Granulomatous Herpes Simplex Encephalitis in an Infant With Multicystic Encephalopathy: A Distinct Clinicopathologic Entity?

Epilepsy surgery for epileptic encephalopathy as a sequela of herpes simplex encephalitis: case report

“…Neuropathological examination of brain tissues is required for the diagnosis of this disease, which is characterized by granulomas consisting of epithelioid macrophages and large multinucleated giant cells that are surrounded by mononuclear inflammatory cells with a prominent plasma cell component, chronic inflammation, and gliosis with extensive mineralization. 1,4,6,7,10,13,18 Chronic granulomatous encephalitis associated with HSV infection generally presents with a biphasic course in older children. 1,6,10,18 Viñas et al 18 described a previously healthy girl who developed HSV-1 encephalitis at the age of 8 years old.…”

“…nfectIon of the central nervous system (CNS) caused by herpes simplex virus (HSV) requires prompt diagnosis and urgent medical attention for treatment. 13 Herpetic encephalitis is often seen as a monophasic acute disease caused by HSV Types 1 and 2 (HSV-1 and HSV-2). The typical clinical presentation is acute encephalopathy and fever in adults and older children, but seizures, lethargy, and fever in infants and younger children.…”

“…The viral antigen and its DNA can be detected in the brain tissue or cerebrospinal fluid (CSF) in the recurrent/relapsing form of HSV encephalitis, and acyclovir remains the treatment of choice. 10,13,18 The diagnosis of HSV encephalitis can be more complex because in some cases, culture, immunohistochemical, and poly-merase chain reaction (PCR) studies are negative because of a postinfectious immune mechanism. 18 In other cases, a progressive clinical course with worsening neurological deficits and seizures can be seen following the initial diagnosis of HSV encephalitis.…”

“…6,10 This clinical course is called "chronic granulomatous HSV encephalitis," which presents as a biphasic disease. 13 Pathological findings are granulomatous inflammation with multinucleated giant cells, gliosis, and multifocal mineralization in the brain parenchyma specimen. 10,13 In this report, we describe an 8-year-old girl with a history of previously treated HSV-1 encephalitis who later developed focal epilepsy.…”

“…13 Pathological findings are granulomatous inflammation with multinucleated giant cells, gliosis, and multifocal mineralization in the brain parenchyma specimen. 10,13 In this report, we describe an 8-year-old girl with a history of previously treated HSV-1 encephalitis who later developed focal epilepsy. Progressive worsening of her behavior and academic functioning were attributed to her electrographic seizures and worsening electroencephalography (EEG) findings accompanied by electrical status epilepticus in slow-wave sleep (ESES).…”