Headaches and chronic pain are amongst the leading cause of morbidity and years lived with disability in the world.[1] Headaches have been classified into various categories based on the International Classification of Headache Disorders criteria.[2] One such headache disorder is a hypnic headache.
Hypnic headache (HH) is a rare headache syndrome that occurs exclusively during sleep, usually at the same time at night. It also has been named before the clockwise headache or alarm clock headache. It is considered a primary headache, according to the international classification of headache disorders.[3]
HH is characterized by attacks of dull headaches that occur after the age of 50 years, at least 15 times per month, and come only during sleep with no associated autonomic symptoms. The attacks wake the patient during sleep and usually last more than 15 min after waking.[4]
Hypnic headache is a chronic disorder that can last for years, with no remission of headache, and 47 percent.[5]
Patients with migraine and hypertension are more likely to develop HH compared to others. Nearly all HH patients had some type of motor activity, such as getting out of bed and eating, drinking, showering, or reading when awakened by the headache attack. Still, they did not typically have the restlessness that is seen with cluster headache and trigeminal autonomic cephalalgias.[6]
While the exact etiology is unknown, the apparent cyclicity and response to lithium suggest a pathophysiologic connection between cluster headache and hypothalamic dysfunction.
Hypnic headache is thought to be about 0.07% of all patients that come to the office with the chief complaint of headache.
In the geriatric population, the ratio is 1.4%, according to an Italian study.[6]
However, in recent years HH is thought to be slightly more common than previous studies. It is estimated to be 0.3% to 0.6% in a French study.[7]
The timing of hypnic headache and the concomitant diabetes insipidus with a feeling of intense thirst that has been reported by few studies favor the involvement of hypothalamic structures.
It has also been reported that sleep-disordered breathing has not been reported to be of high prevalence in patients diagnosed with HH. Continuous positive airway pressure and nocturnal oxygen supplementation did not improve symptoms in patients with HH.
The onset of HH attacks are associated with rapid eye movement sleep, and this association could connect HH to arterial hypertension and low oxygenation that occur during REM sleep.
The suprachiasmatic nucleus (SCN) remains a possible region involved in the origin of HH. As body ages, the cell numbers in the suprachiasmatic nucleus decrease significantly, which leads to a decrease in melatonin. The release of melatonin will be affected by the reduced SCN cells. The SCN projects to and receives afferents from brainstem periaqueductal gray, which means a pain modulating link is possible.[8][9]
Patients usually describe the headache as frequent recurrent headaches occurring only during sleep. They cause wakening and last for up to 4 hours. There are no characteristic associated symptoms and not attributed to other pathology. The formal criteria require according to ICHD-3 headaches that (1) develop only during sleep and cause awakening, (2) occur greater than or equal to 10 days per month for greater than or equal to 3 months, (3) last greater than or equal to 15 minutes for up to 4 hours after awakening, and (4) have no associated cranial autonomic symptoms or restlessness.[10] Additionally, the headache should not be accounted for by another ICHD-3 diagnosis.
Other characteristic features of the HH include [11]:
Evaluation of patients with nocturnal headaches must include ruling out conditions like cerebral tumors, giant cell arteritis, obstructive sleep apnea (OSA), and other sleeping disorders.[13]
MRI brain with/without contrast is needed for patients to rule out any structural abnormality like tumors, etc. Overnight polysomnography (sleep) study is necessary to rule out OSA and other sleeping disorders. Once any organic cause of the headache is ruled out, the diagnosis is mostly clinical based on the ICHD-3 diagnostic criteria. MRI brain may also show grey matter volume reduction in the hypothalamus.
Treatment and management of headache disorders is a challenging task and requires a thorough evaluation.[14] While there is a lack of good quality clinical data for the management of hypnic headache, the following medications have shown benefit in the management of hypnic headaches.
It has been reported that around 17% of patients achieve spontaneous remission over the course of times; however, most patients continue to suffer from it chronically. Another study has reported that 40% of patients achieve freedom from these headaches with treatment. In another published study, 53% of patients enter an episodic course following treatment.[10]
There are no reported long term neurologic sequelae to this condition.[26]
As the pathology and exact etiology of these headaches are still unclear, the prevention of these headaches is still not possible. However, prophylactic strategies have demonstrated good outcomes in suppressing the headache attacks.
The diagnosis of hypnic headache is essential as there are many differential diagnoses that are very similar. History taking is the most critical step in the evaluation of these patients and also proper work up to rule out all secondary causes of such headaches like cerebral tumors, etc.
All members of the healthcare team should be familiar with this condition as it is not very common, but they do occur in the real world and must be well managed.
[1] | Jain S,Malinowski M,Chopra P,Varshney V,Deer TR, Intrathecal drug delivery for pain management: recent advances and future developments. Expert opinion on drug delivery. 2019 Aug; [PubMed PMID: 31305165] |
[2] | Olesen J, From ICHD-3 beta to ICHD-3. Cephalalgia : an international journal of headache. 2016 Apr; [PubMed PMID: 26162775] |
[3] | Lanteri-Minet M,Donnet A, Hypnic headache. Current pain and headache reports. 2010 Aug; [PubMed PMID: 20512536] |
[4] | The International Classification of Headache Disorders: 2nd edition. Cephalalgia : an international journal of headache. 2004; [PubMed PMID: 14979299] |
[5] | Liang JF,Wang SJ, Hypnic headache: a review of clinical features, therapeutic options and outcomes. Cephalalgia : an international journal of headache. 2014 Sep; [PubMed PMID: 24942086] |
[6] | Holle D,Naegel S,Obermann M, Hypnic headache. Cephalalgia : an international journal of headache. 2013 Dec; [PubMed PMID: 23832130] |
[7] | Donnet A,Lantéri-Minet M, A consecutive series of 22 cases of hypnic headache in France. Cephalalgia : an international journal of headache. 2009 Sep; [PubMed PMID: 19250282] |
[8] | Holle D,Naegel S,Krebs S,Gaul C,Gizewski E,Diener HC,Katsarava Z,Obermann M, Hypothalamic gray matter volume loss in hypnic headache. Annals of neurology. 2011 Mar; [PubMed PMID: 21446025] |
[9] | Mathews S,Jain S, Anatomy, Head and Neck, Cricoid Cartilage 2020 Jan; [PubMed PMID: 30969643] |
[10] | Evers S,Goadsby PJ, Hypnic headache: clinical features, pathophysiology, and treatment. Neurology. 2003 Mar 25; [PubMed PMID: 12654950] |
[11] | Goadsby PJ, Unique Migraine Subtypes, Rare Headache Disorders, and Other Disturbances. Continuum (Minneapolis, Minn.). 2015 Aug; [PubMed PMID: 26252589] |
[12] | Dodick DW,Mosek AC,Campbell JK, The hypnic ( [PubMed PMID: 9595209] |
[13] | Peatfield RC,Mendoza ND, Posterior fossa meningioma presenting as hypnic headache. Headache. 2003 Oct; [PubMed PMID: 14511280] |
[14] | Jain S,Silberstein SD, Invited Commentary on Preventive Anti-Migraine Therapy (PAMT). Current treatment options in neurology. 2019 Mar 14; [PubMed PMID: 30868470] |
[15] | Ivañez V,Soler R,Barreiro P, Hypnic headache syndrome: a case with good response to indomethacin. Cephalalgia : an international journal of headache. 1998 May; [PubMed PMID: 9642499] |
[16] | Seidel S,Zeitlhofer J,Wöber C, First Austrian case of hypnic headache: serial polysomnography and blood pressure monitoring in treatment with indomethacin. Cephalalgia : an international journal of headache. 2008 Oct; [PubMed PMID: 18498399] |
[17] | Al Khalili Y,Jain S, Carbamazepine Toxicity 2020 Jan; [PubMed PMID: 29939629] |
[18] | Marziniak M,Voss J,Evers S, Hypnic headache successfully treated with botulinum toxin type A. Cephalalgia : an international journal of headache. 2007 Sep; [PubMed PMID: 17645763] |
[19] | Son BC,Yang SH,Hong JT,Lee SW, Occipital nerve stimulation for medically refractory hypnic headache. Neuromodulation : journal of the International Neuromodulation Society. 2012 Jul; [PubMed PMID: 22376140] |
[20] | Deer TR,Grider JS,Lamer TJ,Pope JE,Falowski S,Hunter CW,Provenzano DA,Slavin KV,Russo M,Carayannopoulos A,Shah JM,Harned ME,Hagedorn JM,Bolash RB,Arle JE,Kapural L,Amirdelfan K,Jain S,Liem L,Carlson JD,Malinowski MN,Bendel M,Yang A,Aiyer R,Valimahomed A,Antony A,Craig J,Fishman MA,Al-Kaisy AA,Christelis N,Rosenquist RW,Levy RM,Mekhail N, A Systematic Literature Review of Spine Neurostimulation Therapies for the Treatment of Pain. Pain medicine (Malden, Mass.). 2020 Feb 8; [PubMed PMID: 32034422] |
[21] | Jain S,Fishman MA,Wu C, Significant cephalad lead migration with use of externally powered spinal cord stimulator. BMJ case reports. 2018 Sep 21; [PubMed PMID: 30244224] |
[22] | Deer TR,Jain S,Hunter C,Chakravarthy K, Neurostimulation for Intractable Chronic Pain. Brain sciences. 2019 Jan 24; [PubMed PMID: 30682776] |
[23] | Jain S,Yuan H,Spare N,Silberstein SD, Erenumab in the treatment of migraine. Pain management. 2018 Nov 1; [PubMed PMID: 30235976] |
[24] | Al Khalili Y,Jain S,Murphy PB, Cervicogenic Headache 2020 Jan; [PubMed PMID: 29939639] |
[25] | Al Khalili Y,Jain S,King KC, Retinal Migraine Headache 2020 Jan; [PubMed PMID: 29939547] |
[26] | Tariq N,Estemalik E,Vij B,Kriegler JS,Tepper SJ,Stillman MJ, Long-Term Outcomes and Clinical Characteristics of Hypnic Headache Syndrome: 40 Patients Series From a Tertiary Referral Center. Headache. 2016 Apr; [PubMed PMID: 27015738] |