Subacute Sclerosing Panencephalitis Causing Rapidly Progressive Dementia and Myoclonic Jerks in a Sexagenarian Woman.

Background: Subacute sclerosing panencephalitis (SSPE) is a disease of childhood and adolescence, but can affect adults. Rapidly progressive cognitive decline, seizures including myoclonic jerks, spasticity, ataxia, visual disturbances, and incontinence are typical manifestations. Case report: A 62-year-old woman who presented with rapidly progressive dementia and myoclonus was diagnosed with SSPE. There was resolution of the movement disorder with clonazepam and valproic acid treatment and some amelioration of cognitive decline after 3 months of therapy with interferon alfa and isoprinosine. Discussion: With the recent rise in measles cases worldwide, any increased incidence of SSPE would require vigilance for early interventions.

Síndrome de Ogilvie / Ogilvie's syndrome

A síndrome de Ogilvie é uma situação clínica rara, caracterizada por uma dilatação aguda do cólon, sem evidência de obstrução orgânica ou processo inflamatório. A associação com doença extracolônica é quase uma constante1. Esta dilatação é rapidamente progressiva podendo culminar na necrose e perfuração do intestino grosso, particularmente do ceco2. A terapêutica deve ser precoce, com medidas clínicas seguidas por descompressão cirúrgica ou endoscópica3. Apresentamos um caso de um paciente com seqüelas neurológicas de panencefalite esclerosante subaguda com quadro de abdome agudo obstrutivo, que foi submetido a laparotomia exploradora com cecostomia.

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

We describe an atypical clinical and electroencephalographic (EEG) pattern observed during the course of subacute sclerosing panencephalitis in a 14 year-old boy. In this patient with a two weeks history of partial complex seizures, the atypical EEG pattern was characterized by an initial left temporal focus which evolved to periodic lateralized epileptiform discharges (PLEDs) and, only during the 3rd and 4th weeks the typical bilateral and generalized periodic complexes appeared.

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.

The influence of inosiplex treatment on the neurological disability of patients with subacute sclerosing panencephalitis.

Subacute sclerosing panencephalitis is a central nervous system degenerative disease that rapidly progresses to death in most untreated cases. In this study we compare the level of neurological disability longitudinally in a group of SSPE patients receiving inosiplex (Isoprinosine) treatment (12) to a historical control group of untreated patients (15). The mean ND did not differ between the groups from onset of SSPE through 21 months. From two years through four and one-half years the inosiplex group had significantly lower ND compared to the nontreatment group. The subjects were then divided into four subgroups: Group 1, rapidly progressing SSPE, not treated; Group 2, slowly progressing SSPE, not treated; Group 3, rapidly progressing SSPE, inosiplex treated; and Group 4 slowly progressing SSPE, inosiplex treated. The rapidly developing groups did not differ in ND at any time. The slowly developing treated group had significantly lower ND than the slowly developing untreated group from two and one-half years to four and one-half years after onset of SSPE. These findings suggest that inosiplex is effective in the slowly developing or chronic form of SSPE.

[Subacute sclerosing panencephalitis with partial remission]. / Panencefalite esclerosante subaguda com remissão parcial.

Subacute progressive panencephalitis is usually a progressive and fatal disease, being uncommon temporary or definitive remissions. A three years old boy, previously vaccinated against measles, developed trembling, progressive and severe mental deterioration, partial seizures and myoclonic jerks. The electroencephalogram showed periodic high amplitude waves concomitantly with myoclonic jerks and the cerebrospinal fluid revealed an increase of the gammaglobulin fraction (16,8), benjoin coloidal reaction shifted to the left and the antimeasles antibody titres were positive (complement fixation text 1:16; neutralization test 1:32). In spite of that, two months after the beginning of the illness the patient showed mental and motor improvement and similar modifications of the electroencephalographic aspects and now, eleven months later, is well, remaining only a slight motor and mental deficiency.