Paroxysmal hemicrania associated to carotid artery dissection: a case report

There are numerous case reports relating trigeminal autonomic cephalalgias to structural injuries. However there is no description of the association between paroxysmal hemicrania and carotid artery dissection. We describe a previously healthy 63-year-old male presented with the onset of severe, throbbing pain in the right frontal region, lasting between 10 and 30 minutes, with a frequency of approximately two to three attacks per day, which began two days before seeking medical care. Pain was associated with ipsilateral tearing, semiptosis and nasal congestion. A cervical arterial magnetic resonance angiography demonstrated left carotid artery dissection in the C1/C2 segment of the left internal carotid artery. The patient became asymptomatic after indomethacin use. We conclude that the possibility of investigating carotid dissection should be considered in patients with paroxysmal hemicrania.

Aura in trigeminal autonomic cephalalgia is probably mediated by comorbid migraine with aura

Objective The presence of aura is rare in cluster headache, and even rarer in other trigeminal autonomic cephalalgias. We hypothesized that the presence of aura in patients with trigeminal autonomic cephalalgias is frequently an epiphenomenon and mediated by comorbid migraine with aura. Methods The study retrospectively reviewed 480 patients with trigeminal autonomic cephalalgia in a tertiary medical center for 10 years. Phenotypes and temporal correlation of aura with headache were analyzed. Trigeminal autonomic cephalalgia patients with aura were further followed up in a structured telephone interview. Results Seventeen patients with aura (3.5%) were identified from 480 patients with trigeminal autonomic cephalalgia, including nine with cluster headache, one with paroxysmal hemicrania, three with hemicrania continua, and four with probable trigeminal autonomic cephalalgia. Compared to trigeminal autonomic cephalalgia patients without aura, trigeminal autonomic cephalalgia patients with aura were more likely to have a concomitant diagnosis of migraine with aura (odds ratio [OR] = 109.0, 95% CI 30.9–383.0, p < 0.001); whereas the risk of migraine without aura remains similar between both groups (OR = 1.10, 95% CI = 0.14–8.59, p = 0.931). Aura was more frequently accompanied with migraine-like attacks, but not trigeminal autonomic cephalalgia attacks. Interpretation In most patients with trigeminal autonomic cephalalgia, the presence of aura is mediated by the comorbidity of migraine with aura. Aura directly related to trigeminal autonomic cephalalgia attack may exist but remains rare. Our results suggest that aura may not be involved in the pathophysiology of trigeminal autonomic cephalalgia.

Indomethacin has no effect on trigeminally provoked parasympathetic output

Background Unlike other non-steroidal anti-inflammatory drugs, indomethacin has been shown to be highly effective in two forms of trigeminal autonomic cephalalgias, hemicrania continua and paroxysmal hemicrania and in some forms of idiopathic stabbing headaches. This specificity is unique in the headache field. Previous findings suggest the involvement of the trigeminal autonomic reflex to play an important role in the pathophysiology of these diseases. Methods 22 healthy participants were enrolled in a double-blind, three-day within-subject design. The participants received indomethacin, ibuprofen or placebo in a randomized order. After an incubation period of 65 min the baseline lacrimation and the lacrimation during intranasal stimulation evoked by kinetic oscillation stimulation were assessed using Schirmer II lacrimation tests. The lacrimation difference in mm was calculated and compared in a repeated measures ANOVA. Results No significant differences were found between the three conditions. Conclusion In our study, neither indomethacin nor ibuprofen had an inhibitory effect on the trigeminal autonomic reflex. We suggest that blocking this reflex may not be the treatment mechanism of indomethacin.

Pediatric-onset trigeminal autonomic cephalalgias: A systematic review and meta-analysis

Background and objective There are five headache disorders composing the trigeminal autonomic cephalalgias (cluster headache, paroxysmal hemicrania, short-lasting unilateral neuralgiform headache attacks with conjunctival injection and tearing (SUNCT), short-lasting unilateral neuralgiform headache attacks with cranial autonomic symptoms (SUNA), and hemicrania continua). Little is known about these disorders in the pediatric population. The objectives of this study are to report the full age ranges of pediatric trigeminal autonomic cephalalgias and to determine if pediatric-onset trigeminal autonomic cephalalgias display similar signs and symptoms as adult onset. Methods Search criteria in Medline Ovid, Embase, PsycINFO, and Cochrane Library were created by a librarian. The remainder of the steps were independently performed by two neurologists using PRISMA guidelines. Inclusion criteria for titles and abstracts were articles discussing cases of trigeminal autonomic cephalalgias with age of onset 18 or younger, as well as any epidemiological report on trigeminal autonomic cephalalgias (as age of onset data was often found in the results section but not in the title or abstract). Data extracted included age of onset, sex, and International Classification of Headache Disorders criteria for trigeminal autonomic cephalalgias (including pain location, duration, frequency, autonomic features, restlessness) and some migraine criteria (photophobia, phonophobia, and nausea). Studies that did not meet full criteria for trigeminal autonomic cephalalgias were examined separately as “atypical trigeminal autonomic cephalalgias”; secondary headaches were excluded from this category. Results In all, 1788 studies were searched, 86 met inclusion criteria, and most ( 56 ) examined cluster headache. In cluster headache, onset occurred at every pediatric age (range 1–18 years) with a full range of associated features. Autonomic and restlessness features were less common in pediatric patients, while migrainous features (nausea, photophobia, and phonophobia) were found at similar rates. The sex ratio of pediatric-onset cluster headache (1.8, 79 male and 43 female) may be lower than that of adult-onset cluster headache. Data for other trigeminal autonomic cephalalgias, while more limited, displayed most of the full range of official criteria. The data for atypical trigeminal autonomic cephalalgias were also limited, but the most common deviations from the official criteria were abnormal frequencies and locations of attacks. Conclusions Trigeminal autonomic cephalalgias can start early in life and have similar features to adult-onset trigeminal autonomic cephalalgias. Specifically, pediatric-onset cluster headache patients display the full range of each criterion for cluster headache (except maximum frequency of six instead of eight attacks per day). However, cranial autonomic features and restlessness occur at a lower rate in pediatrics. Additional information is needed for the other trigeminal autonomic cephalalgias. As for expanding the ICHD-3 criteria for pediatric-onset trigeminal autonomic cephalalgias, we have only preliminary data from atypical cases, which suggests that the frequency and location of attacks sometimes extend beyond the official criteria. Trial Registration: This study was registered as a systematic review in PROSPERO (registration number CRD42020165256).

The Promising Effect of Nerve Decompression in Trigeminal Autonomic Cephalalgias: Report of Case Series

Trigeminal Autonomic Cephalalgias (TAC) are excruciating headaches with limited treatment options. The chronic forms of TACs, including chronic cluster, chronic paroxysmal hemicrania, and hemicrania continua, are disabling conditions. In addition to drug therapy, there are some studies regarding nerve blocking and nerve stimulation with acceptable results. Here we report four cases of decompression nerve surgery with promising results on pain control in these difficult to treat headaches.

Long-Term Outcome of Indomethacin Treatment in Pediatric Patients with Paroxysmal Hemicrania—A Case Series

Paroxysmal Hemicrania is a rare form of primary headache in children and adolescents, belonging to the group of trigeminal autonomic cephalalgias. Patients suffer from severe, short-lasting unilateral headaches accompanied by symptoms of the autonomic system on the same side of the head. The short duration of attacks distinguishes Paroxysmal Hemicrania from other trigeminal autonomic cephalalgias. Indomethacin is the treatment of choice, and its effectiveness provides a unique diagnostic criterion. However, the long-term outcomes in children are highly underreported. In this case-series, n = 8 patients diagnosed with Paroxysmal Hemicrania were contacted via telephone 3.1 to 10.7 years after initial presentation. A standardized interview was conducted. n = 6 patients were headache-free and no longer took indomethacin for 5.4 ± 3.4 years. The mean treatment period in these patients was 2.2 ± 1.9 years. Weaning attempts were undertaken after 1.7 ± 1.3 months; in n = 3 patients, more than one weaning attempt was necessary. n = 2 patients were still taking indomethacin (4.5 and 4.9 years, respectively). Both unsuccessfully tried to reduce the indomethacin treatment (two and six times, respectively). Adverse effects appeared in n = 6 (75%) patients and led to a discontinuation of therapy in n = 2 patients. Our long-term follow-up suggests that in a substantial proportion of pediatric patients, discontinuing indomethacin therapy is possible without the recurrence of Paroxysmal Hemicrania.

Comorbid Sleep Disorders and Headache Disorders

Sleep disorders are closely intertwined with different kinds of headache disorders. In some forms of headaches, this association is profound, such as in hypnic headache, where headaches only occur during sleep, or in cluster headache, which has connection to the REM sleep. In other headaches, the association with sleep is more subtle, but nevertheless, very relevant–for instance in migraine, where sleep deprivation or excessive sleep may act as a trigger for migraine, while sleep has a curative effect on the migraine attack. This chapter focuses in the relationship between sleep disorders and headaches focusing on the five primary forms of headaches: migraine, tension-type headache, paroxysmal hemicrania, hypnic headache, and secondary form of headaches such as obstructive-sleep-apnea-related headaches and medication overuse headaches (MOH).