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Lance-Adams syndrome is a rare but devastating disorder characterized by rest, action, and stimulus-sensitive myoclonus after cardiorespiratory arrest. We aimed to present a case of multidrug-resistant Lance-Adams syndrome that was successfully treated with oral phenobarbital therapy.We report a previously healthy 11-year, 6-month-old boy was referred to our pediatric intensive care unit because of severe hypoxic ischemic brain injury due to sudden cardiorespiratory arrest. On the 15th day of hospitalization, he developed myoclonic jerks involving his limbs, trunk, and eyes. Despite many antiseizure medications in different combinations, myoclonic jerks persisted. Then, phenobarbital was started, and myoclonic jerks disappeared the next day. At the final evaluation, additional phenobarbital treatment was continued for 6 months and the patient remained myoclonus-free during this time.To the best of our knowledge, this case is the first report describing significant improvement with phenobarbital in a patient with multidrug-resistant Lance-Adams syndrome. We suggest that phenobarbital is an effective option and should be kept in mind in patients with multidrug-resistant Lance-Adams syndrome.

Background: The discovery of anti-myelin oligodendrocyte glycoprotein (MOG)-IgG and anti-aquaporin 4 (AQP4)-IgG and the observation on certain patients previously diagnosed with multiple sclerosis (MS) actually have an antibody-mediated disease mandated re-evaluation of pediatric MS series. Aim: To describe the characteristics of recent pediatric MS cases by age groups and compare with the cohort established before 2015. Method: Data of pediatric MS patients diagnosed between 2015 and 2021 were collected from 44 pediatric neurology centers across Türkiye. Clinical and paraclinical features were compared between patients with disease onset before 12 years (earlier onset) and ≥12 years (later onset) as well as between our current (2015–2021) and previous (

Chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) is a rare autoimmune peripheral nervous system disease characterized by progressive or relapsing symmetric weakness and sensory dysfunction. It may rarely cause cranial nerve neuropathy. Although cranial nerve involvement is rarer in CIDP than Guillain-Barré syndrome (GBS), it may occur in rapid-onset and relapsed courses. We present an 8-year-old boy with hypoglossal nerve involvement, which has not been previously described in children. No accompanying disorder or antibodies were identified. This report is the first case in the literature for hypoglossal nerve involvement in children with CIDP. This report expands on the phenotypic features of CIDP in children and highlights that CIDP may represent with hypoglossal nerve involvement in children.

Opsoclonus myoclonus ataxia syndrome (OMAS) is a rare neuroinflammatory disorder. We aimed to retrospectively evaluate clinical and laboratory data and outcomes of 23 children diagnosed with OMAS in two children's hospitals between 2010 and 2021.There were 14 boys and 9 girls aged 4-113 months, median 24 months. Ten (43.5%) children had paraneoplastic causes: neuroblastoma/ganglioneuroblastoma (n = 9), acute lymphoblastic leukemia (n = 1). Three children had a postinfectious cause (upper respiratory tract infection in 2, EBV infection in 1) and two had a history of vaccination (varicella in 1, hepatitis A and meningococcal in 1). No underlying factor was identified in 8 (34.8%) children. Speech disorders were more frequent in patients with neural tumors than in those without (p = 0.017). Intravenous immunoglobulin and steroids were effective as initial treatment in most children. Rituximab resulted in at least mild improvement in all 6 children with persistent or recurrent symptoms. Nine (39%) children experienced at least one relapse. Neurological sequelae were detected in 13 (57%) children. There was no significant correlation between clinical characteristics and outcome, except for higher risk of relapse in case of incomplete recovery after first attack (p = 0.001).Acute lymphoblastic leukemia, vaccines against hepatitis A and meningococci can be included among antecedent factors in OMAS. Among clinical symptoms, speech problems might point to the likelihood of an underlying neoplasm in OMAS. Intravenous immunoglobulin and steroids may be chosen for initial treatment while rituximab can increase the chance of recovery in case of persistent or recurrent symptoms. The presence of relapse was associated with poor outcome.

We aimed to report a severe and rare pediatric rhabdomyolysis case associated with a dual viral infection.A 13 year-old, healthy girl presented with the complaints of fever, abdominal pain, weakness and dark-colored urine. She was diagnosed with rhabdomyolysis based on clinical signs and laboratory findings. The diagnosis was confirmed by serological tests and real-time polymerase chain reaction for Epstein-Barr virus (EBV) and cytomegalovirus (CMV), respectively. Other potential genetic, metabolic and infectious causes were evaluated meticulously but no evidence was found. This case is also important as it is the first reported case to our knowledge on rhabdomyolysis associated with EBV and CMV co-infection in children.The presented case experienced tetraplegia due to the severe muscular damage and muscle power returned to normal range after 3 months. This suggests that EBV and CMV may have exert synergistic effects leading to more severe inflammation and degeneration.ZIEL: Unser Ziel war es, einen schweren und seltenen Fall von Rhabdomyolyse im Kindesalter zu berichten, der im Zusammenhang mit einer Koinfektion mit zwei Viren auftrat. FALL: Ein 13-jähiges gesundes Mädchen kam mit Fieber, Bauchschmerzen, Schwäche und dunklem Urin zur Aufnahme. Anhand der klinischen Symptomatik und Laborergebnisse wurde die Diagnose Rhabdomyolyse gestellt. Mittels serologischer Tests und Real-Time-Polymerase-Kettenreaktion erfolgte der Nachweis von EpsteinBarr-Virus (EBV) und Zytomegalievirus (CMV) und die Bestätigung der Diagnose. Andere mögliche genetische, metabolische und infektiöse Ursachen wurden genauestens untersucht, ließen sich jedoch nicht nachweisen. Der Fall ist auch deshalb wertvoll, weil es sich um den ersten Fall von Rhabdomyolyse im Rahmen einer EBV- und CMV-Koinfektion in Kindsalter handelt. SCHLUSSFOLGERUNG: Im vorgestellten Fall kam es durch die schwere Muskelschädigung zu einer Tetraplegie. Bis zur Normalisierung der Muskelkraft vergingen drei Monate. Dies weist darauf hin, dass das EBV und CMV einen synergistischen Effekt ausgeübt haben könnten, der dann zu schwereren entzündlichen und degenerativen Reaktionen führte.

We aimed at evaluating acute neurologic complications (ANC) and clinical outcome at a 2-year follow-up in children after extracorporeal membrane oxygenation (ECMO).We conducted a single-center, retrospective review of our patient cohort aged between 1 month and 18 years at the time of ECMO support (between June 2014 to January 2017). Outcome analysis included ANC and their clinical consequences.The Pediatric Overall Performance Category (POPC) and Pediatric Cerebral Performance Category (PCPC) were used for neurologic assessment performed at discharge from the hospital and at 2nd year follow-up.There were 35 children who required ECMO. The median ECMO time was 9 days (range 2-32 days). Decannulation from ECMO was achieved in 68.6% of patients, and overall, 42.8% survived (15 patients), The incidence of ANC in the surviving patients was 40% (6 children). ANC were intracranial hemorrhage, seizures, cerebral infarction, which occurred in one, two and three of the 15 surviving patients respectively (6.6, 13.3 and 20%). A higher rate of organ failure was related to death (p=0.043), whereas duration on ECMO was a risk factor for the development of ANC (p0.05). At hospital discharge, the 14 patients evaluated had normal development or -mild disability in 73.2%, and at the 2-year follow-up, 93.4% had these scores.Children who receive ECMO have a risk to develop ANC, which was related to the length of ECMO treatment, while survival was related to less organ failure, Long-term neurological outcome was good in our patient cohort.In dieser Studie wollten wir akute neurologische Komplikationen (ANK) und ihre Folgen in einer 2-jährigen Beobachtung bei Kindern nach extrakorporaler Membranoxygenierung (ECMO) beschreiben und bewerten.Patienten in einem Alter zwischen 1 Monat und 18 Jahren, die zwischen Juni 2014 und Januar 2017 eine ECMO Unterstützung benötigten, wurden in diese Einzelzentrums- und retrospektive Studie aufgenommen. Die Ergebnisanalyse umfasste akute neurologische Komplikationen und ihre Folgen. Zur Bewertung wurden die ‚Pediatric Overall Performance Category‘ (POPC) und die ‚Pediatric Cerebral Performance Category‘ (PCPC) bei der Entlassung und bei der Nachuntersuchung im 2. Jahr verwendet.Es wurden 35 Kinder mit der ECMO behandelt. Die durchschnittliche Verweilzeit unter ECMO war neun Tage (2–32 Tage). Eine Beendigung der ECMO wurde bei 68,6%der Patienten erreicht und es überlebten insgesamt 43% (15 Kinder). Die Inzidenz von ANK bei den Überlebenden betrug 40% (6 Kinder): intrakranielle Blutung, zerebraler Anfall, Hirninfarkt, die bei einem, zwei, bzw drei der 15 überlebenden Kindern auftraten (6,6, 13,3 and 20%). Eine höhere Rate an Organversagen war mit dem Tod assoziiert (p=0,043), wohingegen die Zeit an der ECMO als Risikofaktor für die Entwicklung ANK identifiziert wurde (p0,05). Score.Zum Zeitpunkt der Entlassung lag der score bei 73,2% der untersuchten 14 Patienten im normalen oder leicht beeinträchtigten Bereich; nach 2 Jahren zeigten 93,4% diese Scores SCHLUßFOLGERUNG: Kinder, die ECMO-Therapie benötigen, haben, abhängig von der Dauer der Therapie, ein Risiko, ANK zu entwickeln. Das Überleben war mit geringer auftretendem Organversagen korreliert. Das neurologische Langzeitergebnis war in unserer Patientenkohorte gut.

The aims of this study were to evaluate the demographic and clinical characteristics of children with epilepsy who discontinued their antiseizure medication (ASM), to determine potential predictors of seizure relapse, to calculate the rate of seizure relapse, and to detect long-term seizure outcomes.A total of 269 seizure-free children with epilepsy who were decided to discontinue their ASM and were followed up for at least 18 months after ASM withdrawal were retrospectively evaluated.The enrolled children had been followed up for a median of 46 months (range 18-126 months; IQR: 29-61) after ASM withdrawal and 90 (33.5%) of their seizures relapsed. The median time to seizure relapse was 8 months (range 0.23-117 months; IQR: 2-25). Seizure relapse occurred in 16.7% of the 90 children at 1 month, 45.6% at 6 months, 62.2% at 1 year, 74.4% at 2 years, and 94.4% at 5 years. Univariate logistic regression analyses revealed six predictors significantly related to relapse: age at first seizure, age at diagnosis of epilepsy, intellectual disability, EEG findings after ASM withdrawal, ASM tapering time, and number of seizures on ASM. In multivariate logistic regression analyses, age at first seizure, intellectual disability, and ASM tapering time were not significantly associated anymore. The other three remained independently predictive. Pharmacological control of seizures with monotherapy was restored in 93.3% of the children with seizure relapse.This study evaluated potential predictors of seizure relapse, some of which have rarely been evaluated in previous studies. Adolescent age at diagnosis, abnormal EEG findings after ASM withdrawal, and high number of seizures on ASM were associated with a higher risk of seizure relapse. Abnormal MRI findings such as malformations of cortical development and hydrocephalus may be potential biomarkers for the risk of seizure relapse.