Evolution of certain typical and atypical features in a case of subacute sclerosing panencephalitis.
نویسندگان
چکیده
Subacute sclerosing panencephalitis (SSPE) is a slowly progressive inflammatory disease of the central nervous system caused by a persistent measles virus usually affecting the childhood and adolescent age group. Clinical features at onset are very subtle and non-specific. Certain atypical features can occur at onset or during the course of illness which can be misleading. Neuroimaging features often are non-specific. Features like myoclonic jerks, cognitive decline and typical EEG findings lead to a strong suspicion of SSPE. Here, we describe the stagewise progression of a case of SSPE in a 14-year-old girl who had myoclonic jerks and cognitive decline at onset. During the course of disease, the patient developed cortical vision loss, atypical extrapyramidal features like segmental and hemifacial dystonia ultimately leading to a bedbound vegetative state. EEG showed typical periodic discharges along with positive cerebrospinal fluid serology for measles.
منابع مشابه
Subacute Sclerosing Panencephalitis in a Child with Recurrent Febrile Seizures
Subacute sclerosing panencephalitis (SSPE) is a devastating disease of the central nervous system (CNS) caused by persistent mutant measles virus infection. The diagnosis of SSPE is based on characteristic clinical and EEG findings and demonstration of elevated antibody titres against measles in cerebrospinal fluid. Subacute sclerosing panencephalitis can have atypical clinical features at the ...
متن کاملNonconvulsive Status Epilepticus on Electroencephalography: An Atypical Presentation of Subacute Sclerosing Panencephalitis in Two Children
Subacute sclerosing panencephalitis is a neurodegenerative disease secondary to measles infection that usually has a typical presentation with progressive myoclonia, cognitive decline, and periodic slow-wave complexes on electroencephalography. We report two pediatric cases who presented with periodic myoclonic jerks and cognitive decline. In both cases, the electroencephalogram showed continuo...
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ورودعنوان ژورنال:
- BMJ case reports
دوره 2012 شماره
صفحات -
تاریخ انتشار 2012