Abstract
Subacute sclerosing panencephalitis (SSPE) is a rare chronic, progressive encephalitis that affects primarily children and young adults, caused by a persistent infection of immune-resistant measles virus. Diagnostic hallmarks include widespread cortical dysfunction on EEG, myoclonus, white matter abnormalities on neuroradiological examination and the presence of IgG anti-measles antibodies in the cerebrospinal fluid. We present the first case of SSPE with signs of peripheral nerve hyperexcitability, observed as extra discharges following the compound motor action potential at motor nerve stimulation. In addition we demonstrate the importance of SSPE in the differential diagnosis of adult patients with psychiatric and neurological symptoms.
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References
Dyken PR (2001) Neuroprogressive disease of post-infectious origin: a review of a resurging subacute sclerosing panencephalitis (SSPE). Ment Retard Dev Disabil Res Rev 7:217–225
Metz H, Gregoriou M, Sandifer P (1964) Subacute sclerosing pan-encephalitis: a review of 17 cases with special reference to clinical diagnostic criteria. Arch Dis Child 39:554–557
Garg RK (2002) Subacute sclerosing panencephalitis. Postgrad Med J 78:63–70
Punga AR, Flink R, Askmark H, Stålberg E (2006) Cholinergic neuromuscular hyperactivity in patients with myasthenia gravis seropositive for MuSK antibody. Muscle Nerve 34:111–115
Besser R, Gutmann L, Dillmann U et al (1989) End-plate dysfunction in acute organophosphate intoxication. Neurology 39:561–567
Oldstone MB, Lewicki H, Thomas D et al (1999) Measles virus infection in a transgenic model: virus-induced immunosuppression and central nervous system disease. Cell 98:629–640
Haltia M, Sourander P, Svennerholm L (1970) Chronic sclerosing panencephalitis. I. A histological and lipid-histochemical study. Acta Neuropathol (Berl) 14:284–292
Procopis PG, Gamstorp I (1972) Nerve conduction velocity in subacute sclerosing panencephalitis. Eur Neurol 7:335–338
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Schreurs, A., Stålberg, E.V. & Punga, A.R. Indication of peripheral nerve hyperexcitability in adult-onset subacute sclerosing panencephalitis (SSPE). Neurol Sci 29, 121–124 (2008). https://doi.org/10.1007/s10072-008-0872-y
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DOI: https://doi.org/10.1007/s10072-008-0872-y