[Prevalence of childhood obstructive sleep apnoea syndrome in a referral sleep unit]. / Prevalencia del síndrome de apnea obstructiva del sueño infantil en una unidad de sueño de referencia.

INTRODUCTION: Obstructive sleep apnoea syndrome (OSAS) affects between 1% and 6% of children. Its diagnosis includes: a) snoring and/or apnoea; and b) an apnoea and hypopnoea index >3/hour obtained by polysomnography (PSG). The main aim of this work is to determine the prevalence of OSAS in our study population. PATIENTS AND METHODS: We conducted a descriptive study with a sample of 151 children aged between 1 and 12 years, who had been referred to the sleep unit of the Hospital General Universitario Gregorio Maranon for a PSG. We analysed the demographic variables sex and age; the clinical variables snoring, apnoeas and tonsillar hypertrophy; and the presence of OSAS based on the polysomnographic diagnostic criterion of an apnoea and hypopnoea index >3/hour. RESULTS: The mean age of the sample was 5.37 years (standard deviation: 3.05) and 64.9% were males. In 90.1% of cases, the reason for the visit was suspected OSAS. Snoring, apnoeas and tonsillar hypertrophy were observed in 73.5, 48.7 and 60% of cases, respectively. OSAS was diagnosed en 19 children (12.6%); in 13.5% of snorers; in 15.1% of those with apnoeas; and in 15.6% of the children with tonsillar hypertrophy. CONCLUSIONS: In our study, the prevalence of OSAS in children was 12.6%, which is higher than that reported in most epidemiological studies that include PSG for the diagnosis of OSAS.

TITLE: Prevalencia del síndrome de apnea obstructiva del sueño infantil en una unidad de sueño de referencia.Introducción. El síndrome de apnea obstructiva del sueño (SAOS) afecta a entre el 1 y el 6% de la población infantil. En su diagnóstico, se incluyen: a) ronquidos y/o apneas; y b) un índice de apneas e hipopneas >3/hora obtenido en la polisomnografía (PSG). El objetivo principal de este trabajo es determinar la prevalencia del SAOS en nuestra población de estudio. Pacientes y métodos. Estudio descriptivo con una muestra de 151 niños con edades comprendidas entre 1 y 12 años, remitidos a la unidad de sueño del Hospital General Universitario Gregorio Marañón para la realización de una PSG. Se analizaron las variables demográficas sexo y edad; las variables clínicas ronquidos, apneas e hipertrofia amigdalar; y la presencia de SAOS basada en el criterio diagnóstico polisomnográfico de un índice de apneas e hipopneas >3/hora. Resultados. La edad media de la muestra fue de 5,37 años (desviación estándar: 3,05) y el 64,9% eran varones. En el 90,1% de los casos, el motivo de consulta fue sospecha de SAOS. Los ronquidos, las apneas y la hipertrofia amigdalar se observaron en el 73,5, el 48,7 y el 60% de los casos, respectivamente. Se diagnosticó SAOS en 19 (12,6%) niños; en el 13,5% de los roncadores; en el 15,1% de los niños con apneas; y en el 15,6% de los niños con hipertrofia amigdalar. Conclusiones. En nuestro estudio, la prevalencia del SAOS en niños fue del 12,6%, superior a la descrita en la mayoría de los estudios epidemiológicos, pero similar a la observada en los que incluyen la PSG para el diagnóstico del SAOS.

Status epilepticus management and mortality risk factors: a retrospective study.

INTRODUCTION: Status epilepticus (SE) is a neurological emergency with relatively high mortality rates. In this study, we analysed the management of SE and identified mortality risk factors that may be addressed with educational interventions or modifications to hospital protocols. METHODS: In this retrospective study, we analysed demographic, treatment, and outcome data from 65 patients (mean age, 59 years [range, 44.5-77]; 53.8% women) who were admitted to our tertiary hospital during an 18-month period and met the 2015 International League Against Epilepsy criteria for SE. RESULTS: Thirty patients (46.2%) had history of epilepsy. The most frequent causes of SE were cerebrovascular disease (27.7%) and systemic infection (16.9%). The following deviations were observed in the administration of the antiepileptic drugs: benzodiazepines were used as first option in only 33 (50.8%) patients; the combination of 2 benzodiazepines was recorded in 7 cases (10.8%); and lacosamide was used as an off-label drug in 5 patients (7.7%). Electroencephalography studies were performed in only 26 patients (40%); and only 5 studies (7.7% of patients) were performed within 12 hours of seizure onset. The mortality rate was 21.5%. Acute stroke and cerebrovascular complications were associated with higher mortality rates, while previous history of epilepsy and admission to intensive care were related to better prognosis (P <  .05). CONCLUSIONS: To improve SE management and reduce mortality rates, training activities targeting emergency department physicians should be implemented, together with elective intensive care admission for patients with multiple mortality risk factors (eg, absence of history of epilepsy, acute stroke, or cardiovascular complications).

Syndrome of transient headache and neurological deficits with cerebrospinal fluid lymphocytosis (HaNDL) in a patient with confusional symptoms, diffuse EEG abnormalities, and bilateral vasospasm in transcranial Doppler ultrasound: A case report and literature review. / Síndrome de cefalea transitoria con déficits neurológicos asociados y pleocitosis en líquido cefalorraquídeo (HaNDL) con cuadro confusional, EEG compatible con afectación difusa y datos de vasoespasmo bilateral en estudio Doppler transcraneal: presentación de un caso y revisión de la literatura.

INTRODUCTION: HaNDL syndrome (transient headache and neurological deficits with cerebrospinal fluid lymphocytosis) is characterised by one or more episodes of headache and transient neurological deficits associated with cerebrospinal fluid lymphocytosis. To date, few cases of HaNDL manifesting with confusional symptoms have been described. Likewise, very few patients with HaNDL and confusional symptoms have been evaluated with transcranial Doppler ultrasound (TCD). TCD data from patients with focal involvement reveal changes consistent with vasomotor alterations. DEVELOPMENT: We present the case of a 42-year-old man who experienced headache and confusional symptoms and displayed pleocytosis, diffuse slow activity on EEG, increased blood flow velocity in both middle cerebral arteries on TCD, and single-photon emission computed tomography (SPECT) findings suggestive of diffuse involvement, especially in the left hemisphere. CONCLUSIONS: To our knowledge, this is the first description of a patient with HaNDL, confusional symptoms, diffuse slow activity on EEG, and increased blood flow velocity in TCD. Our findings suggest a relationship between cerebral vasomotor changes and the pathophysiology of HaNDL. TCD may be a useful tool for early diagnosis of HaNDL.

Status epilepticus management and mortality risk factors: A retrospective study. / Manejo y factores de riesgo de mortalidad del estatus epiléptico: estudio retrospectivo.

INTRODUCTION: Status epilepticus (SE) is a neurological emergency with relatively high mortality rates. In this study, we analysed the management of SE and identified mortality risk factors that may be addressed with educational interventions or modifications to hospital protocols. METHODS: In this retrospective study, we analysed demographic, treatment, and outcome data from 65 patients (mean age, 59 years [range, 44.5-77]; 53.8% women) who were admitted to our tertiary hospital during an 18-month period and met the 2015 International League Against Epilepsy criteria for SE. RESULTS: Thirty patients (46.2%) had history of epilepsy. The most frequent causes of SE were cerebrovascular disease (27.7%) and systemic infection (16.9%). The following deviations were observed in the administration of the antiepileptic drugs: benzodiazepines were used as first option in only 33 (50.8%) patients; the combination of 2 benzodiazepines was recorded in 7 cases (10.8%); and lacosamide was used as an off-label drug in 5 patients (7.7%). Electroencephalography studies were performed in only 26 patients (40%); and only 5 studies (7.7% of patients) were performed within 12 hours of seizure onset. The mortality rate was 21.5%. Acute stroke and cerebrovascular complications were associated with higher mortality rates, while previous history of epilepsy and admission to intensive care were related to better prognosis (P <.05). CONCLUSIONS: To improve SE management and reduce mortality rates, training activities targeting emergency department physicians should be implemented, together with elective intensive care admission for patients with multiple mortality risk factors (eg, absence of history of epilepsy, acute stroke, or cardiovascular complications).

[Frontal seizures recorded by magnetoencephalography. Case report]. / Crisis frontales registradas mediante magnetoencefalografía. Caso clínico.

INTRODUCTION: This paper reports the usefulness of magnetoencephalography (MEG) in the anatomical localization of the onset and spread of seizures. CASE REPORT: In a 34-year-old male patient who suffered from drug-resistant complex partial seizures (sometimes generalized) with loss of awareness, magnetic resonance imaging revealed a probable left frontobasal cortical dysplasia. Ictal scalp electroencephalogram showed left frontotemporal theta waves. Electrocorticography (ECoG) registered interictal polyspike discharges and located the seizure onset in the lateral orbital side of the left frontal lobe. Three seizures were registered by MEG, clinically similar to the ones usually experienced by the patient. MEG ictal spike dipole location showed seizure onset coming from the left inferior frontal gyrus (as the ECoG), spreading on to other frontal areas, insula and temporal lobe, all in the left hemisphere. CONCLUSION: MEG may be considered as a useful diagnosis modality in the study of partial seizure physiopathology as well as in its presurgical evaluation.

[Hereditary sensory and autonomic neuropathies. The neurophysiological and pathological aspects of two cases with congenital insensitivity to pain]. / Neuropatías sensitivas autonómicas hereditarias. Dos casos con insensibilidad congenita al dolor; aspectos neurofisiológicos y patológicos.

AIM: Two patients suffering from congenital insensitivity to pain were studied. They corresponded to types IV and V of the 'hereditary sensory and autonomic neuropathies' (HSAN) classification. CASE REPORTS: The first case showed important autonomic dysfunctions, such as anhidrosis, hyperthermia, skin and bone trophic impairment, and mental retardation; the second one only exhibited alterations in pain and temperature sensibilities. In both, chronic indolent corneal ulcers were also present. Conventional neurophysiological evaluation of the neuromuscular system was normal, but an afferent disturbance of the blink reflex (BR) was evident in both. The sympathetic skin response was absent in the HSAN type IV case and normal in the HSAN type V. Notable reduction of the small myelinated fibres, associated to almost no unmyelinated fibres in the first case, were found in the sural nerve biopsies. CONCLUSIONS: So far there haven't been described BR abnormalities in patients with congenital insensitivity to pain, which should be related to a trigeminal sensory impairment, which could explain the corneal ulcers that suffered these cases. BR studies should be included in the neurophysiological evaluation of the suspected small fibre neuropathies even when there are no facial symptoms shown.

[Modified aspiration needle muscular biopsy in neuromuscular diseases. Use and advantages over open surgical biopsy]. / Estudio de las enfermedades neuromusculares por biopsia percutánea mediante aguja con aspiración modificada. Utilidad y ventajas sobre la biopsia quirúrgica.

OBJECTIVE: To evaluate aspiration needle muscular biopsy as an alternative to surgical open biopsy, so we focus on its results and tolerance. PATIENTS AND METHODS: We studied 150 patients with muscular pathology of every kind, and aged between 10 and 86 years, using a modified Allendale/Liverpool needle which by our indication has a built-in lateral funnel that provides faultless aspiration in 100% of cases. Percutaneous biopsy neuromuscular disorders study with modified aspiration Allendale needle: use and advantages over surgical biopsy. RESULTS: The biopsy, which was very well tolerated by the patients, children in particular, left no scar and produced fine samples for standard, immunohistochemical, ultrastructural, biochemical and genetic investigation. In children beyond 10 years of age no general anesthetics was required and in many 4-10 years neither. A number of unsuspected cases of mitochondrial or inflammatory myopathy were detected. Patients with cramps or unclearly defined clinical picture did, however, show frequent morphological pathology. In just three cases samples were defective; all others produced changes of diagnostic or prognostic value with 8% without morphologically abnormal changes. CONCLUSIONS: Modified aspiration needle biopsy is the choice method to study muscle. The samples are of excellent quality allowing for any kind of morphological, biochemical or genetic investigation. The procedure is routinely very well tolerated by patients so is very superior to open surgical biopsy, that we still use for infants in selected cases.

[Refractory temporal epilepsy. An analysis of 33 cases submitted to surgery]. / Epilepsia temporal farmacorresistente. Analisis de 33 casos intervenidos quirurgicamente.

AIM: To analyze clinical manifestations, ictal, interictal and intraoperative EEG monitoring in patients with medical treatment refractory temporal epilepsy successfully controlled surgically. PATIENTS AND METHODS: Retrospective analysis, 33 patients. Mean age: 36 ± 11.40; number of monthly seizures: 9 ± 14.23; mean duration of disease: 22 years; number of anti-epileptic drugs: 3 ± 0.93; average monitoring duration on Intensive Video-EEG Monitoring Unit: 6.42 ± 3.61 days. Scalp EEG was recorded with surface electrodes placed according to the 10-20 international system. RESULTS: 158 seizures were recorded, most of them focal complex. 82% of patients presented aura. Disturbances of consciousness appeared in 94.3%, being more precocious than the automatisms in the majority of the sample. The most frequent and precocious automatisms were oromandibular. Intercritical: epileptiform activity was observed in 87.9% of cases; sharp waves in 93.1%, increasing with sleepiness in 97%. Polyspikes during REM sleep in 21.2%. Bilateral epileptiform activity in 21.1%. Ictal activity: changes on EEG activity was observed previous to clinical manifestation in more than 69%. Type of ictal onset: flattening (46%), rhythmic slow activity (41.7%). Ictal onset was focal in 48.9%. CONCLUSIONS: Detailed knowledge of clinical and electrical manifestations of temporal lobe epilepsies would allow a precocious diagnosis and a reduction of accessing time of these patients to surgical treatment in case of pharmaco-resistance.