Acute stroke care during the COVID-19 pandemic. Ictus Madrid Program recommendations. / Atención al ictus agudo durante la pandemia por COVID-19. Recomendaciones Plan Ictus Madrid.

INTRODUCTION: The COVID-19 pandemic has resulted in complete saturation of healthcare capacities, making it necessary to reorganise healthcare systems. In this context, we must guarantee the provision of acute stroke care and optimise code stroke protocols to reduce the risk of SARS-CoV-2 infection and rationalise the use of hospital resources. The Madrid Stroke multidisciplinary group presents a series of recommendations to achieve these goals. METHODS: We conducted a non-systematic literature search using the keywords "stroke" and "COVID-19" or "coronavirus" or "SARS-CoV-2." Our literature review also included other relevant studies known to the authors. Based on this literature review, a series of consensus recommendations were established by the Madrid Stroke multidisciplinary group and its neurology committee. RESULTS: These recommendations address 5 main objectives: 1) coordination of action protocols to ensure access to hospital care for stroke patients; 2) recognition of potentially COVID-19-positive stroke patients; 3) organisation of patient management to prevent SARS-CoV-2 infection among healthcare professionals; 4) avoidance of unnecessary neuroimaging studies and other procedures that may increase the risk of infection; and 5) safe, early discharge and follow-up to ensure bed availability. This management protocol has been called CORONA (Coordinate, Recognise, Organise, Neuroimaging, At home). CONCLUSIONS: The recommendations presented here may assist in the organisation of acute stroke care and the optimisation of healthcare resources, while ensuring the safety of healthcare professionals.

[Choreoathetosis after Herpes simplex encephalitis]. / Coreoatetosis tras encefalitis herpética.

After Herpes simplex encephalitis, 25% of cases may have a relapse, rarely as a choreoathetosic movement disorder. The anatomic basis for herpes simplex virus encephalitis-associated movement disorders remains poorly understood, but the hypothesis is that it may be due to a post-infectious immune-mediated process. We report an 8-month-old boy, with herpes simplex encephalitis type 1, who started with an extrapyramidal Syndrome, presenting with choreoathetosis and ballistic movements, three weeks after onset. These new symptoms were attributed to a post-infectious immune-mediated process. We treated our patient with corticosteroids at high dose and gamma-globulins, in addition to a new course of Acyclovir. Sedation was required to control the intense choreoathetosic movements. Tetrabenazine was also tried, unsuccessfully. We studied a mutation on the toll like receptors (TLR3), which has been related to susceptibility for the disease, which was negative.

[Temporal lobe epilepsy. Aetiological classification in 61 paediatric patients]. / Epilepsia del lóbulo temporal. Clasificación etiológica en 61 pacientes en edad pediátrica.

INTRODUCTION: There are very few studies on the aetiology of temporal lobe epilepsy (TLE) in childhood. The purpose of the present study is to analyse the data of 61 children diagnosed with TLE, in order to describe the aetiology of TLE in children seen in a neuropaedriatic clinic. We also discuss the currently proposed classification. PATIENTS AND METHODS: A retrospective analysis was carried out on patients diagnosed with TLE. Patients consisted of 61 children less than 15 years old. RESULTS: Patients were classified into three groups: Group 1 (symptomatic temporal lobe epilepsy) consisted of 25 patients (40.98 %) with any temporal lesion on neuroimaging (tumours, malformations or infections) or significant history; Group 2 (Mesial temporal sclerosis) consisted of 17 patients (27.86 %), a history of simple and complex febrile seizure were common in this group; and Group 3 (Cryptogenic epilepsy) consisted of 19 patients (31.15 %) with no abnormalities on neuroimaging or significant history. CONCLUSION: To our knowledge, this is the largest paediatric series of childhood new-onset TLE assessed only by MRI in the literature. We have modified the previous aetiological classification in order to make the groups more realistic.

[Cerebral palsy: the concept and population-based registers]. / Parálisis cerebral: concepto y registros de base poblacional.

INTRODUCTION: Cerebral palsy (CP) is the most common cause of motor disability in the paediatric age. For several decades, a number of developed countries have kept registers that have been used to conduct population-based studies of CP. In Spain, however, little attention has been paid to the epidemiology of CP. AIM: To review the concept of CP today and to stimulate greater interest in researching into CP based on the experience of other countries. DEVELOPMENT: The different definitions of CP cover motor sequelae secondary to an isolated brain injury that occurs in a developing brain. CP registers were started as a means of monitoring the prevalence of CP and planning suitable care for patients. Over the last decade projects have been carried out that involve the coordinated efforts of several centres; this provides a larger population for study and reduces the chances of confusion with respect to the terminology employed. In this regard, one notable line of work is that of the European CP group (SCPE), which gathers information about children with CP in 15 countries. Spain has been taking part in this project since 2003 through a group of researchers from the Hospital 12 de Octubre in Madrid. CONCLUSIONS: From the very definition of the disorder, CP is a complex condition. A population-based study of CP in Spain should help to arouse a renewed interest in this condition in our country.

[Obstetric brachial plexus palsy: incidence, monitoring of progress and prognostic factors]. / Paralisis braquial obstetrica: incidencia, seguimiento evolutivo y factores pronosticos.

INTRODUCTION: Obstetric brachial plexus palsy is related with shoulder dystocia, and its main risk factor is macrosomia. Its incidence is estimated to be between 0.1 and 6.3 cases per 1,000 live newborn infants. Most cases are resolved but can give rise to permanent functional deficiency, which means that there is an interest to identify possible prognostic factors. PATIENTS AND METHODS: We conducted a descriptive study of newborn infants with obstetric brachial plexus palsy born in our hospital between the years 2011 and 2015. Maternal, perinatal and obstetric variables, as well as the type of lesion, were collected and were related with the possibility of recovery at six months. RESULTS: Altogether 32 cases were diagnosed, which represents an incidence of 1.44‰ of live newborn infants. 59% were males and 37.5% of them were macrosomic. The most frequent disorder was injury to the plexus at the proximal level (94%). 44% suffered from shoulder dystocia, and 47% still had sequelae at the sixth month. The antecedent of shoulder dystocia was related with a poor prognosis for recovery. CONCLUSIONS: The incidence of obstetric brachial plexus palsy has remained stable in recent years. The percentage of children who present sequelae at six months is significant. Prospective studies are needed to be able to establish the long-term prognostic factors of this pathology.

TITLE: Paralisis braquial obstetrica: incidencia, seguimiento evolutivo y factores pronosticos.Introduccion. La paralisis braquial obstetrica se relaciona con la distocia de hombros, y su principal factor de riesgo es la macrosomia. Su incidencia se estima entre 0,1 y 6,3 casos por 1.000 recien nacidos vivos. La mayoria de los casos se resuelve, pero puede provocar deficit funcional permanente, por lo que es de interes identificar posibles factores pronosticos. Pacientes y metodos. Estudio descriptivo de los recien nacidos con paralisis del plexo braquial obstetrica nacidos en el hospital entre los años 2011 y 2015. Se han recogido variables maternas, perinatales, obstetricas y del tipo de lesion, y se han relacionado con la posibilidad de la recuperacion a los seis meses. Resultados. Se diagnosticaron 32 casos, lo que supone una incidencia del 1,44‰ de recien nacidos vivos. El 59% fueron varones, y el 37,5%, macrosomicos. La afectacion mas frecuente fue la lesion del plexo a nivel proximal (94%). El 44% sufrio distocia de hombros, y el 47% permanecio con secuelas al sexto mes. El antecedente de distocia de hombros se relaciono con mal pronostico de recuperacion. Conclusiones. La incidencia de paralisis braquial obstetrica se mantiene estable en los ultimos años. El porcentaje de niños que presentan secuelas a los seis meses es relevante. Son necesarios estudios prospectivos para poder establecer los factores pronosticos a largo plazo de esta patologia.

[Neuropsychiatric disorders associated with streptococci: a case report]. / Trastornos neuropsiquiátricos asociados a estreptococo.

Compulsive neuropsychiatric disorders are common in children and the most frequent is Gilles de la Tourette syndrome. Recently, a new disease has been described: the PANDAS syndrome (pediatric autoimmune neuropsychiatric disorders associated with streptococci). The etiology of this syndrome is uncertain but it has been associated with recent group A streptococcal infection (GAS). After an episode of pharyngitis, a boy aged 3 years and 9 months showed abrupt onset of a variety of neurobehavioral problems such as tics (consisting of elevation of the head and ipsilateral shoulder, winking, and grimaces) and compulsions (such as repeatedly hitting objects). A complete biochemical study was performed, including Cu and ceruloplasmin (which had normal values), antistreptolysin O (ASLO) and anti-DNAse (showing elevated values). Pharyngeal culture revealed GAS. The child was initially treated with valproic acid, but his subsequent improvement coincided with penicillin treatment for 10 days. Three months afterwards, ASLO values were reduced and at the 6-month follow-up the improvement was maintained even after suspension of valproic acid. Immunohistochemical studies were requested. PANDAS syndrome was first described in 1998 in a group of children who presented 1) obsessive compulsive disorders and/or tics, 2) episodic course with abrupt exacerbations, 3) abnormal results of neurologic examination (choreiform movements), and 4) temporal relation between GAS infection and onset of symptoms. The etiology of this syndrome is unclear, and it has been postulated that certain streptococcal antigens trigger antibodies which, through a process of molecular mimicry, cross-react with epitopes on the basal ganglia of susceptible hosts, such as the B8/17 antigen, among others. Current recommendations include penicillin treatment of each exacerbation with positive throat culture, and more aggressive therapies (intravenous immunoglobulin or plasmapheresis) when symptoms are severe. We believe that the case presented herein is a probable PANDAS syndrome, which would be the first case described in Spain.

[Acute disseminated encephalomyelitis. Experience of a tertiary hospital in Spain]. / Encefalomielitis aguda. Experiencia de un hospital terciario español.

INTRODUCTION: To describe the epidemiological, clinical, microbiological, neuroimaging and laboratory features, treatment, and outcome in a cohort of children with acute disseminated encephalomyelitis (ADEM). PATIENTS AND METHODS: Retrospective chart review was performed of children with a diagnosis of ADEM over a 23-year period in a tertiary hospital in Spain. RESULTS: Twelve cases were identified. Ten cases (83%) occurred after 1992. Nine patients (75%) presented between April and September. The mean age was 6 years. Nine patients (75%) were male. Fifty percent of the patients had a history of infectious disease or vaccination. The most frequent nonspecific symptom was fever in 75%. The most frequent neurological manifestations were motor deficits and altered consciousness in 75%. Cerebrospinal fluid abnormalities were found in 83%. All patients had at least one brain scan and one brain magnetic resonance imaging (MRI) scan. Three patients underwent spinal MRI. The sensitivity of MRI was greater than that of the scanner in the diagnosis of ADEM. An etiologic diagnosis was made in four patients: Mycoplasma pneumoniae, beta hemolytic streptococcus group A, Epstein-Barr virus and measles-mumps-rubella vaccination. Eleven patients were treated with corticosteroids and one was treated with intravenous immunoglobulin therapy. One patient died while 75 % of the patients had a good outcome. CONCLUSIONS: ADEM is in an infrequent disease in children. The clinical features are similar to those of infectious encephalitis. Etiologic diagnosis is difficult to establish but this entity is usually preceded by an infection. The neuroimaging test of choice to establish the diagnosis is MRI. In most patients, the prognosis is good.

[Neurological complications of systemic cancer and its treatment]. / Complicaciones neurológicas del cáncer sistémico y de su tratamiento.

INTRODUCTION: Therapeutic advances have attained excellent results in the battle against systemic cancer. This has meant cure for many and greater survival. However, these achievements have led to a dramatic increase in neurological complications due to the cancer itself and the surgical treatment on its own or combined with chemotherapy and radiotherapy. These complications, which are very common in childhood neoplasias, have had much less attention than those of adults, although recently excellent reviews have begun to be published. OBJECTIVE: In this paper we review the various complications of systemic cancer and its treatment in childhood, emphasizing the differences from that of adults.

[Unilateral isolated paralysis of the soft palate: a case report and a review of the literature]. / Parálisis aislada unilateral del velo del paladar: presentación de un caso y revisión de la literatura.

INTRODUCTION: Unilateral isolated paralysis of the soft palate is a rare clinical entity. CASE REPORT: We describe the case of a 12 year old girl who presented acute dysphagia, a nasal voice and regurgitation of liquids into the nose. Exploration revealed right velopalatine insufficiency with normal gag reflex and pharyngeal sensitivity. All the complementary studies, including magnetic resonance, lumbar puncture and viral serology tests, were normal. There are 28 similar cases in the literature, with the following characteristics: acute onset, appearing in infancy (96%), predominance in males (79%), recent respiratory infection (35%) and an excellent prognosis for recovery (85%). CONCLUSION: This is probably a case of acute cranial mononeuropathy with a viral aetiology

[Neurological impairment in children with HIV infection]. / Afectación neurológica en niños con infección VIH.

We retrospectively checked 53 paediatric patients suffering from infection with human immunodeficiency virus (HIV) registered in our Centre between the years 1987 and 1993, and evaluated the appearance of HIV encephalopathy. We noted important neurological signs in eleven patients (20.7%) ten of whom had HIV infection via vertical transmission and one as a result of contamination from haemoderivatives. In this review we give a detailed description of neurological signs, the moment of onset of these signs and their possible relationship with the state of the HIV infection. We also analyzed the resulting neuroradiological findings as well as any abnormalities in cerebrospinal fluid. Follow-up period ranged from one month to two and a half years from the moment of onset of the appearance of encephalopathy. Although most of our patients showed a clear improvement after oral or intravenous treatment with zidovudine, this improvement generally proved to be short-lived. The mortality rate in our HIV encephalopathy series was 81.8%, this figure being reached two and a half years after encephalopathy. The appearance of neurological signs in HIV patients therefore represents a very gloomy prognostic factor in the evolution of the disease.

[Neuromuscular gait disorders in pediatric age]. / Trastornos de la marcha de origen neuromuscular en la edad pediátrica.

UNLABELLED: Alterations in gait are a frequent cause of consultation in neuropaediatric units. They have several different causes. One subgroup of special interest concerns those which have their origin in a neuromuscular disease. We studied the experience of the Child Neurology Unit at the Hospital 12 October from 1980 to 1993. MATERIAL AND METHODS: between 1980 and 1993 a total of 176 were studied because they showed alterations in their gait caused by a neuromuscular disease. Frequency and clinical features were analysed (sex, age at the beginning of the deterioration, duration of the latter etc.). RESULTS AND CONCLUSIONS: the alterations in gait were separated into two groups: Group I: backward or non-acquired gait. Group II: deterioration in gait. IIa: temporary. IIb: definitive. The most frequent cause in Group I was spinal muscular atrophy and in Group II, muscular dystrophy linked to sex. To sum up, muscular diseases are far more important than the neuropathic ones, except for Group IIa where acute demyelinating motor polyneuritis was to be found at the top of the list of frequent occurrences.

[The prognosis of epilepsy in children with generalized spikes and wave disc charges in electroencephalographic recording]. / Pronóstico de la epilepsia en niños con trazado electroencefalográfico de descargas de punta-onda generalizadas.

INTRODUCTION: Epilepsy is a chronic disorder. The prognosis of one particular subtype, that of children with generalized spike and wave discharges (P-O) on the electroencephalogram (EEG), is unknown. OBJECTIVE: To determine the prognosis of children with epilepsy with P-O on EEG and the factors which affect this. PATIENTS AND METHODS: All EEGs done over ten years (39,322) were reviewed, and the epileptic patients under 14 years old identified. Absence and recurrence of crises were evaluated and the factors influencing them analyzed. Minimum significance was p < 0.05. The chi square test, Student's t test and Kaplan-Meier method were used. Logistic regression models were devised. RESULTS: Of the children studied, 78% became crisis-free. After monovariant and multivariant analysis the only conditions which fitted the regression models were: abnormal pregnancy; West syndrome or learning difficulties; having certain types of crises, myoclonic, clonic-tonic, partial simple or complex crises; a normal EEG with hypsarrhythmia, slow or fast P-O and requiring multiple medication. When medication ceased, 31.4% had recurrence of the episodes. On monovariant and multivariant analysis, protective factors were absence of attacks and risk factors were a family history of epilepsy or an EEG with rapid P-O. CONCLUSIONS: The overall prognosis for epileptic children with P-O is good. There are factors which improve or worsen the pronosis, but these cannot be modified.

[Cerebral cavernomas in childhood. clinical presentation and diagnosis]. / Cavernomas cerebrales en la infancia. Presentación clínica y diagnóstico.

INTRODUCTION: Intracranial vascular malformations are congenital lesions due to alterations in the development of the arteriolocapillary network. Traditionally they are divided into four types according to their histological characteristics: arteriovenous malformations, cavernous angiomas or cavernomas, venous angiomas and telangectasias. The cavernomas are multilobulated lesions which are clearly delimited and contain blood at different stages of evolution. Histologically they are composed of sinusoidal spaces lined by endothelium and closely interlinked, without intervening nervous tissue. They are usually found at a supratentorial level, and less frequently in the posterior fossa. PATIENTS AND METHODS: We made a retrospective review of the histories of 11 patients aged between 2 and 10 years, diagnosed as having intracranial cavernomas in which imaging techniques lead to the diagnosis of intracranial cavernoma. In two patients there were multiple cavernomas. We analysed the method of diagnosis and clinical features on presentation. RESULTS: The clinical features were varied. In six patients the onset of the disorder was with epileptic seizures, three had progressive neurological deficits, one patient had headache and vomiting which progressed to coma and another had a history of non specific fainting and deficit. All but one of the cavernomas were supratentorial. CONCLUSIONS: Cavernomas are a type of vascular malformation with specific histological features. The usual clinical features are convulsions and parenchymatous bleeding. They are generally supratentorial. The appearance of MR has permitted diagnosis of asymptomatic cavernomas and is currently considered to be the technique of choice for diagnosis.

[Fast and spectacular clinical response to plasmapheresis in a paediatric case of anti-NMDA encephalitis]. / Respuesta clinica rapida y espectacular a plasmaferesis en un caso pediatrico de encefalitis anti-NMDA.

INTRODUCTION: Autoimmune encephalitis against N-methyl-D-aspartate (NMDA) receptors is being diagnosed more and more frequently in the paediatric age. It should be suspected in children with psychiatric symptoms, encephalopathy, abnormal movements or epileptic seizures. Paraneoplastic cases are less frequent than in adults. CASE REPORT: We report the case of a boy, 2.5 years of age, with subacute encephalopathic signs and symptoms and epileptic seizures followed by behaviour disorders, neurological regression, dyskinesias and insomnia. Results of a cerebrospinal fluid study were normal, the magnetic resonance scan of the head revealed a focal periventricular lesion and diffuse leptomeningeal uptake; moreover, the serial electroencephalograms showed high-amplitude delta activity interspersed with generalised intercritical epileptiform activity. The patient was given empirical treatment with high doses of corticoids and intravenous immunoglobulins with no response. After showing up positive for antibodies against the NMDA receptor, plasmapheresis was begun, which led to his swift and spectacular recovery. After more than 18 months' follow-up, his sequelae are limited to mild behavioural and language alterations. He has had no relapses and has not needed any kind of maintenance treatment. CONCLUSIONS: Anti-NMDA encephalitis is a treatable disorder and, sometimes, the first evidence of an underlying neoplasia, which makes its early recognition and treatment essential. Treatment of the non-paraneoplastic forms are based on immunotherapy: glucocorticoids, intravenous immunoglobulins, plasmapheresis and immunosuppressants. Plasmapheresis can bring about a fast, spectacular improvement.

Varón de 8 años con cefalea frontal izquierda, lagrimeo y rinorrea ipsilateral; porque no toda cefalea en niños es migraña / An 8 year-old boy with left frontal headaches, watery eyes and ipsilateral rhinorrhoea; the reason why not all headaches in children is migraine

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Hemicorea de Sydenham como manifestación de fiebre reumática / Sydenham's hemichorea as a manifestation of rheumatic fever

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