Tuesday, January 14, 2020

Cataplexy


A British teen reportedly suffers from a rare disorder that causes uncontrollable muscle paralysis when she exhibits strong emotions such as laughter.

Speaking to the New York Post, Billie Hodgson, 17, said she was officially diagnosed with cataplexy, a neurological disorder linked to narcolepsy, last spring but first noticed symptoms at age 14 when she “fell to her knees” when laughing with a friend.

Cataplexy causes “sudden and uncontrollable muscle weakness or paralysis” that is often “triggered by a strong emotion, such as excitement or laughter,” states the National Sleep Foundation. Stress, anger, and fear can also be triggers.

“Without much warning, the person loses muscle tone and can have a slack jaw, broken speech, buckled knees or total weakness in their face, arms, legs, and trunk. A person experiencing total cataplexy stays awake and is aware of what is happening, but cannot move,” it adds, noting the episodes typically last for a couple of minutes.

The teen says the condition leads her “not to be involved in funny situations to avoid cataplexy, especially in front of those who are not as familiar with it.”

Though Billie said she has "to make light” of the rare disorder, she did note that it has impacted her social life and her future career plans. Cataplexy has prevented her from driving and has forced her to scrap her dream of becoming a midwife; the teen reportedly has trouble controlling her hand movements as a result of cataplexy, she told the Post.

There is no cure for the disorder, but it can be managed with “good sleep hygiene practices and the use of mediation as necessary,” according to Medical News Today.

“It’s so hard to explain how it feels when I collapse,” Billie added. “It’s like you have no control over your body.”

https://www.foxnews.com/health/rare-narcolepsy-linked-disorder-paralyzes-teen-laughter

Kallweit U, Bassetti CL. Pharmacological management of narcolepsy with and without cataplexy. Expert Opin Pharmacother. 2017 Jun;18(8):809-817. 

Abstract

Narcolepsy is an orphan neurological disease and presents with sleep-wake, motoric, neuropsychiatric and metabolic symptoms. Narcolepsy with cataplexy is most commonly caused by an immune-mediated process including genetic and environmental factors, resulting in the selective loss of hypocretin-producing neurons. Narcolepsy has a major impact on workableness and quality of life. Areas covered: This review provides an overview of the temporal available treatment options for narcolepsy (type 1 and 2) in adults, including authorization status by regulatory agencies. First- and second-line options are discussed as well as combination therapies. In addition, treatment options for frequent coexisting co-morbidities and different phenotypes of narcolepsy are presented. Finally, this review considers potential future management strategies. Non-pharmacological approaches are important in the management of narcolepsy but will not be covered in this review. Expert opinion: Concise evaluation of symptoms and type of narcolepsy, coexisting co-morbidities and patients´ distinct needs is mandatory in order to identify a suitable, individual pharmacological treatment. First-line options include Modafinil/Armodafinil (for excessive daytime sleepiness, EDS), Sodium Oxybate (for EDS and/with cataplexy), Pitolisant (for EDS and cataplexy) and Venlafaxine (for cataplexy (off-label) and co-morbid depression). New symptomatic and causal treatment most probably will be completed by hypocretin-replacement and immune-modifying strategies.



No comments:

Post a Comment