Coexistence of neuropathic corneal pain, corneal nerve abnormalities, depression, and low quality of life.

PURPOSE: To evaluate the quality of life (QoL), mental health conditions and corneal morphology in neuropathic corneal pain (NCP) subjects without a significant ocular surface disease. METHODS: A composite questionnaire was administered to 228 consecutive subjects, assessing the pain intensity, duration, and quality using a modified version of the Self-Administered Leeds Assessment of Neuropathic Symptoms and Signs (S-LANSS) and Pain Detect (PD) questionnaires. Subjects diagnosed with possible central NCP and two sub-groups of patients diagnosed with peripheral ocular pain completed an additional battery of mental health questionnaires and were examined by In Vivo Confocal Microscopy (IVCM). RESULTS: Of the 76 subjects that reported chronic ocular pain (duration >1 month), 53 were classified with probable NCP. Nine subjects without signs that justify the pain and non-responding to topical anaesthesia, were considered affected by central NCP. In these patients, a significant negative correlation was found between the presence pain and the mental component of the QoL (R2 = 0.733), and a positive correlation between the severity of pain the presence post-traumatic stress disorder (R2 = 0.83) and depression (R2 = 0.93). Although neuromas and sprouting had higher frequency in the central NCP group compared the control groups, these differences was not statistically different. CONCLUSIONS: The assessment of ocular pain characteristics using multiple questionnaires and IVCM may help to recognize differences between nociceptive and neuropathic pain. An association between pain intensity and mental health condition may guide the therapeutical choices.

Efficacy of a long-term acting triptan for Headache attributed to aeroplane travel: a case report.

Headache attributed to aeroplane travel (AH) is a well-defined nosological entity whose diagnostic criteria have been published in the third provisional International Classification of Headache Disorders (ICHD) and confirmed in the definitive version. Despite the severe intensity of pain, less than half of the AH cases described used medications for preventing the attack. The most frequent prophylactic therapy spontaneously used by sufferers are simple analgesics, non-steroidal anti-inflammatory drugs (NSAIDs), and nasal decongestants, achieving a complete or partial benefit in about 50% of patients. A complete response of AH to fast-acting triptans has been reported. We describe the case of a 37-year-old migrainous woman suffers from AH in about 75% of her flights who preempted the attacks by using a long-acting triptan (frovatriptan). Giving triptans' mechanism of action, an involvement of the trigemino-vascular system in the pathogenesis of AH could be advanced.

The relevance of migraine in the clinical spectrum of mitochondrial disorders.

Recent scientific evidence suggests a link between migraine and brain energy metabolism. In fact, migraine is frequently observed in mitochondrial disorders. We studied 46 patients affected by mitochondrial disorders, through a headache-focused semi-structured interview, to evaluate the prevalence of migraine among patients affected by mitochondrial disorders, the possible correlations between migraine and neuromuscular genotype or phenotype, comorbidities, lactate acid levels and brain magnetic resonance spectroscopy. We explored migraine-related disability, analgesic and prophylactic treatments. Diagnoses were achieved according to International Classification of Headache Disorders, 3rd edition. Lifetime prevalence of migraine was 61% (28/46), with high values in both sexes (68% in females, 52% in males) and higher than the values found in both the general population and previous literature. A maternal inheritance pattern was reported in 57% of cases. MIDAS and HIT6 scores revealed a mild migraine-related disability. The high prevalence of migraine across different neuromuscular phenotypes and genotypes suggests that migraine itself may be a common clinical manifestation of brain energy dysfunction. Our results provide new relevant indications in favour of migraine as the result of brain energy unbalance.

A prospective study on osmophobia in migraine versus tension-type headache in a large series of attacks.

BACKGROUND: In literature, osmophobia is reported as a specific migrainous symptom with a prevalence of up to 95%. Despite the International Classification of Headache Disorders 2nd edition proposal of including osmophobia among accompanying symptoms, it was no longer mentioned in the ICHD 3rd edition. METHODS: We conducted a prospective study on 193 patients suffering from migraine without aura, migraine with aura, episodic tension-type headache or a combination of these. After a retrospective interview, each patient was asked to describe in detail osmophobia, when present, in the following four headache attacks. RESULTS: In all, 45.7% of migraine without aura attacks were associated with osmophobia, 67.2% of migraineurs reported osmophobia in at least a quarter of the attacks. No episodic tension-type headache attack was associated with osmophobia. It was associated with photophobia or phonophobia in 4.3% of migraine without aura attacks, and it was the only accompanying symptom in 4.7% of migraine without aura attacks. The inclusion of osmophobia in the ICHD-3 diagnostic criteria would enable a 9.0% increased diagnostic sensitivity. CONCLUSION: Osmophobia is a specific clinical marker of migraine, easy to ascertain and able to disentangle the sometimes challenging differential diagnosis between migraine without aura and episodic tension-type headache. We recommend its inclusion among the diagnostic criteria for migraine as it increases sensitivity, showing absolute specificity.

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

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.

Prevalence of headache attributed to aeroplane travel in headache outpatient populations: An Italian multicentric survey.

BACKGROUND: To assess the prevalence of headache attributed to aeroplane travel (AH) in patients referred to Italian Headache Centres. MATERIAL AND METHOD: 869 consecutive patients visiting six Italian headache centres during a 6 month-period (October 2013 to March 2014) were enrolled in the survey. Among them, 136 (15.6%) had never flown and therefore were excluded from the study. The remaining 733 patients (f = 586, m = 147; age 39.1 ± 17.3) were asked about the occurrence of headache attacks during flight; those who answered the question positively filled in a detailed questionnaire that allowed the features of the attacks to be defined. RESULTS: Headache attacks during the flight was reported by 34/733 subjects; four presented attacks fulfilling ICHD-3 beta (1) criteria for migraine without aura and therefore were not further considered. The features of the remaining 30 (4.0%; m = 18, f = 12, age 36.4 ± 7.3) completely fulfilled the ICHD-3 beta criteria for AH. In more detail, the pain was unilateral (fronto-orbital: n = 23; fronto-parietal: n = 7; without side-shift: n = 25, with side-shift: n = 5), lasting up to 30 min in 29 subjects. All the patients reported the pain as very severe or unbearable and landing as the phase of travel in which the attack appeared. In four cases, a postictal, milder, dull headache could last up to 24 hours. Accompanying symptoms were present in eight cases (restlessness: n = 5; conjunctival injection and tearing: n = 2; restlessness + ipsilateral conjunctival injection and tearing: n = 1). The fear of experiencing further attacks negatively affected the propensity for future flights in 90.0% of subjects (n = 27). In all the patients, AH onset did not coincide with the first flight experience. Concomitant migraine without aura was diagnosed in 24, tension-type headache in four, migraine without aura + tension-type headache in two cases; none suffered from cluster headache. Five subjects reported AH on each flight, 20 in > 50% of flights, five occasionally. Despite the severe intensity of the pain, only one third of this sample spontaneously reverted to a pharmacological treatment; the most useful strategy combines a decongestant nasal spray plus the intake of a simple analgesic 30 min before the estimated attack. Spontaneous manoeuvres were applied by 18 patients (Valsalva-like: n = 12; compression: n = 2; both manoeuvres: n = 4), more often without significant improvement. These data confirm our previous finding on the clinical features of AH. CONCLUSION: AH was found in 4.0% of a multicentre, large sample of patients with flight experiences. Although limited to a sample of patients followed in six Italian headache centres, to the best of our knowledge these are the first epidemiological data on AH gathered by direct interview. If properly investigated, AH seems to be a not infrequent condition, which, when diagnosed, could probably be prevented in many cases.

Headache Attributed to Aeroplane Travel: An Historical Outline.

INTRODUCTION: Headache attributed to aeroplane travel (AH) is one of the new nosological entries in the International Classification of Headache Disorders (ICHD) 3 beta. MATERIALS AND METHODS: This study retraces the history of headache related to flight, from the initial description to the modern reports, on the basis of original sources. RESULTS: Head pain related to flight has been reported since the beginning of flight era. However, most of those early cases are easily recognized as secondary to an exceptional barotrauma and/or a sinusitis concomitant with the flight. Instead, contemporary research identified a new nosological entity, AH, which by definition occurs in normal flying conditions and in absence of any sinus pathology. Moreover, we identified recently similar forms of headache, triggered by sudden changes of external pressure (rapid descent from mountain, mountain descent headache, MDH); and ascent from diving, diving ascent headache (DAH). CONCLUSION: Head pain related to flight has been reported since the origin of aviation, but it was caused by an exceptional barotrauma or a respiratory infection. Instead, AH occurs in normal flying conditions. Two newly identified conditions, MDH and DAH, appear to share a common pathogenetic mechanism with AH: namely the imbalance between intrasinusal and external air pressure. Therefore, we assert that an expansion of this entity of AH is something that should be considered in the next ICHD.

The pathological spectrum behind migraine aura status: a case series.

BACKGROUND: The recently released International Classification of Headache Disorders-3rd edition (1) includes migraine aura status (MAS) among the complications of migraine (A1.4.5). It is defined as the recurrence of at least three auras over a period of 3 days, in a patient suffering from migraine fulfilling criteria for 1.2 Migraine with aura (MA) or one of its subtypes. CASE SERIES: We describe three cases of MAS secondary to an organic brain lesion: a migrainous infarction, an acute ischemic stroke secondary to a vertebral artery dissection, and an inflammatory demyelinating disease of the central nervous system. CONCLUSIONS: In front of a patient with a MAS, an organic lesion of the brain must be suspected, until a complete negative vascular and neuroradiological diagnostic workup has been performed. A spectrum of underlying pathologies (vascular or demyelinating diseases, epileptic or degenerative conditions) may cause a MAS-like clinical onset. The variability of aura symptoms may result in a real diagnostic challenge.

Sports, physical activity and headache in the classical age: historical descriptions from the first sports textbook, "De arte gymnastica", by Girolamo Mercuriale.

BACKGROUND AND OBJECTIVES: The relationship between physical activity, sports and headache presents a growing interest, testified by numerous papers recently published. The correlation between headache and sporting activities or physical exercise dates back to the classical age. We aim at promoting the development of more studies focused on the relationship between headache and physical activity. METHODS: We analysed the book "De arte gymnastica", written by Girolamo Mercuriale (Forlì, Italy, 1530-1606), and considered the first "sports medical textbook". We discuss these classical literature findings in the light of the International Classification of Headache Disorders, 3rd edition. RESULTS: The Author's work derives from the systematic revision of Greek, Roman and Arabic literatures about the matter. Despite some references to inveterate headaches or cold-related pains, Mercuriale does not gather specific clinical characterisations of different types of headache. However, interestingly, he reports detailed descriptions of how the same sport, or the same physical activity, could cause or give relief from head pain, depending on the precise way of practising. Mercuriale summarises 18 sports or physical activities that can give relief from headache; conversely, running or heavy activities, such as boxing, appear among the 12 contraindicated sports for people suffering from headache. CONCLUSIONS: "De arte gymnastica", by Girolamo Mercuriale, is the first textbook on sports medicine. Headache if often cited along the treatise: different sports and physical activities, or various ways of practising the same action could produce opposite effects for people suffering from headache.

Acute-on-chronic subdural hematoma: the death of the famous XIX century soprano Maria Malibran-a study of the sources.

Maria Malibran (1808-1836) is one of the most famous sopranos of the nineteenth century. In 1825, along with her father, the renowned tenor Manuel Garcia, she introduced the Italian opera in America for the first time. The European debut in Paris (1828) definitively crowned her as a star. Thus, she was requested by the most famous European theaters. In July 1836, during an equestrian excursion in London, she fell from her horse dashing her head against the ground, resulting in a state of insensibility. Since that accident, she had suffered from continual headache and nervous attacks, but she continued to work. In September 1836, she attended a music festival in Manchester, but her health rapidly worsened: episodes of nervous attacks, headache, and fainting occurred with higher frequency. At the end of a representation, she was attacked by violent convulsions. In the following days, she was laid in a kind of stupor. Afterward, she died at the age of 28. The hypothesis that prolonged efforts during her performance could have provoked a rebleeding of a pre-existent chronic subdural hematoma should be taken into account as a possible cause of death.

When migraine mimics stroke: A systematic review.

BACKGROUND: Migraine with aura may mimic an acute ischemic stroke, so that an improper administration of thrombolytic treatment can expose migrainous patients to severe adverse effects. METHODS: This systematic review quantifies the relevance of migraine with aura among stroke mimics, checking for thrombolysis' safety in these patients. We reviewed the literature after 1995, distinguishing from studies dealing with stroke mimics treated with systemic thrombolysis and those who were not treated with systemic thrombolysis. RESULTS: Migraine with aura is responsible for 1.79% (CI 95% 0.82-3.79%) of all the emergency Stroke Unit evaluations and it represents 12.24% (CI 95% 6.34-22.31%) of stroke mimics in the group not treated with systemic thrombolysis. 6.65% (CI 95% 4.32-9.78%) of systemic thrombolysis administrations are performed in patients without an acute ischemic stroke. Migraine with aura is responsible for 17.91% of these (CI 95% 13.29-23.71%). The reported rate of adverse events seems extremely low (0.01%). CONCLUSION: Migraine with aura is the third most common stroke mimic, following seizures and psychiatric disorders; it is responsible for about 18% of all improper thrombolytic treatments. Despite the absence of strong supporting data, thrombolysis in migraine with aura seems to be a procedure with an extremely low risk of adverse events.

A lost track in ICHD 3 beta: A comprehensive review on osmophobia.

Background Osmophobia (Os) has been reported to be much more prevalent in migraine (M) than in other primary headaches, and its high specificity in the differential diagnosis between M and tension-type headache (TTH) has been reported. Os was included in the ICHD II Appendix as a diagnostic criterion of M. It disappeared in ICHD-3 beta. To understand this choice, we reviewed the literature after 2004. Methods This was a systematic review. We searched in PubMed, MEDLINE and Cochrane library for "osmophobia", "odour/odorphobia AND headache", "odour/odor hypersensitivity AND headache" and "olfactory hypersensitivity AND headache". Results 112 papers cited Os as an accompanying symptom of headache; 16 focused on Os in M diagnosis. With the data from 40 articles, we calculated the pooled prevalence of Os in 14,360 patients (2281 pediatric) affected by M (n = 12,496) and TTH (n = 1864). In M, the prevalence was 48.5% (CI 95% 41.4 to 55.8%) in adults and 23.4% (CI 95% 15.7 to 33.4%) in pediatric patients; in TTH, the prevalence was 8.9% (CI 95% 4.6 to 13.5%) in adults and 7.9% (CI 95% 3.3 to 18.1%) in pediatric patients. Ten of these papers allowed us to calculate the sensibility and specificity of Os in differential diagnosis between M and TTH. In adults, the value of specificity was 94.1% (CI 95% 88.9 to 96.9%), and sensitivity was 51.4% (CI 95% 38.4 to 64.2%). In pediatric patients, specificity was 92.0% (CI 95% 81.9 to 96.7%), and sensitivity was 22.1% (CI 95% 10.1 to 41.8%). Conclusion The literature endorses the inclusion of Os among M diagnostic criteria. On this ground, the decision to remove Os from ICHD 3 beta appears unjustified and a revision of this choice is recommended.

Aeroplane headache, mountain descent headache, diving ascent headache.. Three subtypes of headache attributed to imbalance between intrasinusal and external air pressure?

Background Headache attributed to aeroplane travel (AH) is currently codified in Chapter 10.1.2 of the International Classification of Headache Disorders 3 beta (ICHD 3 beta). After our previous papers, the investigation was enlarged to 200 patients. Material and methods People affected by AH contacted us by mail and agreed to fill in a detailed anonymous questionnaire that allowed a thorough definition of the headache features. Results This extended case series allows us to confirm most of the clinical features of AH, reported in our previously published article, and to partially modify others. New results mainly regard the higher occurrence of accompanying symptoms and the presence of a long lasting mild headache phase, usually not exceeding 24 h, that follows the short lasting acute pain, the duration of which has been confirmed as less than 30 min. Moreover, headache attacks identical to those of AH are reported in a portion of patients, triggered by the ascent during free/snorkeling or scuba diving and rapid descent from high mountains. Conclusion The coexistence of AH with headache triggered by different environmental factors (ascent during free/scuba diving and descent from high mountains) indicates a common causal mechanism, that is, the imbalance between intrasinusal and external air pressure. We propose to classify them together, within Chapter 10 " Headache attributed to disorders of homoeostasis", under a unique heading " Headache attributed to imbalance between intrasinusal and external air pressure".

Cluster headache: When to worry? Two case reports.

Background The clinical criteria for cluster headache (CH) are included in Chapter 3 of the International Classification of Headache Disorders, 3rd beta edition (ICHD-III). CH may sometimes be secondary to other pathologies. Case reports We report two patients in whom the clinical features of CH initially fulfilled the ICHD-III criteria, but who later presented some radical modifications in headache natural history as a result of a secondary pathology. The first case of CH was secondary to a pontine cavernous angioma and the second to a cerebral venous thrombosis. Conclusion We highlight the importance of clinical modifications of CH that could suggest clinical investigations should be performed or repeated to exclude a secondary pathology in a previously diagnosed cluster headache. Some of the pathological mechanisms of CH and brain lesions are discussed.