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The Tangled Web of Complex Febrile Seizures: A Common But Complex Conundrum

Definitions and terminology of complex febrile seizures - is  a  subclassification required
Rekha Mittal

While there is now clarity on most aspects of the immediate / long term management and prognosis of simple febrile seizures (SFS), the same is not true for complex febrile seizures (CFS). Characteristics of CFS are one or more of any the following: focal features, duration more than 10 or 15 minutes or recurrence within 24 hours of the febrile illness. However, recent literature suggests that each of these parameters may require  different investigation and immediate and long-term management protocols. Confusion also arises from the operational definition of status epilepticus (SE), which is defined as a seizure lasting for more than 5 minutes.  There may thus be a case for a FS to be called CFS if the duration is more than 5 minutes, instead of 15 minutes. Febrile SE, defined as seizures with fever lasting 30 minutes or more, may also lie within the spectrum of CFS, as some children who initially present with CFS of the prolonged type, may have febrile SE in subsequent episodes.  Pre-existing neurological deficits, which also affect immediate and long-term management of febrile seizures, are not a descriptor for CFS.  FS + and GEFS + is another group, where FS either continue beyond 5-6 years of age or are accompanied by afebrile seizures; these seizures may also have simple or complex characteristics. To reconcile these issues, we will suggest that a subclassification is required for CFS for added clarity, which may be helpful for both researchers and clinicians.

 

Diagnostic testing (EEG, MRI, CSF studies) in Complex Febrile Seizures: when and why, and are they overdone?
Puneet Jain

Children who experience simple febrile seizure and are well-appearing do not require routine diagnostic testing. However, the decision-making is unclear for complex febrile seizures. Pediatricians and neurologists are frequently in a dilemma regarding the extent of investigations indicated in children with complex febrile seizures. This talk will review the available literature and provide guidance in this regard, supplemented with case scenarios. Based on published literature, the yield of lumbar puncture or neuroimaging in a child with complex febrile seizures is low; however, these must be considered in presence of an abnormal neurological examination.
Recent literature on association between HHV 6 and complex febrile seizures will be discussed. EEG usually has no role in acute management of febrile seizures. However, it should be considered in children with multiple risk factors for epilepsy (e.g. developmental delay, family history).  The optimal timing of the investigations is not clear, and literature regarding this will be reviewed. The talk will also focus on the need for these investigations in febrile status epilepticus. Data from FEBSTAT study will be summarized.

 

Prognostication and counseling families of children with Complex Febrile seziures
Suvasini Sharma

Families of children with complex febrile seizures are often worried about the risks of febrile seizure recurrence, development of epilepsy, intellectual disability and death. As per retrospective studies, 5-15% of children with complex febrile seizures develop epilepsy. The risk factors for the development of epilepsy in children with complex febrile seizures include the presence of underlying developmental delay/neurological abnormalities and a family history of epilepsy. In this talk, the evidence for the prediction of all these outcomes will be discussed, so that an informed counseling can be done. The role of clinical factors such as age at onset, presence of family history, co-morbid developmental delay, EEG and MRI abnormalities, and anti-seizure medications in the prediction of recurrence of febrile seizures, future development of epilepsy, and neurodevelopmental outcomes will be explored. The role of genetic testing in predicting the outcomes and the rare risk of SUDEP in this population will also be discussed. The aim is to summarize an evidence-based counseling framework for prognostication and counseling families of children with complex febrile seizures.

 

Long term management : is there a right choice – no prophylaxis vs intermittent prophylaxis vs continuous prophylaxis with antiseizure medication
Jo Wilmshurst

Whilst most Febrile seizures (FS) are self-limited and resolve with age, they can be recurrent, resulting in caregiver distress and frequent demand for intervention. Simple febrile seizures (SFS) do not warrant prophylactic ASM, but when the events are recurrent, the management becomes more contentious. There is no consistency in the recommendations of existing guidelines. A number of them support use of prophylactic short term ASM at the start of a febrile illness (diazepam, topiramate, levetiracetam), but these often acknowledge adverse effects. A Cochrane review also concluded that reduced recurrence rates for children with FS was evident with use of intermittent diazepam and continuous phenobarbital, but associated with adverse effects in up to 30%. Based on the self-limited nature of recurrent FS, and the high prevalence of adverse effects of these drugs, caregivers should be fully informed of the self-limited nature of the condition and supported with adequate emergency care plans, the most widely supported recommendation is that SFS do not require intervention with ASMs. The other intervention supported by some guidelines is access to rescue therapy for FS which are no longer simple FS. The role for regular ASM is generally not supported by guidelines; the Japanese guidelines are one of the few that are more open to regular phenobarbital for recurrent FS (more than 3) and in the setting of marked parental anxiety.