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Contemporary Encephalitis Lethargica: Phenotype, laboratory findings and treatment outcomes

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Abstract

Background

Encephalitis lethargica (EL) is a CNS disorder that manifests with lethargy sleep cycle disturbances, extrapyramidal symptomatology, neuropsychiatric manifestations, ocular features and cardio-respiratory abnormalities. Although there have been no reported outbreaks of EL recently, a number of reports show that cases of EL are still encountered regularly. Against this background we conducted a study aiming to elucidate the clinical characteristics, describe laboratory/ neuroimaging findings (MRI, PET) and present treatment options and outcomes in sporadic EL.

Methods

Patients were diagnosed over a period of 3 years using proposed diagnostic criteria. Extensive laboratory and imaging tests were performed for exclusion of other causes. Anti-neuronal antibodies against human basal ganglia were detected with western immunoblotting and 18F-FDG PET imaging was performed. Selected cases were videotaped.

Results

Our patients (M/F: 5/3) ranged from 2–28 years (mean 9.3 ± 9.5). Encephalopathy, sleep disturbances and extrapyramidal symptoms were present in all cases. Laboratory investigations revealed CSF leukocytosis in 5/8 patients and anti-BG Ab in 4/7 patients. MRIs revealed structural abnormalities in 7/8 cases. 18F-FDG PET showed basal ganglionic hypermetabolism in 4/7 patients. Treatment approaches included immunomodulating and symptomatic therapies. We report no mortality from EL in our series.

Conclusions

There seems to be little doubt that cases of EL still occur. Diagnosis may be based on clinical suspicion and laboratory/imaging tests may lead to early initiation of immunomodulating and supporting therapies. We suggest that in addition to anti-BG Abs FDG PET should be considered as a diagnostic tool for EL.

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Authors and Affiliations

  1. Dept. of Pediatrics, University of Miami, Miller School of Medicine, Miami, USA

    Robert Lopez-Alberola MD

  2. Dept. of Radiology, Division of Nuclear Medicine, University of Miami, Miller School of Medicine, Miami, USA

    Michael Georgiou MD & George N. Sfakianakis MD

  3. Division of Movement Disorders, Dept. of Neurology, University of Miami, Miller School of Medicine, 1501 NW 9th Avenue (NPF), Room 2017, Miami, FL, 33136, USA

    Robert Lopez-Alberola MD, Carlos Singer MD & Spyridon Papapetropoulos MD, PhD

Authors
  1. Robert Lopez-Alberola MD
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  2. Michael Georgiou MD
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  3. George N. Sfakianakis MD
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  4. Carlos Singer MD
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  5. Spyridon Papapetropoulos MD, PhD
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Correspondence to Spyridon Papapetropoulos MD, PhD.

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Lopez-Alberola, R., Georgiou, M., Sfakianakis, G.N. et al. Contemporary Encephalitis Lethargica: Phenotype, laboratory findings and treatment outcomes. J Neurol 256, 396–404 (2009). https://doi.org/10.1007/s00415-009-0074-4

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  • DOI: https://doi.org/10.1007/s00415-009-0074-4

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