Mania as a presenting clinical feature of subacute sclerosing panencephalitis

Autor: Mine Hayriye Sorgun, Rezzak Yilmaz, Nursel Aydin, Seyda Erdogan
Rok vydání: 2015
Předmět:
Zdroj: Acta Neurologica Belgica. 115:803-805
ISSN: 2240-2993
0300-9009
Popis: Subacute sclerosing panencephalitis (SSPE) is a rare,slowly progressive disease resulting in severe neurologicaldeficit and death. Presentation with pure psychiatricsymptoms has been rarely reported in the literature [1–3].We describe a SSPE case of 20-year-old male who pre-sented with manic episode. His mother brought him to ourhospital with abnormal repetitive movements in his handsand legs and irritability. As his mother reported, he had beena successful student until he wanted to drop out of school2 years ago. He became more irritable and angry after then.First he began to go to gym but after only a week he got a jobinarestaurant.Heworkedforlonghoursandsleptverylittle.He spent his money intemperately and bought himself newclotheseveryday.Heboughtredshoeseventhoughheneverliked this color before. He was full of energy and never felttired. Then, he quitted his job and did not come home fordays. One day, his family learned that he was in prison be-cause of larceny. His mother took him to a psychiatrist and adiagnosis of manic episode was made; treatment with olan-zapine was started. As she reported, he became a differentperson in the next 6 months. He had urinary incontinence,abnormal involuntary movements and miscalculations. Hismedical history was unremarkable with regard to diseasesand substance abuse. His family history was uninformative.In his neurological examination, he was not oriented to timeand place, and impairment was detected in all cognitivefunctions. He also had generalized myoclonia and dystonia.His blood count, renal and liver function tests, erythrocytesedimentation rate, serum levels of vitamin B12 and ceru-loplasmin were within normal limits. Antibody against glu-tamic acid decarboxylase was negative. Magnetic resonanceimaging (MRI) of the brain revealed diffuse atrophy. Elec-troencephalogram showed generalized delta waves withhigh amplitude and a periodicity at every 5 s (Fig. 1). Hiscerebrospinal fluid (CSF) was acellular, with normal pres-sure, protein and sugar levels but with elevated anti-measlesIgG antibodies. Measles IgG index, which is a specificmarker of antibody production in CSF, was 2.25 (IgG index[1.5 is associated with antibody production in CSF). A di-agnosis ofSSPE wasmade.He wastreated with isoprinosineat a dose of 100 mg/kg/d and carbamazepine at a dose of400 mg/d. Generalized myoclonia showed well response tothe treatment but generalized dystonia stayed stable. After1 year, his MRI of the brain was performed again. Hyper-intense lesions were revealed in the splenium of corpuscallosum on T2 and fluid-attenuated inversion recovery(FLAIR) sequences (Fig. 2). On follow up, he died in thesecond year of his admission to our hospital.Subacute sclerosing panencephalitis is a progressivedisease of the central nervous system caused by defectivemeasles virus. The disease begins insidiously. The firstsymptoms are usually mild intellectual deterioration andbehavioral changes with decline in school success. As thedisease advances, myoclonic jerks, ataxia, partial andgeneralized seizures, dystonia or dyskinesia and dementiamay occur eventually leading to a decorticated state anddeath [1–4].
Databáze: OpenAIRE
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