neurology today

According to a study published on November 2 in Neurology suggests.

“Our work amplifies the findings of three previous reports of influenza-associated death cases in SCN1A-DS, and [it] highlights how susceptible patients with SCN1A-DS are to the life-threatening sequelae of influenza infection,” wrote lead author Katherine B. Howell, PhD, of the Royal Children’s Hospital, University of Melbourne, and Murdoch Children’s Research Institute, all in Melbourne, Australia “Neurological complications of influenza are more common and more severe in people with SCN1A-DS than in general pediatric patients. [sic] population.”

According to the researchers, 7 to 120 out of 10,000 children in the general population are hospitalized with the flu each year, and 9 to 20 percent end up developing neurological complications, such as simple febrile seizures. Most patients recover despite the severity of the complications, the researchers said, but those with pre-existing neurological complications generally have higher rates of flu-related neurological complications and even death.

Dr. Howell and colleagues sought to determine the range and frequency of complications from influenza infection in people with SCN1A-DS, a developmental, epileptic encephalopathy that researchers say occurs in 6.5 per 100,000 live births. According to the study, vaccine-near seizures or vaccine-near status epilepticus (SE) occur in 27 to 40 percent of people with the disease.

“As a result, rates of vaccination hesitancy and refusal as well as incomplete vaccination are high in this patient population,” the researchers said. “However, [Dravet syndrome] is also associated with a high risk of seizures and SE with febrile illness, underscoring the critical need to protect patients against the sequelae of vaccine-preventable illnesses.

The researchers found the 82 study participants through neurologists at two Melbourne hospitals and through searches of EEG databases, the Victorian PAEDS FluCan database and the program database. of Genetic Research in Epilepsy from the University of Melbourne. They reviewed medical records and spoke to families of patients seen at Austin Hospital Dravet Clinic in 2019 and 2020 to identify participants who had the flu; they then confirmed these infections via pathology reports.

“Influenza infection is common, occurring in a quarter of our cohort of individuals with SCN1A-DS,” the researchers said.

Specifically, 21 people had a total of 24 documented influenza infections (17 type A and seven type B) at a median age of 4 years. Seventy-five percent sought treatment in a hospital for SE or exacerbations of seizures.

“One child died of influenza pneumonia and long-term neurological sequelae were observed with four infections,” the researchers said. [SE]; brain imaging in two showed cerebral edema, with one also having imaging features of laminar necrosis. All have ongoing neurological deficits from baseline, with one having profound global impairment.”

Six participants developed sequelae of influenza infection at a median age of 7.5 years; seizures occurred for less than a month to several times a week. Two patients developed sequelae after brief hospital stays.

“Follow some [two to eight] years after influenza infection found that sequelae remained severe in two individuals and were moderately severe in one, affecting most or all domains of function,” the researchers said. “The most severely affected individual is unresponsive, has poor head control and is fed via gastrostomy. The sequelae were cumulative in the individual with two infections, who needed help to move around and feed himself by gastrostomy after his first infection, and who needed a wheelchair to move around after the second.

While patients with SCN1A-DS could potentially develop vaccine-near seizures (VPS) or vaccine-near SE, the researchers concluded that “it is likely that influenza vaccination is safer than ‘influenza infection in patients with SCN1A-DS’.

“Our data suggest that influenza vaccine can be safely administered in this population, although it is unclear whether antipyretics and additional ASM [antiseizure medications] should be used consistently,” they said. “Prior to influenza vaccination in children with SCN1A-DS, it may be prudent to review the regular ASM regimen, discontinue contraindicated ASMs where possible, and ensure a management plan emergency seizures, including rapid administration of benzodiazepines. , is in place.”

In an accompanying editorial, Katherine C. Nickels, MD, and Elaine C. Wirrell, MD, FRCP, Divisions of Childhood and Adolescent Neurology and Epilepsy, Department of Neurology, Mayo Clinic, stated that researchers have demonstrated that antipyretics or other ASMs can mediate the exacerbation of seizures that can occur in patients with SCN1A-DS when they receive a vaccination.

“While all children can experience acute encephalopathy with influenza, those with Dravet syndrome are likely to be at greater risk of permanent neurological sequelae, even with aggressive treatment. … Therefore, the benefits of a proactive approach to routine vaccinations far outweigh the risks,” said Drs Nickels and Wirrell. “All children can suffer from acute encephalopathy with viral illnesses, such as influenza, making adherence to Vaccination essential for all.

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