Abstract
Subacute Sclerosing Panencephalitis (SSPE) is a chronic complication of measles. It is a neurodegenerative disorder characterized by cognitive and memory deterioration, behaviour abnormalities, involuntary movements and repetitive myoclonic jerks and a steady motor decline. We report a case of a 7-year-old girl with subacute sclerosing panencephalitis presenting with myoclonic jerks, regression of motor milestones and speech. The child was not immunised for measles. Magnetic resonance imaging revealed multiple variable-sized ill-defined T2W and FLAIR hyperintense areas are seen involving the white matter of the bilateral fronto-parietal lobes. Electroencephalograph findings and CSF analysis confirmed the diagnosis of subacute sclerosing panencephalitis.
Highlights
Subacute Sclerosing Panencephalitis (SSPE) is a neurodegenerative disorder due to a persistent infection with a mutated wild measles virus that is harboured intracellularly in the central nervous system for several years. [1,2] After 7-10 years the virus regains virulence and attacks the cells in the central nervous system resulting in inflammation and cell death, leading to a neurodegenerative process
Electroencephalogram showed large-amplitude periodic complexes, these periodic complexes repeat at fairly regular 4– 10 second intervals and have 1:1 relationship with myoclonic jerks [Figure 1], magnetic resonance imaging (MRI) showed multiple variable-sized ill-defined T2W and FLAIR hyperintense areas are seen involving the white matter of the bilateral fronto-parietal lobes [Figure 2], No restriction on diffusionweighted images or blooming on gradient images is noted
We discuss a case of SSPE with myoclonic jerks and white matter hyperintensities of bilateral fronto-parietal lobes classifying it as a stage 2 disease
Summary
Subacute Sclerosing Panencephalitis (SSPE) is a neurodegenerative disorder due to a persistent infection with a mutated wild measles virus that is harboured intracellularly in the central nervous system for several years. [1,2] After 7-10 years the virus regains virulence and attacks the cells in the central nervous system resulting in inflammation and cell death, leading to a neurodegenerative process. Subacute Sclerosing Panencephalitis (SSPE) is a neurodegenerative disorder due to a persistent infection with a mutated wild measles virus that is harboured intracellularly in the central nervous system for several years. We discuss a 7-year old girl with SSPE presenting with myoclonic jerks, regression of motor milestones and speech. The patient developed jerky movements of head each occurring at an interval of 5-10 mins This lasted for a year which gradually involved upper limbs and lower limbs for one month. There was a regression of motor-milestones and speech She can stand with support and can speak 2-3 word phrases. Development of the child was normal till 6 years of age On examination, she was conscious, oriented to person. Anti-measles antibodies titres were elevated in both blood and CSF [Table 1] With these findings a diagnosis of SSPE is confirmed most likely Stage II. Other drugs like Isoprinosine, interferons are not prescribed due to financial constraints
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