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Conference Paper: Klein Levin Syndrome is a steroid-responsive, non-N-methyl-D-aspartate receptor-mediated encephalitis

TitleKlein Levin Syndrome is a steroid-responsive, non-N-methyl-D-aspartate receptor-mediated encephalitis
Other TitlesKlein Levin Syndrome is a steroid-responsive, non NMDA receptor-mediated encephalitis
Authors
KeywordsMedical sciences
Pediatrics medical sciences
Psychiatry and neurology
Issue Date2012
PublisherMac Keith Press. The Journal's web site is located at http://www.mackeith.co.uk/journal.html
Citation
The Joint 12th International Child Neurology Congress (ICNC 2012) and 11th Asian and Oceanian Congress of Child Neurology (AOCCN), Brisbane, Australia, 27 May-1 June 2012. In Developmental Medicine and Child Neurology, 2012, v. 54 n. suppl. s4, p. 143, abstract C3-0034 How to Cite?
AbstractOBJECTIVE: Klein Levin Syndrome is a rare disorder with periodic hypersomnia, cognitive and behavioural disturbance. It is postulated that it is triggered by a viral illness or post-infectious autoimmune encephalitis. With an increasing awareness of autoimmune encephalitis in the past 10years, especially N-methyl-D-aspartate receptor (NMDAR) mediated encephalitis, testing for the presence of NMDAR antibodies in patients with Klein Levin Syndrome should give us more understanding about the relationship between the two disease entities. DESIGN: CASE REPORT. A 15year-old boy with Klein Levin Syndrome presented with episodic attacks of repetitive excessive masturbation, hypersomnia, compulsive water drinking, and fluctuation in blood pressure. He had enjoyed good health in the past and was completely normal in between the attacks. The disease onset was related to a recent upper respiratory tract infection. Extensive work up was unrevealing, excluding microbiological, metabolic and autoimmune causes. Electroencephalogram showed nonspecific frontal intermittent rhythmic delta activities. Magnetic resonance imaging of the brain was normal. Serum and cerebrospinal fluid were sent for NMDAR antibodies during an episodic attack and were negative. Intravenous pulsed methylprednisolone (Pfizer, Puurs, Belgium) was given for 3days, followed by a course of oral prednisolone (APT Pharma, Hong Kong). The boy went into remission afterwards. However, the neuropsychiatric symptoms recurred upon tapering of the oral steroid. Currently, the condition is well controlled with lithium (Norgine, Harefield, UK). CONCLUSION: We support the hypothesis that Klein Levin Syndrome is an autoimmune encephalitis that is steroid-responsive. However the underlying mechanism is not NMDAR antibody mediated. Further study on a larger group of patients would help to elucidate the underlying aetiology.
DescriptionConcurrent Poster Sessions - Stream C: Neuroimmunology
This journal suppl. entitled: Special Issue: Abstracts of the 12th International Child Neurology Congress and the 11th Asian and Oceanian Congress of Child Neurology ... 2012
Persistent Identifierhttp://hdl.handle.net/10722/153122
ISSN
2015 Impact Factor: 3.615
2015 SCImago Journal Rankings: 1.636

 

DC FieldValueLanguage
dc.contributor.authorWong, Sen_US
dc.contributor.authorCheung, Pen_US
dc.contributor.authorWong, Ven_US
dc.contributor.authorChan, Ben_US
dc.date.accessioned2012-07-16T09:57:34Z-
dc.date.available2012-07-16T09:57:34Z-
dc.date.issued2012en_US
dc.identifier.citationThe Joint 12th International Child Neurology Congress (ICNC 2012) and 11th Asian and Oceanian Congress of Child Neurology (AOCCN), Brisbane, Australia, 27 May-1 June 2012. In Developmental Medicine and Child Neurology, 2012, v. 54 n. suppl. s4, p. 143, abstract C3-0034en_US
dc.identifier.issn0012-1622-
dc.identifier.urihttp://hdl.handle.net/10722/153122-
dc.descriptionConcurrent Poster Sessions - Stream C: Neuroimmunology-
dc.descriptionThis journal suppl. entitled: Special Issue: Abstracts of the 12th International Child Neurology Congress and the 11th Asian and Oceanian Congress of Child Neurology ... 2012-
dc.description.abstractOBJECTIVE: Klein Levin Syndrome is a rare disorder with periodic hypersomnia, cognitive and behavioural disturbance. It is postulated that it is triggered by a viral illness or post-infectious autoimmune encephalitis. With an increasing awareness of autoimmune encephalitis in the past 10years, especially N-methyl-D-aspartate receptor (NMDAR) mediated encephalitis, testing for the presence of NMDAR antibodies in patients with Klein Levin Syndrome should give us more understanding about the relationship between the two disease entities. DESIGN: CASE REPORT. A 15year-old boy with Klein Levin Syndrome presented with episodic attacks of repetitive excessive masturbation, hypersomnia, compulsive water drinking, and fluctuation in blood pressure. He had enjoyed good health in the past and was completely normal in between the attacks. The disease onset was related to a recent upper respiratory tract infection. Extensive work up was unrevealing, excluding microbiological, metabolic and autoimmune causes. Electroencephalogram showed nonspecific frontal intermittent rhythmic delta activities. Magnetic resonance imaging of the brain was normal. Serum and cerebrospinal fluid were sent for NMDAR antibodies during an episodic attack and were negative. Intravenous pulsed methylprednisolone (Pfizer, Puurs, Belgium) was given for 3days, followed by a course of oral prednisolone (APT Pharma, Hong Kong). The boy went into remission afterwards. However, the neuropsychiatric symptoms recurred upon tapering of the oral steroid. Currently, the condition is well controlled with lithium (Norgine, Harefield, UK). CONCLUSION: We support the hypothesis that Klein Levin Syndrome is an autoimmune encephalitis that is steroid-responsive. However the underlying mechanism is not NMDAR antibody mediated. Further study on a larger group of patients would help to elucidate the underlying aetiology.-
dc.languageengen_US
dc.publisherMac Keith Press. The Journal's web site is located at http://www.mackeith.co.uk/journal.html-
dc.relation.ispartofDevelopmental Medicine and Child Neurologyen_US
dc.subjectMedical sciences-
dc.subjectPediatrics medical sciences-
dc.subjectPsychiatry and neurology-
dc.titleKlein Levin Syndrome is a steroid-responsive, non-N-methyl-D-aspartate receptor-mediated encephalitisen_US
dc.title.alternativeKlein Levin Syndrome is a steroid-responsive, non NMDA receptor-mediated encephalitis-
dc.typeConference_Paperen_US
dc.identifier.emailWong, V: vcnwong@hku.hken_US
dc.identifier.authorityWong, V=rp00334en_US
dc.description.naturelink_to_OA_fulltext-
dc.identifier.doi10.1111/j.1469-8749.2012.04283.x-
dc.identifier.hkuros200483en_US
dc.identifier.hkuros200572-
dc.identifier.volume54-
dc.identifier.issuesuppl. s4-
dc.identifier.spage143, abstract C3-0034-
dc.identifier.epage143, abstract C3-0034-
dc.publisher.placeUnited Kingdom-
dc.customcontrol.immutablesml 150211-

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