Red Ear Syndrome.

The Red Ear syndrome (RES) is an intriguing syndrome originally described for the first time nearly 20 years ago. RES is characterized by unilateral/bilateral episodes of pain and burning sensation of the ear, associated with ipsilateral erythema. RES episodes are indeed isolated in some patients, but they can occur in association with primary headaches, including in particular migraine in the developmental age. Although the underlying pathophysiological mechanisms are still uncertain, in the recent years the described comorbidities have aroused increasing interest because of possible clinical implications. Moreover, RES seems to be more often associated with clinical features of migraine partially provoked by the involvement of the parasympathetic system. This clinical association has shed new light on the pathophysiology of RES, supporting the hypothesis of a shared pathophysiological background, for example, through the activation of the trigeminal autonomic reflex. Current therapies of RES will be also discussed. Finally, we will resume the more controversial aspects of this relatively new and probably underestimated neurological syndrome.

Prevalence of red ear syndrome in juvenile primary headaches.

BACKGROUND: Previous studies have suggested a relationship between 'red ear syndrome' (RES) and pediatric migraine. Aims of this study were (i) to assess the frequency, specificity and sensitivity of RES in a population of pediatric migraineurs and (ii) to establish the pathophysiological mechanisms of RES associated with migraine. METHODS AND RESULTS: A total of 226 children suffering from headache (aged 4-17 years) were enrolled. One hundred and seventy-two (76.4%) were affected by migraine, the remaining 54 (23.6%) by other primary headaches. RES was followed significantly more frequently by migraine (23.3%; p < .0001), and was characterized by high specificity and positive predictive value (96.3 and 95.3%, respectively). According to the univariate statistical analysis, RES showed a statistically significant association with male gender, throbbing quality of the pain, vomiting and phonophobia. It was confirmed by a multivariate stepwise logistic regression model only for the throbbing quality of the pain, vomiting and male gender. CONCLUSIONS: Our study showed that (i) in children, RES is a highly specific sign for migraine. In addition, the evidence of an association of RES with some migraine features partially provoked by the parasympathetic system supports the hypothesis of a shared pathophysiological background (e.g. via the activation of the trigeminal-autonomic reflex).

Osmophobia in migraine classification: a multicentre study in juvenile patients.

AIMS: This study was planned to investigate the diagnostic utility of osmophobia as criterion for migraine without aura (MO) as proposed in the Appendix (A1.1) of the International Classification of Headache Disorders (ICHD-II, 2004). METHODS: We analysed 1020 patients presenting at 10 Italian juvenile headache centres, 622 affected by migraine (M) and 328 by tension-type headache (TTH); 70 were affected by headache not elsewhere classified (NEC) in ICHD-II. By using a semi-structured questionnaire, the prevalence of osmophobia was 26.9%, significantly higher in M than TTH patients (34.6% vs 14.3%). RESULTS: Osmophobia was correlated with: (i) family history of M and osmophobia; and (ii) other accompanying symptoms of M. By applying these 'new' criteria, we found an agreement with the current criteria for the diagnosis of migraine without aura (MO) in 96.2% of cases; 54.3% of previously unclassifiable patients received a 'new' diagnosis. CONCLUSIONS: In conclusion, this study demonstrates that this new approach, proposed in the Appendix (A1.1), appears easy to apply and should improve the diagnostic standard of ICHD-II in young patients too.

Migraine and cranial autonomic symptoms in children and adolescents: a clinical study.

The frequency of cranial autonomic symptoms in children affected by primary headaches is uncertain. The aim of our study was to estimate the frequency of symptoms in pediatric headaches and correlate it with main migraine characteristics. A questionnaire investigating the presence of cranial autonomic symptoms was administered to all children with primary headache for 2 years. A total of 230 children with primary headache (105 males, 125 females) were included. Two hundred two children were affected by migraine and 28 (12.2%) by other primary headaches. Cranial autonomic symptoms were significantly complained by migraineurs (55% vs. 17.8%) (P < .001) and by children with higher frequency of migraine attacks (odds ratio = 2.6, confidence interval = 1.4-4.7, P = .001). Our findings show that cranial autonomic symptoms are rather common during pediatric migraine attacks. The association between cranial autonomic symptoms and higher frequency of attacks might suggest the role of the trigeminal-autonomic reflex in migraine pathophysiology.

Transient topographical amnesia and cingulate cortex damage: a case report.

Transient topographical amnesia (TTA) is the temporary inability to find one's way in familiar or unfamiliar surroundings due to the inability to use well known environmental landmarks for route finding. The syndrome has not been described as having any obvious aetiology and has been thought to be caused by a vascular deficit in right hemispheric structures which are crucial for topographic recognition, i.e. parietal association and parahippocampal cortex. The patient described in the present study complained of several critical episodes of TTA and tonic rigidity of the left limbs. Neuropsychological assessment was normal except for a deficit in spatial memory tasks. Magnetic resonance (MR) imaging of the brain showed an angioma at the border of areas 24d and 23 of the right cingulate cortex. Because area 23 is strategically located in a network that links the parietal associative (area 7a) and parahippocampal cortices, and because these cortical areas are involved in topographical orienting processes, we suggest that a transient functional inactivation of the network caused by epileptic discharges spreading from the damaged cingulate cortex towards the parahippocampal and parietal association cortex could account for the spatial disorder. Similar discharges spreading from area 24d towards the primary motor cortex and/or the spinal cord could account for the episodes of tonic rigidity of the left limbs.

[Time course analysis of the effects of intrapeduncular injection of kainic acid on amygdaloid paroxysmal activity]. / Analisi temporale degli effetti dell' inoculazione entopeduncolare di acido kainico sull' attivita' parossistica amigdaloidea.

Intrapeduncolar intrapeduncolar entopeduncolar injection of kainic acid determines an early decrease of the evoked amygdaloid paroxysmal activity, and a later increase of the AD length. The diphasic effect of the injection of kainic acid into the entopeduncular nucleus is likely due to the structural analogy of this drug with a neuroexcitant amino acid, monosodium glutamate. The inhibitory role of the entopeduncolar nucleus on the paroxysmal phenomena evoked in the amygdala is emphasized.

Modifications induced on the amygdaloid paroxysmal activity by entopeduncolar or nigral injection of kainic acid, in the cat.

Entopeduncolar or nigral injection of kainic acid determines an early decrease of the evoked amygdaloid paroxysmal activity and a later increase of the after discharge duration. This biphasic effect is likely due to the structural analogy of the drug with monosodium glutamate, a neuroexcitant amino acid. The data suggest that the basal ganglia exert a tonic control on the amygdaloid activity.

Migraine headaches in adolescents: a student population-based study in Monreale.

We assessed the prevalence of migraine headaches in an epidemiological survey of an 11 to 14-year-old student population. Migraine headaches were classified on the basis of questionnaires and neurological examination using the operational diagnostic criteria of the International Headache Society. Prevalence of migraine without aura (IHS code 1.1) was 2.35%; that of migraine with aura (IHS code 1.2) was 0.62%. Migraine without aura was equally distributed among males and females, whereas migraine with aura was preponderant in the female cohort. The prevalence of migraine headaches in males was constant through the ages studied, whereas the prevalence of migraine headaches in females reached a peak at age 12 and plateaued over the following two years. Although the new IHS classification criteria of migraines are reliable and exhaustive, some subcriteria may not be valid in a juvenile population. For instance, the duration of the pain in young migraineurs is often briefer than in adults, and the intensity of pain was almost always described as moderate or severe. Therefore, in order to increase the reliability and comprehensiveness of the IHS classification, minor modifications should be made.

The IHS classification criteria for migraine headaches in adolescents need minor modifications.

The operational and diagnostic criteria for migraine and all other headache disorders released in 1988 by the International Headache Society are universally considered reliable and exhaustive. These criteria, however, cannot be considered as satisfactory for population-based studies on migraine prevalence, especially if adolescents are the subjects of the study. Using these diagnostic criteria, we conducted an epidemiological study in order to assess the prevalence of migraine headache in a student population aged 11 to 14 years. Our survey made it possible to code IHS 1.1 (migraine without aura) in 2.35%, IHS 1.2 (migraine with aura) in 0.62%, IHS 1.7 (migrainous disorders not fulfilling migraine criteria) in 1.52%, and IHS 13 (headache not classifiable) in 1.38% of the examined pupils. In adolescents, the low prevalence estimates of migraine headache coded IHS 1.1 and the relatively high prevalence estimates of headaches coded IHS 1.7 and IHS 13 have appeared to be a consequence of the rigidity of some operational diagnostic criteria of the recent IHS classification rather than of the geographical, environmental, or socioeconomical peculiarities of the cohort. Therefore, in order to improve the reliability and the exhaustiveness of the IHS classification by increasing its sensitivity, we believe that minor modifications of the diagnostic criteria are necessary. Within these revised criteria, the subitem "moderate or severe intensity" of pain headache should become mandatory, whereas the lower limit of the criterion "duration of pain" should be reduced to 1 hour.

Trigeminal neuralgia and cerebellopontine-angle lipoma in a child.

Trigeminal neuralgia and cerebellopontine-angle lipomas are very rare in children. We describe the history and findings of an 8-year-old boy with right trigeminal neuralgia and a lipoma detected by magnetic resonance imaging at the level of the root-entry zone of the right seventh cranial nerve. We propose a possible mechanism of infiltration of the trigeminal rootlets by the lipoma.