Susceptibility to herpes simplex virus type 1 (HSV-1) encephalitis (HSE-1) in otherwise healthy individuals, in the course of primary infection, can be caused by single-gene inborn errors of Toll-like receptor 3 (TLR3) dependent, interferon (IFN)-α/β-mediated immunity,^1,2 or by single-gene inborn errors of snoRNA31.³ These variations underlie infections of the forebrain, whereas mutations of DBR1 underlie infections of the brainstem.³ HSV-2 encephalitis (HSE-2) is typically observed in neonates, albeit also rarely in older children and adults.⁴ Its manifestations include altered level of consciousness, cranial neuropathies or more extensive brainstem encephalitis, hemiparesis, hemisensory loss, and permanent neurologic deficit.⁴ MRI in HSE-2 may show normal findings, nonspecific white matter, orbitofrontal, mesial temporal lobe, or brainstem lesions. Inborn errors of immunity underlying HSE-2 have not been described.

在原发感染过程中，健康的个体对单纯疱疹病毒1型（HSV-1）脑炎（HSE-1）的易感性可能是由依赖Toll样受体3（TLR3）的单基因先天性错误引起的，干扰素（IFN）-α/β介导的免疫力^1,2或snoRNA31的单基因先天性错误。³这些变异是前脑感染的基础，而DBR1突变是脑干感染的基础。³ HSV-2脑炎（HSE-2）通常在新生儿中观察到，尽管在大龄儿童和成人中也很少见。⁴其表现包括意识水平改变，颅神经病或更广泛的脑干脑炎，偏瘫，半感觉丧失和永久性神经功能缺损。⁴ HSE-2的MRI可能显示正常发现，非特异性白质，眶额叶，颞中叶或脑干病变。还没有描述HSE-2潜在的先天性免疫错误。