Headache Resembling Hemicrania Continua Caused by Pituitary Adenoma



Fig. 24.1
Magnetic resonance imaging showing pituitary macroadenoma



Pituitary hormone tests revealed elevated growth hormone levels. Asking the patients about signs of acromegaly she confirmed recent changes in her face and hands. The ophthalmological examination was normal apart from a minimal visual field defect consistent with a chiasmal lesion.

Surgery was scheduled on February 26, 2009, but the patient said that she would not be able to stand the headache up to this date. Considering that analgesics and NSAIDs had failed, occipital and supraorbital nerve block with a long-acting steroid was performed resulting in complete resolution of the headache for 4 weeks. After transsphenoidal resection of the pituitary adenoma, a residual tumour encircling the right carotid artery had remained. Therefore, the patient underwent gamma knife treatment on June 30, 2009.

The patient was not seen in the headache outpatient clinic up to November 2009, when she presented again, because of persisting continuous right-sided headache associated with conjunctival injection and ptosis during periods of pain exacerbations. Again, an occipital nerve block was performed with significant improvement of headache. At follow-up 4 weeks later, a rounder and fuller face was noticed, and therefore, occipital nerve block was not repeated. Instead, topiramate was described. When the patient presented again in April 2010, she reported about side-shifting headaches with fullness of the ear and subjective hearing impairment. She had stopped topiramate because of side effects. During further follow-up, she continued to have chronic headache, but the side locked strictly to the right side with cranial autonomic symptoms changed to a bilateral headache being most severe on the right frontal and orbital regions and not accompanied with cranial autonomic symptoms anymore. MRI showed complete resolution of the residual tumour and pituitary hormone tests had become normal. She failed several prophylactic treatments and underwent inpatient treatment for medication overuse headache. At the last follow-up visit in April 2014, she reported almost daily headaches of varying intensity and bilateral localisation with a maximum in the right frontal and orbital regions, frequent use (but not overuse) of dexibuprofen and some benefit of amitriptyline 25 mg taken 2 h before bedtime.

In conclusion, this is the complex history of a patient with pre-existing migraine with aura who developed first daily headaches, headache resembling tension-type headache and later a continuous, strictly unilateral side-locked headache with exacerbations and ipsilateral cranial autonomic symptoms leading to the detection of a pituitary macroadenoma. After surgery and gamma knife treatment, headache evolved first to a unilateral headache with side shift and later to a bilateral headache with its intensity being most prominent on the right fronto-orbital region without cranial autonomic symptoms complicated by medication overuse requiring inpatient treatment. Obviously, the resolution of the hemicrania continua like headache has paralleled with the resolution of the pituitary macroadenoma, thus suggesting a causal relation.



24.2 Diagnosis of Hemicrania Continua


Hemicrania continua was first described by Sjaastad and Spierings in 1984. Since then case reports, small cases series and the largest case series up till now published by Cittadini and Goadsby in 2009 broadened the spectrum of hemicrania continua, but caused also discussions on how to define true hemicrania continua.

With respect to the International Classification of Headache Disorders (ICHD), hemicrania continua was not included in the first edition published in 1988; it was listed among other primary headaches (ICHD-2 4.7) in the 2004 edition, and finally it has been classified as trigeminal autonomic cephalalgia (ICHD-3 beta, 3.4) in 2013 [5]. According to ICHD-3 beta, the diagnosis of hemicrania continua requires a unilateral headache, present for more than 3 months showing pain exacerbations and accompanied by one or more of the following symptoms or signs on the side of the pain: conjunctival injection and/or tearing, nasal congestion and/or rhinorrhoea, eyelid oedema, sweating/flushing in the forehead and face, sensation of fullness in the ear and miosis and/or ptosis. Additionally or alternatively to these symptoms, and signs headache may be accompanied by a sense of restlessness or agitation, or movement may worsen the pain. Furthermore, an absolute response to therapeutic doses of indomethacin is mandatory, and the headache may not better be accounted for by another ICHD-3 diagnosis.

Compared to the initial two patients described by Sjaastad and Spierings [13], other patients diagnosed with hemicrania continua showed intermittent, not continuous pain, side shifting or even bilateral localisation of the headache, and a broader spectrum of cranial autonomic symptoms [2,7,10,11]. Furthermore, there are reports about headaches exactly resembling the phenomenology of hemicrania continua, but showing no or no absolute response to indomethacin [7,11]. All these observations have been included in the ICHD-3 criteria. Definite hemicrania continua still requires an absolute response to indomethacin, but lack of an (absolute) response allows to diagnose probable hemicrania continua, provided that all other ICHD-3 beta criteria of hemicrania continua are fulfilled.

Currently the revisions of the diagnostic criteria of hemicrania continua in ICHD-3 beta are under discussion. Antonaci and Sjaastad [1] argue that headache must be side locked and inclusion of the movement criteria is the consequence of misunderstanding hemicrania continua. They also argue that a response to 50 mg indomethacin intramuscularly is mandatory and a dose of 100–200 mg as given in ICHD- 3 beta is not only too high, but also harmful. Furthermore, Antonaci and Sjaastad do not agree with the inclusion of autonomic features beyond those reported in the initial patients. Goadsby [4] advocates the ICHD-3 beta criteria entitling his reply “Hemicrania continua – building on experience and clinical science”.


24.3 Differential Diagnosis of Hemicrania Continua


Hemicrania continua must be differentiated from (1) (chronic) migraine, (2) half-sided tension-type headache, (3) cluster headache and paroxysmal hemicrania and (4) secondary headaches. In a series of 528 patients of a headache clinic, Ramón et al. [9] identified 100 patients with unilateral, side-locked headaches. According to ICHD-II, 8 patients had hemicrania continua, 64 had other primary headaches and the remaining patients had secondary headaches. The most common diagnosis was cluster headache in 38 patients; other trigeminal autonomic cephalalgias were diagnosed in 7 patients, episodic and chronic migraine in 6 and 5 subjects, respectively, and other primary headaches in 8 patients. Secondary headaches comprised 12 different diagnoses, with cervicogenic headache being most common (n = 10). In a series of 63 patients with unilateral headaches beyond migraine and cluster headache presenting to a general neurological outpatient clinic, 38 % were classified as having a primary headache, 13 % as a secondary headache and the remaining 49 % could not be classified according to ICHD-II [10]. In the Vågå study, Sjaastad and Bakketeig [12] found one possible case of hemicrania continua among 1838 parishioners in the age group 18–65 years. Further epidemiological data on hemicrania continua are not available.
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Jul 4, 2016 | Posted by in HEAD AND NECK SURGERY | Comments Off on Headache Resembling Hemicrania Continua Caused by Pituitary Adenoma

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