Orofacial Migraine and Other Idiopathic Non-Dental Facial Pain Syndromes: A Clinical Survey of a Social Orofacial Patient Group

: Background: Orofacial pain syndromes (OFPs) are a heterogeneous group of syndromes mainly characterized by painful attacks localized in facial and oral structures. According to the International Classification of Orofacial Pain (ICOP), the last three groups (non-dental facial pain, NDFP) are cranial neuralgias, facial pain syndromes resembling primary headache syndromes, and idiopathic orofacial pain. These are often clinical challenges because the symptoms may be similar or common among different disorders. The diagnostic efforts often induce a complex diagnostic algorithm and lead to several imaging studies or specialized tests, which are not always necessary. The aim of this study was to describe the encountered difficulties by these patients during the diagnostic-therapeutic course. Methods: This study was based on the responses to a survey questionnaire, administered to an Italian Facebook Orofacial Patient Group, searching for pain characteristics and diagnostic-therapeutic care courses. The questionnaire was filled out by patients affected by orofacial pain, who were 18 years and older, using a free online tool available on tablets, smartphones, and computers. Results: The sample was composed of 320 subjects (244F/76M), subdivided by age range (18-35 ys: 17.2%; 36-55 ys: 55.0%; >55 ys 27.8%). Most of the patients were affected by OFP for more than 3 years The sample presented one OFP diagnosis in 60% of cases, more than one in 36.2% of cases, and 3.8% not classified. Trigeminal neuralgia is more represented, followed by cluster headaches and migraines. About 70% had no pain remission, showing persisting background pain (VAS median = 7); autonomic cranial signs during a pain attack ranged between 45 and 65%. About 70% of the subjects consulted at least two different specialists. Almost all received drug treatment, about 25% received four to nine drug treatments, 40% remained unsatisfied, and almost 50% received no pharmacological treatment, together with drug therapy. Conclusion: To the authors' knowledge, this is the first study on an OFP population not selected by a third-level specialized center. The authors believe this represents a realistic perspective of what orofacial pain subjects suffer during their diagnostic-therapeutic course and the medical approach often results in unsatisfactory outcomes

Non-invasive Brain Stimulation in Pediatric Migraine: A Perspective From Evidence in Adult Migraine

Pediatric migraine remains still a challenge for the headache specialists as concerns both diagnostic and therapeutic aspects. The less ability of children to describe the exact features of their migraines and the lack of reliable biomarker for migraine contribute to complicate the diagnostic process. Therefore, there's need for new effective tools for supporting diagnostic and therapeutic approach in children with migraine. Recently, promising results have been obtained in adult headache by means of application of neurostimulation techniques both for investigating pathophysiological mechanisms and also for therapeutical applications. Non-invasive brain stimulation (NIBS) techniques like transcranial magnetic stimulation (TMS) and transcranial direct current stimulation (tDCS) indeed proved to be generally safe and showing also some evidence of efficacy particularly for the symptomatic treatment. On such basis, in the last years increasing interest is rising in scientific pediatric community to evaluate the potential of such approaches for treatment pediatric headaches, particularly in migraine, even if the evidence provided is still very poor. Here we present a perspective for application of TMS and tDCS technique in children migraine principally based on evidence coming by studies in adults

Study on “Atypical” Migraine Auras in the Pediatric Age: The Role of Cortical Spreading Depression and the Physiopathogenetic Hypothesis Arising from Our Clinical Cases

Migraine is a complex neurologic disorder by which several systems of the central nervous system (autonomous system, affective, cognitive, sensory and motor system) may be affected on different levels. About a fourth of migraine patients have migraine auras. The most common aura is the visual aura followed by the sensorial aura but motor deficits, as well as deficits of higher cortical centers (disorders of thinking, orientation, coherence, or concentration), may occur as well. In analogy with a headache diary, an aura diary can deliver important help in the diagnostic process of rare migraine manifestations and prevent the under-diagnosis of unusual migraine manifestations. Complex migraine manifestations are a diagnosis of exclusion, and a broad diagnostic work-up is necessary in order to exclude dangerous neurologic pathologies. In addition, here, we discuss the atypical clinical presentation and possible physio-pathogenetic related aspects of these atypical migraine aura features in the developmental age. In addition, we wanted to stress and analyze the clinical aspects of our children/adolescents with atypical auras, which seem to be more difficult to frame with the mechanisms originally proposed to explain the physio-pathogenetic relationship between CSD and aura. Finally, we discuss in detail the complex aspects of this topic on the basis of available data and propose new terminology: “Multiple, Synchronous and Asynchronous, Cortical and Subcortical Spreading Depression”

Effectiveness of a digital platform for sharing knowledge on headache management: a two-year experience

It is crucial that all headache specialists receive adequate training. Considering the unsatisfactory results obtained with standard updating courses and the growing need for continuing professional education, a digital platform was developed as a training tool. The platform has been active since 1 October 2014. It is readily accessible to doctors by free registration. Users have access to all the material available on the platform, which includes scientific articles, e-books, presentations and images. Users can share their own material and clinical cases directly. At the time of this study, the platform had 37 users. In the second year following its launch 316 files were downloaded and five discussions were started. These saw 22 contributions. Fifteen of the 37 members did not perform any action on the platform. In total, 74 files were uploaded in the second year of activity, but 90% of the contributions came from a very small group of users. There were no significant differences in use of the platform between members of the Italian Society for the Study of Headache and other specialists. Even though the platform appears to be an easily accessible, interactive and inexpensive instrument, the higher number of downloads than uploads suggests that it is used passively

Two distinct phenotypes, hemiplegic migraine and episodic Ataxia type 2, caused by a novel common CACNA1A variant

Background: To investigate the genetic and environmental factors responsible for phenotype variability in a family carrying a novel CACNA1A missense mutation. Mutations in the CACNA1A gene were identified as responsible for at least three autosomal dominant disorders: FHM1 (Familial Hemiplegic Migraine), EA2 (Episodic Ataxia type 2), and SCA6 (Spinocerebellar Ataxia type 6). Overlapping clinical features within individuals of some families sharing the same CACNA1A mutation are not infrequent. Conversely, reports with distinct phenotypes within the same family associated with a common CACNA1A mutation are very rare. Case presentation: A clinical, molecular, neuroradiological, neuropsychological, and neurophysiological study was carried out in proband and his carrier mother. The new heterozygous missense variant c.4262G > A (p.Arg1421Gln) in the CACNA1A gene was detected in the two affected family members. The proband showed a complex clinical presentation characterized by developmental delay, poor motor coordination, hemiplegic migraine attacks, behavioral dysregulation, and EEG abnormalities. The mother showed typical episodic ataxia attacks during infancy with no other comorbidities and mild cerebellar signs at present neurological evaluation. Conclusions: The proband and his mother exhibit two distinct clinical phenotypes. It can be hypothesized that other unknown modifying genes and/or environmental factors may cooperate to generate the wide intrafamilial variability

Is a digital platform useful in headache training? A 4-year Italian experience

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A Narrative Review of Visual Hallucinations in Migraine and Epilepsy: Similarities and Differences in Children and Adolescents

Since the earliest descriptions of the simple visual hallucinations in migraine patients and in subjects suffering from occipital lobe epilepsy, several important issues have arisen in recognizing epileptic seizures of the occipital lobe, which often present with symptoms mimicking migraine. A detailed quantitative and qualitative clinical scrutiny of timing and characteristics of visual impairment can contribute to avoiding mistakes. Differential diagnosis, in children, might be challenging because of the partial clinical, therapeutic, and pathophysiological overlaps between the two diseases that often coexist. Ictal elementary visual hallucinations are defined by color, shape, size, location, movement, speed of appearance and duration, frequency, and associated symptoms and their progression. The evaluation of the distinctive clinical features of visual aura in migraine and visual hallucinations in occipital epilepsy could contribute to understanding the pathogenetic mechanisms of these two conditions. This paper aims to critically review the available scientific evidence on the main clinical criteria that address diagnosis, as well as similarities and differences in the pathophysiological mechanisms underlying the visual impairment in epilepsy and migraine

Migraine in children under 6 years of age: A long-term follow-up study

Background: Early starting of migraine seems predictive for less favorable outcome in later ages, however follow-up investigations are very few and all with short-term prospective period. We report here the longest follow-up study in a population of children presenting with migraine under the age of 6. Methods: We followed-up 74 children under 6 years of age, referred for headache to our department between 1997 and 2003. The study was carried out between October 2016 and March 2018. Headache diagnoses were made according to the IHS criteria. Results: 23/74 patients, 31% of the original cohort, were found at follow-up in a period ranging between 15 to 21 years after the first visit. Seven of them were headache free. The remaining 16 patients had migraine. In the migraine group, the localization of pain changed in 75% of the subjects, 11/16 (68.7%) had allodynia and 9/16 (56.25%) had cranial autonomic symptoms. Conclusions: Our results suggest that the onset of migraine at very young age represents unfavorable prognostic factor for persistence of the disease at later ages. Some clinical features may change during clinical course, and the active persistence of the disorder may lead to an increase in allodynia

Two cases of cluster headache effectively treated with levetiracetam

Cluster headache (CH), which is characterized by recurrent attacks of short-lasting excruciating pain accompanied by signs of autonomic dysfunction (ICHD-II) (Headache Classification Subcommittee of the IHS, 2004), is the most severe of the primary headache syndromes. CH is the most common of the trigeminal autonomic cephalalgias (TACs), whose pathophysiology has not been completely elucidated (Leone and Bussone, 2009). The treatment of CH is still debated. Verapamil (a calcium antagonist) and lithium are the first-option drugs. Several drugs have been proposed as alternative treatments, but only some of these showed limited efficacy. Recently, a new-generation antiepileptic drug, topiramate (200 mg/day), was reported to ameliorate CH (Leone et al., 2003). Here we describe two cases of CH that became unresponsive to classical treatment and were effectively treated with levetiracetam (LEV)