Has clinical activity in paediatric neurology changed in the past 11 years? / ¿Ha cambiado la actividad asistencial de la neurología pediátrica en 11 años?

INTRODUCTION: We believe that the demand for paediatric neurology (PN) care has increased over the past decade, and that reasons for requesting consultations have also changed. The objective of this study is to complete a registry study to profile the demand for PN care in 2013 and compare results to those from a study performed in 2002. METHODS: A prospective registry of PN healthcare activities was completed at Hospital Universitario de Getafe in 2013. Results were compared with those from a prospective registry study conducted in 2002. RESULTS: The number of visits increased from 1,300 in 2002 to 1,982 in 2013 (a 52.46% increase), and from 32.6 visits per 1,000 children to 57.48 (a 76.32% increase). Outpatient consultations accounted for 92.2% of all PN consultations in 2013. Currently, attention deficit-hyperactivity disorder (ADHD) is the most frequent diagnosis (27.6% in 2013 vs. 8.1% in 2002). Although the percentage of headache consultations has decreased (19% in 2013 vs. 22% in 2002), headache was still the most common reason for an initial visit in 2013 (32.1%), followed by ADHD (19.1%). Epilepsy remains the most frequent diagnosis in hospitalised patients (30.3% in 2013 vs. 36.7% in 2002). CONCLUSIONS: PN is fundamentally an outpatient activity that has increased considerably in recent years. This increase is mainly due to neurodevelopmental disorders, especially ADHD. We might state that the role of ADHD in PN is comparable to that of dementia in general neurology.

[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.

[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.

[Mononeuropathy in chronic lymphatic leukaemia]. / Mononeuropatía en la leucemia linfática crónica.

INTRODUCTION: Chronic lymphatic leukaemia (CLL) is the most frequent form of leukaemia in the adult population in western countries. Only 7.2% of the complications of CLL are neurological and most of them are secondary to an infection by herpes zoster virus. CASE REPORT: We report the case of a 71-year-old female with B-type CLL in stage IV or type C that was progressing and becoming diffuse large B-cell lymphoma (Richter's syndrome), who developed an incomplete axonotmesis of the left peroneal nerve and numerous violet-coloured nodules under the skin in the left knee. Magnetic resonance imaging showed signs of diffuse infiltration into the subcutaneous tissue and the muscles of the left leg; a biopsy study of one of the subcutaneous nodules revealed a lymphoid infiltration by large B-cells. In this patient, the injury to the left peroneal nerve was probably secondary to a lymphoid infiltration of the nerve from adjacent infiltrated soft tissues. CONCLUSION: Peripheral neuropathy due to direct infiltration can be a neurological complication of CLL that has not be reported to date, but which is known to occur in other lymphoproliferative processes.

[Migraine in childhood: a trivial condition?]. / La jaqueca en la infancia: una patologia banal?

INTRODUCTION: Migraine is seen as being a trivial disease, and more so in childhood, but in many cases it has a detrimental effect on the patient's quality of life. PATIENTS AND METHODS: Prospective study. All the patients were evaluated by the same neuropaediatrician and all of them satisfied diagnostic criteria for migraine. 127 children were examined. The mean age was 9.4 years, with an interval of 3-14 years; there were no differences between sexes. 67 males and 60 females. RESULTS: The mean length of time the episodes lasted was 22.5 h. The most frequently observed clinical features were: hemicranial localisation, 44.4%; throbbing, 74.4%; photophobia, 74.8%; phonophobia, 83.5%; nausea-vomiting, 63.5%; and aura, 14.3%; with predominance of acutely intense visual and sensory symptoms (74%), functional repercussions in 87% and absence from school in up to 36.9% of cases. 16% of patients have had episodes of status migrainous. At the time of the visit 46% had several attacks a week; 13.7% once a week; 16.1% fortnightly; 13.7 % monthly; 5.6% every three months; and others, 4.8%. 48.7% of the patients were given preventive treatment, which was wholly effective in 48%, partially effective in 35% and not at all effective in 15.4%. CONCLUSIONS: Migraine in childhood is not a trivial pathology. It is disabling: it interferes with their daily life in 85% of cases, causes them to miss school in almost 40% of patients and nearly 50% of them have several episodes a week. A similar figure required prophylactic treatment that was seen to be very effective.

[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.

[Paediatric neurology health care activity in a tertiary hospital. A study comparing it with adult neurology and non-neurological paediatrics]. / Actividad asistencial de la neurología pediátrica en un hospital de nivel 3. Estudio comparativo con la neurología de adultos y la pediatría no neurológica.

INTRODUCTION: Paediatric neurology (PN) remains 'hidden' somewhere between paediatrics and neurology in almost all health care centres and this makes it difficult to allocate resources in a proper manner. AIMS: Our objective in this study was to analyse PN health care activity and compare it with adult neurology (AN) and with non-neurological paediatrics (NNP). MATERIAL AND METHODS: PN health care activity registry for the year 2002. AN and NNP data were collected from the hospital's Computer Service. RESULTS: In all, 1300 PN visits were made, 428 of which were new and 872 successive, which represent 16.2% of paediatric visits (30% new, 13.3% successive) and 10.3% of neurological visits (12.4% new, 10.2% successive). The rates of new and successive visits in PN are: 32.6, 10.7 and 21.9 per 1000 children, and in AN: 44.2, 12.5 and 31.7 per 1000 adults. A total number of 94 hospitalised children were attended, 3.3% of all paediatric admissions and 9.2% of the total neurological attention in inpatients. The rates are 2.36 per 1000 children in PN and 3.9 per 1000 adults in AN. There is greater demand among smaller children. The most frequent pathologies are headaches in clinical visits and epilepsy in hospitalised patients. CONCLUSIONS: PN is essentially a service used by outpatients. It accounts for a high percentage of hospital paediatric visits. The activity rates per 1000 children and per 1000 adults are similar. The increased birth rate raises PN activity to a level that is higher than would normally be desirable, due to the growth in the population.

[The present state of paediatric neurology in the Autonomous Community of Madrid: variability in the distribution of resources]. / Situacion de la neurologia pediatrica en la Comunidad Autonoma de Madrid: variabilidad en la distribucion de los recursos.

INTRODUCTION: Paediatric neurology (PN) lies halfway between neurology and paediatrics, with no official acknowledgment. It is therefore difficult to determine exactly how resources are shared out. AIMS: The aim of this study is to analyse the public resources devoted to PN in the different health care areas within the Autonomous Community of Madrid (ACM). MATERIALS AND METHODS: In May 2002 we carried out a survey among child neurologists (CN) from the 9 independent health care districts within the ACM. RESULTS: A total of 28 CN work for the public health service in the ACM, eight of whom are employed on a part time basis. Only 53% are owners. The ratio of CN per 100000 inhabitants (inhab.) is 0.55 (0.45 after correction for part time work). The figure varies from one area to another from 0.07 to 1 CN/100000 inhab. (1/12000 1/220000 children). Part time neurologists work in the outer districts of the ACM. In most areas it is difficult to carry out diagnostic tests and these become even complicated when the child is small and requires some anaesthetic procedure. Neuropsychological assessment is one of the basic evaluations performed in PN and this is not easily performed in most centres. CONCLUSIONS: The CN/100000 inhab. ratio in the ACM is lower than that commonly recommended. The distribution of resources varies greatly and does not match demographic criteria. Temporary and part time jobs are commonplace in the peripheral areas. It is difficult for most centres to perform diagnostic tests.

[A descriptive analysis of 81 patients referred to a neurology clinic for syncope]. / Análisis descriptivo de 81 pacientes remitidos por síncope a una consulta de neurología.

INTRODUCTION: Syncope is a frequent medical problem which is disabling, potentially serious and difficult to treat. Although patients with syncope are often sent to Neurology clinics for investigation we have found no published report analysing this. OBJECTIVE: To analyze the aetiology of the cases of syncope referred to a Neurology Clinic and also the diagnostic usefulness of the investigations requested, particularly those of neuroimaging, electroencephalogram (EEG) and vascular studies. PATIENTS AND METHODS: A prospective study of the patients referred for syncope to a Neurology Outpatient Clinic. The patients were classified into three groups: 1. Epileptic seizure, if the clinical history suggested the possibility of a convulsion rather than a syncope. 2. Syncope of neurological origin, when the syncope is due to a neurological disorder. 3. Non neurological cause of the syncope when the syncope was not due to neurological disease. RESULTS: We included 81 patients, who made up 4.3% of the patients seen in the Neurology Clinic. Epileptic seizures made up 10% and the other 90% were due to non neurological causes. There was 0% usefulness of neuroimaging investigations and vascular studies. The EEG showed epileptiform changes in 9% of the patients, with non neurological syncopes. CONCLUSION: In patients with syncope neurological investigations are not very useful.

Generalized spike-and-wave patterns in children: clinical correlates.

All electroencephalograms performed in our institution between 1980 and 1990 were reviewed. The clinical characteristics of children with epilepsy and generalized spike-and-wave (SW) patterns were analyzed. The SW patterns were classified according to their frequency. Electroencephalograms of 154 children with epilepsy revealed SW patterns. Absence seizures were the most common first seizure, but partial seizures were frequent. More than 40% had several types of seizures. Sixty percent of the epileptic syndromes were generalized, but almost 25% were partial. The typical SW pattern was associated with absence seizures, a normal examination and computed tomographic scan, idiopathic generalized epilepsies, monotherapy, freedom from seizures, and lack of recurrence. The slow SW pattern was associated with West syndrome; a younger age at seizure onset; atonic, myoclonic, tonic, and partial simple seizures; an abnormal examination and computed tomographic scan; cryptogenic or symptomatic generalized epilepsy or symptomatic partial epilepsy; polytherapy; and poor seizure control. The fast SW pattern was associated with secondary generalized, partial, tonic-clonic, and complex partial seizures; a normal computed tomographic scan; cryptogenic partial epilepsy; isolated seizures; and seizure recurrence. Epilepsy with a typical SW pattern should be considered benign, epilepsy with a slow SW pattern malignant, and epilepsy with a fast SW pattern treacherous.

[Febrile seizures: is EEG useful?]. / Tiene sentido realizar un EEG en las crisis febriles?

OBJECTIVE: The purpose of this study was to determine the clinical relevance of electroencephalograms (EEG) with generalized spike-wave (S-W) in pediatric patients, especially in children with febrile seizures. PATIENTS AND METHODS: One hundred and seventy pediatric patients with S-W were found from a register of 39,322 consecutive EEGs performed in an EEG laboratory dedicated to general clinical practice. Patients that only suffered febrile seizures were not considered epileptic. RESULTS: Of the 170 patients, 154 (90.6%) were epileptic and 16 (9.4%) were not. Mean follow-up was 4.5 years. Twelve of the 16 non-epileptic children with S-W suffered febrile seizures. CONCLUSIONS: The presence of S-W in EEGs is uncommon. Almost 10% of the children with S-W were not epileptic patients and none of them suffered from epilepsy during the follow-up period. Performance of an EEG on a child without epilepsy, especially those with febrile seizures, can be a factor of confusion.

[Perinatal antecedents, psychomotor retardation, learning difficulties and family history. Are they a risk factor for epilepsy?]. / Antecedentes perinatales, retraso psicomotor, dificultades del aprendizaje e historia familiar. Son un factor de riesgo para la epilepsia?

OBJECTIVE: Epilepsy has been associated with certain circumstances such as perinatal pathology, learning difficulties, head trauma, infections of the central nervous system, febrile seizures and family history of epilepsy. The objective of our study was to analyze the association of the mentioned circumstances with epilepsy in a group of children with generalized spike-wave (S-W) in the electroencephalogram (EEG). PATIENTS AND METHODS: One hundred seventy pediatric patients with, S-W were found from a register of 39,322 consecutive EEGs. The patients were classified into 2 groups: epileptic and non-epileptic. Both groups were compared for the presence of known risk factors of epilepsy. RESULTS: Of the 170 patients, 154 (90.6%) were epileptic and 16 (9.4%) were not. Mean follow-up was 4.5 years. Neither past medical antecedents nor family history was found to be associated with epilepsy. CONCLUSIONS: The risk of epilepsy in children with S-W in the EEG is not associated with problems in pregnancy, the birth or neonatal period, learning difficulties or family history of epilepsy.

[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.

[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.

[Axilla skin biopsy in the diagnosis of Lafora's disease]. / Utilidad de la biopsia de piel axilar en el diagnóstico de la enfermedad de Lafora.

Skin biopsy is the method of choice for the diagnosis of Lafora's disease. The presence of PAS (+) inclusions characteristic of Lafora's disease has been thought to be more evident in aprocrine glands of the axillary skin than in the duct cells of the eccrine glands. We describe 4 patients with Lafora's disease diagnosed by axilla skin biopsy, confirming the usefulness of this procedure.