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.

The relationship between epilepsy and migraine is complex and remains to be determined. We report 3 cases that address 2 questions on this topic. The first and second cases showed an association between migraine without aura and the onset of epileptic seizures. The third case report describes a patient in whom migraine with aura occurred and was followed by the development of status epilepticus, which occurred 2 or 3 hours after the attack of migraine with aura. We discuss the present definition of migralepsy and reassess its definition by suggesting possible extensions to its current definition.

Drugs used for prophylactic therapy of primary headaches can cause undesirable effects with long-term treatment; weight gain is often observed in clinical practice, which in some cases can become a relevant problem. In the medical literature, data on this side effect lack studies specifically assessing this parameter and thus are only partial and not always concordant [1-5].

Posterior scleritis is a rare cause of ocular pain, due to scleral inflammation, presenting with periocular pain, pain on movement and decreased vision. Although anterior scleritis may be associated with this condition, ocular signs may be absent. We report a case of posterior scleritis, presenting with right-sided ocular and periocular pain, exacerbated by ocular movements, irradiating to the ipsilateral temple and zygoma, not associated with visual disturbances at onset. Diagnosis was made with ultrasonography and confirmed by brain and orbital MRI. Differential diagnosis of facial pain, in particular, affecting the periorbital region, is discussed.In the presence of ocular pain, even in the absence of ocular signs, an ophthalmologic consultation should be performed.

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