Telemedicine- versus outpatient-based initiation and management of ketogenic diet therapy in children with drug-resistant epilepsy during the COVID-19 pandemic.

INTRODUCTION: Initiation of ketogenic diet therapies (KDT) for pediatric epilepsy is usually done on an inpatient basis and the diet is managed during clinical appointments following a protocol of visits and routine tests. Because of the 2019 coronavirus disease (COVID-19) pandemic and the associated lock-down measures, we switched from outpatient to telemedicine-based KDT initiation. OBJECTIVE: To explore the feasibility, effectiveness, and safety of online KDT initiation and follow-up by comparing a group of children with drug-resistant epilepsy that was managed by telemedicine compared to a group that was treated on an outpatient basis. MATERIALS AND METHODS: An observational study was conducted in two groups of patients with drug-resistant epilepsy who initiated KDT and were followed up with an online versus an outpatient modality by the interdisciplinary KDT team of Hospital Pediatria JP Garrahan in Buenos Aires, Argentina. Dietary compliance, ketosis, retention rate, adverse effects, number of contacts, and clinical outcome were evaluated at 1, 3, and 6 months on the diet. RESULTS: Overall, 37 patients were included, of whom 18 started the KD by telemedicine and 19 on an outpatient basis. Minimum follow-up of the patients was 6 months. All patients received the classic ketogenic diet. No statistical differences between the two groups regarding efficacy and safety of the diet were found. CONCLUSIONS: Our results support the feasibility and safety of initiating and management of KDT by telemedicine. Patients and their families should be carefully selected in order to guarantee a good outcome.

[Update on the adverse effects during therapy with a ketogenic diet in paediatric refractory epilepsy]. / Actualizacion sobre los efectos adversos durante la terapia con dieta cetogenica en la epilepsia refractaria pediatrica.

INTRODUCTION: The ketogenic diet is a non-pharmacological treatment that has been used as a therapeutic alternative for the management of refractory epilepsy since 1921. It is a diet, high in fats and low in carbohydrates, which is used to treat paediatric refractory epilepsy, and is effective in around 50% of the patients that begin to follow it. It is a nutrition plan that is imbalanced in terms of both macro- and micro-nutrients, which can give rise to nutritional deficits in energy, proteins, minerals and vitamins, as well as an excess of lipids. There is therefore a risk of it causing undesirable side effects both at the outset and in later stages of the treatment. DEVELOPMENT: The most frequent adverse effects that may occur either in acute form or in later stages are described, and tools that can be used in their management and prevention during treatment are proposed for paediatricians and ketogenic diet teams. CONCLUSIONS: Some adverse effects are difficult to interpret and may manifest due to involvement at different levels of the organism, thereby arousing doubts as to whether they are caused by the diet, the antiepileptic medication or by the patient's own intercurrent complications. It is important to follow the study and evaluation frequency protocol in order to detect and prevent these effects, as well as to consult reference centres in order to evaluate the cost-benefit of continuing the treatment or not.

TITLE: Actualizacion sobre los efectos adversos durante la terapia con dieta cetogenica en la epilepsia refractaria pediatrica.Introduccion. La dieta cetogenica es una terapia no farmacologica que se ha usado como alternativa para el tratamiento de la epilepsia refractaria desde 1921. Es una dieta alta en grasas y baja en hidratos de carbono, que se utiliza en el tratamiento de la epilepsia refractaria pediatrica, efectiva en alrededor del 50% de los pacientes que la inician. Se trata de un plan alimentario desequilibrado tanto en macro como en micronutrientes, que puede causar deficits nutricionales en energia, proteinas, minerales y vitaminas, y exceso de lipidos, con riesgo de ocasionar efectos secundarios no deseados tanto en el inicio del tratamiento como de forma tardia. Desarrollo. Se describen los efectos adversos mas frecuentes que se presentan en forma aguda o tardiamente, y se brindan herramientas para su manejo y prevencion durante el tratamiento para pediatras y equipos de dieta cetogenica. Conclusiones. Algunos efectos adversos son de dificil interpretacion, y pueden ser manifestacion por afectacion a diferentes niveles del organismo, poniendo en duda si son secundarios a la dieta, a la medicacion antiepileptica o a intercurrencias del propio paciente. Es importante seguir el protocolo de frecuencia de estudios y evaluaciones para detectar y prevenir estos efectos, y consultar a centros de referencia evaluando el coste-beneficio de continuar o no el tratamiento.

Focal polymicrogyria in mother and son.

This 9-year-old boy was admitted at the age of 2 with a diagnosis of congenital hemiparesis while the rest of physical and neurological examination was normal. His score in the Wechsler intelligence scale was 80. Right fronto-parietal cortical dysplasia with hemisphere atrophy was evident by computerized tomography scanning and magnetic resonance imaging. The latter, also disclosed abnormal thick cortex which was interpreted as polymicrogyria or pachygyria. Karyotype was normal. He had a hemifacial motor seizure at the age of 7. At the age of 8 frequent atonic or inhibitory seizures were presented. Asymmetric bilateral spike discharges with high voltage in the right hemisphere during the EEG recording were found. His mother, a 35-year-old woman (Full scale; Adult intelligence scale: 85) also had congenital hemiparesis. She never had seizures and her EEG was normal. Magnetic resonance imaging disclosed right fronto-parietal cortical dysplasia with ipsilateral hemisphere atrophy. Karyotype was normal. Our cases should be interpreted as a familial presentation of the anomaly, probably with autosomal-dominant transmission.

Atypical evolution in childhood epilepsy with occipital paroxysms (Panayiotopoulos type).

We report, on two, school-age girls with clinical and electroencephalographic features of early onset childhood epilepsy with occipital paroxysms (CEOP) of the "Panayiotopoulos type" that showed atypical evolution. Neurological examination and brain imaging were normal in both. One child presented at age 2.5 years episodes of oculocephalic deviation, and ictal vomiting during nocturnal sleep. The EEG showed left occipital spikes during wakefulness and sleep. One year later, frequent inhibitory seizures appeared in the lower limbs causing, "pseudoataxic gait". At the same time she presented with behavioral disturbances and aphasia. EEG showed bilateral spike-waves while awake and continuous spike-waves during slow sleep (CSWSS). After switching AEDs to benzodiazepines, control of seizures along with improvement of behavior, and partial restoration of cognitive functions were achieved. The CSWSS disappeared and the last EEG at age 8 years only showed only isolated right occipital spikes. The other girl had a personal and familial history of febrile seizures. At 4 years of age she presented the first non-febrile seizures during sleep, with oculocephalic deviation and ictal vomiting, followed by a generalized tonic-clonic seizure. Partial control of seizures was obtained with antiepileptic drugs. At age 7, the child began to have weekly episodes of oculocephalic version, occasionally with secondary generalization. Repeated inhibitory seizures and absences also appeared. EEG showed frequent bilateral spikes occupying predominantly the posterior regions while awake, and CSWSS. At 7.5 years the same electro-clinical picture persisted. Ethosuximide was added to sodium valproate and clobazam. Fifteen days later, the seizures disappeared and the EEG showed less frequent bilateral occipital spikes. She is now 9 years old and she has been seizure-free for 18 months. Her present neuropsychological profile shows mild mental retardation. The two children with typical electroclinical features of "Panayiotopoulos Type" CEOP developed an atypical evolution which, to our knowledge, has not been described previously.

[A particular type of epilepsy in patients with congenital hemiparesis associated with polymicrogyria or unilateral pachygyria]. / Un tipo particular de epilepsia en pacientes con hemiparesia congénita asociada a polimicrogiria o paquigiria unilateral.

INTRODUCTION: Magnetic resonance has permitted the recognition of cortical dysplasias in patients with congenital hemiparesia and epilepsy. OBJECTIVE: To study the clinic-EEG characteristics and course of epilepsy in patients with congenital hemiparesia and unilateral polymicrogyria. METHODS AND RESULTS: We analyzed the clinical histories of 11 patients seen between 1990 and 1996. We studied 6 girls and 5 boys aged between 5 and 13 years, with a follow-up period of from 1 to 6 years. The epilepsy began at between 1 and 6 years old with partial motor seizures. On EEG there were frontotemporal spikes in 9 cases, temporooccipital in 1 and parieto-occipital in another. All 11 patients had hemiparesia, with slight mental retardation in 9 patients and moderate mental retardation in 2. The CT/MR brain scan showed unilateral polymicrogyria. At between 2 and 8 years of age, all 11 patients developed subintrant atonic crises with a pseudo-ataxic gait, absences in 7 patients and myoclonia in 3. Awake EEG showed bilateral asymmetrical spikes. During sleep 7 patients had continuous spikewave discharges and 4 had frequent asymmetrical bilateral spikes. Four patients relapsed. Five patients are free of crises, five have sporadic crises and one continues to have daily crises. CONCLUSIONS: These patients had hemiparetic cerebral paralysis, slight mental retardation and epilepsy. At about the age of 6 a peculiar electro-clinical condition developed. Response to treatment was satisfactory, although the follow-up period is still not long.

[Idiopathic partial epilepsy with occipital paroxysms]. / Epilepsias parciales idiopáticas con paroxismos occipitales.

INTRODUCTION: Partial benign epilepsy of childhood with occipital paroxysms (EPBI-O) described by Gastaut in 1982, has been recognized in the latest Classification of Epilepsy and Epileptic Syndromes as being a form of idiopathic partial epilepsy. In 1989 Panayiotopoulos described a different form of idiopathic occipital epilepsy. OBJECTIVE: We analyzed the electro-clinical characteristics of EPBI-O in order to identify the different forms of idiopathic occipital epilepsy. MATERIAL AND METHODS: This prospective study included 74 patients who fulfilled the diagnostic criteria of EPBI-O. The patients were evaluated between January 1990 and June 1996 by means of clinical and electro-encephalic examinations during a follow-up period of between 6 months and 6.5 years. RESULTS: We identified two groups. Group I: eighteen patients with visual crises and post-ictal migrainous episodes, with or without motor crises, predominantly when awake and with the onset of these crises at 8.7 years old. On EEG there were P-O occipital paroxysms which reacted to eyelid opening and group II: fifty-six patients with crises of vomiting followed by oculo-encephalic version, predominantly during sleep and with an average age of 4.9 years when these crises started to occur. On EEG there were occipital spikes with identical morphology to that of the benign epilepsy of childhood with spikes of Roland (EPBI-R). Two patients had associated crises of EPBI-O of Group II and EPBI-R with independent occipital and Roland spikes. These formed a third group. CONCLUSIONS: This study confirms the existence of two variantes of EPBI-O; the 'Gastaut' type and the 'Panayiotopoulos' type with a more benign and more frequent course.

[Acquired epileptic aphasia]. / Afasia epiléptica adquirida.

OBJECTIVE: To evaluate clinical and EEG features, as well as treatment and progression in fifteen patients with a diagnosis of acquired epileptic aphasia. PATIENTS AND METHODS: The population comprised nine male and six female patients, whose mean age was 14.5 (r = 8.11-20 years). All were on routine antiepileptic drugs. Inclusion criteria were acute, subacute or chronic aphasia, lacking signs of motor deficit or demonstrable brain lesion, but displaying bilateral spikes or generalized spike-wave discharges. Throughout, sleep and waking EEG, neuroimaging, brainstem auditory evoked potentials and neuro-psychological evaluations were performed. Cortical brainstem auditory evoked potentials were carried out in ten cases. RESULTS: Median age at onset of verbal auditory agnosia was 5.6 years (r = 1.1-8.6 years), which eleven cases developed epileptic seizures at a median age of five years. Waking and sleep EEG were abnormal but brainstem auditory evoked potentials were normal throughout. Cortical brainstem auditory evoked potentials in ten patients displayed P300 wave and vertex potential alterations. Five cases received 1-3 mg/kg/day prednisone during 6-12 months, with almost complete speech recovery in four. At the last follow-up, language impairment was mild in five patients, moderate in five and severe in three, while two children recovered normal speech. CONCLUSIONS: Early acquired epileptic aphasia treatment is advisable with valproic acid, benzodiazepines or ethosuximide alone or in combinations, supplementary with corticoids for at least six months in the absence of clinical response and/or EEG improvement. Globally, seven out of fifteen patients overcame their speech disorder.