P65 – 2865: Basal ganglia inflammation and a severe dystonic-akinetic syndrome after mycoplasma pneumonia infection in a 6 years old boy

European Journal of Paediatric Neurology(2015)

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摘要
Objective Severe CNS diseases like encephalitis, myelitis or radiculitis are described frequently associated with mycoplasma pneumonia (Mp) infections. There are some case reports of patients with post-infectious bilateral basal ganglia necrosis. In most cases, polymerase chain reaction (PCR) in CSF for Mp is negative. Standard treatments are mostly restricted to antibiotics and symptomatic therapies. Results A 6 years old boy was admitted to our hospital two weeks after a mild respiratory infection because of mutism and swallowing problems. He was alert, compliant and able to walk unaided. The movements were slowly and dystonic, deep tendon reflexes were augmented. Cerebral MRT revealed bilateral signal alteration of the striatum. Within the next days his state deteriorated. He lay in bed without spontaneous movements, with rigor on all extremities, unable to speak nor to swallow. Under the initial suspicion of a thiamine transporter defect, high doses of thiamine and biotine were given without visible impact within the following days. Mp serology showed up to be positive. So we decided to start an immune modulating therapy with corticoids and intravenously immunoglobulins (IVIG). The PCR for Mp in CSF was negative. For the next days, the neurological condition of the boy did not change. Although autoantibodies could not be found, an autoimmune disease was postulated and plasmaexchange (PE) was performed six times followed by four rituximab administrations. The day after the last PE the boy started to move and within a week, he was able to speek and walk unaided again. Conclusion Because of the prompt clinical recovery after PE we postulate an autoimmune aetiology of this postinfectious neurological disease after mycoplasma pneumonia infection although we could not find autoantibodies. Immunmodulatory therapy with corticosteroids and IVIG was not enough to alter the clinical state, only serial plasma exchanges led to fast improvements of the neurological condition.
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