Case Report
 

By Dr. Daniel E Jacome
Corresponding Author Dr. Daniel E Jacome
Dartmouth Hitchcock Medical Center Department of Neurology, One Burnham Street, Suite 2 - United States of America 01376
Submitting Author Dr. Daniel E Jacome
NEUROLOGY

Orgasmic Headache, New Daily Persistent Headache, Hemochromatosis, Idiopathic Intracranial Hypertension, Migraine,Meningioma

Jacome DE. Post-Orgasmic New Daily Persistent Headache In a Patient With Hemochromatosis and Idiopathic Intracranial Hypertension Without Papilledema. WebmedCentral NEUROLOGY 2012;3(11):WMC003859
doi: 10.9754/journal.wmc.2012.003859

This is an open-access article distributed under the terms of the Creative Commons Attribution License(CC-BY), which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited.
No
Submitted on: 30 Nov 2012 05:51:45 AM GMT
Published on: 30 Nov 2012 08:33:17 PM GMT

Abstract


A 56 year old female with hemochromatosis-HFE C282Y homozygous gene mutation and remote history of infrequent isolated visual auras developed new daily persistent headache (NDPH) following a single orgasm. Headache was eventually ameliorated by amytriptiline and topiramate after 18 months following the precipitating event. Her neurological examination and ancillary imaging procedures including head CT, brain MRI-MRA and MRV were normal except for a very small parietal meningioma representing an incidental finding. There was no evidence of reversible vasoconstriction syndrome (RCVS). Her cerebrospinal (CSF) pressure was elevated compatible with idiopathic intracranial hypertension (IIH) without papilledema. There was no sign of bleeding in the CSF but her protein content was mildly increased in the absence of oligoclonal bands. Hemochromatosis (HC) predisposes to migraine in women. The origin of this patient’s headache remains unclear, but the association of primary post-orgasmic NDPH, hemochromatosis and IIH represents a unique clinical presentation.

Introduction


Orgasmic headache (OH) is the classic form of sexual headache typically benign, often recurrent but self-limited, not periodic or predictable, normally lasting only for a few hours. The headache is usually severe (“thunderclap” or “explosive”) and of rapid development during the climax of sexual activity. It is precipitated by either intercourse or masturbation. Pre and post-orgasmic headaches are also identified (1, 2). Sexual headache may be classified as a Valsalva-induced or exertion type of headache; however, cases of more ominous significance has been reported in the literature. They include examples of subarachnoid hemorraghe, embolic stroke, intracranial hematoma, basilar artery dissection and basilar artery stenosis (3, 4).

New daily persistent headache (NDPH) is one of the presentations of chronic daily headache (CDH). The other three categories are chronic migraine, chronic tension-type headache and hemicrania continua (5). According to Li and Rozen, NDPH must be bilateral or global, with pain features that may be pulsating, pressure-like, or both, and accompanied by sound and light supersensitivity in 60 to 70% of the cases. Headache must last a minimum of one month and should occur at least 15 days a month, for more than one month. Headaches should last at least for four hours every day in the symptomatic days. By definition the headache will be of new onset; stated differently, NDPH should not be diagnosed in someone already suffering from other types of CDH (5).

Nick and Bakouche reported that primary post-coital headache rarely persists for up to a few days (6). To my knowledge, no examples of longer duration (weeks or months) persistent primary orgasmic-induced headache, as a variant of NDPH, has been described. Of parallel interest, a 41 year old patient with hemochromatosis (HC) due to a homozygous C282Y mutation was reported with hemicrania continua lasting for several months, slowly evolving from episodic hemicrania, with no apparent precipitant. His headache was substantially ameliorated by venesection (7). 

Case Report


A 56 year old nurse was seen in consultation after she developed a paroxysmal excruciating (“thunderclap”) headache while achieving sexual orgasm. The headache was bilateral, occipital in location, radiated to the temples, was severe and throbbing in nature. It was not associated with photophobia, or vomiting. The day after the initial episode she was drowsy and her blood pressure (BP) was 160/104 mm Hg. She had no history of headache but only of rare isolated visual auras. The headache persisted with a lesser intensity but continued daily for 3 months. After 3 month she had headaches only 2 to 3 times a week, relieved by common analgesics. She experienced no more OH after the first episode. Initially she was given Gabapentin but she developed a rash. She chose then not to take additional prophylactic medications. After 18 month the original occipital daily headache returned on a daily basis, although not with the initial severity, and was accompanied by non-pulsating tinnitus. She was given verapamil following her neurological re-evaluation but she developed a rash again. Amitryptiline 10 mg h.s. reduced the intensity of her headache. She could not tolerate a larger dose. Additional improvement was achieved with the addition of topiramate at a dose of 50 mg b.i.d.

Her past history included atypical chest pain with a negative cardiac evaluation including a coronary angiogram and echocardiogram. She had been diagnosed 2 years earlier with phenotypic HC due to homozygous mutation HFE C 282-Y. Liver biopsy demonstrated iron overload with an iron index of 2.2 but no cirrhosis. Her initial ferritin and iron saturation levels were elevated. She was treated by venesection ever 3 weeks initially and subsequently every 3 to 6 months according to her blood work. Venesections did not suppress or ameliorated her headaches. Additional history included mild hypertension, sciatica, anxiety disorder, vitamin D deficiency and familial essential tremors. She had no family history of HC and genetic testing on her two children was negative. She took atenolol 50 mg a day and vitamin D 2.000 IU a day. On physical examination her BP was 110/74 and her pulse was 64. Her BMI was 23.6. Her neurological examinations on several occasions were normal except for mild postural tremor of the fingers. There was no meningismus. Her initial head CT and brain MRI showed an ovoid 9 mm, partially calcified, mildly enhancing, extra-axial left parietal lesion representing a small meningioma; otherwise imaging was normal without signs of generalized or peri-tumoral edema or hemorraghe, parenchymal bleed or hydrocephalus. Brain MRA and MRV with contrast were normal. Repeat brain MRI, MRA and MRV 18 months after the initial imaging studies were completed, showed no changes, including on the size and appearance the suspected small meningioma. There was no sign of RCVS and no vascular anomalies were identified in the initial and follow-up studies. Her EEG showed a normal background with no epileptic discharges, periodic complexes or sustained focal slowing. Her CSF opening pressure, once she returned 18 month later from the original visit complaining of a recurring occipital headache and tinnitus, was elevated at 28 cm of water. She had no cells and only mild elevation of her CSF protein at 57 mg/DL.  There were zero oligoclonal bands in the CSF.

Discussion


Hemochromatosis is an autosomal recessive blood disorder due to various genetic mutations, resulting in elevated absorption of iron and secondary progressive accumulation of iron in the liver, pancreas and pituitary gland, among several organs. In the brain, excessive iron overload causes neurodegeneration by way of cumulative oxidative neuronal injury (8). High prevalence of headache seems to exist in women with HC, in particular on those harboring the H63D polymorphism of the HFE gene (9). Iron accumulation in the peri-aqueductal gray (PAG), an area sub-serving pain modulation given its concentration of substance P, orexin, oxytocin, endogenous opiates and endocannabinoids, is believed to result in progressive neurodegeneration (8,9,10, 11). The latter mechanism has been hypothesized as the source for recurrent headache in HC, and in another venue, for the transformation of episodic headache into CDH, by way of creating central sensitization (i.e., induction of chronic central pain) (7,9,10).

The etiology of orgasmic headache has not been ultimately defined. It has been explained on basis of several potential mechanisms that are not mutually exclusive: 1. Reversible cerebral vasoconstriction syndrome (RCVS) (12) 2. Paroxysmal hypertension with loss of cerebral vascular auto-regulation during climax.  3. Underlying undiagnosed intracranial venous stenosis resulting in transient intracranial hypertension during orgasm (13) 4. Migraine diathesis. 5.  Valsalva maneuver during coitus inducing maximal pulsing distention of the intracranial vasculature (‘microdissections”?). 6. Unsuspected Arnold Chiari malformation (ACM) or cerebellar tonsilar ectopia with transient impaction on the spinal canal interrupting CSF outflow (14) 7. Ill-defined hormonal release or release of pro-inflammatory cytokines during intercourse, inducing acute “post-orgasm illness syndrome”, potentially relieved by non-steroidal anti-inflammatory agents. (15). No one of these potential mechanisms however, will explain persistent post-orgasmic headache; therefore the justification to diagnose this patient with primary post-orgasmic NDPH.

The etiology of NDPH is also of multifactorial origin (5). Secondary forms of NDPH that could also apply to secondary orgasmic persistent headache include intracranial bleed, acute cerebral venous thrombosis and acute CSF leaks from unsuspected arachnoid traumatic tears during sexual activity causing low CSF pressure. NDPH could equally result from unsuspected ACM complicated by irreversible impaction of the tonsils of the cerebellum into the cranio-cervical junction (16).

Although one patient with a giant subarachnoid cyst suffered from OH, meningiomas have not been associated to OH (17). This patient’s menigioma was very small, causing no mass effect,  bleeding or hydrocephalus. It was believed to represent an incidental finding totally unrelated to her intracranial hypertension.

In is uncertain as to the exact nature of this patient primary post-orgasmic NDPH. It is conceivable that her IIH was significant only as a chronic subclinical accompaniment of her HC that was present all along, or constituted a delayed complication from her OH. Certainly, she exhibited none of the conditions listed above that precipitate NDPH. Conversely, it can be speculated in the absence of micro-bleeds, vasoconstriction, cerebral venous sinus stenosis or thrombosis, that HC and IIH predisposed her to develop an orgasmic headache that ultimately acted upon immediate response genes, up-regulating her PAG nocioceptive neurotransmitters receptors. It could be inferred that the latter mechanism, lead into a condition of chronic central pain sensitization, albeit of fluctuating nature, perpetuated by her previously asymptomatic intracranial hypertension (10,11,18). If subclinical neurodegeneration of the PAG was present in the background prior to the appearance of her headache, it remains to be demonstrated.

References


1. Frese A, Rhamann A, Gregor N, Biehl K, Husstedt IW, Evers S (2007) Headache associated with sexual activity: prognosis and treatment options. Cephalalgia 27: 1265-1270
2. Ostergaard JR, Kraft M. (1992) Natural course of benign coital headache. BMJ 305: 1129.
3. Finnelli PF (1993) Coital cerebral hemorrhage. Neurology 43:2683-2685
4. Delasobera BE, Osborn SR, Davis JE (2009) Thunderclap headache with orgasm: a case of basilar artery dissection associated with sexual intercourse. J Emerg Med 20: 1-5
5. Li D, Rozen TD (2002) The clinical characteristics of new daily persistent headache. Cephalalgia 22: 66-69
6. Nick J, Bakouche P. (1980) Headache related to sexual intercourse. Sem Hop 56: 621-628
7. Gaul C, Krummernerl P, Tamke B, Kornhuber M (2007) Chronic daily headache in hereditary hemochromatosis treated by venesection. Headache 47: 926-928
8. Benkovic SA, Connor JR (1993) Ferritin, transferring, and iron in selected regions of the adult and aged rat brain. J Comp Neurol 338: 97-113
9. Rainero I, Rubino E, Rivoiro C, Valfre W, Binello E, Zampella E, De Martino P, Gentile S, Fenoglio P, Savi L, Gallone S, Pinessi L (2007) Hemochromatosis gene (HFE) polymorphisms and migraine: an association study. Cephalalgia 27: 9-13
10. Welch KMA, Nagesh V, Aurora SK, Gelman N (2001) Periaqueductal gray matter dysfunction in migraine: Cause or the burden of illness? Headache 41: 629-637
11. Ho YC, Lee HJ, Tung LW, Liao YY, Fu SY, Teng SF, Liao HT, Mackie K, Chiou LC (2011) Activation of orexin 1 receptors in the periaqueductal gray of male rats leads to antinocioception via retrograde endocannabinoid ( 2- arachidonoglycerol)-induced disinhibition. J Neurosci 31: 14600-14610
12. Valenca MM, Valencs LPPA, Bordini CA, Farias da Silva W, Leite GP, Antunes-Rodrigues J, Speciali JG (2004) Cerebral vasospasm and headache during sexual intercourse and masturbatory orgasms. Headache 44: 244-248
13. Quattrone A, Bono F, Oliveri RL, Gambardella A, Pirritano D, Labate A, Lucisano A, Valentino P, Zappia M, Aguglia U, Lavano A, Fera F, Pardatscher K (2001) Cerebral venous thrombosis and isolated intracranial hypertension without papilledema in CDH.Neurology  57:3136
14. Martins HA, Ribas VR, Lima MD, Oliveira DA, Viana MT, Ribas KH, Valenca MM (2010)     Headache precipitated by Valsalva maneuvers in patients with congenital Chiari I malformation. Arq Neuropsiquiatr 68: 406-409
15. Ashby J, Goldmeir D (2010) Postorgasm illness syndrome-a spectrum of illnesses. J Sex Med 7: 1976-1981
16. Mea E, Chiapparini L, Leone M, Franzini A, Messina G, Bussone G ( 2011) Chronic daily headache in the adults: differential diagnosis between symptomatic Chiari I malformation and spontaneous intracranial hypotension. Neurol Sci 32 Suppl 3: S291-294
17. Kang SY, Choi JC, Kang JH, Lee JS (2012) Huge supratentorial arachnoid cyst presenting as an orgasmic headache. Neurol Sci 33-639-641
18. De Simone RD, Ranieri A, Fiorillo, C, Bilo L, Bonavita V (2010) Is idiopathic intracranial hypertension without papilledema a risk factor for migraine progression? Neurol Sci 31: 411-415

Source(s) of Funding


Self funded

Competing Interests


None

Disclaimer


This article has been downloaded from WebmedCentral. With our unique author driven post publication peer review, contents posted on this web portal do not undergo any prepublication peer or editorial review. It is completely the responsibility of the authors to ensure not only scientific and ethical standards of the manuscript but also its grammatical accuracy. Authors must ensure that they obtain all the necessary permissions before submitting any information that requires obtaining a consent or approval from a third party. Authors should also ensure not to submit any information which they do not have the copyright of or of which they have transferred the copyrights to a third party.
Contents on WebmedCentral are purely for biomedical researchers and scientists. They are not meant to cater to the needs of an individual patient. The web portal or any content(s) therein is neither designed to support, nor replace, the relationship that exists between a patient/site visitor and his/her physician. Your use of the WebmedCentral site and its contents is entirely at your own risk. We do not take any responsibility for any harm that you may suffer or inflict on a third person by following the contents of this website.

Comments
0 comments posted so far

Please use this functionality to flag objectionable, inappropriate, inaccurate, and offensive content to WebmedCentral Team and the authors.

 

Author Comments
0 comments posted so far

 

What is article Popularity?

Article popularity is calculated by considering the scores: age of the article
Popularity = (P - 1) / (T + 2)^1.5
Where
P : points is the sum of individual scores, which includes article Views, Downloads, Reviews, Comments and their weightage

Scores   Weightage
Views Points X 1
Download Points X 2
Comment Points X 5
Review Points X 10
Points= sum(Views Points + Download Points + Comment Points + Review Points)
T : time since submission in hours.
P is subtracted by 1 to negate submitter's vote.
Age factor is (time since submission in hours plus two) to the power of 1.5.factor.

How Article Quality Works?

For each article Authors/Readers, Reviewers and WMC Editors can review/rate the articles. These ratings are used to determine Feedback Scores.

In most cases, article receive ratings in the range of 0 to 10. We calculate average of all the ratings and consider it as article quality.

Quality=Average(Authors/Readers Ratings + Reviewers Ratings + WMC Editor Ratings)