Abstracts

Rationale: We report a 9-hour-old ex-31+3wks baby girl upon presentation, with a history of prematurity, prenatally diagnosed ventriculomegaly, and found to have congenital HSV infection (purulent vesicles). Subsequent neuroimaging revealed PVL, extensive cystic encephalomalacia, and abnormal electrographic activity in the context of largely absent cortex on neuroimaging, raising the diagnostic dilemma of electrographically identifying seizures vs nonspecific cortical dysfunction.


Methods: An undiagnosed neonate was evaluated in the neonatal ICU. Consent from the mother was obtained for a case report. EEG, neuroimaging, and lab testing were assessed along with a retrospective chart review.


Results: A 9-hour-old ex-31w3d GA female born via NSVD was found to have blistering erythematous desquamating skin lesions, diffuse extensive cystic brain lesions, periventricular leukomalacia (noted on head US), right eye chorioretinitis and bilateral congenital cataracts.

On lumbar puncture, the ME panel was positive for HSV-2, and the patient was started on acyclovir. Abnormal myoclonic jerking movements were noted. EEG revealed frequent rhythmic runs with no clear clinical correlate, although she did exhibit limb stiffening and jerking that occasionally occurred during the runs.

A head ultrasound performed at 5 hours of life demonstrated periventricular leukomalacia and extensive cysts throughout the cortex of the brain. MRI Brain, T2 Axial imaging showed essentially near complete absence of brain parenchyma with relative sparing of the occipital poles and minimal cerebellar tissue. The ventral pons and cerebellar peduncles had cystic changes as well, with the medulla mostly intact.


Conclusions:
Intrauterine HSV encephalitis is a rare presentation of neonatal HSV encephalitis. Assuming an incidence of neonatal HSV encephalitis being 1 in 5000 deliveries, 95% of them occur from direct lesional contact during childbirth, while the remaining 5% were acquired intrauterine. The incidence of intrauterine HSV infection is less than 1 in 100,000 deliveries [1]. This case highlights furthermore a unique presentation of intrauterine HSV encephalitis on neuroimaging with near complete absence of brain parenchyma, as well as electrographically on EEG, which poses a dilemma for anticonvulsant management. This patient had multiple recurrent seizures, with most of them not having a clear clinical correlate. She was treated with Phenobarbital, Fosphenytoin, and Levetiracetam with minimal improvement. It is likely that the electrographic activity seen on EEG in correlation with her MRI brain findings could be representative of a non-epileptogenic disconnected dysfunctional cortex, rather than a treatable seizure.


References

[1] Marquez L, Levy ML, Munoz FM, Palazzi DL. A report of three cases and review of intrauterine herpes simplex virus infection. Pediatr Infect Dis J. 2011 Feb;30(2):153-7. doi: 10.1097/INF.0b013e3181f55a5c. PMID: 20811312.


Funding: None