"IH has long been considered a rare disease, believed to be 10 times less frequent than narcolepsy. The prevalence of narcolepsy (with cataplexy) is estimated between 1/3,300 and 1/5,000. Although the true prevalence of IH is unknown, it is estimated at 1/10,000 to 1/25,000 for the long sleep form and 1/11,000 to 1/100,000 without long sleep. A more precise estimate "is complicated by a lack of clear biologic markers" and a lack of "unambiguous diagnostic criteria. "Because of the rarity of IH, research into the condition is limited "Patients are rare, researchers and scientists involved in the field are few and research findings are therefore scarce." "In Europe and in North America there is now a public health concern about helping patients and families affected by these rare diseases. Due to the complexity of the disease, they often experience difficulties to be diagnosed and often face social and professional consequences." Research
GABA-directed medications
There is ongoing research into the efficacy of gamma aminobutyric acid A (GABAA) receptor antagonists for the treatment of IH. Research findings suggest that the GABA neurotransmitter system plays a significant role in the etiology of primary hypersomnias, such as IH and Narcolepsy Type 2. Given the possible role of hyperactive GABAA receptors in IH, medications that could counteract this activity are being studied to test their potential to improve sleepiness. These currently include clarithromycin and flumazenil.Flumazenil, a GABAA receptor antagonist is approved by the FDA for use in anesthesia reversal and benzodiazepine overdose.
Hyperglycinemia may refer to one of two related inborn amino acid disorders that are characterized by elevated levels of glycine in the blood. Propionic acidemia, also known as "ketotic glycinemia"
Glycine encephalopathy, also known as "non-ketotic hyperglycinemia"