Herpes simplex encephalitis

Herpes simplex (HSV) encephalitis is the most common cause of fatal sporadic fulminant necrotising viral encephalitis and has characteristic imaging findings. 

Two subtypes are recognised which differ in demographics, virus and pattern of involvement. They are 1:

  1. neonatal herpes encephalitis
  2. childhood and adult herpes encephalitis

This article concerns itself with the latter. For a discussion of the former, please refer to the article neonatal herpes simplex encephalitis.

Epidemiology

Childhood and adult herpes encephalitis is usually due to HSV-1 (90%) with the rest due to HSV-2 5. There is no particular age, sex or seasonal predilection.

Clinical presentation

The presentation is unfortunately relatively non-specific consisting of fever, headaches, focal neurological deficits, seizures, and altered or decreased level of consciousness.

Diagnosis is established with PCR of CSF, although the combination of the clinical scenario, CSF demonstrating pleocytosis and elevated protein, and appropriate imaging is usually highly suggestive and permits commencement of treatment.

Pathology

HSV is an obligatory intracellular virus that enters via infecting nasopharyngeal cells into the sensory branch of lingual nerve then ascends to trigeminal ganglion and remains latent for a lifetime. Reactivation in the case of immunosuppression, trauma, or other stresses can result in fulminant haemorrhagic necrotising encephalitis. HSV has a high affinity for limbic systems with bilateral but asymmetrical involvement.

Histology

Perivascular cuffs of lymphocytes, large inclusions in neurons and glial cells called "owl's eye", neurophagia, necrosis and haemorrhage are characteristics of HSE.

Radiographic features

In the immunocompetent adult patient, the pattern is quite typical and manifests as a bilateral asymmetrical involvement of the limbic system, medial temporal lobes, insular cortices and inferolateral frontal lobes. The basal ganglia are typically spared, helping to distinguish it from a middle cerebral artery infarct.

Extralimbic involvement is more prevalent in children than in adults, seen most commonly in the parietal lobe, with sparing of basal ganglia. Eventually, it results in marked cystic encephalomalacia and volume loss in affected areas.

In immunocompromised patients, involvement can be more diffuse, and more likely to involve the brainstem 5.

CT

Early diagnosis is difficult and a 'normal' scan should not dissuade from the diagnosis. If findings are present, they typically consist of subtle low density within the anterior and medial parts of the temporal lobe and the island of Reil (insular cortex) 2. If scanned later then the changes may become more apparent and even progress to haemorrhage. 

Contrast enhancement is uncommon during the first week of the disease. After that patchy low-level enhancement may be seen 5.

MRI

Affected areas, however, have a similar appearance, in terms of signal characteristics:

  • T1
    • may show general oedema in the affected region
    • if complicated by subacute haemorrhage there may be areas of hyperintense signal
  • T1 C+ (Gd)
    • enhancement is usually absent early in the disease
    • enhancement occurs later and is variable in pattern 5
      • gyral enhancement
      • leptomeningeal enhancement
      • ring enhancement
      • diffuse enhancement
  • T2
    • hyperintensity of affected white matter and cortex
    • more established haemorrhagic components may the hypointense
  • DWI/ADC
  • GE/SWI
    • may demonstrate blooming if haemorrhagic (rare in neonates, common in older patients)

Treatment and prognosis

Mortality ranges dramatically depending on how early treatment is instituted. Even in patients who are young and otherwise well, and only lethargic still have a mortality of 25%. Older patients or those comatose at the time treatment is started invariably have a much poorer outcome 3. Overall mortality is over 70% with only 2.5% of affected patients every fully recovering 5.

Treatment is with intravenous antivirals (e.g. acyclovir).

Differential diagnosis

General imaging differential considerations include:

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Article Information

rID: 1410
Section: Pathology
Synonyms or Alternate Spellings:
  • Adult herpes simplex encephalitis
  • Adult HSV encephalitis
  • HSV encephalitis
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    Case 10: with multifocal haemorrhage
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