Adult-onset subacute sclerosing panencephalitis presenting as a degenerative dementia syndrome

Frings, Markus; Blaeser, Ingo; Kastrup, Oliver

doi:10.1007/s00415-002-0720-6

Cited by 19 publications

(13 citation statements)

References 6 publications

Supporting

Mentioning

Contrasting

Order By: Relevance

“…Diagnosis depended on a high index of suspicion, as well as patient access to either specialized neurologists or a physician familiar with the rare disease SSPE. The numerous case reports and reviews of SSPE underscore the complexity of the clinical aspects of the disease and the challenges associated with the recognition and diagnosis of SSPE, particularly adult-onset SSPE and SSPE that follows a fulminating course [1,2,19,52,53].…”

Section: Discussionmentioning

confidence: 99%

Subacute Sclerosing Panencephalitis: More Cases of This Fatal Disease Are Prevented by Measles Immunization than Was Previously Recognized

et al. 2005

View full text Add to dashboard Cite

Section: Discussionmentioning

confidence: 99%

Subacute Sclerosing Panencephalitis: More Cases of This Fatal Disease Are Prevented by Measles Immunization than Was Previously Recognized

et al. 2005

View full text Add to dashboard Cite

“…Recent studies indicate that after the measles virus enters the human body, the structure of the virus begins to change. Structural changes in the M protein, which is formed as a result of a long-lasting latent period; glycoprotein (F), which causes membrane fusion; and hemagglutinin (H) have been observed in virus particles in the CNS (1,4). It has been demonstrated that the mutant virus develops as a result of these structural changes and can replicate in brain tissue via protein synthesis; it can then infect the brain, which lacks an immune system.…”

Section: Discussionmentioning

confidence: 99%

“…Although SSPE affects individuals at a wide range of ages, with a range of 2 to Turk J Neurol 2015;21:112-6 DO I:10.4274/tnd.03789 32 years reported in the literature, it is most often observed in those aged between 5 and 15 years (1). Nearly 50% of patients were reported to have had early measles infections, especially before 2 years of age, or measles vaccinations (2,3,4,5). The disease rapidly progresses; patients typically die 6-24 months after symptom onset (2).…”

Section: Introductionmentioning

confidence: 99%

Process From Diagnosis To Treatment Of 3 SSPE Cases Presented At The Late Age

Bican¹,

Bora²

2015

tnd

View full text Add to dashboard Cite

Subacute sclerosing panencephalitis (SSPE) is diagnosed with clinical evaluation including history and physical evaluation along with electroencephalogram (EEG), magnetic resonance imaging of the brain and cerebrospinal fluid (CSF) analysis. A total of 3 patients with SSPE (mean age=25.3 years) who were admitted to the Department of Neurology at Uludağ University Faculty of Medicine, were evaluated retrospectively. Clinical staging was performed using Risk and Haddad's classification. Time to SSPE diagnosis from initial symptoms ranged from 10 days to 3 months; the patients presented to a physician two months after their symptoms were recognized; weakening of mental capacities attracted the attention of members of their families. EEG examinations revealed slow baseline activity and penetrating slow discharges with regular high amplitudes every 8-10 seconds. Although immunoglobulin (Ig) M for measles was negative, IgG was positive in the cerebrospinal fluid and serum samples of all patients. All patients received isoprinosine, and various doses of valproic acid and clonazepam during their treatment. One patient died 13 months after the diagnosis, the other two continue to be followed up. SSPE infection should be considered in central nervous system-involved rapidly-progressing late-onset adult diseases because there has recently been a rapid increase in the number of reported cases of SSPE.Keywords: Subacute sclerosing panencephalitis, prognosis, late presentation Subakut slerozan panensefalit (SSPE) elektroensefalografi (EEG), kranyal manyetik rezonans (MR) görüntüleme ve klinik belirtileri değerlendirip, hikayeleri, fizik muayenedeki değişikliği ve EEG ve beyin omurilik sıvısı (BOS) incelemeleri ile tanı konur. Uludağ Üniversitesi Tıp Fakültesi, Nöroloji Kliniği'nde toplam 3 SSPE'li (yaş ortalaması=25,3) hasta retrospektif olarak değerlendirildi. SSPE tanısı semptomların başlangıcından 10 gün ile 3 ay arasında konuldu. EEG incelemelerinde 8-10 saniye süreli yüksek amplitüdlü yavaş dalgalar ve temel aktivitede yavaşlama gözlendi. BOS örneklerinde kızamık IgM negatif iken IgG pozitif, serumda hepsi pozitif idi. Valproik asit ve klonazepam değişik dozlarda ve sürede kullanıldı tüm olgulara Isoprinosine verildi. Bir olgu 13 ay sonra kaybedildi, diğer olgular takip edilmektedir. SSPE enfeksiyonu santral sinir sistemini tutup hızlıca progrese olmaktadır ve son zamanlarda geç yaşta görülen SSPE olgularının sunumları hızla artmaktadır.

show abstract

“…104 It may be present in VaD, CBD, 60 DLB, 37 prion diseases, 29 and HIV. 62 Other rare causes include SSPE, 31 familial encephalopathy with neuroserpin inclusion bodies, 23 familial British dementia, 89 hereditary hemochromatosis, 67 mitochondrial disease, 90 Gaucher disease, 71 sialidosis, 92 Kufs disease, 73 Lafora body disease, 93 and PLOSL. 97,98 Ophthalmologic assessment may provide a clue to the underlying disease process.…”

Section: Other Neurologic Symptoms/signs Presentmentioning

confidence: 98%