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More than typical sleepiness during day. Increased reflexes. Low glucose levels in cerebral spinal fluid. Decreased immune function. Repeated seizures without recovery between them. Do you have more information about symptoms of this disease? We want to hear from you. Do you have updated information on this disease? Treatment Treatment. Related Diseases Related Diseases.
Conditions with similar signs and symptoms from Orphanet. Differential diagnosis includes: other viral causes of encephalitis, varicella zoster virus, enteroviruses etc ; bacterial infections, including ricketsial disease, mycoplasma pneumonia, chlamydophila infections; autoimmune diseases, such as paraneoplastic limbic encephalitis, acute disseminated encephalomyelitis, Rasmussen subacute encephalitis; and other causes including space occupying lesion and non-infectious encephalopathy.
Visit the Orphanet disease page for more information. Research Research. Clinical Research Resources ClinicalTrials. Click on the link to go to ClinicalTrials. Please note: Studies listed on the ClinicalTrials. We strongly recommend that you talk with a trusted healthcare provider before choosing to participate in any clinical study. Organizations Organizations. Organizations Supporting this Disease. Do you know of an organization?
Learn More Learn More. Click on the link to view information on this topic. NORD is a patient advocacy organization for individuals with rare diseases and the organizations that serve them. If your healthcare providers think that a newborn has herpes encephalitis resulting from infection with HSV2 while passing through the birth canal, they may check samples of the baby's blood and spinal fluid.
Treating the cause of your infection is the primary treatment. Since most cases of meningoencephalitis are caused by the herpes virus, the antiviral acyclovir is used to treat it.
You may need to take this medicine through an intravenous IV line for 10 to 14 days. Your healthcare provider may also give you medicine to reduce swelling in the brain and to treat or prevent seizures. With treatment, most people with this disease start to improve within a day or two and tend to recover fully within about a month.
But without treatment, very serious complications can set in, including death. Even with treatment, some people with severe cases may have long-term brain damage. They may have trouble thinking, controlling their body, and hearing, seeing, or speaking. They may need to take medicines for a long time, and they may require long-term care.
Avoiding herpes virus infections in the first place can help you prevent herpes meningoencephalitis. Ways to avoid infections from herpes viruses include:. Abstain from sex or have only one sex partner who has been tested for the virus and isn't infected.
Neonates and older children are treated with a higher dose of IV aciclovir for 21 days. Immunocompromised persons may require a higher dose with a longer duration. Oral aciclovir prophylaxis has been shown to reduce relapse in children after the initial treatment with IV acyclovir.
In rare instances of IV acyclovir non-availability, IV ganciclovir could be used. Acyclovir resistance is usually managed with IV foscarnet or cidofovir. Acyclovir could be used in pregnant patients whenever the potential benefits of treatment outweigh the potential risks.
A prospective registry on acyclovir use for 15 years did not show any increased incidence of fetal malformations in the women with first-trimester exposure.
Patients with issues regarding airway, breathing, and hemodynamics need to be shifted to the intensive care unit ICU for further monitoring and management. Those with significantly impaired neurology need ICU admission for intubation to facilitate airway protection. Other indications for ICU admissions are persistent seizures or features of increased intracranial pressure ICP for which intubation and ventilation may be required.
Seizures are initially treated with benzodiazepines, with longer-acting agents for example, levetiracetam, carbamazepine, or fosphenytoin added subsequently. Increased ICP is usually treated with head-end elevation and mannitol, frusemide, or hypertonic saline as per institutional protocol. The use of adjuvant corticosteroids in HSE continues to be controversial. The potential benefit of corticosteroids in suppressing immune-mediated damage is questioned by its equal potential to cause enhanced viral replication due to the same immune suppression.
Many authors reserve corticosteroids only for patients with significant edema with a mass effect. One nonrandomized trial showed outcome benefits in the corticosteroid group at 3 months. Differential diagnoses should enlist conditions that could mimic encephalopathy or encephalitis. These include:. A prospective multinational, randomized placebo-controlled trial was conducted among 87 HSE patients to evaluate the potential benefit of adjuvant oral valacyclovir 6 grams per day in the reduction of neuro-psychological sequelae assessed at 12 months.
Valacyclovir was administered for 3 months in continuation with standard IV acyclovir therapy for all patients in the treatment arm. The clinical benefit of additional valganciclovir for 3 months was negated by the lack of clinical benefits observed in this study. The clinical benefits of adjuvant corticosteroid in HSE treatment continue to be controversial. A non-randomized retrospective study on 45 HSE patients where adjuvant corticosteroids were added to acyclovir did a stepwise logistic regression analysis and concluded that the predictors of poor outcome were advanced age, GCS at the time of acyclovir initiation and non-initiation of adjuvant corticosteroid.
Dex-Enceph is an ongoing randomized control trial evaluating the clinical benefit of 4 days of 10 mg dexamethasone 6 hourly in addition to acyclovir treatment, with the primary endpoint being the impact on a verbal memory score.
Herpes simplex encephalitis in adults is associated with significant morbidity and mortality. Morbidity and mortality are significant in neonates and children, whether treated or untreated. Even though the standard mental status examination is within normal limits, many suffer from dysnomia and difficulty for new learning, especially via visual and verbal media. Short term complications include cerebral edema, status epilepticus, increased intracranial pressure, aspiration pneumonitis, cerebral venous thrombosis, cerebral infarction, and diabetes insipidus.
Long term sequelae include neurological deficits with varying severity for example, aphasia, ataxia, dysphasia, amnesia , cognitive, behavioral, physical, and neuropsychiatric abnormalities.
Autoimmune encephalitis with antibodies directed against the N-methyl-D-aspartate receptor needs to be considered in any patient with recent history HSE presenting with recurrent neuro symptoms. A neurology consult is a must for expert evaluation and management. Infectious disease consult is ideal if no cause for the encephalitis could be established after initial workup, and especially if the patient is not adequately improving or is deteriorating.
Neurosurgeons may need to be involved if significant brain involvement with midline shift occurs or a brain biopsy is planned rarely indicated or performed currently. Rehabilitation consult has to be given for short term as well as long term neurorehabilitation. Similarly, in children, apart from neonatology or pediatrician involvement for patients belonging to this age group, pediatric infectious disease and neurology experts may have to be involved.
Herpes simplex encephalitides have significant morbidity and mortality despite prompt detection and antiviral treatment. Significant neurologic sequelae occur in neonates due to HSV-2 infection even with treatment. No available strategies currently prevent HSE in older children or adults. Person to person spread has not been described. Prophylactic treatment of close contacts and isolation precautions are not indicated. HSV-1 causes encephalitis in adults and children beyond the neonatal period.
It is the most common cause of life-threatening sporadic encephalitis across the globe. HSV-2 causes encephalitis is predominant in neonates and immunocompromised patients. Herpes simplex encephalitis HSE has significant morbidity and mortality, even with early diagnosis and treatment.
Immunocompromised patients or patients in extremes of age might present with subtle or atypical symptoms or signs. Behavioral, cognitive, or personality changes could easily be misdiagnosed as a psychiatric disorder. Immunocompromised patients and immunocompetent adults in the early part of illness may not show evidence of CSF pleocytosis. HSE is a neurologic emergency. A high index of suspicion among attending physicians, rapid diagnostic workup, and early diagnosis will result in early initiation of IV acyclovir in all suspected or diagnosed cases, which could further decrease morbidity and mortality.
HSV encephalitis management requires close coordination between the treating interprofessional team. Interprofessional discussions and coordination between various specialties are necessary to improve patient outcomes.
Internists, emergency physicians, neurologists, neurosurgeons, infectious disease specialists, intensivists, pharmacists, physiatrists, psychologists, and psychiatrists are usually involved in the care.
Herpes simplex encephalitis is a neurologic emergency that requires a high degree of suspicion, rapid diagnostic workup, and treatment. Patients might need intubation either for airway protection in case of a significant drop in consciousness or persistent seizures.
Lumbar puncture needs to done promptly after brain imaging rules out intracranial hypertension or space-occupying lesions, and CSF analysis should be reported as soon as possible. Intravenous acyclovir needs to be administered as soon as possible in all suspected or confirmed cases of HSV encephalitis.
Intensive care unit admission is indicated once the patient is intubated or requires other organ supports. Continued neurology review is a must, and infectious disease consultation will be indicated if no other cause could be established despite initial evaluations, especially if the patient is not improving or is deteriorating. HSE causes significant morbidity in the survivors. After the acute phase, there needs to be continued follow up by the rehabilitation and neurology team.
A psychiatry or psychology consultation may be needed not only for patients but also for the family members to cope up with the stress of long term rehabilitation. This book is distributed under the terms of the Creative Commons Attribution 4. Turn recording back on. National Center for Biotechnology Information , U. StatPearls [Internet]. Search term. Affiliations 1 Manipal Hospitals. Introduction Herpetic infections have been well documented even in ancient Greek literature.
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