RESUMO
Subacute sclerosing panencephalitis (SSPE) is a slowly progressive brain disorder caused by mutant measles virus. SSPE affects younger age groups. SSPE incidence is proportional to that of measles. High-income countries have seen substantial decline in SSPE incidence following universal vaccination against measles. SSPE virus differs from wild measles virus. Measles virus genome recovered from the autopsied brain tissues demonstrates clustered mutations in virus genome particularly in the M gene. These mutations destroy the structure and functioning of the encoded proteins. Complete infectious virus particle has rarely been recovered from the brain. Human neurons lack required receptor for entry of measles virus inside the neurons. Recent in vitro studies suggest that mutations in F protein confer hyperfusogenic properties to measles virus facilitating transneuronal viral spread. The inflammatory response in the brain leads to extensive tissue damage. Clinically, SSPE is characterized by florid panencephalitis. Clinically, SSPE is characterized by cognitive decline, periodic myoclonus, gait abnormalities, vision loss, and ultimately to a vegetative state. Chorioretinitis is a common ocular abnormality. Electroencephalography (EEG) shows characteristic periodic discharges. Neuroimaging demonstrates periventricular white matter signal abnormalities. In advanced stages, there is marked cerebral atrophy. Definitive diagnosis requires demonstration of elevated measles antibody titers in cerebrospinal fluid (CSF). Many drugs have been used to stabilize the course of the disease but without evidence from randomized clinical trials. Six percent of patients may experience prolonged spontaneous remission. Fusion inhibitor peptide may, in the future, be exploited to treat SSPE. A universal vaccination against measles is the only proven way to tackle this menace currently.
Assuntos
Panencefalite Esclerosante Subaguda/diagnóstico , Panencefalite Esclerosante Subaguda/etiologia , Biomarcadores , Encéfalo/diagnóstico por imagem , Encéfalo/patologia , Encéfalo/virologia , Tronco Encefálico/diagnóstico por imagem , Tronco Encefálico/patologia , Tronco Encefálico/virologia , Diagnóstico Diferencial , Gerenciamento Clínico , Suscetibilidade a Doenças , Eletroencefalografia , Feminino , Predisposição Genética para Doença , Humanos , Imuno-Histoquímica , Vírus do Sarampo/fisiologia , Neuroimagem/métodos , Fenótipo , Gravidez , Prognóstico , Panencefalite Esclerosante Subaguda/epidemiologia , Panencefalite Esclerosante Subaguda/terapia , Internalização do VírusRESUMO
Subacute sclerosing panencephalitis (SSPE) is a rare delayed complication of measles virus infection in infancy. We present here 7 month pregnant lady who had SSPE. She delivered low birth-weight baby prematurely which died on 3rd day of delivery. Patient died due to sepsis one month after admission.
Assuntos
Encéfalo/diagnóstico por imagem , Vírus do Sarampo , Complicações Infecciosas na Gravidez , Sepse/etiologia , Panencefalite Esclerosante Subaguda , Adulto , Líquido Cefalorraquidiano/imunologia , Diagnóstico Diferencial , Eletroencefalografia/métodos , Evolução Fatal , Feminino , Humanos , Imageamento por Ressonância Magnética/métodos , Vírus do Sarampo/imunologia , Vírus do Sarampo/isolamento & purificação , Administração dos Cuidados ao Paciente/métodos , Gravidez , Complicações Infecciosas na Gravidez/diagnóstico , Complicações Infecciosas na Gravidez/fisiopatologia , Complicações Infecciosas na Gravidez/terapia , Resultado da Gravidez , Panencefalite Esclerosante Subaguda/complicações , Panencefalite Esclerosante Subaguda/diagnóstico , Panencefalite Esclerosante Subaguda/fisiopatologia , Panencefalite Esclerosante Subaguda/terapiaRESUMO
MEASLES VIRUS AND ASSOCIATED CENTRAL NERVOUS SYSTEM: Sequelae Renee Buchanan, Daniel J. Bonthius Seminars in Pediatric Neurology Volume 19, Issue 3, September 2012, Pages 107-114 Worldwide, measles remains one of the most deadly vaccine-preventable diseases. In the United States, enrollment in the public schools requires that each child receives 2 doses of measles-containing vaccine before entry, essentially eliminating this once endemic disease. Recent outbreaks of measles in the United States have been associated with importation of measles virus from other countries and subsequent transmission to intentionally undervaccinated children. The central nervous system complications of measles can occur within days or years of acute infection and are often severe. These include primary measles encephalitis, acute postinfectious measles encephalomyelitis, measles inclusion body encephalitis, and subacute sclerosing panencephalitis. These measles associated central nervous system diseases differ in their pathogenesis and pathologic effects. However, all involve complex brain-virus-immune system interactions, and all can lead to severe and permanent brain injury. Despite better understanding of the clinical presentations and pathogenesis of these illnesses, effective treatments remain elusive.
Assuntos
Encefalomielite Aguda Disseminada , Sarampo , Panencefalite Esclerosante Subaguda , Criança , Humanos , Vírus do Sarampo/fisiologia , Sistema Nervoso Central , Sarampo/complicações , Sarampo/epidemiologia , Sarampo/prevenção & controle , Panencefalite Esclerosante Subaguda/epidemiologia , Panencefalite Esclerosante Subaguda/terapia , Panencefalite Esclerosante Subaguda/complicações , Encéfalo , Encefalomielite Aguda Disseminada/complicaçõesAssuntos
Pediatria/história , Panencefalite Esclerosante Subaguda/diagnóstico , Panencefalite Esclerosante Subaguda/história , Criança , Pré-Escolar , História do Século XX , Humanos , Lactente , Recém-Nascido , Irlanda , Vacina contra Sarampo , Panencefalite Esclerosante Subaguda/terapia , Estados UnidosAssuntos
Distonia/fisiopatologia , Rabdomiólise/fisiopatologia , Panencefalite Esclerosante Subaguda/fisiopatologia , Adolescente , Diagnóstico Diferencial , Distonia/diagnóstico , Distonia/terapia , Humanos , Masculino , Rabdomiólise/diagnóstico , Rabdomiólise/terapia , Panencefalite Esclerosante Subaguda/terapiaRESUMO
Isikay S. The behavior pattern of parents of patients with subacute sclerosing panencephalitis concerning alternative medicine. Turk J Pediatr 2017; 59: 288-294. The aim of the study was to examine the attitude of the parents of Subacute Sclerosing Panencephalitis (SSPE) patients regarding alternative treatment methods and compare with those of the parents of epilepsy patients. The study comprised 39 SSPE and 53 epilepsy patients who were under follow-up in Gaziantep Children`s Hospital. A questionnaire designed to inquire about the knowledge (13 questions) and behavior (11 questions) of parents about alternative medicine methods was given to the caregiver of all patients. The ratio of parents using alternative medicine methods was 29/39 (74.4%) in the SSPE group and 8/53 (15.1%) in the epilepsy group. Less than half of the parents of SSPE patients reported talking about it with their doctors. These results show parents facing a chronic debilitating disease frequently seek benefit from alternative methods. Most define this treatment as complementary to the established medical treatment. However, potential and unrecognized adverse events of alternative methods and their interference with regular medical treatment can be of importance, especially because treating physicians are seldom informed about concurrent use.
Assuntos
Atitude Frente a Saúde , Terapias Complementares/estatística & dados numéricos , Epilepsia/terapia , Conhecimentos, Atitudes e Prática em Saúde , Panencefalite Esclerosante Subaguda/terapia , Adolescente , Adulto , Criança , Feminino , Humanos , Masculino , Pais , Inquéritos e Questionários , Turquia , Adulto JovemRESUMO
Encephalitis is the most frequent neurological complication of measles virus infection. This review examines the pathophysiology of measles infection and the presentations, diagnosis and treatment of the four types of measles-induced encephalitis including primary measles encephalitis, acute post-measles encephalitis, measles inclusion body encephalitis and subacute sclerosing panencephalitis. The early symptoms of encephalitis may be non-specific and can be mistakenly attributed to a systemic infection leading to a delay in diagnosis. This review provides a summary of the symptoms that should cause health care workers to suspect measles-induced encephalitis.
Assuntos
Encefalite Infecciosa/virologia , Sarampo , Doença Aguda , Humanos , Imunidade Coletiva , Encefalite Infecciosa/diagnóstico , Encefalite Infecciosa/terapia , Vacina contra Sarampo , Panencefalite Esclerosante Subaguda/diagnóstico , Panencefalite Esclerosante Subaguda/terapia , Panencefalite Esclerosante Subaguda/virologiaRESUMO
Regional cerebral glucose metabolism was studied in a 15-year-old boy with subacute sclerosing panencephalitis before and after therapy with human interferon beta, using positron emission tomography of fluorine 18-2-fluoro-2-deoxyglucose. At first examination, metabolism was symmetrically decreased in the thalamus, cerebellum, and all cortical areas except prerolandic motor cortex, but increased in lentiform nucleus. A computed tomographic scan was normal. Six months later, bilateral focal necrosis centered in the previously hypermetabolic putamen was demonstrated by computed tomography and magnetic resonance imaging. The caudate nucleus and the superoposterior part of the putamen were spared, still showing increased metabolism. Corresponding with some clinical improvement, cortical glucose consumption rates had returned to a normal level.
Assuntos
Encéfalo/metabolismo , Glucose/metabolismo , Panencefalite Esclerosante Subaguda/metabolismo , Adolescente , Encéfalo/diagnóstico por imagem , Desoxiglucose/análogos & derivados , Radioisótopos de Flúor , Fluordesoxiglucose F18 , Humanos , Interferon Tipo I/uso terapêutico , Masculino , Panencefalite Esclerosante Subaguda/diagnóstico por imagem , Panencefalite Esclerosante Subaguda/terapia , Tomografia Computadorizada de EmissãoRESUMO
Thirteen patients with subacute sclerosing panencephalitis (S.S.P.E.) at different stages of the disease were admitted for transfer factor treatment. The transfer factor was prepared from non-selected blood bank donors. The activity of the transfer factor was tested in patients with diseases other than S.S.P.E. and was found to be either clinically or immunologically active. Regardless of the number of transfer factor units applied a significant influence on the course of the disease was not apparant. The observed intermittant improvement of 3 patients was considered as spontaneous remission which is known to occur occasionally in S.S.P.E. The humoral and cellular immune response before and after transfer factor therapy did not reveal significant changes which could be correlated with transfer factor therapy.
Assuntos
Panencefalite Esclerosante Subaguda/terapia , Fator de Transferência/uso terapêutico , Adolescente , Anticorpos Antivirais/análise , Criança , Pré-Escolar , Feminino , Humanos , Imunidade Celular , Imunoglobulina A/líquido cefalorraquidiano , Imunoglobulina G/líquido cefalorraquidiano , Imunoglobulina M/líquido cefalorraquidiano , Masculino , Sarampo/complicações , Vírus do Sarampo/imunologia , Testes Cutâneos , Panencefalite Esclerosante Subaguda/imunologiaRESUMO
PURPOSE: To evaluate the progression of CT and MR changes of the brain in subacute sclerosing panencephalitis (SSPE) as a basis for assessing the effects of different types of therapy. METHODS: Fifty-two patients with SSPE were examined, 44 with MR imaging and 42 with CT of the brain on one or more occasions. A total of 92 MR and 67 CT studies were performed. RESULTS: Correlation between the clinical status and the MR findings in admission was poor. Of 20 patients with clinically advanced disease, only 8 had marked MR abnormalities; 6 had normal or almost normal findings on MR examinations. Two of 4 patients with clinically mild disease had advanced MR changes. The progression of the MR findings appeared to follow a constant pattern. The earliest pathologic finding was focal, high-T2-intensity white matter changes; later atrophic changes followed. The atrophy lagged behind the white matter changes and was thus mild when white matter changes were moderate or severe. In the most advanced stage, when the patient was in a neurovegetative state, an almost total loss of white matter had usually taken place. At this stage, the corpus callosum was also thin. Basal ganglia changes, usually involving the putamina, were seen in one third of patients and cortical gray matter changes were seen in one fourth of patients examined with MR imaging. In 2 of 20 patients, MR changes regressed in parallel with clinical improvement following therapy, but in 5 patients clinical improvement was accompanied by progression of MR changes. CONCLUSION: The progress of MR abnormalities seen in patients with SSPE seems to follow a constant pattern, but the severity of MR changes does not always correlate well with the clinical findings. Caution must therefore be used when evaluating the effects of therapy.
Assuntos
Encéfalo/patologia , Imageamento por Ressonância Magnética , Panencefalite Esclerosante Subaguda/diagnóstico , Tomografia Computadorizada por Raios X , Adolescente , Atrofia , Criança , Feminino , Seguimentos , Humanos , Masculino , Fibras Nervosas Mielinizadas/patologia , Sensibilidade e Especificidade , Panencefalite Esclerosante Subaguda/terapia , Resultado do TratamentoRESUMO
Oligoclonal IgG bands were analyzed in matching pairs of cerebrospinal fluid (CSF) and serum from 12 subacute sclerosing panencephalitis (SSPE) patients, using isoelectric focusing and immunofixation. Each patient was given isoprinosine, and four of the 12 patients were given alpha-interferon in addition. Two to 4 serial CSF and serum samples were collected from each SSPE patient during periods ranging from 1 to 16 months. In 3 SSPE patients a small number of new oligoclonal bands were seen in the follow-up CSF samples. In the other 9 SSPE patients there was no change in CSF band patterns between initial and follow-up specimens. Band patterns in serum remained unchanged between initial and follow-up samples. Although all 12 SSPE cases had higher IgG indices and increased rate of intra blood-brain barrier (BBB) IgG synthesis in comparison to patients with other neurological diseases, the values did not significantly differ between the first and follow-up specimens. We conclude that treatment of SSPE patients with isoprinosine or with isoprinosine and alpha-interferon had no significant effect on the CSF oligoclonal band profiles or IgG synthesis within the central nervous system.
Assuntos
Proteínas do Líquido Cefalorraquidiano/análise , Imunoglobulina G/líquido cefalorraquidiano , Inosina Pranobex/uso terapêutico , Interferon-alfa/uso terapêutico , Panencefalite Esclerosante Subaguda/imunologia , Adolescente , Adulto , Formação de Anticorpos/efeitos dos fármacos , Barreira Hematoencefálica , Proteínas do Líquido Cefalorraquidiano/biossíntese , Criança , Terapia Combinada , Feminino , Humanos , Imunoglobulina G/biossíntese , Imunoglobulina G/sangue , Fatores Imunológicos/uso terapêutico , Inosina Pranobex/farmacologia , Interferon-alfa/farmacologia , Masculino , Esclerose Múltipla/sangue , Esclerose Múltipla/líquido cefalorraquidiano , Doenças do Sistema Nervoso/sangue , Doenças do Sistema Nervoso/líquido cefalorraquidiano , Panencefalite Esclerosante Subaguda/sangue , Panencefalite Esclerosante Subaguda/líquido cefalorraquidiano , Panencefalite Esclerosante Subaguda/tratamento farmacológico , Panencefalite Esclerosante Subaguda/terapia , Resultado do TratamentoRESUMO
A 14-year-old pregnant Caucasian girl presented with a 1-week history of dementia. She had presented 1 year prior with acute unilateral visual impairment and was noted to have macular degeneration of presumed infective aetiology. This evolved to a pigmentary macular lesion. During the course of the current presentation she developed myoclonic jerks. An electroencephalogram revealed periodic spike and slow wave complexes at 1-2 second intervals. Blood and cerebrospinal fluid examination showed raised anti-measles IgG antibody titres. Intrathecal synthesis of anti-measles virus antibody was demonstrated unequivocally and a diagnosis of subacute sclerosing panencephalitis was made. A healthy male infant was delivered by elective caesarean section at 33 weeks' gestation. She continued to deteriorate clinically despite treatment with intraventricular alpha-interferon. She had not had primary immunization against measles.
Assuntos
Coriorretinite/diagnóstico , Demência/diagnóstico , Complicações Infecciosas na Gravidez/diagnóstico , Panencefalite Esclerosante Subaguda/diagnóstico , Adolescente , Anticorpos Antivirais/sangue , Anticorpos Antivirais/líquido cefalorraquidiano , Encéfalo/patologia , Cesárea , Coriorretinite/complicações , Demência/complicações , Progressão da Doença , Eletroencefalografia , Eletromiografia , Feminino , Humanos , Imageamento por Ressonância Magnética , Vírus do Sarampo/imunologia , Gravidez , Panencefalite Esclerosante Subaguda/complicações , Panencefalite Esclerosante Subaguda/terapiaRESUMO
Subacute sclerosing panencephalitis (SSPE) is a neurodegenerative disease of childhood that is due to a persistent measles infection. Owing to a nationwide immunization program in the United States, the incidence has decreased considerable, coexistent with the decline in natural measles infection. The disease, now fully understood, still represents a great international problem. Clinical presentation, etiology, pathogenesis, prevention, and treatment are updated in this article.
Assuntos
Panencefalite Esclerosante Subaguda/diagnóstico , Adolescente , Amantadina/uso terapêutico , Anticorpos Antivirais/líquido cefalorraquidiano , Encéfalo/patologia , Criança , Pré-Escolar , Eletroencefalografia , Feminino , Humanos , Inosina Pranobex/uso terapêutico , Masculino , Vírus do Sarampo/imunologia , Cuidados Paliativos , Sistema de Registros , População Rural , Panencefalite Esclerosante Subaguda/epidemiologia , Panencefalite Esclerosante Subaguda/terapia , Proteínas Virais/imunologiaRESUMO
Subacute sclerosing panencephalitis (SSPE), a neurodegenerative disease caused by a persistent "slow virus infection" with a mutated measles virus, is endemic in much of the developing world. Its incidence will increase in the USA, not only in immigrants, but also because of the 1988-1990 measles epidemic. This report reviews the pathogenesis, clinical and laboratory diagnosis, and future perspectives in treatment and prevention.
Assuntos
Panencefalite Esclerosante Subaguda/diagnóstico , Adolescente , Adulto , Encéfalo/patologia , Criança , Pré-Escolar , Vírus Defeituosos/genética , Humanos , Lactente , Interferon-alfa/administração & dosagem , Vírus do Sarampo/genética , Mutação/genética , Exame Neurológico/efeitos dos fármacos , Vírus SSPE/genética , Panencefalite Esclerosante Subaguda/terapia , Panencefalite Esclerosante Subaguda/virologiaRESUMO
Two patients with subacute sclerosing panencephalitis (SSPE) were treated with intraventricular alpha interferon (IFN-alpha) via an Ommaya reservoir for 20-57 months. The clinical course of the disease was followed for 20-67 months. Clinical improvement was observed after daily intraventricular administration of IFN in one case. There were no serious complications or side effects during interferon therapy except for the fever. Intraventricular administration of IFN appears superior to intrathecal administration for long-term treatment in several respects and is considered to be a potential therapeutic modality for SSPE.
Assuntos
Interferon Tipo I/uso terapêutico , Panencefalite Esclerosante Subaguda/terapia , Criança , Implantes de Medicamento , Humanos , Interferon Tipo I/administração & dosagem , Masculino , Fatores de TempoRESUMO
Subacute sclerosing panencephalitis (SSPE) is a rare infectious central nervous system (CNS) disease with a poor prognosis. We reported on the case of an adolescent girl with SSPE and characteristic periodic electroencephalographic (EEG) complexes. Her neurological deficits including generalized myoclonic seizures improved after intraventricular interferon (IFN) treatment. However, unusual EEG patterns consisting of bisynchronous occipital spikes preceding periodic complexes developed in follow-up EEGs.
Assuntos
Eletroencefalografia , Panencefalite Esclerosante Subaguda/diagnóstico , Panencefalite Esclerosante Subaguda/fisiopatologia , Adolescente , Feminino , Humanos , Injeções Intraventriculares , Interferons/administração & dosagem , Lobo Occipital/fisiopatologia , Prognóstico , Panencefalite Esclerosante Subaguda/terapiaRESUMO
This is a case report of a 10-year-old boy with subacute sclerosing panencephalitis (SSPE). He initially developed visual disturbance and macular degenerative changes of the right eye at the age of 8 years, followed by chorioretinitis of the left eye, and his neurological symptoms deteriorated rapidly from the age of 10 years. He was diagnosed as having SSPE, as judged on cerebrospinal fluid examination for measles virus RNA by reverse transcription-polymerase chain reaction (RT-PCR), at the second stage of Jabbour's classification on admission. Although high intensity lesions were observed in the right occipital and temporal lobes, especially around the optic radiation, on T2-weighted brain MRI before the start of intrathecal interferon-alpha (IFN-alpha) therapy, they had disappeared at about two months after the treatment. Chorioretinitis (and/or macular degeneration) should be considered in the differential diagnosis of SSPE, permitting early IFN therapy.
Assuntos
Coriorretinite/complicações , Panencefalite Esclerosante Subaguda/complicações , Criança , Coriorretinite/diagnóstico , Coriorretinite/terapia , Diagnóstico Diferencial , Humanos , Interferon gama/administração & dosagem , Degeneração Macular/diagnóstico , Degeneração Macular/etiologia , Degeneração Macular/terapia , Masculino , Panencefalite Esclerosante Subaguda/diagnóstico , Panencefalite Esclerosante Subaguda/terapiaRESUMO
A 10-year-old boy with subacute sclerosing panencephalitis was treated with intravenous gamma-globulin and inosiplex and followed for 18 months. Clinical improvement, demonstrated by decreasing scores on the Neurologic Disability Index, was observed. There were no side effects. We recommend intravenous immune globulin as an alternative therapy in the treatment of subacute sclerosing panencephalitis.