Case Report of a Patient With Idiopathic Hypersomnia and a Family History of Malignant Hyperthermia Undergoing General Anesthesia

Abstract

The pathophysiologic underpinnings of idiopathic hypersomnia and its interactions with anesthetic medications remain poorly understood. There is a scarcity of literature describing this patient population in the surgical setting. This case report outlines the anesthetic considerations and management plan for a 55-year-old female patient with a known history of idiopathic hypersomnia undergoing an elective shoulder arthroscopy in the ambulatory setting. In addition, this case offers a unique set of considerations and conflicts related to the patient having a family history of malignant hyperthermia. A combined technique of general and regional anesthesia was used. Anesthesia was maintained with total intravenous anesthesia via the use of propofol and remifentanil. The depth of anesthesia was monitored with entropy. There were no perioperative complications.

Perioperative Considerations in a Patient with Kleine–Levin Syndrome Undergoing a Neurosurgical Procedure under General Anesthesia

“…reduce in frequency and severity by middle age. [1][2][3] During an episode, the patient could exhibit excessive sleep, hallucinations, delusions, hyperphagia, derealization (a striking feeling of unreality), irritability, and hypersexuality 3,4 (►Table 3). Our patient's overnight polysomnography report was normal with no sleep-related breathing disorders.…”

“…10 Specific postoperative considerations related to KLS include (1) close monitoring in the PACU for symptom exacerbations, (2) limiting the use of sedative medications when possible, (3) optimizing multimodal analgesia, and (4) counseling the patient regarding postanesthetic care prior to discharge. 3…”

“…It is associated with cognitive impairment, derealization, apathy, and behavioral changes. It occurs in adolescents, with a predominance in males, and is associated a history of birth or developmental problems 3 ( Fig. 1 ).…”

“…Episodes might be triggered by infection, alcohol intake, or sleep deprivation, and usually reduce in frequency and severity by middle age. 1 2 3 During an episode, the patient could exhibit excessive sleep, hallucinations, delusions, hyperphagia, derealization (a striking feeling of unreality), irritability, and hypersexuality 3 4 ( Table 3 ).…”

Central Disorders of Hypersomnolence, Restless Legs Syndrome, and Surgery With General Anesthesia: Patient Perceptions

“…Kleine-Levin syndrome, also a central disorder of hypersomnolence, refers to a syndrome consisting of bouts of recurrent hypersomnia separated by periods of relative normalcy. Potential concerns for the perioperative management of patients with central disorders of hypersomnolence raised by case reports and small case series include: (1) the appearance of peri-operative cataplexy, sleepiness, or other disease-specific symptoms; (2) the chronic use of psychotropic and/or vasoactive medications, including amphetamines, in patients with these disorders; and (3) delayed emergence from anesthesia (Mesa et al, 2000 ; Peláez et al, 2004 ; Burrow et al, 2005 ; Fischer et al, 2006 ; Ozkose et al, 2007 ; Staikou et al, 2007 ; Doyle and Wilkinson, 2008 ; Dahaba et al, 2009 ; Morimoto et al, 2011 ; Stoicea et al, 2014 ; Tzabazis et al, 2015 ; Aflaki et al, 2017 ). The concern regarding delayed emergence may be particularly relevant to a subgroup of patients with central disorders of hypersomnolence who demonstrate the presence of a positive allosteric modulator of gamma-aminobutyric acid Type A (GABA-A) receptors within spinal fluid (Rye et al, 2012 ).…”