Encefalitis autoinmune posherpética. Caso clínico pediátrico / Autoimmune post-herpes simplex encephalitis. A pediatric clinical case report

La encefalitis por virus herpes simple (VHS) es una causa frecuente de encefalitis grave y potencialmente fatal. La encefalitis autoinmune posherpética (EAPH) afecta a un porcentaje de los pacientes que han presentado encefalitis herpética (EH) y se caracteriza por la aparición de nuevos síntomas neurológico/psiquiátricos, y/o por el empeoramiento de los déficits adquiridos durante la infección viral dentro de un lapso temporal predecible. Se produce por un mecanismo no relacionado con el VHS, sino por fenómenos autoinmunes, y es susceptible de tratamiento con inmunomoduladores. Se presenta el caso de un varón de 5 años de edad con EAPH que requirió tratamiento inmunomodulador, de primera y segunda línea, con buena evolución y remisión de los síntomas.

Herpes simplex virus (HSV) encephalitis is a common cause of severe and potentially fatal encephalitis. Autoimmune post-herpes simplex encephalitis (AIPHSE) affects a percentage of patients who developed herpes simplex encephalitis (HSE) and is characterized by the onset of new neurological/psychiatric symptoms and/or worsening of deficits acquired during the herpes infection within a predictable time frame. It is caused by a mechanism not related to HSV, but by autoimmune conditions, and is susceptible to treatment with immunomodulators. Here we describe the case of a 5-year-old boy with AIPHSE who required first- and second-line immunomodulatory treatment, with an adequate course and remission of symptoms.

Encefalite herpética associada a mieloma múltiplo: desafio diagnóstico / Herpetic encephalitis associated with multiple myeloma: a diagnostic challenge

O mieloma múltiplo é a segunda neoplasia onco-hematológica mais comum, correspondendo a 1% das neoplasias malignas. Trata-se de uma condição subdiagnosticada, assim como a encefalite herpética. Entretanto, é comum a relação do mieloma com infecções, em decorrência do acometimento da imunidade humoral. A encefalite viral herpética tem como principal agente o vírus do herpes simples 1. O caso foi descrito baseado em um raciocínio clínico, visando contribuir para uma melhor caracterização do quadro clínico e do diagnóstico de duas entidades clínicas que possuem baixa suspeição diagnóstica e elevada morbimortalidade. Durante o curso da investigação, foram propostas variadas etiologias como responsáveis pelo rebaixamento do nível de consciência, levando a uma pesquisa de um espectro mais amplo de hipóteses diagnósticas, as quais precederam a confirmação do diagnóstico final. O quadro clínico atípico descrito apresentou obstáculos à suspeição diagnóstica correta, pois a ausência dos sinais e sintomas característicos de ambas as doenças levaram à pesquisa de um arsenal mais amplo de diagnósticos diferenciais. Assim, o atraso no diagnóstico e o início tardio do tratamento foram fatores que contribuíram para o prognóstico reservado do paciente. A associação dessas doenças é pouco descrita na literatura, de modo que mais estudos acerca do assunto se fazem necessários. (AU)

Multiple myeloma is the second most common onco-hematologic neoplasm, accounting for 1% of malignant neoplasms. As herpetic encephalitis, it is an underdiagnosed condition. However, the relation of myeloma with infections is common, due to the involvement of humoral immunity. Herpetic encephalitis has as its main etiological agent the herpes simplex virus 1. The case was described based on a clinical reasoning, aiming to contribute to a better characterization of the clinical picture and diagnosis of two entities that have low rates of diagnostic suspicion and high rates of morbidity and mortality. During the course of investigation, several etiologies were proposed as responsible for the decreased level of consciousness, leading to a search for a broader range of diagnostic hypotheses, which preceded confirmation of the final diagnosis. The atypical clinical picture described presented obstacles to the correct diagnostic suspicion, as the absence of symptoms and signs characteristic of both diseases led to the search for a broader arsenal of differential diagnoses. Thus, delayed diagnosis and late treatment were factors that contributed to the patient's reserved prognosis. The association of these diseases is poorly described in the literature, so further studies on that subject are required. (AU)

Hemorragia intracerebral temprana como presentación atípica de encefalitis por herpes virus simple tipo 1 / Early intracerebral hemorrhage as an atypical presentation of herpes simplex-1 virus encephalitis

RESUMEN La encefalitis por herpes virus simple es la causa más común de encefalitis esporádica letal en el mundo. Una complicación poco usual es la hemorragia intracerebral. Se describe el caso de un preescolar de tres años que ingresa con cuadro clínico de encefalitis aguda, con hallazgos de hemorragia intracerebral temprana en región occipital y estudio en líquido cefalorraquídeo positivo para herpes virus tipo 1, que recibe tratamiento con aciclovir; sin embargo, cursa con evolución tórpida, incremento de hemorragias intracerebrales y desenlace fatal.

ABSTRACT Herpes simplex encephalitis is the most common cause of sporadic lethal encephalitis in the world. Intracerebral hemorrhage is an uncommon complication. The case of a 3-year-old preschooler who was admitted with acute encephalitis clinical picture is described. The patient presents findings of early in tracerebral hemorrhage in the occipital region and a positive cerebrospinal fluid study for herpes virus type 1, which is treated with acyclovir; however, it presents a torpid evolution, increased intracerebral hemorrhages and fatal outcome.

Craniectomía descompresiva en encefalitis herpética: caso clínico / Decompressive craniectomy in herpes simplex encephalitis. Clinical case

Introduction: The morbidity and mortality of herpes simplex encephalitis (HSE) have decreased with the use of acyclovir. However, some patients develop focal hemorrhagic necrosis and edema in the temporal lobe, with a subsequent elevation of intracranial pressure. Clinical cases: We report the clinical outcomes of two children with HSE who developed severe intracranial hypertension and impending uncal herniation refractory to profound sedation, osmotic agents and moderate hyperventilation. Decompressive craniectomy allowed an effective control of intracranial pressure and a favorable neurological outcome at discharge in both patients. Conclusions: Decompressive craniectomy could be considered as a rescue treatment strategy in patients with life-threatening intracranial hypertension due to severe herpetic encephalitis.

Introducción: La morbimortalidad de la encefalitis herpética ha disminuido con el uso de aciclovir. Sin embargo, algunos pacientes pueden evolucionar con necrosis focal hemorrágica y edema cerebral con posterior elevación de la presión intracraneal. Casos clínicos: Reportamos el curso clínico de dos niños con encefalitis herpética complicada con hipertensión intracraneal refractaria al tratamiento con sedación profunda, agentes osmóticos e hiperventilación. Ambos pacientes desarrollaron signos de enclavamiento uncal por lo que se realizó una craniectomía descompresiva precoz, que permitió un control de la presión intracraneal y una evolución neurológica favorable al alta. Conclusiones: La craniectomía descompresiva podría ser considerada como una alternativa terapéutica de rescate en pacientes con encefalitis herpética e hipertensión endocraneana refractaria a la terapia medica.

Aphasia and herpes virus encephalitis: a case study / Afasia e herpes vírus: estudo de caso

CONTEXT: Meningoencephalitis early in life, of any etiology, is a risk factor for development of subsequent sequelae, which may be of physical, psychiatric, behavioral or cognitive origin. Anomia is a language abnormality frequently found in such cases, and other language deficits are rarely described. The aim of this study was to describe the cognitive and linguistic manifestations following a case of herpetic meningoencephalitis in a 13-year-old patient with eight years of schooling. CASE REPORT: The patient underwent a speech-language audiology assessment nine months after the neurological diagnosis. The battery of tests included the Montreal-Toulouse Language Assessment test protocol (MT Beta-86, modified), the description from the Cookie Theft task of the Boston Diagnostic Aphasia Examination (BDAE), an informal assessment of the patient's logical and mathematical reasoning, and the neuropsychological subtests from the WAIS-III scale, which assess working memory. The patient presented mixed aphasia, impairment of short-term memory and working memory, and dyscalculia. This case also presented severe cognitive and linguistic deficits. Prompt diagnosis is crucial, in order to enable timely treatment and rehabilitation of this neurological infection and minimize the cognitive deficits caused by the disease.

CONTEXTO: Meningoencefalite em idade precoce, de qualquer etiologia, é fator de risco para o desenvolvimento de sequelas posteriores, que podem ser de origem física, psíquica, comportamental ou cognitiva. Anomia é uma manifestação linguística frequente nesses casos e outras alterações de linguagem raramente são descritas. O objetivo do estudo foi descrever as manifestações linguísticas e cognitivas pós-meningoencefalite herpética de um paciente com 13 anos de idade e oito anos de escolaridade. RELATO DE CASO: O paciente passou por avaliação fonoaudiológica nove meses após o diagnóstico neurológico. A bateria de avaliação incluiu o Protocolo de Avaliação de Linguagem Montreal Toulouse (MT Beta-86 modificado), a descrição da prancha Roubo dos Biscoitos do Teste de Boston para o diagnóstico da afasia, avaliação informal do raciocínio lógico-matemático e os subtestes neuropsicológicos da escala WAIS-III que avaliam a memória de trabalho. O paciente apresentou afasia mista, alteração da memória de curto prazo e da memória de trabalho e discalculia. Apresentou também alterações cognitivas e linguísticas graves. O diagnóstico precoce tem fundamental importância para o tratamento oportuno e a reabilitação dessa infecção neurológica e também para minimizar as alterações cognitivas causadas pela doença.

Relapse of herpes simplex encephalitis presenting as choreoathetosis.

We report a case of herpes simplex virus (HSV) encephalitis (HSE) in an 11-year-old boy who recovered with acyclovir therapy but developed relapse after 2 weeks. Choreoathetosis was the presenting feature of relapse. Response to antiinflammatory treatment was excellent. To the best of our knowledge, this is the first case of HSE relapse presenting with choreoathetosis reported from India. We describe the patient and review the literature on HSE and HSE relapse.

Enfermedad de Creutzfeldt-Jakob en un paciente interpretado como encefalitis por herpes simple / Creutzfeldt-Jakob disease in a patient with possible herpes simplex encephalitis

Se presentó un paciente con una enfermedad de Creutzfeldt-Jakob, al cual se le realizó un diagnóstico presuntivo inicial de encefalitis por herpes simple, que comenzó con trastornos conductuales, agresividad, alucinaciones visuales y pérdida progresiva de la visión, empeoró de forma paulatina, aparecieron movimientos involuntarios inespecíficos y estado de postración, convulsiones técnico-clínicas del hemicuerpo izquierdo, paresia homolateral, poca respuesta al dolor con empobrecimiento de la mímica facial e imposibilidad para localizar el estímulo nociceptivo y discreta rigidez nucal. Se le realizaron estudios microbiológicos para pesquisaje virológico incluido herpes virus, bacterias y hongos, así como análisis citoquímico del líquido cefalorraquídeo y resultaron normales, en el electroencefalograma aparecieron alteraciones que señalaron actividad paroxística asimétrica. El paciente evolucionó tórpidamente y falleció, se presenta el resultado de los estudios anatomopatológicos de la microscopia óptica

This is the case of a patient presenting with Creutzfeldt-Jakob disease with an initial presumptive diagnosis of herpes-simplex encephalitis started with behavioral disorders, aggression, visual hallucinations and a progressive loss of vision, worsening in a gradual way, non-specific unintentional movements and prostration status, left hemi-body tonic-clonic convulsions, homolateral paresis, a low response to pain with a poor facial expression and inability to locate the nociceptive stimulus and a light nuchal stiffness. Microbiologic studies were conducted for a virology screening including herpesvirus, bacteria and fungi, as well as, cytochemistry of cerebrospinal fluid with normal results, in electroencephalogram appeared alterations related to an asymmetric paroxysmal activity. Patient evolved with torpidity and died. Anatomic-pathologic studies result of optical microscopy is showed

Basal ganglia involvement in herpes simplex encephalitis.

Herpes simplex encephalitis (HSE) is a leading cause of sporadic, nonepidemic viral encephalitis in children and adults. We report a very rare case of HSE with involvement of bilateral thalamus, putamen, upper pons and midbrain, with development of extrapyramidal symptoms which responded to corticosteroid therapy. A 15-mth-old female baby admitted with complaint of fever for 5 days and generalised tonic clonic seizure 10 hours before admission. On clinical examination patient was drowsy, temperature was 39.4 oC and vitals were stable with signs of increased intracranial tension. There were no signs of meningeal irritation. Patient gradually become unconscious in the next few hours and pupils were constricted bilaterally with development of atonia in all four limbs and neck muscles. Doll’s eye phenomenon was absent.

Aprendizaje de nombres en una paciente con amnesia anterógrada / Names learning in an anterograde amnesic patient

En este trabajo se describe la aplicación de algunas técnicas empleadas para la rehabilitación de la memoria en personas con daño cerebral, con el fin de facilitar el aprendizaje de nombres de personas cercanas y conocidas en una paciente de 55 años, universitaria, diestra y quien como secuela de una encefalitis herpética presentó lesión isquémica en territorio frontotemporal izquierdo y severas alteraciones cognoscitivas y funcionales. Aunque la aplicación combinada de técnicas favorece los procesos de aprendizaje, éstos suelen ser lentos y desgastantes en personas con amnesia severa. No obstante, los resultados apoyan la propuesta de la conservación de la memoria implícita en esta población y favorecen el planteamiento de perspectivas de intervención.

In this work, we describe the application of some techniques used for the memory rehabilitation in people with brain damage, in order to facilitate learning the names of close and known persons in a 55 year old patient, college graduate, right handed, with herpetic encephalitis who presented injury in left frontotemporal region and severe cognitive and functional alterations. As show in the results, of the six names selected for the intervention, it was possible to work with three of them and to obtain significant results in two. Although the mix of techniques favors the learning processes, this process is usually slow in people with severe amnesia. However, these results support the proposal of the implicit memory conservation and favor the intervention from this perspective.

Kluver-Bucy syndrome--a rare complication of herpes simplex encephalitis.

A twelve-year-old female was admitted with history of high fever, recurrent vomiting and repeated convulsion for 2 days and altered consciousness for one day. Cranial CT scan showed intraparenchymal haemorrhage involving both temporal lobes and right basal ganglia region without mass effect. Serology was reactive against IGM HSV1. Injection acyclovir was started at a dose of 10 mg/kg 8 hourly intravenously. Patient regained consciousness on fourth day but speech was altered. Abnormal behavioural symptoms were noticed. EEG showed generalised spike and slow waves and sharp and slow wave discharge more in the temporal region. The patient was given clonidine and carbamazepine. She also received behavioural therapy and parental counselling. She was followed up for six months and maintaining well.

Acute intracerebral haematoma--an unusual presentation of herpes simplex encephalitis.

A young man presented with acute headache, behavioral abnormality and CT scan evidence of intracerebral hematoma. Detailed investigation established its infective origin due to herpes simplex virus type-I. The patient made an excellent recovery following a course of acyclovir. Frank intracerebral haematoma in the form of well defined mass on CT scan in patient with herpes simplex encephalitis is extremely rare.

[Herpes simplex virus encephalitis during immunosuppressive treatment of ulcerative colitis]

Today, with the advent of new medications, treatment of Ulcerative colitis [UC] has been markedly improved. Immunosuppressive drugs used in therapy predispose patients to opportunistic infections. A 22-year-old woman was admitted to emergency department due to acute exacerbation of UC and decreased level of consciousness. She was under treatment with 5-aminosalicylate, prednisolone and azathioprine. In neurological evaluation, patient had cerebral herniation. Non-contrast CT scan revealed multiple hemorrhagic areas in both frontal lobes. Right frontal craniotomy was performed emergently. Histopathologic evaluation of brain tissue was reported as "Herpes simplex encephalitis". Polymerase chain reaction [PCR] assay was also positive for HSV DNA. Immunosuppressive drugs such as azathioprine have a pivotal role in the treatment of resistant and/or severe cases of UC. Prevalence of infectious complications was reported to be 7.4%, 1.8% of which were severe [including herpes zoster encephalitis] .Our review of literature indicates that no case of herpes simplex encephalitis following immunosuppressive therapy for UC has been reported

Craniectomía descompresiva en una paciente con encefalitis herpética asociada a hipertensión intracraneana refractaria / Decompressive craniectomy in a patient with herpetic encephalitis associated to refractory intracranial hypertension

Herpetic encephalitis is the most common cause of viral encephalitis in our country. Pathological studies show progressive necrosis and edema in specific territories of the brain. The mortality of herpetic encephalitis was reduced from 70% to 20% with the use of intravenous aciclovir in the first three days of illness. However, almost 50% of patients develop a neurological deficit. One of the most important causes of death in herpetic encephalitis is the refractory intracranial hypertension. There are anecdotal reports of patients with refractory intracranial hypertension due to herpetic encephalitis that were treated with decompressive craniectomy with good results. We report a 21 years old female patient with herpetic encephalitis and refractory intracranial hypertension that was successfully treated with a decompressive craniectomy (Rev Méd Chile 2003; 131: 1434-8).

Cerebral aneurysmal dilatation in an infant with perinatally acquired HIV infection and HSV encephalitis

Although most children with human immunodeficiency virus (HIV) infection have neurological dysfunction, in childhood the incidence of symptomatic cerebrovascular disease is low. Cerebral aneurysmal arteriopathy in childhood AIDS has been reported in the past and considered to have a relatively long latency following the primary infection. We report a 1 month-old infant with congenitally acquired HIV infection, and herpes encephalitis; she presented a sudden cardiorespiratory arrest followed by coma and was found to have a giant saccular aneurysm of the left basilar artery. Literature review showed that cerebral aneurysmal artheriopathy is an unusual manifestation in newborns and infants and this case is possibly the youngest patient reported with aneurysma, herpes encephalitis and AIDS. The role of HIV and herpes simplex infections in the pathogenesis of this lesion is discussed

Clínica e tratamento das infecçöes pelos vírus Herpes Símplex tipo 1 e 2 / Clinical manifestations and treatment of infections caused by Herpes Simplex 1 and 2 viruses

As infecçöes pelos vírus herpes símplex säo conhecidas da raça humana desde a antiguidade. Estes vírus säo responsáveis por uma gama de infecçöes que väo desde erupçöes cutâneas até quadros graves de encefalite. Atualmente, um antiviral específico para estes vírus, o aciclovir, está disponível para o tratamento destas infecçöes. Nesta revisäo, o uso do aciclovir é discutido em relaçäo às diversas manifestaçöes da infecçäo herpética.