RT Journal Article
SR Electronic
T1 Adult-onset subacute sclerosing panencephalitis
JF Practical Neurology
JO Pract Neurol
FD BMJ Publishing Group Ltd
SP 346
OP 350
DO 10.1136/practneurol-2020-002880
VO 21
IS 4
A1 Sruthi S Nair
A1 K V Vysakha
A1 Ramshekhar N Menon
A1 Soumya Sundaram
YR 2021
UL http://pn.bmj.com/content/21/4/346.abstract
AB Subacute sclerosing panencephalitis (SSPE) is a lethal slow viral disease of the central nervous system caused by a defective measles virus. The onset is mostly in childhood, manifesting clinically as decline in academic performance, behavioural changes, motor dysfunction and myoclonus. Adult-onset SSPE is rare and can present as rapidly progressive dementia. We present a young man of Indian origin with adult-onset SSPE with rapidly progressive dementia but no localising neurological signs. The diagnostic clues were parieto-occipital white matter changes on MR brain scan and history of childhood fever with rash. High titres of antimeasles antibody in cerebrospinal fluid confirmed the diagnosis. The long latency from primary measles virus infection to symptom onset can be misleading in adults. SSPE should be considered in adults with dementia, especially in tropical countries where vaccination coverage is suboptimal.