Report of two narcoleptic patients with remission of hypersomnolence following use of prednisone

Coelho, Fernando Morgadinho Santos; Pradella-Hallinan, Márcia; Alves, Giovanni; Bittencourt, Lia; Tufik, Sérgio

doi:10.1590/s0004-282x2007000200028

Cited by 17 publications

(9 citation statements)

References 6 publications

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“…5 Furthermore, early diagnosis close to symptom onset may become critical as more is discovered about possible autoimmune etiologies of narcolepsy 19,20 and immunomodulating treatments that can alter disease trajectory. 21,22 Morrish et al conducted a crosssectional survey of 215 narcolepsy patients in the United Kingdom and reported that narcolepsy was diagnosed in 66% of patients within 5 y of symptom onset. 4 In multivariate regression modeling, the study authors found that absence of cataplexy and longer symptom duration were present, resulting in delayed diagnosis.…”

Section: Discussionmentioning

confidence: 99%

Listening to the Patient Voice in Narcolepsy: Diagnostic Delay, Disease Burden, and Treatment Efficacy

Maski

Steinhart

Williams

et al. 2017

Journal of Clinical Sleep Medicine

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Study Objectives: Describe common symptoms, comorbidities, functional limitations, and treatment responsiveness among patients with narcolepsy. Investigate the effect of pediatric onset of narcolepsy symptoms on time to diagnosis of narcolepsy and presence of comorbid depression. Methods: Cross-sectional survey of 1,699 people in the United States with self-reported diagnosis of narcolepsy. We utilized mixed-methods data analyses to report study findings. Results: Most participants reported receiving a diagnosis of narcolepsy more than 1 y after symptom onset. We found that the strongest predictor of this delayed diagnosis was pediatric onset of symptoms (odds ratio = 2.4, p < 0.0005). Depression was the most common comorbidity but we detected no association with pediatric onset of narcolepsy symptoms. Overall, participants reported that fatigue and cognitive difficulties were their most burdensome symptoms in addition to sleepiness and cataplexy. The majority of participants reported residual daytime fatigue and/or sleepiness despite treatment. Most participants reported they could not perform at work or school as well as they would like because of narcolepsy symptoms. Conclusions: This study provides unique insight into the narcolepsy disease experience. The study quantifies the problem of diagnostic delay for narcolepsy patients in the United States and highlights that symptoms are more likely to be missed if they develop before 18 y of age. These results suggest that narcolepsy awareness efforts should be aimed at parents, pediatric health care providers, school professionals, and children/adolescents themselves. Disease burden is high because of problems with fatigue, cognition, and persistence of residual symptoms despite treatment.

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Section: Discussionmentioning

confidence: 99%

Listening to the Patient Voice in Narcolepsy: Diagnostic Delay, Disease Burden, and Treatment Efficacy

Maski

Steinhart

Williams

et al. 2017

Journal of Clinical Sleep Medicine

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“…Plasmapheresis,80 corticosteroids,81,82 and intravenous immunoglobulin infusions83 have been used in case reports and small studies with mixed results (plasmapheresis did not improve narcolepsy; corticosteroids helped daytime somnolence in two cases, did not help in one case; intravenous immunoglobulin (IVIG) infusions helped cataplexy but not other symptoms in ¼ of the cases, but not the other symptoms). Plasmapheresis and steroids were utilized close to onset of symptoms, while IVIG was used within 6 months of diagnosis.…”

Section: Hypocretin-based Therapiesmentioning

confidence: 99%

New developments in the management of narcolepsy

Abad

Guilleminault

2017

NSS

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Narcolepsy is a life-long, underrecognized sleep disorder that affects 0.02%–0.18% of the US and Western European populations. Genetic predisposition is suspected because of narcolepsy’s strong association with HLA DQB1*06-02, and genome-wide association studies have identified polymorphisms in T-cell receptor loci. Narcolepsy pathophysiology is linked to loss of signaling by hypocretin-producing neurons; an autoimmune etiology possibly triggered by some environmental agent may precipitate hypocretin neuronal loss. Current treatment modalities alleviate the main symptoms of excessive daytime somnolence (EDS) and cataplexy and, to a lesser extent, reduce nocturnal sleep disruption, hypnagogic hallucinations, and sleep paralysis. Sodium oxybate (SXB), a sodium salt of γ hydroxybutyric acid, is a first-line agent for cataplexy and EDS and may help sleep disruption, hypnagogic hallucinations, and sleep paralysis. Various antidepressant medications including norepinephrine serotonin reuptake inhibitors, selective serotonin reuptake inhibitors, and tricyclic antidepressants are second-line agents for treating cataplexy. In addition to SXB, modafinil and armodafinil are first-line agents to treat EDS. Second-line agents for EDS are stimulants such as methylphenidate and extended-release amphetamines. Emerging therapies include non-hypocretin-based therapy, hypocretin-based treatments, and immunotherapy to prevent hypocretin neuronal death. Non-hypocretin-based novel treatments for narcolepsy include pitolisant (BF2.649, tiprolisant); JZP-110 (ADX-N05) for EDS in adults; JZP 13-005 for children; JZP-386, a deuterated sodium oxybate oral suspension; FT 218 an extended-release formulation of SXB; and JNJ-17216498, a new formulation of modafinil. Clinical trials are investigating efficacy and safety of SXB, modafinil, and armodafinil in children. γ-amino butyric acid (GABA) modulation with GABAA receptor agonists clarithromycin and flumazenil may help daytime somnolence. Other drugs investigated include GABAB agonists (baclofen), melanin-concentrating hormone antagonist, and thyrotropin-releasing hormone agonists. Hypocretin-based therapies include hypocretin peptide replacement administered either through an intracerebroventricular route or intranasal route. Hypocretin neuronal transplant and transforming stem cells into hypothalamic neurons are also discussed in this article. Immunotherapy to prevent hypocretin neuronal death is reviewed.

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“… 67 Similar negative results were reported in a 29-year-old woman who received intravenous methylprednisolone (IVMP) for a transverse myelitis of possible autoimmune etiology nine years after the onset of NT1. 68 Conversely, Coelho et al 69 described two men with a longstanding NT1 history, who reported the disappearance of EDS, and in one case also of cataplexy, upon prednisone treatment (40 mg/day) for other inflammatory conditions (inflammatory intestinal disease and asthma). However, these cases were not corroborated by CSF hypocretin-1 levels measurements nor the improvement was confirmed by repeated sleep studies after treatment and, in consideration of the long interval between disease onset and treatment, it is likely that the arousing effects of the steroids played a major role in the control of EDS.…”

Section: Immunotherapiesmentioning

confidence: 99%

“…0.6 m, SOREMs 5 ESS unchanged, MSLT: s.l. 0.0, SOREMs 4/4 N/A None [ 70 ] Case report 1 10, M 3 months after CPL onset IVIG 1g/kg/d over 2 days ESS N/A; CPL ≥1/d, hcrt-1<40, MSLT N/A ↓ EDS ↓ CPL hcrt-1 <40 MSLT N/A Reappearance of symptoms after steroid suspension Headache, fever, and flushing Followed by PO 1.3 mg/kg/d for 3 w and tapering N/A N/A Weight gain and acne with irritant dermatitis [ 69 ] Case series 1 22, M 9 years Inflammatory intestinal disease PO 40 mg/d ESS 15, CPL frequent s.l. 4 m SOREMS 2 Symptoms disappearance with withdrawal of methylphenidate, imipramine N/A None 2 42, M 9 years Asthma PO 40 mg/d for a fortnight ESS 18, MSLT: s.l.…”

Section: Immunotherapiesmentioning

confidence: 99%

“…However, the improvement of cataplexy that occurred during IVMP treatment suggests that steroids could exert an immunomodulating effect, explaining the improvement of cataplexy in these cases 71 and in one of the previously reported cases. 69 Nevertheless, corticosteroids have several effects on the central nervous system, 72 therefore, although unlikely, a direct anticataleptic activity, ie, through their action on noradrenergic and serotoninergic neurotransmission, cannot be excluded. 73 PLEX is a very well-established treatment for antibody-mediated disorders.…”

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confidence: 99%

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Reviewing the Clinical Implications of Treating Narcolepsy as an Autoimmune Disorder

Giannoccaro¹,

Liguori²,

Plazzi³

et al. 2021

NSS

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Narcolepsy type 1 (NT1) is a lifelong sleep disorder, primarily characterized clinically by excessive daytime sleepiness and cataplexy and pathologically by the loss of hypocretinergic neurons in the lateral hypothalamus. Despite being a rare disorder, the NT1related burden for patients and society is relevant due to the early onset and chronic nature of this condition. Although the etiology of narcolepsy is still unknown, mounting evidence supports a central role of autoimmunity. To date, no cure is available for this disorder and current treatment is symptomatic. Based on the hypothesis of the autoimmune etiology of this disease, immunotherapy could possibly represent a valid therapeutic option. However, contrasting and limited results have been provided so far. This review discusses the evidence supporting the use of immunotherapy in narcolepsy, the outcomes obtained so far, current issues and future directions.

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Report of two narcoleptic patients with remission of hypersomnolence following use of prednisone

Abstract: This article focuses on 2 clinical case reports of narcoleptic patients who experienced an absence of excessive sleepiness during treatment of other illnesses with 40 mg daily intake of prednisone.

Cited by 17 publications

References 6 publications

Listening to the Patient Voice in Narcolepsy: Diagnostic Delay, Disease Burden, and Treatment Efficacy

Listening to the Patient Voice in Narcolepsy: Diagnostic Delay, Disease Burden, and Treatment Efficacy

New developments in the management of narcolepsy

Reviewing the Clinical Implications of Treating Narcolepsy as an Autoimmune Disorder

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