Red ear syndrome (RES) is a rare disorder characterized by attacks of unilateral ear pain during which the ear becomes red. Episodes can occur spontaneously, or be triggered, in most cases, by rubbing or touching the ear. Both duration and frequency are variable. RES has been explained by a dysfunction of cervical spinal nerves (C3 root) and a dysregulation with disinhibition of brainstem trigemino-autonomic circuits, leading to sympathetic inhibition and parasympathetic hyperactivity producing vasodilation. We describe 6 new cases of RES with different characteristics. Although all presented the cardinal symptom of red ear, the headache patterns were suggestive of other primary headaches (migraine or cluster headache). Therapeutic response was obtained when directed to the associated primary headache phenotype, suggesting that RES may be a phenomena associated with different headaches, rather than a syndrome in itself.
ObjectiveTo evaluate the response to CPAP in patients with chronic insomnia disorder (CID) with OSAS in an unselected patient population including all OSAS severity groups. As a secondary objective, we also wanted to evaluate the differences between patients that improve insomnia symptoms with CPAP and patients that do not improve, specifically evaluating possible gender differences. MethodsRetrospective study of patients with a diagnosis of OSAS treated with CPAP and CID at the first clinical visit, selected from a database of an outpatient sleep clinic of University Hospital. ResultsFrom a database of total of 827 patient, 90 patients were identified with OSAS and CID (53.3% women). Middle / moderate OSAS was diagnosed in 68.9% and severe OSA in 31.1%. Most patients (61.1%) improved insomnia symptoms after CPAP therapy. In the responders group, 58.2% had initial insomnia, 63.6% middle insomnia and 12.7% late insomnia. Responders to CPAP were more frequently women (women 61.8%, men 38.2%, p = 0.035) and there was no other difference between responders and non-responders. On subgroup analysis, this difference was significant only in severe OSAS (women 88.9%, men 31.6%, p = 0.013). ConclusionIn most patients with CID and OSA, there is a consistent reduction of insomnia symptoms with the CPAP use. This factor emphasizes the importance of performing PSG in CID. Insomnia in men with severe OSAS responds less frequently to CPAP suggesting that in these cases the insomnia phenotype is less dependent on the respiratory symptoms.
Aim: This study aimed to characterize, clinically and neurophysiologically, a series of patients with gelastic seizures (GS), including both adults and children. Methods: We retrospectively collected patients with GS from epilepsy clinics of five tertiary hospital centres within a single country. Patients were selected through relatives’/caregivers’ descriptions, home video and/or video‐EEG monitoring. GS were identified through ictal semiology. Results: Thirty‐five patients were enrolled; 62.9% had initial GS in infancy, 14.3% in adolescence and 22.8% at adult age. Twenty‐six had abnormal MRI: eight presented with hypothalamic hamartoma (HH) and 16 non‐HH lesions that included different structural aetiologies and genetic, metabolic and immune aetiologies. All patients with HH had their first GS in infancy or adolescence. For the remaining aetiologies, GS started in infancy in 59.3%, in adolescence in 11.1% and at adult age in 29.6%. Video‐EEG data was available for analysis in 11 patients, including seven patients with a non‐HH MRI lesion. The ictal onset topography on scalp video‐EEG was usually concordant with the MRI lesion (in 6/7 patients) and the most frequent ictal onset was fronto‐temporal. In two patients, both video‐EEG and MRI suggested a parietal and occipital epileptogenic zone. Conclusion: Aetiologies and patterns of affected topography unrelated to HH are common in patients with GS, and all age groups may manifest with this type of ictal semiology. This ictal manifestation has no lateralizing value and, despite a clear preponderance for hypothalamic, frontal and temporal lobe origins, other brain areas, namely the parietal and occipital lobes, should be considered.
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