Neuropharmacology of progressive myoclonus epilepsy: response to 5-hydroxy-L-tryptophan

Epilepsia
M R PranzatelliD Franz

Abstract

Low concentrations of the serotonin metabolite 5-hydroxyindoleacetic acid (5-HIAA) in cerebrospinal fluid (CSF) of patients with progressive myoclonus epilepsy (PME) suggest hypofunctional serotonergic neurotransmission. To study this hypothesis, we enrolled 6 patients with PME [Unverricht-Lündborg disease (U-L), mitochondrial encephalomyopathy, or Lafora disease] in a controlled, double-blinded, dose-ranging, cross-over add-on pilot clinical trial of 5-hydroxy-L-tryptophan (L-5-HTP) plus carbidopa after 2 other patients had received open-label L-5-HTP for compassionate use. Prestudy CSF 5-HIAA concentrations were low (< 20 ng/ml) in 6 patients regardless of the etiology of PME. One patient with U-L disease showed clinical improvement and a fivefold increase in CSF 5-HIAA, and 1 with Lafora disease showed a twofold increase in CSF 5-HIAA without improvement. A patient with Lafora disease reported enough improvement in myoclonus-evoked convulsions to continue chronic use of the drug. One patient with mitochondrial encephalomyopathy developed status epilepticus during treatment with L-5-HTP. As a group, patients had no statistically significant changes in myoclonus evaluation scale scores, subjective and objective measures of ata...Continue Reading

References

Mar 1, 1975·Lancet·D ChadwickE H Reynolds
Jun 12, 1978·Annals of the New York Academy of Sciences·R A BrowningR L Simonton
Jan 13, 1977·The New England Journal of Medicine·M H Van WoertM B Bowers
Nov 23, 1979·European Journal of Pharmacology·E Chung Hwang, M H Van Woert
Oct 14, 1992·JAMA : the Journal of the American Medical Association·S L Nightingale
May 1, 1990·Australian and New Zealand Journal of Ophthalmology·D McKayF Taylor
Jan 1, 1985·Epilepsia·M Hallett
Oct 27, 1972·Brain Research·L K NgI J Kopin
Apr 1, 1973·Journal of the Neurological Sciences·C GuilleminaultD Cousin
Jun 1, 1980·Neurology·J H Wood
Sep 1, 1981·Acta Pharmacologica Et Toxicologica·I MagnussenM H Van Woert
Jan 1, 1982·European Journal of Clinical Pharmacology·I Magnussen, M H Van Woert
Dec 1, 1982·Psychiatry Research·H G WestenbergH M van Praag
May 1, 1981·Neurology·J J KellyJ R Daube
Oct 2, 1980·The New England Journal of Medicine·E M SternbergC K Osterland
Jan 1, 1993·Journal of Neurology, Neurosurgery, and Psychiatry·M R PranzatelliJ M Martens
Apr 1, 1993·Movement Disorders : Official Journal of the Movement Disorder Society·F Sellal, E Hirsch
Feb 1, 1993·Annals of Neurology·S G KalerW A Gahl
Apr 1, 1993·Movement Disorders : Official Journal of the Movement Disorder Society·E Tate, M R Pranzatelli
Jan 1, 1993·Epilepsia·S F BerkovicF Andermann

❮ Previous
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Citations

Nov 30, 2000·Current Treatment Options in Neurology·S Frucht
May 1, 1996·Clinical Neurology and Neurosurgery·M R PranzatelliA Wheeler
Nov 5, 1997·Biological Psychiatry·J R StevensP Szot
Dec 18, 2001·Pediatric Neurology·M R Pranzatelli, E D Tate
Dec 1, 1996·Epilepsy Research·J R StevensP Szot
Sep 29, 2011·Child Neuropsychology : a Journal on Normal and Abnormal Development in Childhood and Adolescence·Carmen SoriaGeorges Dellatolas
Jun 10, 2010·CNS Drugs·Thomas S Monaghan, Norman Delanty
Dec 6, 2001·Current Treatment Options in Neurology·Basim M. Uthman, Andreas Reichl
Jun 29, 2013·Child and Adolescent Psychiatric Clinics of North America·Charles W Popper
Jan 11, 2007·Journal of Neurochemistry·Gyorgy BagdyRita Jakus
Jun 6, 2003·Seminars in Pediatric Neurology·Michael R Pranzatelli
Oct 11, 2008·Epilepsy Research·Euclides Maurício Trindade-FilhoEsper Abrão Cavalheiro
Jan 2, 2013·Clinical Neurophysiology : Official Journal of the International Federation of Clinical Neurophysiology·E VisaniS Franceschetti
Oct 28, 1997·American Journal of Medical Genetics·I N BespalovaM Burmeister
Sep 1, 1996·Journal of Child Neurology·M R Pranzatelli
May 8, 2019·Expert Opinion on Pharmacotherapy·Josef Finsterer
Jun 30, 2018·Current Neuropharmacology·Faheem Hyder PottooMohammad Azam Ansari
Dec 24, 2019·Experimental Neurology·Alexandra N PetrucciGordon F Buchanan

❮ Previous
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