Subacute Sclerosing Panencephalitis: Results of the Canadian Paediatric Surveillance Program and review of the literature

Campbell, Craig; Levin, Simon; Humphreys, Peter; Walop, Wikke; Brannan, Renee

doi:10.1186/1471-2431-5-47

Cited by 45 publications

(28 citation statements)

References 48 publications

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“…The recently reported incidence of SSPE in one of the developed countries (Canada) was 0.06/million children/year [1]. However, the incidence of SSPE still remains high in Turkey and in some poor and developing countries like Papua New Guinea and India [2,3].…”

Section: Epidemiologymentioning

confidence: 98%

Subacute sclerosing panencephalitis

2008

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Subacute sclerosing panencephalitis (SSPE) is a subacute encephalopathy of childhood and young adolescence. Infrequently, SSPE can occur in adults and pregnant women. It is caused by an aberrant measles virus, known as the SSPE virus. SSPE virus differs from wild-type measles viruses in the form of several mutations affecting the viral genome. The matrix gene is most commonly affected by these mutations. The characteristic clinical manifestations of SSPE include behavioral changes, cognitive decline, myoclonic jerks, seizures, abnormalities in vision, bilateral pyramidal signs and coma. Ocular changes may occur in up to 50% of patients. The most characteristic ophthalmological lesion is necrotizing retinitis. Cortical blindness can be the early feature of SSPE. The diagnosis of SSPE is often difficult in the early stages. In a typical case diagnosis is based on clinical, electroencephalographic, and cerebrospinal fluid findings. At present, there is no effective treatment to completely cure SSPE. Oral isoprinosine and intrathecal or intraventricular alpha-interferon may prolong survival to some extent. Immunization against measles is currently the most effective strategy against SSPE.

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Section: Epidemiologymentioning

confidence: 98%

Subacute sclerosing panencephalitis

2008

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“…The exact mechanism producing an acute fulminant course is not known. Several factors such as exposure to measles at an early age, viral virulence, impaired host defense mechanisms, and concurrent infections with other viruses, have been suggested as responsible for producing a rapid course of the disease 7,19 . The disease is fatal in 90-95% of cases 13 .…”

Section: Discussionmentioning

confidence: 99%

“…In the Canadian Paediatric Surveillance Pro-gram, Campbell et al 7 diagnosed four cases of SSPE, of whom one was previously immunized (25%) and presented with an atypical EEG pattern. Salehi et al 8 reported three atypical cases of SSPE in immunized patients.…”

Section: Discussionmentioning

confidence: 99%

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Atypical subacute sclerosing panencephalitis: case report

Cruzeiro

Vale

Pires

et al. 2007

Arq. Neuro-Psiquiatr.

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-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.KEY WORDS: subacute sclerosing panencephalitis, measles, immunization.Panencefalite esclerosante subaguda: relato de caso atípico RESUMO -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.PALAVRAS-CHAVE: panencefalite esclerosante subaguda, sarampo, imunização.

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“…The disease incidence varies in different countries according to the routine immunization policies of the countries (2)(3)(4)(5). For instance, the disease is seen as 0.01 per million in the USA (2), 0.06 per million in Canada (3), 0.461 per million in Turkey (4), and 11 per million in Japan (5).…”

Section: Introductionmentioning

confidence: 99%

Clinical Characteristics of Oral Structures and Feeding Function in Subacute Sclerosing Panencephalitis

Arslan¹,

Alemdaroğlu²,

Demir³

et al. 2016

Türk Fizyoterapi Ve Rehabilitasyon Dergisi

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Purpose: The aim was to investigate the characteristics of oral structures and feeding in patients with Subacute Sclerosing Panencephalitis (SSPE).Methods: Twenty SSPE patients were included. Clinical evaluation of oral structures and oral hygiene were performed. Each patient was requested to take liquid, pudding and solid food consistencies during feeding evaluation. The Karaduman Chewing Performance Scale (KCPS) was used to determine the level of chewing function. The 3-ounce water swallow test was used to screen patients for aspiration risk. Results:The mean age was 13.76±1.92 years, of which 55% were male. Seven children (35%) had open mouth posture at rest, 5 (25%) had open bite, 12 (60%) had high arched palate, and 8 (40%) had tongue thrust. Eighteen children (90%) had oral hygiene problems. Feeding positions were variable with 1 child fed in supine, 6 in semi-sitting position, and 13 in upright sitting position. Findings during clinical feeding evaluation revealed the following: food acceptance (20, 100%), liquid intake (20, 80%), pudding intake (17, 85%), and solid food intake (5, 25%). Five children (25%) were in level I, 7 children (35%) were in level II, 3 children (15%) were in level III, and 5 children (25%) were in level IV according to the KCPS. Four children (25%) failed in 3 ounce water swallow test.Discussion: Problems with oral structures, oral hygiene, chewing and swallowing function can be seen in SSPE patients. Thus, clinical oral structure and feeding functions should be considered to evaluate in routine evaluation procedure in patients with SSPE. Tartışma: SSPE hastalarında oral yapılar, oral hijyen, çiğneme ve yutma fonksiyonunda problemler görülebilmektedir. Bu yüzden, SSPE hastalarının rutin değerlendirmesinde klinik oral yapı ve beslenme fonksiyonlarının değerlendirilmesi de düşünülmelidir.

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Subacute Sclerosing Panencephalitis: Results of the Canadian Paediatric Surveillance Program and review of the literature

Cited by 45 publications

References 48 publications

Subacute sclerosing panencephalitis

Subacute sclerosing panencephalitis

Atypical subacute sclerosing panencephalitis: case report

Clinical Characteristics of Oral Structures and Feeding Function in Subacute Sclerosing Panencephalitis

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