Acute bilateral vision loss as the presenting feature of subacute sclerosing panencephalitis.

A 12-yr-old boy with an atypical presentation of subacute sclerosing panencephalitis (SSPE) is described. Bilateral macular chorioretinitis preceded the neurological symptoms by 3 weeks. Both visual and neurological features had an acute onset. Clinicians need to be aware that macular chorioretinitis in a child may be the heralding feature of SSPE.

Atypical subacute sclerosing panencephalitis: case report

Subacute sclerosing panencephalitis (SSPE) is a progressive inflammatory disorder of the central nervous system with both poor prognosis and high mortality. The disease has been related to a persistent and aberrant measles virus infection and no effective treatment has been available. We report a case of SSPE with atypical features including seizures at onset and a fulminant course in a 8 years-old boy who had been previously immunized against measles.

Panencefalite esclerosante subaguda (PES) é uma doença inflamatória e progressiva do sistema nervoso central com prognóstico reservado e alta mortalidade. A doença tem sido relacionada com a infecção persistente e anormal pelo vírus do sarampo e não há tratamento específico disponível. Relatamos um caso de PES com características atípicas representadas por início do quadro com crises convulsivas e apresentação fulminante em menino de 8 anos previamente imunizado contra o vírus do sarampo.

Panencefalitis esclerosante subaguda: experiencia en la consulta de neuropediatría del Hospital J.M. de Los Ríos / Subacute sclerosing panencephalitis: experience in the ambulatory clinic of the Hospital J.M. de Los Rios

La panencefalitis esclerosante subaguda (PEES), o enfermedad de Van Boager, es una encefalopatía lentamente progresiva, originada por la infección persistente por una forma mutante del virus del sarampión, que ocasiona una desmielinización inflamatoria multifocal del sistema nervioso central. Es conocido que infecciones antes de los 2 años de edad aumentan el riesgo de padecer PEES, no demostrándose hasta la actualidad casos secundarios a vacunación. Presentar una revisión de cinco casos clínicos, que consultaron el Hospital de Niños "J.M de Los Ríos", en el periodo comprendido entre los años 1990-2005, con diagnóstico de PEES; en quienes se analiza la forma de presentación, manifestaciones clínicas, hallazgos en los estudios serológicos, de imágenes y evolución. Todos los pacientes tuvieron antecedentes de infección por sarampión cuatro de ellos antes de los dos años de edad. La edad promedio de presentación fue de 7 años 3 meses, con una media para el período de latencia de 5,2 años. En relación a las manifestaciones clínicas iniciales, en todos hubo crisis epilépticas mioclónicas, en dos de ellos trastornos conductuales y en otros dos somnolencia. Los estudios electroencefalográficos mostraron un patrón periódico en todos los casos. Los t¡tulos de anticuerpos antisarampión positivos en suero y líquido cefalorraquídeo confirmaron el diagnóstico en cuatro casos. La evolución fue fatal en un caso y desfavorable en los otros, con compromiso en áreas motoras, sensoriales y cognitivas. El tratamiento hasta los momentos sigue siendo preventivo, erradicando el sarampión y manteniendo un plan de vacunación.

Subacute sclerosing panencephalitis (SSPE), or disease of Boager Goes, is a slowly progressive encephalopathy originated by the persistent infection of a mutant form of the measles virus, which causes an inflammatory multifocal demielinization of the central nervous system. It is known that infections before 2 years of age increase the risk of SSPE. Cases following vaccination have not been demonstrated. To present a review of five children with SSPE attended at the Children's Hospital ¨J.M de Los Ríos" between 1990 and 2005. The initial presentation, clinical manifestations, serologic and imaging findings as well as the clinical outcome were analysed. All patients had a precedent measles infection, four of them before two years of age. The average age of presentation was 7 years 3 months, with an average for the period of latency of 5.2 years. In relation to the clinical initial manifestations, myoclonic events were common to all patients, two of them presented with cognitive deterioration and two with drowsiness. The EEG studies demonstrated a periodic boss in all cases. Anti measles antibodies titers in serum and spinal liquid confirmed the diagnosis in four cases. The outcome was fatal in one case and unfavourable in the others, whom presented motor, sensory and cognitive deterioration. Treatment at the present time continues to lie in preventive measures, supporting the plan of vaccination and aiming to the eradication of measles.

Subacute sclerosing panencephalitis presenting as acute disseminated encephalomyelitis.

Subacute sclerosing panencephalitis (SSPE) is a chronic encephalitis of childhood and young adolescence due to persistent measles virus infection of the central nervous system. In majority of cases onset occurs from 5-15 years of age. In a nonimmunized population the average onset is 8 years. Children with SSPE had experienced natural infection with the rubeola virus at an early age, half before age 2 years. SSPE generally occurs 5-10 years after measles infection. In the early stages of the disease behavioral and personality changes is followed by myoclonic jerks and convulsions. In late stages dementia, stupor and coma develops. Diagnosis is achieved by typical clinical findings, measles antibody titer increase in cerebrospinal fluid (CSF) and serum, high amplitude, slow, sharp waves in EEG. Prognosis is poor and death ensues in about 3 yr after the diagnosis. Here it is presented a 7-years-old boy with involuntary movements in both hands, drop attacks while walking, ataxia and stupor. Due to suggestive radiological and clinical findings and a history of recent mumps infection he was thought to have acute disseminated encephalomyelitis initially and given treatment. But due to clinical deterioration and detection of anti measles IgG in serum and CSF, SSPE diagnosis was confirmed. With this SSPE case presenting initially as ADEM, the authors tried to emphasize that presentation of SSPE may clinically and radiologically be diverse and a thorough differential diagnosis is mandatory for a definite diagnosis.

Diagnostic dilemmas in fulminant subacute sclerosing panencephalitis (SSPE).

This report describes an eleven-year-old boy with atypical features of subacute sclerosing panencephalitis (SSPE), a rare complication of measles. He had only visual symptoms for 2 months followed by rapid neurological worsening to a vegetative state in 10 days. A diagnosis of SSPE was made based on the history of measles, characteristic ocular findings, compatible magnetic resonance imaging and electroencephalographic changes, and elevated ratio of cerebrospinal fluid to serum anti-measles antibody titers.

Subacute sclerosing panencephalitis presenting as optic neuritis.

Subacute sclerosing panencephalitis (SSPE) is a rare progressive neurologic disease affecting both grey and white matter of the brain in children and young adults. One such case which involved the visual system is described here.

Panencefalitis esclerosante subaguda: reporte de un caso en Chile / Subacute sclerosing panencephalitis: a case report in Chile

La panencefalitis esclerosante subaguda postsarampión (PEES) es en la actualidad una entidad infrecuente en aquellos países donde existen programas regulares de vacunación contra el sarampión. Presentamos un caso, con evolución rápida a la muerte, en el cual fueron observados hallazgos característicos. Entre los más relevantes: títulos elevados de anticuerpos antisarampión en el líquido cefalorraquídeo (LCR), patrón periódico en el EEG, fenómenos de desmielinización progresiva en el scanner y resonancia nuclear magnética cerebral, asociados a la presencia constante de crisis mioclónicas. A pesar de una terapia continua con alfa-interferón intratecal, cimetidina en dosis inmunorreguladoras y con antiviral, se observó un deterioro progresivo del paciente. El estudio neuropatológico reveló compromiso a nivel de los centros semiovales de los hemisferios frontales, de predominio izquierdo

Panencefalitis esclerosante subaguda de inicio en edad adulta: presentación atípica en 2 pacientes / Adult onset subacute sclerosing panencephalitis: atypical presentation in 2 cases

Subacute sclerosing panencephalitis is an infrequent central nervous system viral disease and is a late manifestation of persistent infection by a mutant form of measles virus. Since it affects mainly children and teenagers, the diagnosis in older ages is difficult. Its main clinical symptoms are cognitive impairment, behavioral disturbances and myoclonia. We report two males, aged 21 and 22 years old, presenting with the disease with atypical manifestations. One had a catatonic syndrome and the other, amaurosis. The recognition of the different presentation forms of the disease, endemic in developing countries, allows an earlier diagnosis and a more efficient treatment, when available

Atypical clinical and electroencephalographic pattern in a patient with subacute sclerosing panencephalitis

Descrevemos o quadro clínico e eletrencefalográfico atípicos observados durante a evoluçäo de panencefalite esclerosante subaguada, em um menino de 14 anos. Neste paciente, com história de crises parciais complexas há duas semanas, o padräo eletencefalográfico atípico foi caracterizado por foco temporal esquerdo que evoluiu para "periodic lateralized epileptiform discharges" (PLEDs), somente durante a 3ª e 4ª semanas surgindo os clássicos complexos periódicos difusos

Neuroimagem em panencefalite esclerosante subaguda - relato de um caso e diagnóstico diferencial / Subacute sclerosing panencephaling - a case report and differential diagnosis

Os autores apresentam um caso de panencefalite esclerosante subaguda, enfocando os aspectos da neuroimagem e o diagnóstico diferencial com outras doenças do sistema nervoso central que têm quadro clínico semelhante.

Serodiagnosis of subacute sclerosing panencephalitis by enzyme linked immunosorbent assay.

Thirty three young patients (23 males and 10 females), between 3-20 years of age, with a clinical suspicion of subacute sclerosing panencephalitis (SSPE) were evaluated over a period of 4 years. A past history of measles was obtained in 42.4% of the cases. One child was immunised against measles. Measles specific antibodies (IgG) were demonstrated in the cerebrospinal fluid (CSF) from 30 (90.9%) of the 33 patients by ELISA. In the remaining three cases the antibodies were detected only in the serum. Serodiagnosis of SSPE by ELISA had a sensitivity of 100%, a specificity of 93.3% and a positive predictive value of 100%.

Panencefalite esclerosante subaguda: relato de caso / Subacute sclerosing panencephalitis: a case report

Os autores fazem uma breve revisäo , quanto ao diagnóstico, aspectos clínicos e evolutivos da Panencefalite Esclerosante Subaguda. A seguir, relatam um caso dessa patologia em uma criança de 5 anos de idade, cuja evoluçäo clínica näo segue os parâmetros clássicos

Prevalence of SSPE: a serological study.

The presence of measles antibodies in serum and cerebrospinal fluid (CSF) of 340 samples from children clinically suspected of subacute sclerosing panencephalitis (SSPE) were studied. One hundred and thirty eight (40%) of these children had SSPE based on the serological evidence. The mean age group of children affected was 8.2 years. The M:F ratio was 5:1. The titres of antibodies ranged from 1:2 to 1:32 in the CSF and from 1:16 to 1:512 in the serum.

Long term survival in subacute sclerosing panencephalitis.

A case of subacute sclerosing panencephalitis (SSPE) with a long survival of 8 years is reported. Long term survival in SSPE has not been previously reported from Sri Lanka.