Aura in Cluster Headache: A Cross-Sectional Study

Ilse F. de Coo, MD; Leopoldine A. Wilbrink, MD; Gaby D. Ie, MD; Joost Haan, MD, PhD; Michel D. Ferrari, MD, PhD


Headache. 2018;58(8):1203-1210. 

In This Article


By August 2014, there were 756 subjects with a questionnaire-based diagnosis of cluster headache in the LUCA database. In 720/756 (95.2%) of these the diagnosis had been separately confirmed by a physician. Six hundred and forty-one of 756 (84.8%) agreed to also fill in the aura questionnaire.

In the aura questionnaire, 88/641 (13.7%) indicated to have aura like symptoms. To be accurate, we contacted these 88 participants by phone to assess the ICHD-III beta criteria for typical aura with headache. By means of the telephone interview, typical aura according the ICHD-III criteria beta was diagnosed in 44/88 participants and no aura in 32/88 (the remaining 12 did not participate in the telephone interview and were defined as unknown). Therefore, a total of 44 participants with cluster headache with aura and 585 participants with cluster headache without typical aura were available for further analysis (Figure 1).

Figure 1.

Flowchart study flow.

Nonresponder analysis revealed no significant differences in baseline characteristics between responders and nonresponders of the questionnaire and if indicated telephone interview (Supporting Information Table S1).

Aura Characteristics in Cluster Headache with Typical Aura.—Visual symptoms were most frequently reported to be part of the aura (27/44; 61.4%), followed by sensory symptoms (17/44; 38.6%), dysarthria (12/44; 27.3%), and dysphasia (10/44; 22.7%; Table 1). There was much overlap, but interestingly, isolated dysarthric, aphasic, and sensory symptoms without visual symptoms were also reported.

Visual symptoms most often (70.4%) started before the cluster headache attack whereas sensory, dysarthric, and aphasic aura symptoms started most often during the headache phase (Table 1).

The median duration was 15 minutes for dysarthric symptoms, 20 minutes for visual symptoms and 25 minutes for both sensory and aphasic symptoms. Aura symptoms were not present during all cluster headache attacks. In participants with aura symptoms, roughly half of the attacks were accompanied or preceded by visual, sensory, and dysarthric symptoms. Aphasic symptoms were reported in a smaller proportion of attacks (Table 2).

Six out of these 44 participants reported also typical aura symptoms without headache, which occurred strictly with visual aura symptoms and fulfilled to the criteria of "typical aura without headache." None of these 6 participants reported to also have migraine.

Three case reports to illustrate the reported aura symptoms:

  • This man suffered from episodic cluster headache since he was 30 years old. He experienced a cluster headache bout every 2 years lasting 6–8 weeks. He had very severe pain right above the left eye together with ipsilateral conjunctival injection, lacrimation, ptosis, and rhinorrhea, with a sense of restlessness and agitation. An attack lasted about 3 hours without intervention and about 30 minutes with the use of oxygen only. About 30 minutes before the cluster headache attacks, he experienced zig-zag figures in his left eye, which spreads gradually over 10 minutes and then stopped. Twenty minutes later the headache started. He reports these visual symptoms in 10% of his cluster headache attacks.

  • A 57-year-old woman had chronic cluster headache attacks, with about 3 attacks a week since the age of 51. The very severe left orbital attacks lasted about 60 minutes without intervention. They were accompanied with restlessness and agitation and ipsilateral conjunctival injection, lacrimation, ptosis, miosis, and nasal congestion or rhinorrhoea. Before every attack, she saw unilateral (left eye) blurry zig-zag figures and after several minutes she also developed a heavy and slurred speech. Both symptoms start 5 minutes before the headache and both disappear after about 20 minutes. We have classified this as both a visual and dysarthric aura.

  • A 58-year-old male was diagnosed with episodic cluster headache and he experienced twice a year a bout of 8–10 weeks. The very severe pain is located periorbitally and above the left eye and is accompanied by restlessness and ipsilateral conjunctival injection, lacrimation, ptosis, eyelid edema, miosis, and nasal congestion or rhinorrhea. Without intervention the attacks last between 1.5 and 3 hours. Before about 80% of his attacks he experienced a slurred speech like being drunk for about 15–20 minutes. About 5 minutes after start of the slurred speech the headache starts. We classified this as dysarthria. His partner has noticed this difference in speech as well.

Comparison Between Participants With Typical Aura and No Aura.—In each group 2/3 had episodic and 1/3 chronic cluster headache. The 2 groups did not differ in demographic and clinical characteristics except for lower alcohol consumption and higher prevalence of frontal pain in participants with cluster headache with typical aura (Table 3). Comorbid migraine was reported in 13.6% (N = 6) of the participants with typical aura, of whom 2 females (33.3%) and 10.4% (N = 61) of participants without typical aura of whom 34 females (55.7%). Those who experienced aura associated with cluster headache attacks experienced (mainly visual) aura associated with migraine in 4 participants and no aura associated with their migraine in 2 participants.