Symptoms of Narcolepsy: Cataplexy

EP: 1.Orexin and the Destabilization of Wakefulness in Narcolepsy

EP: 2.Symptoms of Narcolepsy: Excessive Daytime Sleepiness

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EP: 3.Symptoms of Narcolepsy: Cataplexy

EP: 4.Symptoms of Narcolepsy: Sleep Paralysis & Hallucinations

EP: 5.Testing and Criteria for Narcolepsy Diagnosis

EP: 6.Psychiatric Comorbidities & Differential Diagnosis in Narcolepsy

EP: 7.Behavioral Interventions and Adaptations in Narcolepsy

EP: 8.Pharmacological Treatment Options for Narcolepsy

EP: 9.Pitolisant and the Role of Histamine in Narcolepsy

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Debra Stultz, MD: We have several other symptoms, but one of the other specific symptoms for narcolepsy is cataplexy. When I was in medical school, and they taught us about narcolepsy—at that time you had to have all the symptoms to get the diagnosis—they showed a video of a man who had narcolepsy. He was standing with his wife, and she would talk about something. He would get emotional, and he would fall to the floor. There was another video of him sitting in the chair, and he would fall to the floor. OK, I got that. When I see that, that’s cataplexy. But cataplexy is much more than that. It can be a vague symptom. It can be any muscle in your body.

When I’m describing it to patients and I’m talking about the symptoms, I’ll start at the top of the head, with drooping eyelids or even blurred vision. I’ve had a couple of ophthalmologists send me narcolepsy patients, and I’m so excited about that. Their jaw droops. The jaw weakness is especially common in pediatric cases. The patient with pediatric narcolepsy will have a loosening of the jaw, and their tongue will protrude a little.

After you’ve seen it for a while, you recognize that it happens with their jaw and have neck weakness, like people bobbing with their head. You can have shoulder weakness, arm weakness, hand weakness. I had 1 patient with narcolepsy who said she would be working on something stressful at work, and she just drops her pen. The most common cataplexy is the knees buckling and that weakness that even your calves can get weak. Patients have told me, “I can’t wear high heels anymore because I might fall.” It can be any muscle, and it can occur with most any emotion. Laughter is the most common, but it can occur with anger, frustration, even sexual excitation. Any strong emotion that precipitates a muscle weakness is what cataplexy is.

Stephen Stahl, MD, PhD: Very fascinating. You made some really important points. You have the idea that cataplexy doesn’t have to be these dramatic things. Most of us have seen these videos in our training.

Debra Stultz, MD: Right.

Stephen Stahl, MD, PhD: Maybe you’ve even seen the Doberman dogs that are bred for cataplexy, and they run across and they collapse, and they get up and they run and collapse again. That’s interesting, but it’s not helpful because you will miss the more subtle forms. That’s a nice set of descriptions that you had there. I don’t see a lot of cataplexy in my own practice, although I did have a case I wrote up a million years ago, last century when I was a neurology resident at UCSF [University of California, San Francisco School of Medicine] and we had a brain stem tumor. That person had disruption of the sleep pathways that caused him to collapse. My neurology attending [physician] was very clever. He collapsed, and everybody thought it was a cardiac event. He was a younger guy, so it wasn’t all that credible. We were working him up for the possibility of a brain stem tumor. On the floor, the guy was completely limp. He opened up his eyes and said, “Move your eyes left and right.” That’s 1 of the ways to tell it wasn’t a heart attack and he was awake, but he couldn’t move them. His eye movements were preserved but had atonia.

Debra Stultz, MD: Right. When people do see the cataplexy, they may be confused that the patient is still aware. They’re aware what’s going on, but they can’t speak. Their eyes may move, and that’s why cataplexy is often thought of a psychiatric disorder. A lot of times when patients have cataplexy, they can sense it is coming on. Almost like when people are about to faint, sometimes they’ll hold their arms out before they faint. Other times people just hit the floor from fainting. The cataplexy is the same way. They can feel it coming on, so they don’t fall as often into something. Then it will be like it must be psychiatric because when they fell they didn’t get hurt. But I don’t think that’s true either. You have to tease it out.

Stephen Stahl, MD, PhD: Yes. What you really have is a normal state in the wrong phase of wakefulness.

Debra Stultz, MD: That’s right.

Stephen Stahl, MD, PhD: We normally go into that state when we dream, so we don’t act. Some people think it’s because you don’t act out your dreams when you’re paralyzed. The reality is that it’s miswired. Somehow, you’re connecting this “atonia”—lack of tone—at the wrong time when you’re awake and you need your tone.

Debra Stultz, MD: Right. Let me make 2 more points about cataplexy before we move on. If you’re seeing a patient, especially if you’re a psychiatrist seeing a patient or anybody seeing a new patient, if they’ve been on antidepressants for a long time, they may have suppressed the cataplexy. You might have to ask them about what happened before. Patients are often not the best historians for this symptom. They go home and talk to their family: “Yeah, that thing you do when you’re laughing.” It’s like, “What thing that I do when I’m laughing?” That’s a symptom you’ve got to keep asking about.

Transcript edited for clarity.

Disclosures:

Dr Stephen Stahl is clinical professor of psychiatry and neuroscience at the University of California Riverside, adjunct professor of psychiatry at the University of California San Diego, and honorary fellow in psychiatry at the University of Cambridge. Over the past 12 months (January 2020 - December 2020), Dr Stahl has served as a consultant to Acadia, Alkermes, Allergan, AbbVie, Arbor Pharmaceuticals, Axovant, Axsome, Celgene, Concert, Clearview, EMD Serono, Eisai Pharmaceuticals, Ferring , Impel NeuroPharma, Intra-Cellular Therapies, Ironshore Pharmaceuticals, Janssen, Karuna, Lilly, Lundbeck, Merck, Otsuka, Pfizer, Relmada, Sage Therapeutics, Servier, Shire, Sunovion, Takeda, Taliaz, Teva, Tonix, Tris Pharma, and Viforpharma; he is a board member of Genomind; he has served on speakers bureaus for Acadia, Lundbeck, Otsuka, Perrigo, Servier, Sunovion, Takeda, Teva, and Vertex; and he has received research and/or grant support from Acadia, Avanir, Braeburn Pharmaceuticals, Eli Lilly, Intra-Cellular Therapies, Ironshore, ISSWSH, Neurocrine, Otsuka, Shire, Sunovion, and TMS NeuroHealth Centers.

Dr Debra Stultz is the Director and Owner of Stultz Sleep and Behavioral Health in Barboursville, West Virginia. Dr Stultz earned her medical degree from Marshall University School of Medicine in Huntington, West Virginia. She completed a residency in psychiatry and a fellowship in child and adolescent psychiatry through West Virginia University at their Charleston Division through Charleston Area Medical Center in Charleston, West Virginia. She is board certified in psychiatry, sleep medicine, and behavioral sleep medicine. With a special interest in Narcolepsy, she treats a variety of sleep disorders and psychiatric issues. She is also the editor for the Clinical TMS Society newsletter, on their Board of Directors, and the chairman of the TMS and Sleep Disorders Affinity Group. Dr Stultz is on the advisory committee for Harmony Biosciences and is a speaker for Harmony Biosciences and Jazz Pharmaceuticals.