Confirmation of new epilepsy syndrome in children with HHV-6 PALE

Confirmation of new epilepsy syndrome in children with HHV-6 PALE

Following a previous report of three children who developed symptomatic generalized epilepsy after HHV-6 PALE, a second group from Spain has confirmed the existence of a novel epilepsy syndrome featuring generalized seizures and epileptic encephalopathy after HHV-6-associated PALE (posttransplant limbic encephalitis) in children. This group observed the development of epileptic spasms in a child four months after HHV-6 PALE. The newly described epilepsy syndrome features generalized seizures and cognitive regression after HHV-6 PALE. Additional clinical indications include presentation of epileptic spasms, lack of CSF & serum reactivity with cultured rat hippocampal neurons and rituximab inefficiency.

Including this patient, three out of all four reported children with generalized epilepsy following HHV-6 PALE were <4 years old and suffered loss of attained language and social skills. The authors suggest an “age-dependent susceptibility of the temporal lobe to HHV-6 related epileptogenic insult,” which differs from the specific memory impairment and TLE described in adults. The authors go on to report that detailed imaging findings between the two groups “indicate that this syndrome may appear in children lacking bilateral radiological involvement in the short-term.” In addition, despite a lack of observed reactivity the authors suggest the potential for an inflammatory process at a tissue level, which is undetectable with the investigations performed at this time. Furthermore, the article contributes to mounting evidence that is beginning to support a role of inflammation in epilepsy as a whole.

For more information on the dangers of HHV-6 reactivation in transplantation, and on the role of HHV-6 in epilepsy, read the full paper  and visit the following HHV-6 Foundation webpages:

HHV-6 & Transplant Complications

HHV-6 & Epilepsy