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      A Case of Off-label Sodium Oxybate in an 11-Year-Old with Narcolepsy

      case-report
      Author(s): 1 , , 1
      Editor(s): ,
      Publication date (Electronic, pub):
      Publication date (Electronic, collection):
      Journal: Cureus
      Publisher: Cureus
      Keywords: case report, narcolepsy, pediatric, off label, sodium oxybate, hypocretin, sleep disorder, excessive sleepiness

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          Abstract

          Narcolepsy is a rare chronic condition which affects sleep architecture. It may manifest in childhood or adolescence by causing excessive daytime sleepiness, sleep attacks, and hallucinations. Research suggests that there is a significant delay in diagnosis with the mean age being 10 to 15 years from onset of symptoms. Although narcolepsy is predominantly associated with loss of hypocretin (orexin), the role of genetics is complementary to the diagnosis. In addition, clinical manifestations of symptoms vary in presentation and severity from case to case. Therefore, this report provides an opportunity to review pediatric narcolepsy including the diagnostic workup and clinical response to sodium oxybate. This particular case describes an 11-year-old boy meeting the clinical and diagnostic criteria for narcolepsy. Clinically, the patient had a very positive response to treatment with sodium oxybate, which at the time of initiating therapy was off-label for patients under the age of 18.

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          Most cited references17

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          The hypocretins: hypothalamus-specific peptides with neuroexcitatory activity.

          L de Lecea, T S Kilduff, C Peyron … (1998)
          We describe a hypothalamus-specific mRNA that encodes preprohypocretin, the putative precursor of a pair of peptides that share substantial amino acid identities with the gut hormone secretin. The hypocretin (Hcrt) protein products are restricted to neuronal cell bodies of the dorsal and lateral hypothalamic areas. The fibers of these neurons are widespread throughout the posterior hypothalamus and project to multiple targets in other areas, including brainstem and thalamus. Hcrt immunoreactivity is associated with large granular vesicles at synapses. One of the Hcrt peptides was excitatory when applied to cultured, synaptically coupled hypothalamic neurons, but not hippocampal neurons. These observations suggest that the hypocretins function within the CNS as neurotransmitters.
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            Delayed diagnosis of narcolepsy: characterization and impact.

            Michael J. Thorpy, Ana Krieger (2014)
            Narcolepsy, a chronic neurologic condition resulting from dysregulation of the sleep-wake cycle, usually has an onset at an early age. However, a long delay until diagnosis has been consistently reported in the literature across countries and several publications have focused on characterizing this delay. Most studies report a mean delay to diagnosis of up to 15 years, with individual cases of >60 years, although a trend over time toward a shorter diagnostic delay has been suggested. While variables associated with this delay have been identified, a lack of symptom recognition resulting in misdiagnosis prior to reaching the narcolepsy diagnosis is the likely underlying reason. This lack of symptom recognition is especially relevant considering the high comorbidity burden that has been shown in patients with narcolepsy as some disorders manifest with symptoms that overlap with narcolepsy. A consequence of delayed diagnosis is delayed treatment, which affects the burden of disease. Substantial detrimental effects on health-care resource utilization, employment, and quality of life have been described after narcolepsy onset and prior to the diagnosis of narcolepsy. This review highlights the importance of closing the diagnostic gap by expanding awareness of narcolepsy and its symptoms.
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              Opiates increase the number of hypocretin-producing cells in human and mouse brain and reverse cataplexy in a mouse model of narcolepsy

              Dick F. Swaab, Jerome M Siegel, Paul F. Worley … (2018)
              The changes in brain function that perpetuate opiate addiction are unclear. In our studies of human narcolepsy, a disease caused by loss of immunohistochemically detected hypocretin (orexin) neurons, we encountered a control brain (from an apparently neurologically normal individual) with 50% more hypocretin neurons than other control human brains that we had studied. We discovered that this individual was a heroin addict. Studying five postmortem brains from heroin addicts, we report that the brain tissue had, on average, 54% more immunohistochemically detected neurons producing hypocretin than did control brains from neurologically normal subjects. Similar increases in hypocretin-producing cells could be induced in wild-type mice by long-term (but not short-term) administration of morphine. The increased number of detected hypocretin neurons was not due to neurogenesis and outlasted morphine administration by several weeks. The number of neurons containing melanin-concentrating hormone, which are in the same hypothalamic region as hypocretin-producing cells, did not change in response to morphine administration. Morphine administration restored the population of detected hypocretin cells to normal numbers in transgenic mice in which these neurons had been partially depleted. Morphine administration also decreased cataplexy in mice made narcoleptic by the depletion of hypocretin neurons. These findings suggest that opiate agonists may have a role in the treatment of narcolepsy, a disorder caused by hypocretin neuron loss, and that increased numbers of hypocretin-producing cells may play a role in maintaining opiate addiction.
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                Author and article information

                Journal
                Journal ID (nlm-ta): Cureus
                Journal ID (iso-abbrev): Cureus
                Journal ID (issn): 2168-8184
                Title: Cureus
                Publisher: Cureus (Palo Alto (CA) )
                ISSN (Electronic): 2168-8184
                Publication date (Electronic, pub): 12 February 2019
                Publication date (Electronic, collection): February 2019
                Volume: 11
                Issue: 2
                Electronic Location Identifier: e4057
                Affiliations
                [1 ] Internal Medicine, Jersey Shore University Medical Center, Neptune City, USA
                Author notes
                Jeffrey A. Miskoff jamiskoff@ 123456yahoo.com
                Article
                DOI: 10.7759/cureus.4057
                PMC ID: 6464478
                SO-VID: e02721eb-24ef-4fe7-aa6a-ff97c47225ab
                Copyright © Copyright © 2019, Miskoff et al.
                License:

                This is an open access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited.

                History
                Date received : 24 January 2019
                Date accepted : 12 February 2019
                Categories
                Subject: Internal Medicine
                Subject: Neurology
                Subject: Pediatrics

                Keywords: case report,narcolepsy,pediatric,off label,sodium oxybate,hypocretin,sleep disorder,excessive sleepiness
                Data availability:
                Keywords: case report, narcolepsy, pediatric, off label, sodium oxybate, hypocretin, sleep disorder, excessive sleepiness

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