point of view. The pathophysiology of migraine and tension headache involve personality and biochemical factors, such as serotonin, which are also common in coeliac disease. OBJECTIVE: To establish the prevalence of headache in children and adolescents with coeliac disease, and any possible relation between these conditions. PATIENTS AND METHODS: We made a randomized selection of 86 patients with coeliac disease. All were on gluten-free diets and had no current gastroenterological symptoms. They had direct interviews and full physical and neurological examinations. The diagnosis of headache was based on criteria of the International Headache Society in 1988. RESULTS: The average age was 12.71 +/- 4.5 years (range 5-24). Headache occurred in 34 (39.5%) of the 86 patients studied. In 18 cases (20.9%) headache was of tension type and in 16 (18.6%) of migraine type. Of the latter, 10 cases had auras and 6 did not. There was no significant sex difference. CONCLUSIONS: An increased prevalence of both migraine and tension headaches was observed in the coeliac patients studied as compared with data published in the literature. In the former patients there was also a lower frequency in histories of migraine in first degree family members. This data is probably related to the personality of the patient or to his family or social circumstances in the case of tension headaches. In the case of migraine it may be due to biochemical factors such as a lowered plasma serotonin, seen both in coeliac disease and in migraine.

[Facial asymmetry with crying: a neurophysiological study and clinical account of this entity]. / Asimetría facial con el llanto: estudio neurofisiológico y recuerdo clínico de esta entidad.

OBJECTIVE: The objectives of this study were to determine in our newborn population the frequency of facial asymmetry during crying or congenital hypoplasia of the depressor anguli oris muscle (DAOM) without associated major malformations and to evaluate the contribution of electrophysiological studies in the diagnosis of this anomaly. PATIENTS AND METHODS: We performed a prospective study of the living newborns with facial asymmetry which did not need to be admitted to the hospital and that were delivered without the use of forceps in our hospital during 4 consecutive years. Newborn evaluation included physical examination, facial nerve electroneurography and DAOM electromyography. RESULTS: This anomaly without major malformations was present in 0.348/1,000 newborns. Electrophysiological studies demonstrated a normal latency in the facial nerve electroneurography and an absence or reduced number of motor unit potential without signs of denervation in the electromyography. CONCLUSIONS: Diagnosis of this entity is important to exclude facial nerve palsy. Only a physical examination is necessary for a correct diagnosis.