First Attack and Clinical Presentation of Hemiplegic Migraine in Pediatric Age: A Multicenter Retrospective Study and Literature Review

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.

Diagnostic and therapeutic aspects of hemiplegic migraine

“…Emotional and intense physical stress, viral infections and head trauma are the more common reported trigger factors for HM attacks. 11 12 Table 1 describes diagnostic criteria for HM, according to the definition of the third edition of International Classification of Headache Disorders. 3…”

“… 20 However, HaNDL is monophasic with resolution in 3 months, while HM recur for decades 62 ; also, only a minority of attacks of HaNDL include visual symptoms, while visual aura is very common in HM. 11 76 …”

Ophthalmological Findings in Joubert Syndrome and Related Disorders

“…38 39 40 41 42 43 44 45 46 47 48 In fact, in recent years, next-generation sequencing technologies, including exome and mRNA sequencing studies, have been shown to be essential in dissecting the pathophysiology of a wide array of childhood disorders. 49 50 51 52 53 54 55 56 57 58 59 This led to a better understanding of the subcellular alterations underlying diseases, including several regulatory networks and genetic together with nongenetic factors. 60 61 62 63 64 65 66 67 68 69 70 Actually, there is still no effective treatment for JS patients, but our increasing understanding of the underlying genetic basis of various JS-related subtypes and the better genotype–phenotype correlations could lead to an advancement of the field in near future.…”

CACNA1A Gene Variants in Eight Chinese Patients With a Wide Range of Phenotypes

“…In rare cases, diplegia has been reported as a motor aura symptom (18). Compared with FHM, SHM patients tends to have less non-motor auras, longer and more severe attacks, manifesting as acute encephalopathy, characterized by hemiplegia, coma, fever, convulsions, and so on (19). It is thought to be associated with increased glutamic acid release, decreased cortical spreading depression (CSD) threshold, and prolonged and repetitive CSD, all of which lead to cytotoxic and vasogenic edema (17,20).…”