Herpes Simplex Encephalitis Clinical Presentation

  • Author: Wayne E Anderson, DO; Chief Editor: Karen L Roos, MD   more...
 
Updated: Jun 17, 2011
 

History

Herpes simplex encephalitis (HSE) is an acute or subacute illness that causes both general and focal signs of cerebral dysfunction. It is sporadic and occurs without a seasonal pattern. Although the presence of fever, headache, behavioral changes, confusion, focal neurologic findings, and abnormal cerebrospinal fluid (CSF) findings are suggestive of HSE, no pathognomonic clinical findings reliably distinguish HSE from other neurologic disorders with similar presentations (see Workup).[23]

Patients may have a prodrome of malaise, fever, headache, and nausea, followed by acute or subacute onset of an encephalopathy whose symptoms include lethargy, confusion, and delirium. The following are typically the most common symptoms of HSE[24] :

  • Fever (90%)
  • Headache (81%)
  • Psychiatric symptoms (71%)
  • Seizures (67%)
  • Vomiting (46%)
  • Focal weakness (33%)
  • Memory loss (24%)

Signs and symptoms of neonatal HSE develop about 6-12 days after delivery, at which time lethargy, poor feeding, irritability, tremors, or seizures may be noted. Those with disseminated disease also have abnormal liver function test results and thrombocytopenia. In contrast to older patients, neonates often have herpetic skin lesions.

The initial presentation may be mild or atypical in immunocompromised patients (eg, those with HIV infection or those receiving steroid therapy).

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Physical Examination

The most frequent findings on physical examination are fever and mental status abnormalities. Meningeal signs may be present, but meningismus is uncommon.

Typical findings on presentation include the following[24] :

  • Alteration of consciousness (97%)
  • Fever (92%)
  • Dysphasia (76%)
  • Ataxia (40%)
  • Seizures (38%) - Focal (28%); generalized (10%)
  • Hemiparesis (38%)
  • Cranial nerve defects (32%)
  • Visual field loss (14%)
  • Papilledema (14%)

A causal or temporal relationship between peripheral lesions (eg, herpes labialis) and HSE does not exist. In addition, many febrile diseases may precipitate herpes labialis. Therefore, the presence or absence of such lesions neither confirms nor excludes the diagnosis.

Unusual presentations occur. Both herpes simplex virus type 1 (HSV-1) and herpes simplex virus type 2 (HSV-2) may produce a more subacute encephalitis, apparent psychiatric syndromes, and benign recurrent meningitis. Less commonly, HSV-1 may produce a brain stem encephalitis, and HSV-2 may produce a myelitis.

Ku et al discussed the unique presentation of HSE in a bilingual patient, who developed global aphasia for 1 language (his most recently learned language) but retained most of his birth language ability.[25]

McGrath et al reported on 4 patients with confirmed HSE, each with an anterior opercular syndrome, and observed that the syndrome (ie, paralysis of the masticatory, facial, pharyngeal, and lingual muscles) occurred as the primary manifestation of HSE in 2 patients and as part of the encephalitis picture in the other 2 patients.[26] The authors suggested that unique presentations (eg, anterior opercular syndrome), should alert the clinician to the possibility of HSE.

Mondal et al reported basal ganglia involvement in a child with HSE, demonstrating extrapyramidal symptoms.[27] Li and Sax reported HSE-associated cerebral hemorrhage in an HIV-positive person.[28]

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Complications

Even in treated cases of HSE, complications and sequelae (both focal and global) are not uncommon. If treatment of HSE is delayed, permanent neurologic deficits may develop in survivors.

Common sequelae among survivors include motor deficits, seizure disorders, and changes in mental status. Cognitive and memory deficits are particularly common. So too are recurrent seizures; some authorities recommend prophylactic treatment with anticonvulsant drugs in patients with severe HSE.

In addition, patients with HSE are subject to the same complications as any other seriously ill and immobilized patients with depressed levels of consciousness (eg, aspiration, deep venous thrombosis, decubitus ulcers).

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Contributor Information and Disclosures
Author

Wayne E Anderson, DO  Assistant Professor of Internal Medicine/Neurology, College of Osteopathic Medicine of the Pacific Western University of Health Sciences; Clinical Faculty in Family Medicine, Touro University College of Osteopathic Medicine; Clinical Instructor, Departments of Neurology and Pain Management, California Pacific Medical Center

Wayne E Anderson, DO is a member of the following medical societies: American Academy of Neurology, American Medical Association, American Society of Law, Medicine & Ethics, California Medical Association, and San Francisco Medical Society

Disclosure: Cephalon Honoraria Speaking and teaching; Pfizer Honoraria Speaking and teaching; King Honoraria Speaking and teaching; Forest Honoraria Speaking and teaching

Specialty Editor Board

Ramon Diaz-Arrastia, MD, PhD  Professor, Department of Neurology, University of Texas Southwestern Medical Center at Dallas, Southwestern Medical School; Director, North Texas TBI Research Center, Comprehensive Epilepsy Center, Parkland Memorial Hospital

Ramon Diaz-Arrastia, MD, PhD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Neurology, New York Academy of Sciences, and Phi Beta Kappa

Disclosure: Nothing to disclose.

Francisco Talavera, PharmD, PhD  Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Medscape Salary Employment

Selim R Benbadis, MD  Professor, Director of Comprehensive Epilepsy Program, Departments of Neurology and Neurosurgery, Tampa General Hospital, University of South Florida College of Medicine

Selim R Benbadis, MD is a member of the following medical societies: American Academy of Neurology, American Academy of Sleep Medicine, American Clinical Neurophysiology Society, American Epilepsy Society, and American Medical Association

Disclosure: UCB Pharma Honoraria Speaking, consulting; Lundbeck Honoraria Speaking, consulting; Cyberonics Honoraria Speaking, consulting; Glaxo Smith Kline Honoraria Speaking, consulting; Pfizer Honoraria Speaking, consulting; Sleepmed/DigiTrace Honoraria Speaking, consulting

Chief Editor

Karen L Roos, MD  John and Nancy Nelson Professor of Neurology, Professor of Neurological Surgery, Department of Neurology, Indiana University School of Medicine

Karen L Roos, MD is a member of the following medical societies: American Academy of Neurology and American Neurological Association

Disclosure: Nothing to disclose.

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Axial proton density-weighted image in 62-year-old woman with confusion and herpes encephalitis shows T2 hyperintensity involving right temporal lobe.
Axial gadolinium-enhanced T1-weighted image reveals enhancement of right anterior temporal lobe and parahippocampal gyrus. At right anterior temporal tip is hypointense, crescentic region surrounded by enhancement consistent with small epidural abscess.
Axial diffusion-weighted image reveals restricted diffusion in left medial temporal lobe consistent with herpes encephalitis. This patient also had positive result on polymerase chain reaction assay for herpes simplex virus, which is both sensitive and specific. In addition, patient had periodic lateralized epileptiform discharges on electroencephalography, which supports diagnosis of herpes encephalitis.
 
 
 
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