The Treatment of Narcolepsy and Cataplexy

1981 ◽  
Vol 15 (4) ◽  
pp. 257-262 ◽  
Author(s):  
R. Keith Campbell

Narcolepsy may affect as many as 250 000 Americans. It involves a neurologic defect in the regulation of sleep and wakefulness. The chief symptoms are sleepiness, inappropriate sleep episodes, and cataplexy. Narcoleptic patients also frequently complain of hypnagogic hallucinations, sleep paralysis, and automatic behavior, as well as disturbed nocturnal sleep. Narcolepsy usually develops in adolescence and is a life-long illness. Satisfactory treatment is not available at the present time. The current treatments of choice include general measures (such as patient and family counseling and frequent napping) and drug treatments, including methylphenidate for sleepiness and sleep episodes and imipramine for cataplexy. Medication dosages must be adjusted for individual patients. Pharmacists have a special responsibility to monitor narcoleptic patients by becoming familiar with the disease and its symptoms and by encouraging patients to follow their drug regimens and to take drug holidays under the supervision of the physician when the drug dose becomes excessive.

CNS Spectrums ◽  
2003 ◽  
Vol 8 (2) ◽  
pp. 120-126 ◽  
Author(s):  
Alan B. Douglass

AbstractDoes narcolepsy, a neurological disease, need to be considered when diagnosing major mental illness? Clinicians have reported cases of narcolepsy with prominent hypnagogic hallucinations that were mistakenly diagnosed as schizophrenia. In some bipolar disorder patients with narcolepsy, the HH resulted in their receiving a more severe diagnosis (ie, bipolar disorder with psychotic features or schizoaffective disorder). The role of narcolepsy in psychiatric patients has remained obscure and problematic, and it may be more prevalent than commonly believed. Classical narcolepsy patients display the clinical “tetrad”—cataplexy, hypnagogic hallucinations, daytime sleep attacks, and sleep paralysis. Over 85% also display the human leukocyte antigen marker DQB10602 (subset of DQ6). Since 1998, discoveries in neuroanatomy and neurophysiology have greatly advanced the understanding of narcolepsy, which involves a nearly total loss of the recently discovered orexin/hypocretin (hypocretin) neurons of the hypothalamus, likely by an autoimmune mechanism. Hypocretin neurons normally supply excitatory signals to brainstem nuclei producing norepinephrine, serotonin, histamine, and dopamine, with resultant suppression of sleep. They also project to basal forebrain areas and cortex. A literature review regarding the differential diagnosis of narcolepsy, affective disorder, and schizophrenia is presented. Furthermore, it is now possible to rule out classical narcolepsy in difficult psychiatric cases. Surprisingly, psychotic patients with narcolepsy will likely require stimulants to fully recover. Many conventional antipsychotic drugs would worsen their symptoms and make them appear to become a “chronic psychotic,” while in fact they can now be properly diagnosed and treated.


Narcolepsy ◽  
2008 ◽  
pp. 87-97 ◽  
Author(s):  
Armando D’Agostino ◽  
Ivan Limosani

Author(s):  
Gert Jan Lammers

Narcolepsy with cataplexy is caused by disturbed cerebral hypocretin (also called orexin) transmission. It results in impaired physiological boundaries of wake and sleep stages and their specific components, leading to clinical symptoms such as excessive daytime sleepiness (EDS), impaired sustained attention, disturbed nocturnal sleep, cataplexy, and hypnagogic hallucinations. This chapter discusses the consequences for daily life of the disorder, the diagnostic challenges, particularly the interpretation of the results of the multiple sleep latency test (MSLT), the presumed cause and pathophysiology, the frequent comorbidities such as obesity, and practical guidelines for optimal nonpharmacological as well as pharmacological treatment.


1971 ◽  
Vol 16 (4) ◽  
pp. 283-293 ◽  
Author(s):  
Roger Broughton

This brief review documents some of the important contributions of recent sleep research to the understanding of a number of neurological conditions. Narcoleptic attacks have been shown to be either episodes of NREM or REM sleep; and cataplexy, sleep paralysis and vivid hypnagogic hallucinations consist of dissociated or inappropriate REM sleep. Important relationships of the hypersomnias and various comas to sleep mechanisms are being increasingly elucidated. Various types of epileptic seizures have been found to be affected differentially by the two types of sleep and by arousal from them; and sleep deprivation may activate or perpetuate epilepsy. Finally, some miscellaneous conditions, such as dyskinesias, cerebrovascular accidents, migraine, and memory and repair functions have been considered. As well as being of pathophysiological interest, much of this new knowledge has a direct diagnostic and therapeutic relevance.


2018 ◽  
Vol 19 (3) ◽  
pp. 174-184
Author(s):  
Paulina Wróbel-Knybel ◽  
Michał Flis ◽  
Rafał Dubiel ◽  
Hanna Karakuła-Juchnowicz

Summary Introduction: Sleep paralysis (SP) is a condition that widely occurs among people all over the world. It has been known for thousands of years and is rooted in the culture of many countries. It arouses strong emotions, though still little is known about it. The clinical picture of the disorder can be very diverse. It is often accompanied by hypnopompic and hypnagogic hallucinations, somatic complaints and the feeling of intense anxiety. A feeling of paralysis in the body with inhibited consciousness is always observed with the experience. SP pathophysiology is not fully understood, however, most theories explaining this phenomenon are based on the assumption that it results from dysfunctional overlap of REM sleep and wakefulness. It is experienced by healthy people, but it is more often associated with somatic and mental disorders, which is why it is becoming an object of interest for researchers. Aim: The aim of this work is to present the most important information about the disorder known as sleep paralysis - its history, cultural context, pathophysiology, prevalence, symptomatology, coexistence with other somatic and mental disorders as well as diagnostics and available forms of prevention and treatment. Materials and methodology: The available literature was reviewed using the Google Scholar bibliographic databases searching the following keywords: sleep paralysis, REM sleep parasomnias, sleep disorder, night terrors and time descriptors: 1980-2018. Results 1. Sleep paralysis has already been described in antiquity, and interpretations related to its occurrence are largely dependent on culture and beliefs. 2. Symptomatology of the disorder is very diverse: both mental and somatic symptoms are present. 3. The pathophysiology of the disorder has not been fully explained. The basis of most theories regarding sleep paralysis is the assumption that it results from the dysfunctional overlap of REM sleep and wakefulness. 4. The prevalence of SP at least once in a lifetime is 7.6% in the general population, although it is estimated that it is much more frequent in people with various mental and somatic disorders. 5. Treatment of SP is associated with a change in lifestyle and the use of pharmacotherapy and psychotherapy.


SLEEP ◽  
2019 ◽  
Vol 43 (3) ◽  
Author(s):  
Hilde T Juvodden ◽  
Marte K Viken ◽  
Sebjørg E H Nordstrand ◽  
Rannveig Viste ◽  
Lars T Westlye ◽  
...  

Abstract Study Objectives To explore HLA (human leukocyte antigen) in post-H1N1 narcolepsy type 1 patients (NT1), first-degree relatives and healthy controls, and assess HLA associations with clinical and sleep parameters in patients and first-degree relatives. Methods Ninety post-H1N1 NT1 patients and 202 of their first-degree relatives were HLA-genotyped (next generation sequencing) and phenotyped (semistructured interviews, Stanford Sleep Questionnaire, polysomnography, and multiple sleep latency test). HLA allele distributions were compared between DQB1*06:02-heterozygous individuals (77 patients, 59 parents, 1230 controls). A subsample (74 patients, 114 relatives) was investigated for associations between HLA-loci and continuous sleep variables using logistic regression. Identified candidate HLA-loci were explored for HLA allele associations with hypnagogic hallucinations and sleep paralysis in 90 patients, and patient allele findings were checked for similar associations in 202 relatives. Results DQB1*06:02 heterozygous post-H1N1 NT1 patients (84.4% H1N1-vaccinated) showed several significant HLA associations similar to those reported previously in samples of mainly sporadic NT1, i.e. DQB1*03:01, DRB1*04:01, DRB1*04:02, DRB1*04:07, DRB1*11:04, A*25:01, B*35:03, and B*51:01, and novel associations, i.e. B*14:02, C*01:02, and C*07:01. Parents HLA alleles did not deviate significantly from controls. The HLA-C locus was associated with sleep parameters in patients and relatives. In patients C*02:02 seems to be associated with protective effects against sleep paralysis and hypnagogic hallucinations. Conclusions Our findings of similar risk/protective HLA-alleles in post-H1N1 as in previous studies of mainly sporadic narcolepsy support similar disease mechanisms. We also report novel allelic associations. Associations between HLA-C and sleep parameters were seen independent of NT1 diagnosis, supporting involvement of HLA-C in sleep subphenotypes.


2020 ◽  
pp. 088307382096865 ◽  
Author(s):  
Indar Kumar Sharawat ◽  
Prateek Kumar Panda

Background: The ongoing COVID-19 pandemic and the lockdown measures have forced clinicians across the world to look on telemedicine. Although migraine as such seems an ideal option for telemedicine, a systematic study reviewing feasibility, efficacy, and advantages of current advanced telecommunication technologies in children with migraine is lacking. Methods: Clinical details of telephonic consultations performed between March 25 and June 4, 2020, including migraine characteristics, analgesic/prophylactic drug regimens, headache frequency/severity/disability, compliance, drug-related/unrelated adverse effects and unavailability of drugs were recorded. Suitable change in the dose/commercial brand of drug regimen and the addition of new drugs were done as required. Voice call, text messages, picture/video messages, and all other possible measures were used to accumulate maximum clinical information in real time. Unless contacted for trivial reasons, most children were later followed up telephonically to ensure health care concern was resolved and to fill up one 6-item trichotomous quality control questionnaire. Results: A total of 146 telephone consultations were performed in 51 participants. Thirty-six patients were identified to have 42 significant clinical events (worsening clinical status/partial response to medications [18; 44%], drug-related adverse effects [10; 19%], unrelated systemic complaints [7; 13%], and worsening of headache due to stress/triggers [7; 13%]). In the rest of the patients, the query of the caregiver included concern regarding COVID-19–related symptoms. Twenty-nine children required increment in drug dose or addition of new drug or commercial brand. Around 90% of caregivers were satisfied with the quality of teleconsultation. Conclusion: Teleconsultation is a feasible and efficacious option, with excellent caregiver satisfaction for children with migraine.


Author(s):  
Joaquín M. Campos ◽  
Claudia Molina

Background: Narcolepsy, also known as Gélineau syndrome, is a chronic and neurological disease that affects 0.05% of the European population, though that percentage could be higher due to the diagnostic difficulties. The main symptom is excessive daytime sleepiness, although it may be accompanied by cataplexy, sleep paralysis and hypnagogic hallucinations. Objective: Nowadays, there is no cure for narcolepsy and the treatment is symptomatic: psychostimulants for the sleepiness by means of amphetamines, methylphenidate or modafinil, and antidepressants and sodium oxybate for treating cataplexy. Method: This is a short review regarding pharmacotherapy for narcolepsy. Result: Hypocretins were discovered in 1998. They are neuropeptides whose deficit is responsible for this symptomatology, has opened up a new field of investigation. Conclusion: Agonists of hypocretins could be a promising therapy against this disease.


Author(s):  
Roger Broughton ◽  
Quais Ghanem ◽  
Yasuo Hishikawa ◽  
Yoshiro Sugita ◽  
Sonia Nevsimalova ◽  
...  

SUMMARY:A questionnaire survey has been made of the life effects of narcolepsy in 180 patients, 60 each from North American, Asian and European populations, with 180 similarly distributed age and sex matched controls. Life-effects were attributed by the patients to the primary symptoms of excessive daytime drowsiness, sleep attacks, cataplexy, vivid hypnagogic hallucinations and sleep paralysis, and also to other frequent symptoms such as visual problems (blurring, diplopia) and memory impairment. Occupational problems were prevalent (over 75%) and included statistically significant deleterious effects upon performance, promotion, earning capacity, fear of or actual job loss and increased disability insurance. Driving was greatly affected and patients fell asleep at the wheel more frequently (66%), had near or actual accidents from drowsiness or falling asleep at the wheel (67%), and could experience cataplexy (29%) or sleep paralysis (12%) while driving. Work or home accidents attributed to sleepiness or sleep (49%) or related to smoking (49%) were much more common in patients. There were also deleterious effects on education, recreation and personality related to the disease. Narcolepsy can produce a variety of life-effects probably more serious and pervasive than, for instance, those of epilepsy, therefore emphasizing the importance of early diagnosis and treatment.


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