Neonatal herpes simplex encephalitis is caused by vertical transmission of infection during passage from birth canal with diffuse cerebral involvement within the first month after birth; in contrast to adult herpes simplex encephalitis, it is commonly related to HSV-2.
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Epidemiology
The incidence of neonatal HSV infection at all is usually low and it varies by country. About 80% of cases are due to HSV-2.
Clinical presentation
There are three types of clinical manifestations related to this infection 2:
skin lesions without any visceral or central nervous system (CNS) involvement, also known as skin, eye and mouth disease
CNS disease (with or without skin lesions, but without involvement of visceral organs); usually this presentation has non-specific signs including decreased level of consciousness, seizures, lethargy and fever
disseminated form characterized as a sepsis with multi-organ failure
Newborn babies are initially asymptomatic for one or two weeks.
The diagnosis is confirmed by detection of HSV DNA in the cerebrospinal fluid.
Radiographic features
It is important to appreciate that the radiographic appearance of neonatal HSV encephalitis is different from its more common adult counterpart. Changes are typically diffuse which can be difficult to identify due to normal immature myelin (see normal myelination) and more commonly involving the cerebral cortex, the deep white matter, and thalamus 4. The medial temporal and inferior frontal lobes may be spared and hemorrhagic change is uncommon but can develop later and is best seen on gradient echo/susceptibility sequences 1.
As sequelae, areas of leukomalacia, diffuse multicystic encephalomalacia, and parenchymal punctate or gyral calcifications may be seen 3.
CT
may be negative in the early course of the disease
in advanced stages may present with extensive areas of parenchymal hypoattenuation, predominantly in the white matter, as a result of edema or necrosis 3
enhancement usually presents in a gyriform pattern
MRI
Affected areas, however, have a similar appearance regarding signal characteristics:
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T1
may show general edema in the affected region
if complicated by subacute hemorrhage there may be areas of hyperintense signal
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T2
hyperintensity of affected white matter and cortex, predominantly due to edema
as previously discussed, the incomplete white matter myelination in the neonate's brain and its high water content may mask subtle signal changes
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DWI/ADC
restriction diffusion is demonstrated in approximately half of all patients, which tends to be diffuse and bilateral
restricted diffusion is common due to cytotoxic edema
restricted diffusion is less intense compared to infarction
beware of T2 shine through due to vasogenic edema
GE/SWI: may demonstrate blooming if hemorrhagic
T1 C+ (Gd): enhancement usually presents in a gyriform pattern
Treatment and prognosis
Neonatal herpes simplex encephalitis is highly lethal (in about 50% of cases) and can cause permanent disability if left untreated 2.
Treatment is with intravenous antivirals (acyclovir is usually the drug of choice).
Sequelae are mostly seen in neurodevelopment, including deafness, vision loss, cerebral palsy, and seizure.