Hypothalamic Activation in Trigeminal Autonomic Cephalgia: Functional Imaging of an Atypical Case

Abstract

We report headache induced BOLD changes in an atypical case of trigeminal autonomic cephalgia (TAC). A 68-year-old patient was imaged using fMRI during three attacks of a periorbital head-pain with a average duration of 3 min. During the attacks, left sided conjunctival injection, rhinorrhea, lacrimation, facial sweating and hypersalivation were apparent. These attacks were usually partly responsive to oxygen administration but otherwise refractory to any drug. The patient described either attacks with a duration of one minute or less or longer attacks persisting for maximum of 20 min with headaches occurring up to 100 times a day. When considering the symptoms, frequency, duration and therapeutic response of the patient's headache, no clear-cut classification to one of the subtypes of trigeminal autonomic cephalgias (cluster headache, paroxysmal hemicrania, SUNCT) or trigeminal neuralgia was possible. The cerebral activation pattern was similar but not identical to those previously observed in cluster headache and SUNCT with a prominent activation in the hypothalamic grey matter. This case study underlines the conceptual value of the term TAC for the group of headaches focusing around the trigeminal-autonomic reflex. Our results emphasize the importance of the hypothalamus as key region in the pathophysiology of this entity.

Botulinum toxin type-A therapy in cluster headache: an open study

“…Accordingly, increased venous blood pressure in the sinus would directly cause the attack by temporarily increasing the pressure on sympathetic fibres running in the neighbourhood with the carotid artery [16]. Alternatively, evidence from PET studies [17,18], voxel-based morphometry [16] and stereotactic hypothalamic deep brain stimulation [20][21][22] suggests that there is a dysfunction of the ipsilateral posterior hypothalamus in CH, which causes a secondary activation of the trigemino-autonomic brainstem pathways [23]. This secondary activation elicits neurogenic inflammation of the large dural vessels, a mechanism that is similar to that during migraine attacks.…”

Update on the diagnosis and management of Trigemino-Autonomic Headaches

“…Indeed, several independant studies uniformly found an activation next to the hypothalamic area activated in cluster headache [4,34,47]. Even in a patient suffering from excrutiating trigemino-autonomic headache attacks, in whom frequency, duration and therapeutic response allowed no clear-cut classification to one of the subtypes of trigeminal autonomic cephalgia, the same prominent activation in the hypothalamic grey matter was found [46]. If this biological model is correct, the underlying cause for trigeminal autonomic cephalagias may indeed be similar and the variation in duration and frequency might be generally dependant on a different disorder of the inferior posterior hypothalamic neurons, perhaps a modulation of neuronal activity or a different involvement of the trigeminovascular system, explaining the relativly different phenotypes of these related syndromes.…”

From neuroimaging to patients’ bench: what we have learnt from trigemino-autonomic pain syndromes

“…Activation of the ipsilateral hypothalamus during attacks was reported in a patient whose headache attacks varied in duration, and could be diagnosed as trigeminal neuralgia, SUNCT, PH, or CH [17].…”