Two Cases of Rapidly Progressive Subacute Sclerosing Panencephalitis

Fulminating Subacute Scierosing Panencephalitis: Case Report and Literature Review

“…The duration of this stage in general is variable, ranging from a few months to a few years.' Myoclonic jerks were described in eight of these patients including our patient (Cases 1, 2, 5, 16,19,23,24,26).182023-26 The jerks herald the onset of the second stage. The myoclonus in SSPE is fairly characteristic, occurring frequently at regular intervals.…”

“…Almost three quarters (18 patients including the present patient) presented with personality changes, mental difficulties, and/or academic failures (Cases 1-3, 5-7, 9-14, 17, 18, 20, 23, 24, 26). [13][14][15][16]19,21,[23][24][25][26][27] The symptoms are typically the initial manifestations representing the first stage of the disease. Because of its rarity, SSPE is often not considered until more typical clinical manifestations appear.…”

Scientific correspondence

“…SSPE usually begins insidiously and follows a subacute course with relentless but slow progression to death. In the world literature, there have been only 23 previously reported cases of fulminant SSPE (Table 1) [2,5,9,10,12,15,18–22,24], for which the time from onset of first symptoms to death ranged from 1 month to 6 months with a median of 2 months, in contrast to a median of 1.8 years for all SSPE [16].…”

“…To our knowledge, there have been 23 previously reported cases of acute fulminant SSPE. Of these, only 11 had autopsy or brain biopsy confirmation, 14 had serological confirmation, four had MRI imaging and one had the genotype sequenced (Table 1) [2,5,9–12,15,18–22,24]. This is the first reported case of acute fulminant SSPE having MRI imaging, serological confirmation, autopsy confirmation with neuropathology and measles virus antigen detection in brain tissue by immunofluoresence, as well as sequencing and identification of the measles virus genotype.…”

A novel, progressive, sclerosing panencephalitis in a horse

“…In man, there are 2 diseases that result in sclerosing encephalitis. Subacute sclerosing panencephalitis (SSPE) is a rare but serious progressive degenerative disease of the central nervous system (Freeman 1969), caused by a persistent measles virus infection (Chen et al 1969;Horta-Barbosa et al 1969) and characterised by progressive dementia, incoordination, ataxia, myoclonic jerks (Jubelt and Miller 1989), personality changes (Tibbles et al 1964), altered mentation (Silva et al 1981), visual disturbances (Nelson et al 1970;Brudet-Wickel et al 1982;Caruso et al 1997) and other focal neurological signs (Jubelt and Miller 1989). Subacute sclerosing panencephalitis typically affects children and adolescents (Jubelt and Miller 1989) and children developing measles at a young age are more prone to SSPE which occurs after a latent period of several years (Dyken 1985).…”