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Herpes simplex encephalitis is the most common cause of sporadic encephalitis in the western world. Patients usually have altered levels of consciousness, altered levels of mentation, fever, headache, and personality changes. These may progress to hemiparesis and seizures. Exact diagnosis must be established by brain biopsy and identification of the virus in biopsy material. There is a great need for a noninvasive test that is positive early in the disease. Without antiviral treatment the mortality rate is greater than 70%, and many survivors have serious disabilities. Both adenine arabinoside and acyclovir decrease death and morbidity, but acyclovir is the preferred drug. With acyclovir about 40% of patients will survive with normal development or minor levels of impairment but more than half of the patients will die or suffer significant impairment. It is essential to treat early; patients who are young and have not reached coma or impaired consciousness may show 65% recovery and return to normal function. Development of new antiviral drugs or other types of therapies is desirable. Herpetic skin lesions are likely to be more confusing than diagnostic because other types of encephalitis with fever often precipitate recurrent herpes that is unrelated to the encephalitis.
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PMID:Antiviral treatment of a serious herpes simplex infection: encephalitis. 333 44

A 28-year-old woman with systemic lupus erythematosus (SLE) suffered recent onset fever, headache, encephalopathy followed by severe, repeated generalized seizures. Investigations revealed limbic encephalitis. Tests for Herpes simplex encephalitis and paraneoplastic encephalomyelitis were negative. High titers anti-ribosomal-P antibodies in the cerebrospinal fluid (CSF) suggested an association with nervous system lupus. No brain biopsy was performed. Treatment was with anti-seizure, anti-viral, and immunomodulating medication.
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PMID:Nervous system lupus mimics limbic encephalitis. 986

Herpes simplex encephalitis is a rare complication of Herpes virus infections. Innate immune mechanisms are the first line of defence encountered by invading infectious agents. A 41-year-old man was admitted to the neurology department with the complaints of fever, headache, vertigo, tinnitus and ataxia. His first brain Magnetic Resonance Imagine showed nodular lesions in the medulla oblongata and the second showed a new left occipital lobe lesion in addition. In sera, Herpes Simplex Virus IgG and M values were positive and liver enzymes were found to be elevated. His diagnosis was Herpes encephalitis with liver involvement. CD19, CD20, CD21, CD22, CD35 receptors were found to be diminished. In this case we want to address that one of the causes of Wallenberg's lateral medullary syndrome can be Herpes simplex virus-1 and probable immune system deficiency can be researched.
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PMID:An atypic herpes encephalitis with Wallenberg's lateral medullary syndrome and CD receptors deficiency. 1516 83

Herpes simplex encephalitis (HSE) is a life-threatening consequence of herpes simplex virus (HSV) infection of the central nervous system (CNS). Although HSE is rare, mortality rates reach 70% in the absence of therapy and only a minority of individuals return to normal function. Antiviral therapy is most effective when started early, necessitating prompt diagnosis. The International Herpes Management Forum (IHMF) has issued guidelines to aid the diagnosis and treatment of HSE. Polymerase chain reaction (PCR) of the cerebrospinal fluid (CSF) is the diagnostic method of choice for HSE, but negative results need to be interpreted in the context of the patient's clinical presentation and the timing of the CSF sampling. CSF virus culture is of little value in all but patients under the age of 6 months. CSF (intrathecal) antibody measurements are not recommended for acute diagnostic purposes. However, demonstration of an intrathecal HSV antibody response may be helpful in retrospective diagnosis or in cases in which CSF is sampled only late after onset of infection and PCR is negative. Serum HSV antibody measurements are not of utility in the diagnosis of HSV encephalitis in adults. In children and young adults, HSV serology may help define whether HSE is part of a primary or a reactivated HSV infection, although the clinical features, therapy, and prognosis of these two forms of HSV encephalitis are similar. The IHMF recommends that all patients with HSE receive intravenous aciclovir 10 mg/kg every 8 h for 14-21 days. Owing to the life-threatening nature of the disease, if there is a delay in diagnostic test results therapy should not be withheld until they become available. After completion of therapy, PCR of the CSF can confirm the elimination of replicating virus, aiding further management of the patient. Clinical trials of other antiviral agents (i.e. adjunctive oral valaciclovir after intravenous aciclovir) for the treatment of HSE are underway. Herpes infection of the CNS, especially with HSV-2, can also cause both monophasic and recurrent aseptic meningitis, as well as myelitis or radiculitis. Limited evidence suggests that aciclovir may be effective in its treatment. Recurrent aseptic meningitis is predominantly caused by HSV-2 infection, and is characterized by self-limited episodes of fever, meningismus and severe headache. Many cases are indistinguishable from cases previously classified as "Mollaret's meningitis", a term that should now be reserved for idiopathic cases of recurrent aseptic meningitis.
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PMID:Herpes simplex virus infections of the central nervous system: encephalitis and meningitis, including Mollaret's. 1531 91

Significant mortality, high incidences of complications and permanent neurological sequel are still noted in patients suffering fro herpetic encephalitis. They result mainly from delayed diagnosis and treatment of the specific cause. The aim of our paper was the analysis o a clinical course of patients with Herpes simplex encephalitis. From 1999 to 2001 7 patients aged 2 weeks to 15 years, treated in Children' Neurology Department of Silesian School of Medicine, were diagnosed to have herpetic encephalitis. Fever, headache, vomiting, as well as alteration of consciousness, all typical for neuroinfection were main clinical symptoms present on admission. Three children presented with respiratory distress requiring admission to Intensive Care Unit. On examination "cold sores" were found in 2 patients, in remaining 5 the history of exposition to herpes labialis was obtained. On neurological examination we found either right or left hemiparesis in all patients, motor aphasia in 2 and left sided central facial nerve palsy in 1. Lumbar puncture revealed lymphocytosis in 5 patients. Anti-HSV type IgG an IgM antibodies were found in serum of all 6 patients, while only in 2 of them were detected in cerebrospinal fluid (CSF). These were the 2 most severely ill children. In 2 patients DNA HSV using PCR (polymerase chain reaction) method was found in CSF and in serum. Magnetic resonance imaging (MRI) of the head confirmed diagnosis. Although herpetic encephalitis is an uncommon, sporadic disease, the diagnosis should be considered in any child with neuroinfection and early treatment started before laboratory confirmation.
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PMID:[Herpes encephalitis at children]. 1576 59

Herpes simplex encephalitis (HSE) is the most frequent cause of sporadic fatal encephalitis in the Western world. Definitive diagnosis by viral PCR of cerebrospinal fluid (CSF) and treatment with aciclovir have improved the prognosis significantly. Nevertheless, the condition is rare and presents with non-specific symptoms that can easily be mistaken for systemic infection or non-infective encephalopathy. We report a case of HSE which was not recognised by four separate doctors, leading to substantial delay in diagnosis and treatment. Our patient presented with fever, headaches, altered behaviour and generalised bradykinesia. This was initially diagnosed as otitis interna (labyrinthitis) and, subsequently, an ischaemic stroke. There was a delay of 10 days in the initiation of aciclovir from symptom onset. MRI and CSF PCR confirmed herpes simplex virus type-1 (HSV-1) infection. The patient improved on aciclovir, but is disabled with word-finding difficulties and cognitive slowing.
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PMID:Diagnostic delay in a case of herpes simplex encephalitis. 2168 59

Herpes simplex encephalitis (HSE) still remains a serious illness with high morbidity and mortality. The characteristic presentation of HSE usually consists of fever, headache, and altered mental function. We present three patients with atypical features of HSE. First, a 48-year-old man with symptomatic posttraumatic epilepsy, who developed a gastrointestinal infection, seizures, and fever. After significant clinical improvement, the patient had fever again and developed a status epilepticus, which led to the diagnosis of HSE. Second, an 84-year-old woman with hyperactive delirium after levofloxacin intake. Cranial computed tomography (CCT) revealed hypodense temporal changes, prompting lumbar puncture and diagnosis of HSE. Third, a 51-year-old diabetic woman presented with fever and acute confusion. As CCT and cell count of cerebrospinal fluid (CSF) were normal, infection and hyperglycemia as initial diagnoses were postulated. Due to aphasic symptoms, the differential diagnosis of a stroke was taken into account. Thus a second lumbar puncture led to the correct diagnosis of HSE. These atypical presentations need a high grade of suspicion and a high willingness to reconsider the initial working diagnosis, in order to prevent a diagnostic delay.
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PMID:Pitfalls associated with the diagnosis of herpes simplex encephalitis. 2391 95

Herpes simplex encephalitis (HSE) is a serious disease with 10-20% mortality and high rate of neuropsychiatric sequelae. This study is a long-term, nationwide study in a single country, Iceland. Clinical data were obtained from patient records and from DNA PCR and antibody assays of CSF. Diagnosis of HSE was classified as definite, possible or rejected based on symptoms, as well as virological, laboratory and brain imaging criteria. A total of 30 definite cases of HSE were identified during the 25 year period 1987-2011 corresponding to incidence of 4.3 cases/106 inhabitants/year. Males were 57% of all patients, median age 50 years (range, 0-85). Fever (97%), cognitive deficits (79%), impaired consciousness (79% with GCS < 13), headache (55%) and seizures (55%) were the most common symptoms. Brain lesions were found in 24 patients (80%) by MRI or CT. All patients received intravenous acyclovir for a mean duration of 20 days. Three patients (10%) died within one year and 21/28 pts (75%) had a Karnofsky performance score of <70% with memory loss (59%), dysphasia (44%), frontal symptoms (44%) and seizures (30%) as the most frequent sequelae. Mean delay from onset of symptoms to treatment was 6 days; this was associated with adverse outcome. In conclusion, the incidence of `HSE is higher than recently reported in a national registry study from Sweden. Despite advances in rapid diagnosis and availability of treatment of HSE, approximately three of every four patients die or are left with serious neurological impairment.
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PMID:Herpes simplex encephalitis in Iceland 1987-2011. 2527 15

A 44-year-old right-handed Caucasian male was initialy diagnosed in 2007 with dermatomyositis (DM) and in 2009 with systemic lupus erythematosus (SLE) (overlap syndrome). He was treated with Methylprednisolone and Hydroxychloroquine. He interrupted the treatment in the last three years. The patient presented with fever (39.8 degrees C), left zoster ophthalmicus, headache and psychomotor agitation. The cerebral CT scan showed left hemispheric hypodense lesions. Herpetic encephalitis was suspected. The patient was referred to the Institute of Infectious Diseases. The patient's neurological status worsened, he presented spastic tetraparesis and aphasia. DW-MRI, ADC, DS and AngioMRI were done, the patient proved to have an ischemic stroke due to acute thrombosis of the left internal carotid artery and multiple watershed infarctions. An infectious pathology, including HSV-1, was excluded by PLEX ID performed from CSF. Acyclovir, anti vitamin K, steroidal intravenous pulse therapy was started. The patient was referred after two weeks to the Department of Neurology. Mild inflammatory syndrome, tests for anti-double stain DNA (dsDNA), anti-Sm, anti-SSA, IgM and IgG anti-cardiolipin antibodies and lupus anticoagulant were positive. He was currently treated with Methylprednisolone (48 mg/d), anti vitamin K, statin, symptomatics. The outcome was favorable, with good laboratory response. Overlap syndrome may be associated with a significant increase in the risk of stroke. Our case presented without clinically susceptible symptoms of stroke but found to have stroke after neurological assessment associated with overlap syndrome (DM and SLE).
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PMID:Ischemic strokes in a young patient with dermatomyositis, systemic lupus erythematosus and secondary antiphospholipid syndrome mimicking herpetic encephalitis. 2550 61

Herpes simplex encephalitis is an acute/subacute illness that causes both general and focal signs of cerebral dysfunction with fever, headache, and confusion as cardinal features. Recurrent herpes simplex meningitis, also known as Mollaret's meningitis, is another manifestation of central nervous system herpetic infection with recurrent episodes of fever, headache, and nuchal rigidity associated with cerebrospinal fluid (CSF) evidence of active herpes simplex infection. Bell's palsy is yet another manifestation of a herpes virus infection in at least some reported cases documented by CSF analysis. We report a case of a 70-year-old male who presented with acute transcortical motor aphasia initiating a stroke work-up that was negative. Physical examination revealed genital vesicles, and the CSF was consistent with active herpes simplex infection.
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PMID:Recurrent Transcortical Motor Aphasia-Another CNS Infectious Syndrome Associated with Herpes Virus Infection. 2695 55


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