Parents' priorities for decision-making of pediatric epilepsy treatments and perceived needs for decision support in multi-ethnic Asian clinical setting: A qualitative analysis.

OBJECTIVE: To identify parents' priorities when making a decision on genetic testing and antiseizure drug (ASD) options for pediatric epilepsy and their support needs for informed decision-making in multi-ethnic Asian clinical settings. METHODS: Qualitative in-depth interviews, using a semi-structured interview guide, were conducted with purposively selected parents of pediatric patients with newly diagnosed epilepsy or known diagnosis of epilepsy (n = 26). Interviews were audio recorded and transcribed verbatim. Thematic analysis was undertaken to generate themes. RESULTS: Parents' narratives showed difficulty assimilating information, while knowledge deficit and emotional vulnerability led parents' desire to defer a decision for testing and ASDs to mitigate decisional burden. Priorities for decisions were primarily based on intuitive ideas of the treatment's risks and benefits, yet very few could elaborate on tradeoffs between risks and efficacy. Priorities outside the purview of the healthcare team, such as children's emotional wellbeing and family burden of ASD administration, were also considered important. Authority-of-medical-professional heuristic facilitated the ASD decision for parents who preferred shared rather than sole responsibility for a decision. Importantly, parents' support needs for informed decision-making were very much related to the availability of support mechanisms in post-treatment decisions owing to perceived uncertainty of the chosen ASD. CONCLUSIONS: Findings suggest that multiple priorities influenced ASD decision process. To address support needs of parents for informed decision-making, more consideration should be given to post-treatment decision support through the provision of educational opportunities, building peer support networks, and developing a novel communication channel between healthcare providers and parents.

Unique Severe HyperEkplexia-Like Apneic Events (SHELAE) Improved by High-Dose Piracetam.

Breath-holding spells are common non-epileptic events with onset between 6 months and 18 months of age that are usually triggered by minor painful events or strong emotions. Symptomatic treatments for breath-holding spells include iron supplementation, glycopyrrolate and piracetam. Hyperekplexia is a rare non-epileptic disorder characterized by generalized hypertonia and exaggerated startle. Prolonged stiffening triggered by startle can lead to desaturation, cardiac asystole and sudden infant death. It is commonly treated with Clonazepam and other anti-epileptic drugs. Piracetam has been reported to be effective in some anecdotal cases. We describe a case of an infant with frequent hyperekplexia-like breath-holding events who failed to respond adequately to glycopyrrolate, pace-maker insertion and clonazepam, who had marked improvement in his symptoms with high dose Piracetam. High dose Piracetam should be considered in infants with similar severe hyperekplexia-like/breath-holding events as it may be beneficial in ameliorating the acute and chronic course in these children.

Favorable Outcomes With Early Interleukin 6 Receptor Blockade in Severe Acute Necrotizing Encephalopathy of Childhood.

BACKGROUND: Outcome in severe acute necrotizing encephalopathy of childhood is poor, with high mortality (30%) and moderate to severe disability in survivors despite the use of intravenous corticosteroids or immunoglobulins. Increased blood interleukin 6 level correlates with poor outcome. METHODS: We report the early use of tocilizumab, a monoclonal antibody against the interleukin 6 receptor, in three patients (aged five, eight, and 10 years) with severe acute necrotizing encephalopathy. RESULTS: All three patients experienced a rapid neurological deterioration associated with febrile viral illnesses and met criteria for severe acute necrotizing encephalopathy with a high risk for death or severe disability. Intravenous methylprednisolone and tocilizumab were administered at 18 to 32 hours of encephalopathy in addition to supportive medical therapy. No side effects were observed with this therapeutic strategy. The two patients with influenza A(H1N1)pdm09 virus-related acute necrotizing encephalopathy had a short illness with excellent clinical and radiological recovery. The patient with influenza B virus-related acute necrotizing encephalopathy and florid hemorrhagic brain lesions had a slow recovery with eventual mild disability despite focal encephalomalacia on follow-up neuroimaging. CONCLUSIONS: The early use of interleukin 6 blockade in acute necrotizing encephalopathy is safe and may have a role in improving outcomes and preventing disability.