Peculiar "reverse narcolepsy" sleep medicine case.

[danielmd06]

Good morning. I have a case that I would appreciate any commentary on. For those of you in academics, I wouldn't mind you drawing this to the attention of relevant faculty.

I have a patient I saw Thursday. She has a peculiar history of insomnia and lack of appetite since a surgical procedure in 2011. But first some background. This is her story...

Ms. A is a 42-year-old right-handed African-American female with a past medical history of migraine headaches, hypertension, hyperlipidemia, obesity (BMI of 33.1 kg/m2), chronic insomnia, and moderate obstructive sleep apnea. This latter was diagnosed by full-night sleep study completed May 12, 2013, with an overall apnea-hypopnea index of 15.0, REM apnea-hypopnea index 29.0, supine apnea-hypopnea index 6.9, oxygen saturation nadir 79%, and 22.9 minutes spent with oxygen saturation less than 88%. She had a titration polysomnogram completed May 26, 2013, which found that BiPAP at 10/6 cm H2O normalized the apnea-hypopnea index to 0.6 with an oxygen saturation nadir of 93%. She was ordered a BiPAP machine and returned to me Thursday for follow-up after having last been seen in clinic on May 1, 2013, with an Epworth Sleepiness Score of 2 (and being ordered her first sleep study). Epworth Sleepiness Score in the office is 2 (normal is less than 10). She loves the BiPAP. She denies trouble with it. Her energy levels and quality of sleep are improved for using BiPAP when she actually sleeps.

But that's not what bothers me here.

She has a history of very short sleep times since a second hysterectomy (for fibroids and bleeding) in 2011. Before this, she would sleep around 6 hours per night. The six hours were totally refreshing for her. After having the second hysterectomy surgery, she immediately noted postoperatively that she slept much less because she was "wired" and she also noticed that her hunger drive diminished. She now sleeps between 0 and 4 hours per night. It is not unusual for her to average 1.5 hours per 24 hour cycle. She feels fine when she awakens, if not exactly completely refreshed. This has been unresponsive to medications, including trazodone or over-the-counter sleep aids. She has no diminished energy levels or side effects of the lack of sleep. She actually isn't complaining about this per se. Just a bit annoyed and perhaps slightly fatigued.

I don't have copies of her operative report, hospital stay, or any history of an MRI brain. She tells me she'll bring in her records from the hospital.

Her neurologic exam is non-focal.

I had previously thought she simply had short sleep times. I didn't get her post-op history and the clue of lack of appetite until this last office visit.

For the non-sleep disorders people, the lateral hypothalamus contains the orexin-producing neurons that stimulate wakefulness and appetite. Loss of these cells gives you that picutre of narcolepsy (and increased appetite). If my patient's symptoms were actually secondary to a stroke, or some manner of injury from the anesthesia or surgery, where would it localize to? What actually could I do about it save watchful waiting? Again, she has no true complaints beyond what I've listed, and I plan on getting the MRI (though this happened two years ago). Probably be denied by insurance.

I'm just curious if you guys believe I'm overthinking this. Where should the lesion actually localize to (providing there is one)? What would you do with her (I know there are some new sleeping medications on the horizon that block orexin receptors)? I cannot find any articles with a similar clinical constellation, are any of you aware of some? Any help from those here, or specific faculty at your home institutions would be appreciated. I am available by PM if you don't wish to post.

Kind regards.

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[michaelrack]

I don't have any neurologic insights. But you may want to consider an atypical endocrine syndrome as the cause of her symptoms (either pituitary or ovarian- check or refer for TSH, prolactin, LH, FSH, assessment of cortisol, basic chem panel). Ovarian dysfunction can cause insomnia; not sure about decreased appetite.

Consider spirometry to look for an underlying pulmonary d/o (curious as to why she needed bipap for pure osa)

Are her BiPAP downloads ok? I often find overnight home oximetry on CPAP/BiPAP helpful to assess how patients are doing (DME's will sometimes do this for free, or it will often be covered by medicare and other insurances).
Did another entity do the titration (if so, maybe there wasn't any supine/REM sample at 10/6 and the patient isn't doing as well as the titration study suggests).

 

[neglect]

I think you are overthinking this. First of all, does this actually bother her? Is there a complaint here? She sleeps well for the <5 hours she does and has no daytime sleepiness. She has less appetite, but you mentioned that she is obese, so it isn't leading to pathologic weight loss. You state she has good energy levels but is fatigued. I'm not sure how you arrived at this distinction, but if she is intermittently fatigued then you can always blame her obesity and move her weight down.

Why isn't this diagnosis: normal variation of being human?

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Personally, I have another question that perhaps people here could answer for me. Where, exactly, is the difference between PVS and MCS? Or is this a meaningless question, akin to asking where MCI ends and AD begins? PVS patients can display pretty complicated behaviors: startling, orienting, blink to threat. They can display primitive localizations. MCS patients can be pretty messed up, and have long periods where they = PVS.

Not that I think this matters. I'm firmly in the camp that thinks that PVS is no consciousness and MCS is worse, because there is some consciousness to experience the hell of immobility, cognitive and emotional disorders, and pain. But I had a case and started reading a morass of academic, poorly written literature at odds with itself.

 

[danielmd06]

I don't have any neurologic insights. But you may want to consider an atypical endocrine syndrome as the cause of her symptoms (either pituitary or ovarian- check or refer for TSH, prolactin, LH, FSH, assessment of cortisol, basic chem panel). Ovarian dysfunction can cause insomnia; not sure about decreased appetite.

Consider spirometry to look for an underlying pulmonary d/o (curious as to why she needed bipap for pure osa)

Are her BiPAP downloads ok? I often find overnight home oximetry on CPAP/BiPAP helpful to assess how patients are doing (DME's will sometimes do this for free, or it will often be covered by medicare and other insurances).
Did another entity do the titration (if so, maybe there wasn't any supine/REM sample at 10/6 and the patient isn't doing as well as the titration study suggests).

She is only taking premarin, and I did talk with her about potential endocrine/hormonal derangements. This was precisely one of the avenues I was thinking along, actually. I did mention thyroid, progesterone, and estrogen abnormalities as possible causes. She is bringing me copies of her reports from the OSH. I cannot recall if she told me her PCP or an endocrinologist was following her after the surgery.

Her BiPAP downloads show a residual AHI of 4.0, and an average usage of 1 hour, 35 minutes, and 32 seconds. She tells me that this is all she is sleeping, and she affirms that she really likes the PAP.

Her titration study was with us, as was her diagnostic. BiPAP was purely for tolerability. She was on BiPAP 8/4, 9/5, and 10/6. All pressures looked good (only the latter two included REM). Oxygenation was great the entire night. The final pressure was selected based on the amount of time she spent on it (and in REM). No supine REM was appreciated on the titration (another reason I favored the higher pressure).

 

[danielmd06]

I think you are overthinking this. First of all, does this actually bother her? Is there a complaint here? She sleeps well for the <5 hours she does and has no daytime sleepiness. She has less appetite, but you mentioned that she is obese, so it isn't leading to pathologic weight loss. You state she has good energy levels but is fatigued. I'm not sure how you arrived at this distinction, but if she is intermittently fatigued then you can always blame her obesity and move her weight down.

Why isn't this diagnosis: normal variation of being human?

I amended my earlier post to include the amount of sleep she is now getting, which is 0-4 hours per night, with an average of around 1.5 hours per night. She tells me it is not unusual for her to stay up at least one night out of every three. Technically, this changes nothing from the story, but helps to emphasize the numeric change in how much sleep she averages now compared to previous.

Back to what you suggest, this is exactly what I considered at first. As long as there is no specific complaint, you really shouldn't treat anything when you're talking about insomnia. But, she had a dramatic difference after a procedure that was like a light switch getting flipped, and while there is a paucity of trouble I found it a curious issue (potentially) from a neuroanatomical standpoint. It seems the history is suggestive of an insult of some manner that caused the change.

Originally, I just labeled her to myself as a short sleeper (ie normal), set about ruling out OSA, and we discussed cognitive behavioral treatments for insomnia such as stimulus control therapy and proper sleep habits and hygiene to maximize what sleep she does get. Then I got the extra clues in the last office visit which intrigued me. Complaints aside, she is certainly on the short side of sleeping, and to have nights where one doesn't sleep at all is not normal in my experience. But I see no reason why her case cannot still be considered normal.

Truthfully, I'll probably do little beyond manage her sleep disordered breathing. But I find her story compelling. The constellation seems to make tantalizing sense.

 

[michaelrack]

Interesting case. Any h/o psychiatric illness, especially bipolar d/o. Is she currently on any meds for headache prophylaxis? You may want to try a sedating anticonvulsant such as VPA at bedtime for insomnia/headache prophylaxis.

 

[Plecopotamus]

I'm a mere student, but why not onset of menopausal symptoms due to unintended oophorectomy /disruption of blood supply by hysterectomy?

 

[neglect]

I amended my earlier post to include the amount of sleep she is now getting, which is 0-4 hours per night, with an average of around 1.5 hours per night. She tells me it is not unusual for her to stay up at least one night out of every three. Technically, this changes nothing from the story, but helps to emphasize the numeric change in how much sleep she averages now compared to previous.

Back to what you suggest, this is exactly what I considered at first. As long as there is no specific complaint, you really shouldn't treat anything when you're talking about insomnia. But, she had a dramatic difference after a procedure that was like a light switch getting flipped, and while there is a paucity of trouble I found it a curious issue (potentially) from a neuroanatomical standpoint. It seems the history is suggestive of an insult of some manner that caused the change.

I agree with you. No further comments, but I don't think you're going to find anything within the sensitivity of any test to give you a firm answer.

 

[neurologist]

The post-surgical change is interesting, and I'm honestly not sure what to make of that.
I do want one piece of added info from you though, which is this:
If I read this right, her super-short sleep time has been since 2011? So how long did she sleep during the PSGs that she had done in 2013? Were those unusually short? Any strikingly abnormal sleep architecture?
I'm wondering about sleep-state misperception here.
Maybe put an actigraph on her?

 

[danielmd06]

The post-surgical change is interesting, and I'm honestly not sure what to make of that.
I do want one piece of added info from you though, which is this:
If I read this right, her super-short sleep time has been since 2011? So how long did she sleep during the PSGs that she had done in 2013? Were those unusually short? Any strikingly abnormal sleep architecture?
I'm wondering about sleep-state misperception here.
Maybe put an actigraph on her?

This was the first thing I checked after her second visit. Nice to know I'm in good company.

They are commensurate with what she reports. The test from 5-12-13 had 108.0 minutes TST, with 35.7% stage N3, and 13.4% stage R. The test from 5-26-13 had a TST of 227.5 minutes, with 38.5% stage N3, and 11.9% stage R. So if anything, she has awesome deep sleep. Plus, she's calm and collected and not complaining. Totally unlike my typical paradoxical insomnia cases.

 

[typhoonegator]

Not to derail things, but I'll have what she's having. I sleep 6 hours most nights and have the same low-grade chronic fatigue that many physicians do. To be able to make do with substantially less and still be a composed member of society sounds like a boon. Does she have any memory or learning deficits? If not, it certainly sounds like she's relatively well-compensated for the change overall.

That said, check an ANA. It's probably lupus.

 

[typhoonegator]

Personally, I have another question that perhaps people here could answer for me. Where, exactly, is the difference between PVS and MCS? Or is this a meaningless question, akin to asking where MCI ends and AD begins? PVS patients can display pretty complicated behaviors: startling, orienting, blink to threat. They can display primitive localizations. MCS patients can be pretty messed up, and have long periods where they = PVS.

Not that I think this matters. I'm firmly in the camp that thinks that PVS is no consciousness and MCS is worse, because there is some consciousness to experience the hell of immobility, cognitive and emotional disorders, and pain. But I had a case and started reading a morass of academic, poorly written literature at odds with itself.

Dude, great topic totally worth a separate thread.

 

[neglect]

Dude, great topic totally worth a separate thread.

It's a situation that's like porn: hard to define but you know it when you see it.