First Attack and Clinical Presentation of Hemiplegic Migraine in Pediatric Age: A Multicenter Retrospective Study and
Irene Toldo1, Francesco Brunello1, Veronica Morao1
1Department of Woman's and Child's Health, Juvenile Headache Centre, University Hospital of Padua, Padua, Italy.
Insights
Pediatric hemiplegic migraine (PHM) presents heterogeneously, with sporadic cases experiencing more severe attacks and familial cases having more frequent ones. Despite early onset and severe attacks, most children showed favorable clinical outcomes.
Area of Science:
- Neurology
- Pediatrics
- Genetics
Background:
- Clinical data on pediatric hemiplegic migraine (HM) are scarce.
- This study details the phenotype of sporadic (SHM) and familial (FHM) pediatric HM.
- A literature review of pediatric HM was conducted.
Purpose of the Study:
- To describe the clinical presentation of pediatric HM at onset and during early disease years.
- To compare phenotypic features between SHM and FHM in children.
- To review existing literature on pediatric HM.
Main Methods:
- Retrospective analysis of 46 pediatric HM cases from 11 Italian centers.
- Patients were categorized into SHM (32) and FHM (14) subgroups.
- Data included age at onset, aura duration, attack frequency, and clinical evolution.
Main Results:
- Mean age at onset was 10.5 years; motor aura averaged 3.5 hours.
- SHM attacks were more prolonged than FHM attacks.
- Sensory and basilar-type auras were common; FHM patients had more frequent attacks, while SHM patients had more severe attacks.
Conclusions:
- This is the first Italian pediatric HM series, revealing heterogeneous onset.
- Children with HM exhibit fewer non-motor auras than adults.
- Most pediatric HM cases, even severe ones, showed favorable clinical evolution.
Abstract:
Background: Data on clinical presentation of Hemiplegic Migraine (HM) are quite limited in the literature, particularly in the pediatric age. The aim of the present study is to describe in detail the phenotypic features at onset and during the first years of disease of sporadic (SHM) and familial (FHM) pediatric hemiplegic migraine and to review the pertinent literature. Results: Retrospective study of a cohort of children and adolescents diagnosed with hemiplegic migraine, recruited from 11 Italian specialized Juvenile Headache Centers. Forty-six cases (24 females) were collected and divided in two subgroups: 32 SHM (16 females), 14 FHM (8 females). Mean age at onset was 10.5 ± 3.8 y (range: 2-16 y). Mean duration of motor aura was 3.5 h (range: 5 min-48 h). SHM cases experienced more prolonged attacks than FHM cases, with significantly longer duration of both motor aura and of total HM attack. Sensory (65%) and basilar-type auras (63%) were frequently associated to the motor aura, without significant differences between SHM and FHM. At follow-up (mean duration 4.4 years) the mean frequency of attacks was 2.2 per year in the first year after disease onset, higher in FHM than in SHM cases (3.9 vs. 1.5 per year, respectively). A literature review retrieved seven studies, all but one were based on mixed adults and children cohorts. Conclusions: This study represents the first Italian pediatric series of HM ever reported, including both FHM and SHM patients. Our cohort highlights that in the pediatric HM has an heterogeneous clinical onset. Children present fewer non-motor auras as compared to adults and in some cases the first attack is preceded by transient neurological signs and symptoms in early childhood. In SHM cases, attacks were less frequent but more severe and prolonged, while FHM patients had less intense but more frequent attacks and a longer phase of active disease. Differently from previous studies, the majority of our cases, even with early onset and severe attacks, had a favorable clinical evolution.
