Brivaracetam add-on treatment in pediatric patients with severe drug-resistant epilepsy: Italian real-world evidence.

PURPOSE: To report the efficacy and tolerability of brivaracetam (BRV) in add-on therapy in pediatric patients with severe drug-resistant epilepsy. Prognostic factors of clinical outcome were also analyzed. METHODS: This Italian multicenter retrospective observational study was conducted on 45 pediatric patients with severe drug-resistant epilepsy, treated with BRV for at least 1 month and with a follow-up >6 months. Demographic, clinical, and treatment variables were assessed at T0 (baseline, BRV introduction) and T1 (6 months after BRV introduction). The response was defined as ≥50% seizure frequency reduction; responders and non-responders were then compared to assess potential prognostic factors. RESULTS: Forty-five patients (M = 28, mean age 12.4+/-4.4 years) were enrolled (focal epilepsy=14; generalized epilepsy=2; epileptic encephalopathy=29). At T1, 19/45 patients (42.2%) were responders (≥50% seizure frequency reduction), with 4 patients (8.9%) achieving a ≥ 75% seizure reduction and 2 patients (4.4%) becoming seizure free. Epilepsy onset at >12 months of age (p = 0.001), disease duration ≤6 years (p = 0.036), and lower seizure frequency at baseline (p = 0.008) were the prognostic factors significantly associated with a better prognosis. No significant difference emerged for demographics, epilepsy types/etiology, intellectual disability, or therapy variables. At T1, 21 patients (46.6%) discontinued BRV, mainly due to lack of efficacy (13 subjects; 28.9%) and adverse events in 8 patients (17.8%). CONCLUSION: Brivaracetam was an effective and tolerated treatment in pediatric patients with severe drug-resistant epilepsy, especially when the seizure onset was at >12 months of age, the epilepsy duration ≤6 years, and the seizure frequency before BRV treatment was low. Further and controlled studies are needed.

The Pediatric Trochlear Migraine: Diagnostic and Therapeutic Implications.

Trochlear Migraine has been recently described as the concurrence of strictly unilateral migraine and ipsilateral trochleodynia with relief of migraine after successful treatment of trochleodynia. This disorder has been interpreted as "cluster-tic syndrome" or "seizure-triggered migraine". Trochlear Migraine is unrecognized and rarely described in childhood. The aim of this study is to review the few cases of Trochlear Migraine reported in the literature in addition to the cases observed in our clinical experience. In particular, our cases showed recurrent attacks of severe and pulsating headache associated with nausea, vomiting, phonophobia, photophobia, and strict trochlear localization of pain. They often presented with alternating side attacks. Therefore, we suggest that the term "Trochlear Migraine" should be reserved for clinical migraine attacks strictly localized in the trochlear region, and we assume that the excessive increase in descriptions of new primary headache syndromes, according to the International Classification of Headache Disorders, can be probably be ascribed to the common physiopathological mechanisms characterizing these forms of migraine.

Migraine and handedness.

Migraine is a typically unilateral disorder in adulthood; however, the reasons for painful lateralization have been little investigated. The possible influence of manual dominance was suggested. We aimed to investigate the localization of pain in migraine attacks in right-handed and left-handed subjects. The retrospective study collected 546 patients with migraine aged between 16 and 65 years, reporting the manual dominance to the Edinburgh test. We included 466 right-handed and 80 left-handed subjects with migraine. We registered 4215 unilateral painful attacks. The right-handers had 3412 unilateral episodes; 62.8% of the attacks were characterized by pain on the right side and 37.2% by pain on the left. The left-handed subjects reported 803 unilateral pain with 63.5% of unilateral pain episodes on the left side and 36.5% of attacks with lateralized pain on the right (p < 0.001). Our data suggest that manual dominance may influence the side of pain lateralization in migraine.

Red ear syndrome in children: Review of literature and report of three cases.

BACKGROUND: Red ear syndrome (RES) is a neurological syndrome that is characterized by attacks of redness and pain that is localized in the earlobe, accompanied by a burning sensation, swelling or otalgia. The exact pathophysiology of RES is not known. Several pediatric cases have been described. They show an extreme variability in clinical presentation and therapeutic response, and therefore there are numerous difficulties in the diagnostic-therapeutic approach and in the comprehension of the physiopathology. The goal of this report is to present three clinical cases of red ear syndrome in children. These cases show various characteristics that can give useful indications regarding the differential diagnosis and the pathogenetic mechanisms that are involved, particularly when they are compared with cases published in the literature. CASE-REPORTS: We report three pediatric RES cases: 1) a boy whose condition offered a typical example of the association that occurs between migraine and RES. 2) a girl with idiopathic RES. 3) a child who suffered RES attacks that showed many similarities with trigeminal autonomic cephalalgias. CONCLUSION: Our clinical series shows the different ways in which RES can be expressed and they support the reported scientific literature. We suggest that the different forms of RES have a common final autonomic pathogenetic mechanism that is activated by parasympathetic hyperactivity and sympathetic inhibition. The different temporal characteristics, frequency, etc. may depend on the activation of distinct physiopathological modules that are related to the pain circuits, as suggested by the modular theory which describes that groups of neurons are defined as a module, where each module is responsible for a symptom and the individual's headache is defined by the activated modules.