sodium-oxybate and Atrophy

sodium-oxybate has been researched along with Atrophy* in 1 studies

Other Studies

1 other study(ies) available for sodium-oxybate and Atrophy

ArticleYear
Significant behavioral disturbances in succinic semialdehyde dehydrogenase (SSADH) deficiency (gamma-hydroxybutyric aciduria).
    Biological psychiatry, 2003, Oct-01, Volume: 54, Issue:7

    We report two adult patients with succinic semialdehyde dehydrogenase deficiency, manifesting as gamma-hydroxybutyric aciduria. For both, the clinical presentation included significant behavioral disturbances and psychosis (hallucinations, disabling anxiety, aggressive behavior, and sleep disorder), leading to multiple therapeutic attempts. Intervention with benzodiazepines appeared most efficacious, resulting in decreased aggression and agitation and improvement in anxiety. A review of 56 published and unpublished studies of SSADH-deficient patients revealed that 42% manifested behavioral disturbances, whereas 13% (predominantly adults) displayed psychotic symptomatology. To explore the potential biochemical basis of these behavioral abnormalities, we studied cerebrospinal fluid derived from 13 patients, which revealed significantly elevated GHB (65- to 230-fold), high free and total GABA (up to threefold), and low glutamine. Although within the control range, homovanillic and 5-hydroxyindoleacetic acids (end products of dopamine and serotonin metabolism, respectively) showed a significant linear correlation with increasing GHB concentration, suggesting enhanced dopamine and serotonin turnover. We conclude that elevated GABA combined with low glutamine suggest disruption of the glial-neuronal glutamine/GABA/glutamate shuttle necessary for replenishment of neuronal neurotransmitters, whereas altered dopamine and serotonin metabolism may be causally linked to the hyperkinetic movement disorders and behavioral disturbances seen in SSADH-deficient patients.

    Topics: Adult; Aldehyde Oxidoreductases; Atrophy; Cerebellum; Chromatography, High Pressure Liquid; Chromatography, Ion Exchange; Female; gamma-Aminobutyric Acid; Globus Pallidus; Glutamic Acid; Hallucinations; Homovanillic Acid; Humans; Hydroxyindoleacetic Acid; Intellectual Disability; Magnetic Resonance Imaging; Male; Metabolism, Inborn Errors; Sodium Oxybate; Statistics as Topic; Succinate-Semialdehyde Dehydrogenase

2003