[Is the incidence of parapneumonic pleural effusion increasing?]. / Está aumentando la incidencia de derrames pleurales paraneumónicos?

INTRODUCTION: Streptococcus pneumoniae is the microorganism most frequently associated with complicated pleural effusion. After the introduction of the heptavalent pneumococcal vaccine, there was a decline in the incidence of invasive pneumococcal disease and, to a lesser extent, in that of pneumonia. However, the incidence of empyema apparently increased. The antipneumococcal heptavalent vaccine was introduced in Spain in 2001. OBJECTIVES: To determine whether the incidence of pleural effusion secondary to pneumonia has increased in hospitalized patients and to examine the possible influence of the antipneumococcal heptavalent vaccine on the incidence rate of parapneumonic effusions. PATIENTS AND METHODS: Patients aged less than 16 years old admitted to our hospital with a diagnosis of pneumonia between 1999 and 2005 were retrospectively reviewed. We calculated the annual incidence rate of pleural effusion with respect to the total number of patients admitted with pneumonia and with respect to patients considered to have probable bacterial pneumonia, based on previously established criteria. RESULTS: A total of 337 patients were analyzed, of which 213 (63.2%) met the criteria for a diagnosis of probable bacterial pneumonia. Pleural effusion was found in 34 patients (15.9%), and 13 of these effusions (38%) were complicated. No clear trend was detected in the annual incidence of probable bacterial pneumonia per 100 admitted patients, although the highest numbers were detected in the last two years of the study period. The percentage of complications (effusions) remained constant (mean: 16.28%). No differences were found in the effusion rate between vaccinated and unvaccinated patients (12.5% vs 18.6%). CONCLUSIONS: The trend in the incidence of parapneumonic pleural effusions was parallel and proportional to that of probable bacterial pneumonia.

[Scrotal hair in infants]. / Vello escrotal en lactantes.

The presence of pubic hair is exceptional in healthy infants of both sexes. In most of the cases described in the literature, the process was self-limited and no etiology was found. Nevertheless, in some patients, this finding has been associated with other manifestations of hyperandrogenism related to potentially serious diseases such as congenital adrenal hyperplasia or virilizing tumors. In the present article, we describe seven infants followed-up in the Pediatric Endocrine Clinic because of scrotal hair. In all patients, the process was self-limited and resolved spontaneously and no hormonal or developmental alterations were observed. Key words: Scrotal hair, infants, virilization.

[The clinical semiology of attention deficit hyperactivity disorder according to age, and the effectiveness of treatments at different ages]. / Semiología clínica del trastorno por déficit de atención con hiperactividad en función de la edad y eficacia de los tratamientos en las distintas edades

INTRODUCTION: Attention deficit hyperactivity disorder (ADHD) is the most important cause of poor academic performance and is also usually associated with behavioural, emotional and sociability disorders. AIM: To analyse the different clinical features and the response to psychostimulant therapy, according to the age. SUBJECTS AND METHODS: We reviewed the cases of 152 children referred to Neuropaediatrics owing to suspected ADHD over a 6-year period. Diagnosis and comorbidity were investigated by the looking at their case history, by examination and observation, as well as by interviewing the parents (DSM-IV criteria), and by applying Conners' Rating Scale for parents and teachers, in addition to neuropsychological tests. In order to improve data processing, we established four groups according to the patient's age at diagnosis and when treatment was started: G1: 3-5; G2: 6-8; G3: 9-11; and G4: 12-15 years. RESULTS: ADHD was diagnosed in 102 of the 152 children; 53% had the combined type, 26% were of the inattentional type, and 20% were hyperactive/impulsive. Poor school performance increased with age and reached 75% in G4. The same occurred with conduct disorders: at 6 years of age, 23% were found to have oppositional defiant disorder, and from the age of 9 onwards over 60% of them had conduct disorders. Up until 12 years of age they showed some improvement with psychostimulants and later the rate of dropouts from medical clinical controls and from treatment was over 40%. CONCLUSIONS: The older patients are and the later ADHD is attended, the more problems they have. If successful preventive measures are to be implemented, it would be advisable to begin treatment even before children start primary education (5 years old), so as to try and avoid the pedagogical and behavioural repercussions observed in these children at the age of 6.

[Variability in the treatment of headaches in visits to the Neuropaediatrics service at six Spanish hospitals]. / Variabilidad en el tratamiento de las cefaleas en las consultas de Neuropediatría de seis hospitales españoles.

INTRODUCTION: Headache is one of the chief reasons for visiting Neuropaediatric units. Variability in clinical practice has been defined as variations in the rates a clinical procedure is employed. The aim of this work is to study the variability in the treatment of children's headaches in six Spanish hospitals. PATIENTS AND METHODS: We conducted a retrospective study involving patients who first visited because of a headache some time in the year 1999. Data were collected in December 2003. The following variables were analysed: type of headache, neuroimaging scans, beginning of prophylaxis, follow-up visits to clinic and number of patients who dropped out of the follow-up. RESULTS: In all, 372 children were attended. 63.4% of the headaches were considered as being migraine-type; 43% of the patients were submitted to neuroimaging and prophylaxis was established in 36.8%. Mean follow-up time in the clinic was 10.6 months and the mean number of visits was 3. 30% had dropped out of the follow-up. In the comparative analysis, significant differences were found in all the variables studied. CONCLUSIONS: Overall results are similar to those found in other series. The differences found between centres can be accounted for by differences in population, differences in health professionals' styles of medical practice or by the nonexistence of clear guidelines regarding a particular procedure.

[Neurocutaneous melanosis]. / Melanosis neurocutánea.

Neurocutaneous melanosis is a sporadic neurocutaneous syndrome characterized by the presence of giant or multiple melanocytic nevi and excessive proliferation of melanotic cells in the central nervous system. Patients usually consult dermatologists because the congenital cutaneous nevi. Most neurologic symptoms appear in the first two years of life and the prognosis in such cases is poor, especially if they are caused by hydrocephalus, which is the main complication. All patients with giant or multiple melanocytic nevi should be seen frequently to detect promptly the eventual development of neurologic involvement.

[Menkes' disease: anatomo-clinical presentation of a case]. / Enfermedad de Menkes: presentación anatomoclínica de un caso.

We describe a case of Menke's disease with severe neurological involvement, convulsive crises and characteristic hair anomalies (scanty, fragile, macroscopically hypopigmented and microscopically kinked) which led to rapid diagnosis. Vascular abnormalities with elongated, twisted arteries, skeletal abnormalities (more wormian cranial bones than usual, lateral spurs of metaphyses) and vesicle diverticuli. Electron microscopy of skeletal muscle showed concentrically laminated bodies, possibly of mitochondrial origin. Respiratory chain enzyme activity was normal. The patient died at the age of two and a half. On necropsy, histological abnormalities characteristics of the illness were seen (loss of neurones in the granular layer of the cerebellum, the neurones of Purkinje had thickening of the dendrites which spread out in the form of a weeping willow, reduplication and fragmentation of the internal elastic layer of muscle arteries). In the cortex of the cerebellum mega-mitochondria with electron-dense bodies, were seen on electron microscopy. This is the first case of Menke's disease described in the Spanish literature which includes pathology and electron microscope studies.

[Unilateral agenesis of the internal carotid artery in childhood: description of a case]. / Agenesia unilateral de la arteria carótida interna en la infancia: descripción de un caso.

INTRODUCTION: Unilateral or bilateral agenesis of the internal carotid artery has not often been described in children since it is usually asymptomatic. CLINICAL CASE: We describe a case of isolated unilateral agenesis of the internal carotid artery in a child of 20 months old who presented with two episodes of status convulsives, the first after a head injury and the second when he was febrile. The anomaly was a casual finding, diagnosed on cerebral magnetic resonance when there was no signal corresponding to carotid blood flow. It was confirmed by echography-doppler and angiographic magnetic resonance. We also describe the neuro-radiological findings which led to the diagnosis of this anomaly. CONCLUSIONS: We consider that unilateral agenesis of the carotid artery was a casual finding in this case. The provisional diagnosis may be made when there is absence of vascular signals on conventional magnetic resonance.

[Diagnosis in severe myoclonic epilepsy in childhood: study of 13 cases]. / Diagnóstico en la epilepsia mioclónica severa de la infancia: estudio de 13 casos.

OBJECTIVE: The purpose of this report is to describe clinical pattern, EEG, outcome and differential diagnosis in severe myoclonic epilepsy in infancy (SMEI). MATERIAL AND METHODS: We report 13 cases initially diagnosed of SMEI and selected according to the following criteria: first seizure between 1 and 12 months of life, frequent seizures resistant to antiepileptic drugs, no previous personal history of disease, normal psychomotor development before the first seizure and normal EEG, CT scanning and laboratory analyses at the beginning. CT and/or MRI were performed in 13 cases, arteriography in 2 patients, MR spectroscopic imaging in 1 child and SPECT in 3 cases. Quantification of enzymatic activities of the mitochondrial respiratory chain was made in 5 patients. RESULTS: Only 8 cases were finally diagnosed of SMEI according to ILAE definition. In two cases, seizures were finally controlled with antiepileptic treatment and EEG abnormalities disappeared. Three patients showed other findings: mesiotemporal sclerosis, angiitis diffusely involving CNS and mitochondrial cytopathy with deficiency of the complex IV. CONCLUSIONS: Although diagnosis of SMEI, based on clinical manifestations, is suspected in most cases from the first year of life, final diagnosis should not be confirmed until steady state, when polymorphous seizures occur. Even then, differential diagnosis should be made with other disorders. Perhaps, further studies should be performed in order to identify and eliminate another etiology.

[Clinical progress of neonatal non-ketotic hyperglycinemia under treatment]. / Evolución de la hiperglicinemia no cetósica neonatal en tratamiento.

INTRODUCTION: Non-ketotic hyperglycinemia is a congenital error in the breakdown of glycine. The most common type is the classical neonatal form, which begins at the age of a few days with symptoms of lethargy, hypotonia, myoclonia, convulsions, apneas and, frequently, ends in death. Survivors usually develop intractable epilepsy and mental retardation. There is no effective treatment for this condition, but trials have been carried out with a therapy that diminishes the levels of glycine, benzoate (BZ), and another that blocks the excitatory effect in N-methyl-D-aspartate receptors: dextromethorphan (DTM). CASE REPORT: We report on the progress of a classical neonatal case, which began at the age of a few hours with hypotonia and stupor, without myoclonias or seizures, but with a suppression wave trace on the electroencephalogram (EEG). Cerebrospinal fluid (CSF) showed glycine levels of 141 micromol/L (the normal level is 6.66 +/- 2.66 micromol/L), with a CSF/plasma ratio of 0.19 (the normal ratio is < 0.02). Treatment was started on the thirteenth day with BZ and DTM, and alertness and eye fixation improved in just three days; at the same time the EEG readings become normal. The glycine level in plasma returned to normal at two months and that in CSF was considerably reduced, although with CSF/plasma levels that were still high. At present the patient is 4 years old, has never had convulsions, EEG results have always been normal, and continues with BZ, DTM, carnitine and diet. The patient has presented a high degree of hypermotoric behaviour, but is currently more attentive and more sociable, has been walking from the age of 35 months and has a quotient in the different areas of development of 40-50. CONCLUSIONS: The clinical progress made by our patient could be said to be anything but negligible, and we therefore recommend that treatment should be started as early as possible after diagnosis.

[Growth charts for the Spanish population with neurofibromatosis type 1]. / Gráficos de crecimiento de la población española con neurofibromatosis tipo 1.

OBJECTIVE: To make the charts of stature and head circumference of Spanish pediatric patients affected with neurofibromatosis type 1 (NF1), to compare them with the ones from a healthy population and to review the possible causes of its alterations. PATIENTS AND METHODS: We obtained the data from 251 Spanish patients (122 women and 129 men) with NF1 seen in seven hospitals between the years 2000 and 2002, with ages between 1 month and 18 years old. The calculation of the 50th centile or median was done using the method of mobile variables, and the 3rd and 97th centiles where calculated from this median. RESULTS: Girls with NF1 have a shorter stature than the healthy ones, which becomes evident after 10 years of age. We have not found differences in the stature between boys with the disease and those without it. In regards to head circumference, in boys and girls we have observed that it is bigger throughout life in patients affected with NF1, and that the 50th centile of healthy people correlates with the 3rd centile of those affected, the 97th centile of the healthy ones with the 50th centile of those affected, being the 3rd centile of the healthy people well below the 3rd centile of the affected ones, and the 97th centile of the affected ones well above the 97th of the healthy people. CONCLUSIONS: The short stature in postpuberal girls and the macrocephaly in both sexes at all ages, are primary characteristics in the NF1 and they are not usually related with other alterations, although they are not specific for this illness.

Response to methylphenidate by adult and pediatric patients with attention-deficit/hyperactivity disorder: the Spanish multicenter DIHANA study.

BACKGROUND: The purpose of this multicenter Spanish study was to evaluate the response to immediate-release methylphenidate by children and adults diagnosed with attention-deficit/hyperactivity disorder (ADHD), as well as to obtain information on current therapy patterns and safety characteristics. METHODS: This multicenter, observational, retrospective, noninterventional study included 730 patients aged 4-65 years with a diagnosis of ADHD. Information was obtained based on a review of medical records for the years 2002-2006 in sequential order. RESULTS: The ADHD predominantly inattentive subtype affected 29.7% of patients, ADHD predominantly hyperactive-impulsive was found in 5.2%, and the combined subtype in 65.1%. Overall, a significant lower Clinical Global Impression (CGI) score and mean number of DSM-IV TR (Diagnostic and Statistical Manual of Mental Disorders Fourth Edition, Text Revision) symptoms by subtype were found after one year of treatment with immediate-release methylphenidate; CGI decreased from 4.51 to 1.69, symptoms of inattention from 7.90 to 4.34, symptoms of hyperactivity from 6.73 to 3.39, and combined subtype symptoms from 14.62 to 7.7. Satisfaction with immediate-release methylphenidate after one year was evaluated as "very satisfied" or "satisfied" by 86.90% of the sample; 25.75% of all patients reported at least one adverse effect. At the end of the study, 41.47% of all the patients treated with immediate-release methylphenidate were still receiving it, with a mean time of 3.80 years on therapy. CONCLUSION: Good efficacy and safety results were found for immediate-release methylphenidate in patients with ADHD.

Levetiracetam as an alternative therapy for Tourette syndrome.

Tourette syndrome is a common childhood-onset neuropsychiatric disorder characterized by chronic tics and frequent comorbid conditions such as attention deficit disorder. Most currently used tic-suppressing drugs are frequently associated with serious adverse events. Thus, alternative therapeutic agents with more favorable side-effect profiles are being evaluated. New hypotheses and recent studies involving GABAergic system in the pathophysiology of Tourette syndrome suppose a reason for the evaluation of GABAergic drugs. Levetiracetam is a drug with an atypical GABAergic mechanism of action that might be expected to improve tics. Although trials performed to evaluate the efficacy of levetiracetam in the treatment of Tourette syndrome have provided conflicting results, it may be useful in some patients. The established safe profile of levetiracetam makes this drug an alternative for treatment if intolerance to currently used drugs appears, but additional evaluation with larger and longer duration controlled studies are necessary to assess the real efficacy in patients with Tourette syndrome.

[Coding in neuropediatrics based on the International Classification Diseases, 9th revision (ICD-9), 5th edition (2006)]. / Codificación en neurología pediátrica basada en la Clasificación Internacional de Enfermedades, 9.(a) revisión (CIE-9), 5.(a) edición (2006).

AIMS: To analyze International Classification Diseases, 9th revision (ICD-9) coding and adapt it, on a consensus basis, to 'reasons for medical consultation', 'diagnoses' and 'procedures' in child neurology. MATERIALS AND METHODS: The most frequent reasons for medical consultation, diagnoses and procedures in neuropediatrics were selected and assigned the most appropriate ICD-9, Clinical Modification (5th ed.) (ICD-9-CM) codes in accordance with this system's coding rules. Disorders were grouped by sections, and allocated to the various members of the working group (13 child neurologists from 10 hospitals in Madrid and environs). RESULTS: Available on the web www.neurologia.com/cie-9. ICD-9-CM codes were assigned to: 158 reasons for medical consultation; 886 diagnoses; 73 diagnostic procedures; and 53 therapeutic procedures. In every case, the most appropriate ICD-9 code was sought for the respective diagnosis. No codes were invented but the working group did take certain liberties with interpretation, which nevertheless showed respect for general ICD-9-CM philosophy and are described in full in the text. CONCLUSIONS: The creation of this ICD-9 adaptation will not only enhance diagnostic coding in child neurology departments, but will also provide them with a useful tool for setting up databases to enable information to be retrospectively analyzed and shared by the different health centers.

Lactancia materna y protección contra las infecciones respiratorias en los primeros meses de vida / Breastfeeding and the protection against respiratory infections in the first months of life

Introducción: entre las ventajas de la lactancia materna (LM) se incluye su papel protector ante infecciones respiratorias (IR). Objetivos: describir el patrón de LM de una cohorte de recién nacidos (RN) y su protección contra las IR en el lactante. Material y métodos: estudio prospectivo de 316 RN, de las áreas 8 y 9 de Madrid, desde el nacimiento mediante llamadas telefónicas quincenales durante el invierno, registrando datos clínicos y epidemiológicos. Si presentaban sintomatología compatible con IR se recogió aspirado nasofaríngeo. Resultados: mil ochocientas sesenta y cinco llamadas (mediana: 4,2 [1-11]) y 106 visitas programadas. Al mes, el 56,1% recibía LM exclusiva; a los tres meses, el 39,4%, y a los cinco meses, el 31,9%. La LM exclusiva se mantuvo 65,1 días (desviación estándar: 43,49) y ningún factor se asoció a una mayor duración. Hubo 89 episodios de IR aguda. Los hermanos escolares multiplican por 1,74 (intervalo de confianza del 95% [IC 95%]: 1,12-2,72) el riesgo de IR en todos los estratos de duración de LM exclusiva. El riesgo de infección se multiplica por cinco (IC 95%: 2,07-12,19) si la LM exclusiva dura menos de un mes, por 9,8 (IC 95%: 4,06-23,66) si dura entre 30 y 60 días y por 3,4 (IC 95%: 1,28-9,19) si dura entre 60 y 90 días. Conclusiones: aunque muchos RN inician LM exclusiva, solo la tercera parte la mantiene a los cinco meses. Ningún factor se asoció a mayor duración de la misma. La LM es un factor protector de IR cuando dura más de 90 días. Se debe fomentar el mantenimiento de la LM más allá del tercer mes, especialmente en aquellos con hermanos escolares (AU)

Background: the protective role against respiratory infections is included among the advantages of breastfeeding (BF). Objective: to describe the pattern of BF in a cohort of newborns and its protective role against respiratory infections (RI) in the infant. Material and methods: a cohort of 316 newborns (NB) from Madrid Health Districts 8 and 9 was followed by telephone calls every fortnight during the winter season recording clinical and epidemiological data. Nasopharyngeal aspirate was obtained in every patient with symptoms compatible with RI. Results: one thousand, eight hundred and sixty-five phone calls [median: 4.2 (1-11)], and 106 scheduled visits were conducted. At the age of 1 month 56.1% of the infants were exclusively breastfed, at 3 months 39.4% and at 5 months 31.9%. The duration of BF was 65.1 days (SD: 43.49) and no factors were associated to a longer duration. Eighty nine acute RI were registered. The presence of siblings in school age increased the risk of RI by 1.74 (CI 95%: 1.12-2.72) in all the strata of duration of exclusive BF. The risk of RI increased by 5 (CI 95%: 2.07-12.19) if breastfeeding lasted less than 1 month, by 9,8 (CI 95%: 4,06-23,66) between 30-60 days and by 3,4 (CI 95%: 1.28-9,19) if it lasted 60-90 days. Conclusions: though many NB begin BF, only a third part of them go on with it at the age of 5 months. No covariant was associated with a longer duration. BF is a protector factor against RI when it lasts more than 90 days. So it must be promoted specially in those infants with siblings in school age (AU)

[Macrocephaly the first manifestation of glutaric aciduria type I: the importance of early diagnosis]. / Macrocefalia como forma de presentación de la aciduria glutárica tipo 1. Importancia de un diagnóstico precoz.

INTRODUCTION: The clinical manifestations of glutaric aciduria type I (GA-I) usually develop during the first two years of life as acute encephalopathic crisis leading to irreversible dystonic. Progressive macrocephaly can be an early clinical sign. We report a 9 month old patient with macrocephaly diagnosed of GA-I in the presyntomatic stage. This early diagnosis and treatment avoided the irreversible neurologic damage associated to this disease. CASE REPORT: A 9 month old male referred to the pediatrics neurology clinic because of macrocephaly with a head circumference of 51 cm (> 97th percentle). At birth this head circumference was 37.5 cm (97th percentile) and showed rapid growth during the first 4 months of life. In the physical exam there was mild hypotonia and no other neurologic alterations with normal psychomotor development. In the work up for macrocephaly urinary organic acids were determined showing a glutaric acid: 78,000 mmol/mol creatinine (normal values: 2-10); 3-hydroxyglutaric acid: 250 mmol/mol creatinine (normal values: 1-12). Cerebral magnetic resonance (MR) performed at 12 months of age showed frontotemporal atrophy with enlargement of subarachnoid spaces, a high signal in T2 in the pallidus nucleus and subdural hematomas. Genetic analysis showed a mutation S 305 L/Q 352 X in GCDH gene. L-carnitine and riboflavin supplementation and a diet with restriction of lysine and tryptophan was started. Intercurrent illnesses were treated with intravenous fluid, glucose and L-carnitine. At 3 years and 6 month of age, he had not shown any encephalopathic crisis, he had a normal psychomotor development and no dystonia. MR shows mild temporal lobe atrophy without basal ganglia alterations. CONCLUSIONS: In GA-I, macrocephaly is an early sign before other neurologic alterations. In patients with little or no neurological symptoms, early treatment may prevent the acute encephalopathic crisis and neurological deterioration, improving the prognosis and may also normalize the basal ganglia neuroradiological alterations. Urinary organic acid analysis should be performed in the work up of macrocephaly of unknown aetiology.

[Treatment of Tourette syndrome and its comorbidity: experience with 17 cases]. / Tratamiento del síndrome de Tourette y su comorbilidad: presentación de 17 casos.

INTRODUCTION: Clinical characteristics and comorbid disorders of Tourette syndrome (TS) are reviewed along with a presentation of our experience with 17 cases. MATERIAL AND METHODS: We carried out a retrospective study of pediatric patients with TS admitted from 1998 to 2004 in Fundación Hospital Alcorcón. RESULTS: Seventeen patients were obtained, 16 of whom were men and there was only 1 woman. Present age ranged from 7 to 17 years old. Most frequent comorbid disorders were attention deficit disorder (ADD) in 9 patients, (53%), obsessive-compulsive disorder in 8 (48%) and anxiety in 7 (41%). Learning disorders were found in 7 patients (41%), 5 of whom have concurrent ADD and 1 severe obsessive compulsive disorder. Psychopharmacological treatment was withdrew in the 2 cases treated with halloperidol due to the presence of severe extrapyramidal symptoms (EPS) and in 3 of the 7 cases treated with pimozide (one of them was withdrawn due to EPS). No EPS was found with atypical neuroleptics, but sedation and weight gain was common. Methylphenidate was administered to 7 patients without an increase in tics. CONCLUSIONS: In our sample the most common comorbid disorders were ADD, obsessive-compulsive disorders, anxiety and learning disorders. Atypical neuroleptics were better tolerated than classic ones, although the incidence of side effects is elevated. Methylphenidate was not associated with tic worsening.

[Scleroderma en coup de sabre with intracranial involvement. A case report]. / Esclerodermia en coup de sabre y participación intracraneal. Presentación de un caso.

Scleroderma en coup de sabre is a descriptive term denoting linear scleroderma of the frontoparietal area of the face and scalp. This form of scleroderma is infrequent, mainly in pediatric patients, but not infrequently associates neurologic symptoms, especially epilepsy. We describe a 15-year-old girl with scleroderma en coup de sabre and a long-standing history of epilepsy. Cranial magnetic resonance examination reaffirms the clinical impression that inflammation is not solely confined to the skin in this form of linear scleroderma.

[Effects of corticosteroids in the management of Duchenne muscular dystrophy: our experience]. / Efecto de los corticoides en el tratamiento de la distrofia muscular de Duchenne.

Objective To evaluate the clinical course in patients with Duchenne muscular dystrophy admitted to our department who received corticosteroid treatment and to compare their course with that in patients who did not receive corticosteroid treatment.Patients and methodsWe performed a retrospective study of 20 pediatric patients with a diagnosis of Duchenne muscular dystrophy who were offered corticosteroid treatment: 10 patients received deflazacort and 10 refused the treatment. The MRC muscular strength scale and Vignos' functional scale were used to evaluate clinical course, which was compared in both groups.ResultsUntreated patients showed progressive worsening. Corticosteroid-treated patients showed disease stabilization both in muscular strength and functional performance. In addition, muscular balance improved in 70 % of these patients, but only 2 % showed functional improvement. The positive effect of steroid treatment had a mean duration of 12 months. Loss of independent gait occurred at similar ages in both groups (10.3 vs. 10.5 years). The results of Achilles' tendon surgery were poor.ConclusionsCorticosteroids produced clinical stabilization and improved muscular strength. Functional improvement was not significant, including loss of gait, probably because this loss also depends on an increase in joint contracture. Good coordination among multiprofessional teams is essential to achieve optimal results.

[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.