Very young age of onset in trigemino-autonomic cephalalgias – case report and review of the literature

Abstract

Background Trigemino-autonomic cephalalgias are very rare disorders and even rarer in children and adolescents. We report the onset of paroxysmal hemicrania in a very young girl and reviewed the scientific literature for similar cases. Findings We describe the case of a 1.6-year-old girl with left-sided headache attacks fulfilling the criteria of paroxysmal hemicrania including prompt responsiveness to indomethacin. In addition, we detected at least two children for every trigemino-autonomic cephalalgias subtype with an age of under 7 years at the onset of the trigemino-autonomic cephalalgias. Remarkable features were a vast majority of chronic course from onset on and left-sided attacks. Conclusion Although very rare, trigemino-autonomic cephalalgias can occur even in very young children under the age of 6 years. This should be known in neuropaediatrics.

Paroxysmal hemicrania in children and adolescents: A systematic review

“…A total of 182 records were identi ed and reduced to 116 after removing duplicates. After screening and the application of the eligibility criteria, 22 articles (10,11,(21)(22)(23)(24)(25)(26)(27)(28)(29)(30)(31)(32)(33)(34)(35)(36)(37)(38)(39)(40) met the inclusion criteria. Figure 1 presents the ow diagram of included and excluded articles of our review according to PRISMA (41).…”

“…Among previous studies in the pediatric population, CPH, EPH, and not-speci ed-PH were reported in 23, 7, and 5 patients, respectively. PH has been described among children and adolescents in various parts of the world including the United States (USA) (10,23,27,35), Canada (29,39), United Kingdom (UK) (21), Germany (22,25,30,31), France (40), Italy (26, 32, 38), Austria (34), Estonia (36, 37), Brazil (24), Portugal (11), Japan (28), and Korea (33), but the races were not speci ed in most of the studies. Unless one study, the gender of all cases was reported (10).…”

“…Despite the ICHD-3 diagnostic criteria for the indomethacin trial, it seems that some cases might benefit from other treatment options as well as indomethacin, 9,28,34,39 while most of the patients showed complete response to the indomethacin trial. 21,22,[24][25][26][27]29,30,32,33,[35][36][37] another option for controlling this type of headache in children and adolescents. 20,23,31,34,37 Future studies should be more focused on comparing treatment modalities and their effectiveness in the pediatric population.…”

“…In these 35 cases that we systematically reviewed, there was no evidence of PH in the family history of children and adolescents whereas we found several cases with family history positive for migraine 25,[32][33][34][35]37 and unspecified headaches, 28,30,33,34 breath-holding, 35 and bipolar disorder. 22 Almost all patients benefited from indomethacin and showed complete response to treatment, 21,22,[24][25][26][27]29,30,32,33,[35][36][37]39 while some needed combination treatment of indomethacin with other medications, such as a combination of topiramate and sumatriptan, 20 verapamil, 23,34 sodium valproate, 37 and topiramate. 37 [Correction added on 22 August 2022, after first online publication: In the preceding sentence, Refs.…”

“…In EPH, attacks occur in periods lasting seven days to one year separated by pain-free periods lasting 1 month or longer. In CPH, attacks occur for more than a year without remission or with remissions lasting less than a month (5,6).…”