Experience of an interdisciplinary management for pediatric Moyamoya disease: application of a novel Hemispheric Surgical Score.

Purpose: The primary purpose of this study was to develop and implement a novel Hemispheric Surgical Score to guide the treatment of pediatric patients with Moyamoya disease (MMD). Additionally, we aimed to describe a comprehensive flowchart for the evaluation, treatment, and follow-up of these patients and to share our experience with the interdisciplinary management of a large pediatric cohort at a referral pediatric hospital.

Methods: We conducted a retrospective observational study using medical records of patients diagnosed with MMD at the Pediatric Hospital "Prof.

[Arterial ischemic stroke in childhood].

Despite advances in the identification and recognition of risk factors for pediatric arterial ischemic stroke, little progress has been made in hyperacute treatment. The most frequent risk factors are arteriopathies, cardiopathies, and thrombophilia. Early confirmation with neuroradiological studies is key to consider reperfusion therapies, which have limited evidence in pediatrics but a good safety profile.

[Malformations of cortical development and epilepsy].

Around 15% of childhood epilepsies are resistant to antiepileptic drugs, 40% of which are caused by malformations of cortical development (MCD). The current classification scheme for MCD is based on the primary developmental steps of cell proliferation, neuronal migration, and cortical organization. Considering the clinic and molecular alterations, a classification based on main pathways disruption and imaging phenotype has been proposed.

[Status epilepticus].

Status epilepticus (SE) is one of the most common neurologic emergencies in pediatrics. It is a condition resulting either from the failure of the mechanisms responsible for seizure termination or from the initiation of mechanisms, which leads to abnormally, prolonged seizures. This definition provides a good guidance, when emergency treatment must be considered.

Thrombectomy for Acute Stroke in Childhood: A Case Report, Literature Review, and Recommendations.

The updated American Heart Association/American Stroke Association guidelines include recommendation for thrombectomy in certain adult stroke cases. The safety and efficacy of thrombectomy in children are unknown. An 8-year-old girl experienced acute stroke symptoms on two occasions while therapeutically anticoagulated on Novalung.

Ketogenic diet in pediatric patients with refractory focal status epilepticus.

Unlabelled: The ketogenic diet (KD) has been used as an alternative treatment for patients with refractory status epilepticus (SE).

Purpose: In this retrospective study we assess the efficacy and tolerability of the KD in patients with refractory SE.

Methods: Between March 1, 2010 and January 1, 2014, 10 patients who met the diagnostic criteria of refractory SE seen at our department were placed on the KD and followed for a minimum of 6 months.

Congenital hemiparesis, unilateral polymicrogyria and epilepsy with or without status epilepticus during sleep: a study of 66 patients with long-term follow-up.

Aim: We retrospectively analysed the electroclinical features, treatment, and outcome in patients with unilateral polymicrogyria (PMG), focussing on epileptic syndrome with or without encephalopathy, with status epilepticus during sleep (ESES) or continuous spikes and waves during slow sleep (CSWS) syndrome.

Methods: From June 1990 to December 2012, 39 males and 27 females, aged 5-26 years, were studied. We did not include patients with bilateral PMG or cases with unilateral PMG associated with other cerebral lesions.

[Therapeutic developments in chronic ataxias].

Autosomal recessive cerebellar ataxias belong to a broader group of disorders known as inherited ataxias. In most cases onset occurs before the age of 20. These neurological disorders are characterized by degeneration or abnormal development of the cerebellum and spinal cord.

[Acute optic neuropathy: differential diagnoses].

Introduction: Functional impairment of the optic nerve is characterized by visual loss, dyschromatopsia, visual field defects, relative afferent pupillary defect, and swelling or atrophy of the optic nerve.

Aim: To describe the spectrum of acute optic neuropathies, focusing on clinical features, diagnosis and treatment with an emphasis on pediatric entities.

Development: Optic neuritis may be monophasic, recurrent, or part of a polysymptomatic demyelinating process.

Febrile infection-related epilepsy syndrome: a study of 12 patients.

Purpose: To analyze the electroclinical features, neuroimaging findings, treatment, and outcome of 12 patients with febrile infection-related epilepsy syndrome (FIRES).

Methods: This is a retrospective study of 12 children with FIRES with a mean time of follow-up of 6.5 years carried out at the Garrahan Hospital of Buenos Aires between 1997 and 2012.

Rasmussen syndrome: an Argentinean experience in 32 patients.

Purpose: The aim of this study is to analyze the electroclinical features, treatment, and evolution of patients with Rasmussen syndrome (RS).

Materials And Methods: We conducted a retrospective, descriptive study in 32 consecutive patients with RS followed between 1990 and 2012.

Results: Twenty boys and 12 girls were included in the study.

Basal ganglia and internal capsule stroke in childhood--risk factors, neuroimaging, and outcome in a series of 28 patients: a tertiary hospital experience.

We present 28 patients with basal ganglia ischemic stroke and describe the main neurological manifestations, neuroimaging findings, risk factors, and outcome. In 23 cases, at least 1 risk factor was identified. A total of 7 cases (25%) had antecedent of varicella infection and 7 cases (25%) had preceding mild head trauma.

Accidental cerebral venous gas embolism in a young patient with congenital heart disease.

This article describes an 11-year-old girl with a diagnosis of Ebstein's anomaly. Glenn and Fontan surgeries were performed successfully. She had a generalized tonic-clonic seizure after peripheral intravenous infusion under pressure.