SSPE (Subacute sclerosing panencephalitis) - the great masquerader

Chheda, Akash; Agrawal, Shruti; Ravat, Sangeeta; Pillai, Rajit; Jain, Neeraj

doi:10.1016/j.jns.2021.119935

Cited by 1 publication

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“…Our review identified dystonia as the presenting feature in 54 (12.8%) of patients (Table S1). Onset of dystonia may be acute or it may develop over the course of the disease 34 . Among 56 patients below 20 years presenting with dystonia at one center, 35 three had SSPE.…”

Section: Resultsmentioning

confidence: 99%

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Movement Disorders in Patients with Subacute Sclerosing Panencephalitis: A Systematic Review

Garg,

Patel,

Sankhla

et al. 2024

Movement Disord Clin Pract

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BackgroundSubacute sclerosing panencephalitis (SSPE) is a complication of measles, occurring after a latency of 4–10 years. It continues to occur in developing countries although resurgence is being reported from developed countries. Characteristic features include progressive neuropsychiatric issues, myoclonus, seizures, movement disorders and visual impairment. Electroencephalography (EEG) typically shows periodic generalized discharges, and elevated CSF anti‐measles antibodies are diagnostic. Movement disorders are being increasingly recognized as part of the clinical spectrum, and range from hyperkinetic (chorea, dystonia, tremor, tics) to hypokinetic (parkinsonism) disorders and ataxia.ObjectivesThis article aims to comprehensively review the spectrum of movement disorders associated with SSPE.MethodsA literature search was conducted in PubMed and EMBASE databases in December 2023 and articles were identified for review.ResultsMovement disorders reported in SSPE included hyperkinetic (chorea, dystonia, tremor and tics), hypokinetic (parkinsonism), ataxia and extraocular movement disorders. Myoclonus, a core clinical feature, was the most frequent “abnormal movement.” Movement disorders were observed in all clinical stages, and could also be a presenting feature, even sans myoclonus. Hyperkinetic movement disorders were more common than hypokinetic movement disorders. An evolution of movement disorders was observed, with ataxia, chorea and dystonia occurring earlier, and parkinsonism later in the disease. Neuroradiological correlates of movement disorders remained unclear.ConclusionA wide spectrum of movement disorders was observed throughout the clinical stages of SSPE. Most data were derived from case reports and small case series. Multicentric longitudinal studies are required to better delineate the spectrum and evolution of movement disorders in SSPE.

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Section: Resultsmentioning

confidence: 99%

“…Onset of dystonia may be acute or it may develop over the course of the disease. 34 Among 56 patients below 20 years presenting with dystonia at one center, 35 three had SSPE. One study reported a child with fulminant presentation, in association with periodic myoclonus.…”

Section: Dystoniamentioning

confidence: 99%