Contemporary Encephalitis Lethargica: phenotype, laboratory findings and treatment outcomes.
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 (18)F-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. (18)F-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.
Dept. of Pediatrics, University of Miami, Miller School of Medicine, Miami 33136, USA.
Robert
R
Lopez-Alberola
Michael
M
Georgiou
George N
GN
Sfakianakis
Carlos
C
Singer
Spyridon
S
Papapetropoulos
D000293:Adolescent; D000328:Adult; D000906:Antibodies / Q000134:cerebrospinal fluid; D001479:Basal Ganglia / Q000276:immunology / Q000473:pathology / Q000503:physiopathology*; D015153:Blotting, Western; D002648:Child; D002675:Child, Preschool; D003937:Diagnosis, Differential; D005260:Female; D019788:Fluorodeoxyglucose F18; D006801:Humans; D007155:Immunologic Factors / Q000627:therapeutic use; D007964:Leukocytosis / Q000134:cerebrospinal fluid; D008279:Magnetic Resonance Imaging; D008297:Male; D010301:Parkinson Disease, Postencephalitic / Q000175:diagnosis* / Q000503:physiopathology* / Q000628:therapy; D049268:Positron-Emission Tomography; D013788:Thalamus / Q000473:pathology; D016896:Treatment Outcome; D055815:Young Adult
D000906:Antibodies; D007155:Immunologic Factors; D019788:Fluorodeoxyglucose F18