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Suggest Treatment For Narcolepsy And Idiopathic Hypersomnia
Question: My daughter ( 19)has been diagnosed with narcolepsy and then Idiopathic hypersomnia. She is currently on modafinil. Her Migraines were also a major issue and an MRI found she has small venous angioma medially on left front lobe. There is no haemosiderin deposition other then within vessals. She was put on blood pressure meds for her severe migraine but they only subsided for a while. The doctor is considering botox for her migraines. Do you think this is the best treatment ? I also was wondering if you have come across anyone that found their Idiopathis hypersomnia was caused by a DVA? She is so young to be this unwell.
Brief Answer:
Narcolepsy and Idiopathic Hypersomnia- REALLY--- Hmmmmm?
Detailed Answer:
Good morning. Let's see if we can't give a few ideas as to how to go about treating your daughter for her problems. First of all.....I have to ask the question as to how she was diagnosed with IH and narcolepsy AT THE SAME TIME. This is actually a violation of "the rules" of sleep disorders. A person with TRUE narcolepsy cannot be diagnosed with IDIOPATHIC HYPERSOMNIA and vice versa since the symptoms of both disorders cross over to the other and preclude each other's diagnosis simultaneously. And so I would have to ask how the NARCOLEPSY was diagnosed. If a MSLT was performed at a certified sleep center and interpreted by a qualified sleep specialist then, I would say that is likely her primary (and only) sleep disorder diagnosis. Secondly, if modafanil is not really treating her condition adequately has she been tried on alternative agents? A newer cousin to modafanil (Pro-vigil) is available and can sometimes work better called NUVIGIL. Has she been tried on that medication? If that doesn't result in adequate treatment of her narcolepsy then, has her neurologist considered XYREM? In my practice, that is one of my GOTO's in my narcoleptic patients with cataplexy (if she has that condition along with narcolepsy). You haven't said whether or not she suffered from cataplexy (sudden collapse as if the person just fell asleep where they stood subsequent to a strong emotion....has that ever happened to your daughter?). I find that in patients for whom modafanil isn't working well that there is a strong possibility the patient may have cataplexy so I look for it by history.
I would ask several questions about the headaches. First of all, are they truly MIGRAINES? There are criteria to fulfill and if she is not truly suffering from migraines then, that may be one reason why her prophylactic blood pressure medication is not working....or the dose may not be high enough or they may be using a medication that is not that effective. In my headache practice I generally start with PROPRANOLOL (NOT ATENOLOL which I find to be quite ineffective compared to propranolol) and I make sure that the patient is titrated to at least 40mg. 3X/day before making a decision to go to another agent.
Also, by consensus of the XXXXXXX Headache Society and the International Headache Society and the XXXXXXX Academy of Neurology, TOPAMAX is the preferred oral agent of choice to PREVENT CHRONIC MIGRAINE HEADACHES meaning greater than 15 headache days per month. Does this describe your daughter's headaches...and are we sure they are migraines? Has she been tried on the tricyclics yet such as NORTRIPTYLINE....I find that to be very useful as well for preventing headaches. BOTOX is a great drug to prevent headaches but I ALWAYS try my patients on:
1. BETA BLOCKERS (good adequate dose of PROPRANOLOL)
2. TCA's (Nortriptyline as opposed to amitriptyline) titrated to at least 50mg. nightly
3. AED's (antiepileptics such as TOPAMAX vs. TEGRETOL vs. VALPROIC ACID in that order) to see if we get acceptable results
4. BOTOX- Finally, if the previous regimens fail then, I will do a good stiff injection regimen of BOTOX and using a FIXED dose pattern as opposed to a FOLLOW THE PAIN PATTERN with reinjection scheduled for 10-12 weeks in the future.
On the venous anomaly. No hemosiderin? Any compression of vital structures or closing off CSF flow anywhere? If not, then, I would look at the entity as likely a NON-ISSUE in terms of the headache picture and I wouldn't mess around with hit. Remember IH should not be diagnosed in tandem with someone who has CONFIRMED NARCOLEPSY.
I agree that she is very young and needs to be on the LEAST amount of medication as possible and so I hope she is being followed by someone "large and in charge" wherever you guys live.
If I've satisfactorily addressed your question then, could you do me the kindest of favors by CLOSING THIS QUERY and be sure to include some fine words of feedback and a 5 STAR rating to our transaction if you feel the response has helped you? Again, many thanks for posing your question.
Do not forget to contact me in the future at: www.bit.ly/drdariushsaghafi for additional questions, comments, or concerns having to do with this topic or others.
This query has utilized a total of 24 minutes of professional time in research, review, and synthesis for the purpose of formulating a return statement.
Narcolepsy and Idiopathic Hypersomnia- REALLY--- Hmmmmm?
Detailed Answer:
Good morning. Let's see if we can't give a few ideas as to how to go about treating your daughter for her problems. First of all.....I have to ask the question as to how she was diagnosed with IH and narcolepsy AT THE SAME TIME. This is actually a violation of "the rules" of sleep disorders. A person with TRUE narcolepsy cannot be diagnosed with IDIOPATHIC HYPERSOMNIA and vice versa since the symptoms of both disorders cross over to the other and preclude each other's diagnosis simultaneously. And so I would have to ask how the NARCOLEPSY was diagnosed. If a MSLT was performed at a certified sleep center and interpreted by a qualified sleep specialist then, I would say that is likely her primary (and only) sleep disorder diagnosis. Secondly, if modafanil is not really treating her condition adequately has she been tried on alternative agents? A newer cousin to modafanil (Pro-vigil) is available and can sometimes work better called NUVIGIL. Has she been tried on that medication? If that doesn't result in adequate treatment of her narcolepsy then, has her neurologist considered XYREM? In my practice, that is one of my GOTO's in my narcoleptic patients with cataplexy (if she has that condition along with narcolepsy). You haven't said whether or not she suffered from cataplexy (sudden collapse as if the person just fell asleep where they stood subsequent to a strong emotion....has that ever happened to your daughter?). I find that in patients for whom modafanil isn't working well that there is a strong possibility the patient may have cataplexy so I look for it by history.
I would ask several questions about the headaches. First of all, are they truly MIGRAINES? There are criteria to fulfill and if she is not truly suffering from migraines then, that may be one reason why her prophylactic blood pressure medication is not working....or the dose may not be high enough or they may be using a medication that is not that effective. In my headache practice I generally start with PROPRANOLOL (NOT ATENOLOL which I find to be quite ineffective compared to propranolol) and I make sure that the patient is titrated to at least 40mg. 3X/day before making a decision to go to another agent.
Also, by consensus of the XXXXXXX Headache Society and the International Headache Society and the XXXXXXX Academy of Neurology, TOPAMAX is the preferred oral agent of choice to PREVENT CHRONIC MIGRAINE HEADACHES meaning greater than 15 headache days per month. Does this describe your daughter's headaches...and are we sure they are migraines? Has she been tried on the tricyclics yet such as NORTRIPTYLINE....I find that to be very useful as well for preventing headaches. BOTOX is a great drug to prevent headaches but I ALWAYS try my patients on:
1. BETA BLOCKERS (good adequate dose of PROPRANOLOL)
2. TCA's (Nortriptyline as opposed to amitriptyline) titrated to at least 50mg. nightly
3. AED's (antiepileptics such as TOPAMAX vs. TEGRETOL vs. VALPROIC ACID in that order) to see if we get acceptable results
4. BOTOX- Finally, if the previous regimens fail then, I will do a good stiff injection regimen of BOTOX and using a FIXED dose pattern as opposed to a FOLLOW THE PAIN PATTERN with reinjection scheduled for 10-12 weeks in the future.
On the venous anomaly. No hemosiderin? Any compression of vital structures or closing off CSF flow anywhere? If not, then, I would look at the entity as likely a NON-ISSUE in terms of the headache picture and I wouldn't mess around with hit. Remember IH should not be diagnosed in tandem with someone who has CONFIRMED NARCOLEPSY.
I agree that she is very young and needs to be on the LEAST amount of medication as possible and so I hope she is being followed by someone "large and in charge" wherever you guys live.
If I've satisfactorily addressed your question then, could you do me the kindest of favors by CLOSING THIS QUERY and be sure to include some fine words of feedback and a 5 STAR rating to our transaction if you feel the response has helped you? Again, many thanks for posing your question.
Do not forget to contact me in the future at: www.bit.ly/drdariushsaghafi for additional questions, comments, or concerns having to do with this topic or others.
This query has utilized a total of 24 minutes of professional time in research, review, and synthesis for the purpose of formulating a return statement.
Above answer was peer-reviewed by :
Dr. Chakravarthy Mazumdar
Thankyou Dr. XXXXXXX Saghafi,
She has had sleep test down in sleep centre and was diagnosed with Narcolepsy ( went into REM within 5mins on all naps. However, second test she did not go into Rem on all 5 naps and has symptoms more like IH. Xyrem and Nuvigil are unavailable in Australia. She is on waiting list for Nuvigil as sleep doctor states it may become available in Australia by end of year. She does not have cateplexy but has narcoleptic symptoms like sleep paralysis and vivid dreams. However, she is very groggy in the mornings ( like IH people) and it takes a massive effort to get her out of bed and standing upright.
NO hemosiderein so therefore her migraines are PROBABLY OR POSITIVELY not linked to the venious anomaly ?? Is there a major possibility that it will bleed or that it could give her a stroke?? Her neurologist states she has full blown migraines.
Your information is invaluable and if you could just quickly answer the above questions and add anything else I will happily provide 5 star and close discussion.
She has had sleep test down in sleep centre and was diagnosed with Narcolepsy ( went into REM within 5mins on all naps. However, second test she did not go into Rem on all 5 naps and has symptoms more like IH. Xyrem and Nuvigil are unavailable in Australia. She is on waiting list for Nuvigil as sleep doctor states it may become available in Australia by end of year. She does not have cateplexy but has narcoleptic symptoms like sleep paralysis and vivid dreams. However, she is very groggy in the mornings ( like IH people) and it takes a massive effort to get her out of bed and standing upright.
NO hemosiderein so therefore her migraines are PROBABLY OR POSITIVELY not linked to the venious anomaly ?? Is there a major possibility that it will bleed or that it could give her a stroke?? Her neurologist states she has full blown migraines.
Your information is invaluable and if you could just quickly answer the above questions and add anything else I will happily provide 5 star and close discussion.
Brief Answer:
Narcolepsy vs. IH
Detailed Answer:
Thank you very much for your clarifications.
And thus my comment about not being able to have NARCOLEPSY and IH present in the same patient. You may need to revise that portion of your presentation in the future so as to not give an impression that the sleep center is actually making these 2 diagnoses in the same patient. Or are they??
It seems to me that in point of fact, they may be challenged at this point to know exactly which diagnosis she has....if she has either entity at all.
The MSLT is only about 57% sensitive in patients being tested for NARCOLEPSY due to testing and environmental variability. However, when a patient is definitely known to possess a SL of less than 5 min. then, we are about 94% certain the diagnosis IS NARCOLEPSY (i.e. 94% specific for the diagnosis of Narcolepsy).
How about SLEEP ONSET REM sleep (SOREM)? In the diagnosis of narcolepsy if a patient definitely is believed not to have CATAPLEXY as part of the syndromic definition then, they MUST have > 2 SOREM's in their study. I will
It has also been shown that the presence of 2 or more of these entities in a tested subject during a sleep study confers a sensitivity for the diagnosis of narcolepsy 84% of the time BUT this time our specificity for the diagnosis in patients with 2+ SOREM's in a study shoots the specificity up to 99%. Does she have any SOREM's?
And so the bottom line using those 2 parameters is that in patients with PATHOLOGICAL DROWSINESS performing a sleep study- the combination of a positive MSLT (less than 5 min. from head to pillow into any stage of sleep) PLUS at least 1 SOREM (REM being the first stage of sleep encountered by patient following head to pillow) makes the likelihood of narcolepsy....extremely high. Between 94-99% to be exact.
The risk of the venous anomaly rupturing or being responsible is very slight. I wouldn't worry about that as the cause of the headaches now or in the future. At least 5-15% of people have such anomalies and never are ever aware of it....I would also consider the use of TRIPTAN MEDICATIONS for the migraines if that's what the headaches are all about. In order to verify that diagnosis you may wish to get your daughter to a HEADACHE SPECIALIST. Triptan medications are things such as SUMATRIPTAN, RIZATRIPTAN, ZOLMITRIPTAN, etc.
Those medications in conjunction with preventative medications which I outlined for you before for the core requirements to deal with chronic or daily migraine headaches. BOTOX is an option but in my headache practice I usually do not go to BOTOX unless the patient fails other regimens first. Also, your daughter should be keeping track of her headaches on what's called a headache log or diary.
Cheers!
Narcolepsy vs. IH
Detailed Answer:
Thank you very much for your clarifications.
And thus my comment about not being able to have NARCOLEPSY and IH present in the same patient. You may need to revise that portion of your presentation in the future so as to not give an impression that the sleep center is actually making these 2 diagnoses in the same patient. Or are they??
It seems to me that in point of fact, they may be challenged at this point to know exactly which diagnosis she has....if she has either entity at all.
The MSLT is only about 57% sensitive in patients being tested for NARCOLEPSY due to testing and environmental variability. However, when a patient is definitely known to possess a SL of less than 5 min. then, we are about 94% certain the diagnosis IS NARCOLEPSY (i.e. 94% specific for the diagnosis of Narcolepsy).
How about SLEEP ONSET REM sleep (SOREM)? In the diagnosis of narcolepsy if a patient definitely is believed not to have CATAPLEXY as part of the syndromic definition then, they MUST have > 2 SOREM's in their study. I will
It has also been shown that the presence of 2 or more of these entities in a tested subject during a sleep study confers a sensitivity for the diagnosis of narcolepsy 84% of the time BUT this time our specificity for the diagnosis in patients with 2+ SOREM's in a study shoots the specificity up to 99%. Does she have any SOREM's?
And so the bottom line using those 2 parameters is that in patients with PATHOLOGICAL DROWSINESS performing a sleep study- the combination of a positive MSLT (less than 5 min. from head to pillow into any stage of sleep) PLUS at least 1 SOREM (REM being the first stage of sleep encountered by patient following head to pillow) makes the likelihood of narcolepsy....extremely high. Between 94-99% to be exact.
The risk of the venous anomaly rupturing or being responsible is very slight. I wouldn't worry about that as the cause of the headaches now or in the future. At least 5-15% of people have such anomalies and never are ever aware of it....I would also consider the use of TRIPTAN MEDICATIONS for the migraines if that's what the headaches are all about. In order to verify that diagnosis you may wish to get your daughter to a HEADACHE SPECIALIST. Triptan medications are things such as SUMATRIPTAN, RIZATRIPTAN, ZOLMITRIPTAN, etc.
Those medications in conjunction with preventative medications which I outlined for you before for the core requirements to deal with chronic or daily migraine headaches. BOTOX is an option but in my headache practice I usually do not go to BOTOX unless the patient fails other regimens first. Also, your daughter should be keeping track of her headaches on what's called a headache log or diary.
Cheers!
Above answer was peer-reviewed by :
Dr. Chakravarthy Mazumdar
Answered by
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