Background There are various studies on experimentally provoked 'ice-cream headache' or 'headache attributed to ingestion or inhalation of a cold stimulus' (HICS) using different provocation protocols. The aim of this study was to compare two provocation protocols. Methods Ice cubes pressed to the palate and fast ingestion of ice water were used to provoke HICS and clinical features were compared. Results The ice-water stimulus provoked HICS significantly more often than the ice-cube stimulus (9/77 vs. 39/77). Ice-water-provoked HICS had a significantly shorter latency (median 15 s, range 4-97 s vs. median 68 s, range 27-96 s). There was no difference in pain localisation. Character after ice-cube stimulation was predominantly described as pressing and after ice-water stimulation as stabbing. A second HICS followed in 10/39 (26%) of the headaches provoked by ice water. Lacrimation occurred significantly more often in volunteers with than in those without HICS. Discussion HICS provoked by ice water was more frequent, had a shorter latency, different pain character and higher pain intensity than HICS provoked by ice cubes. The finding of two subsequent HICS attacks in the same volunteers supports the notion that two types of HICS exist. Lacrimation during HICS indicates involvement of the trigeminal-autonomic reflex.

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Objective: Cerebrovascular abnormalities have been reported in adult patients with Pompe disease. The objective was to study these abnormalities by (1) determining the diameter and mean flow velocity (MFV) of large cerebral arteries and (2) estimating cerebral blood flow (CBF), resistance index (RI) and cerebrovascular reactivity (CVR) as functions of resistance vessels.Methods: In ten adults with Pompe disease and twenty controls, the diameter, peak systolic (PSV) and enddiastolic velocities (EDV) of arteries supplying the brain were quantified by MR angiography and sonography. MFV, RI and CBF were calculated. CVR in the middle cerebral artery (MCA) was determined by hyperventilation and acetazolamide injection.Results: MR angiography revealed dilation of cerebral arteries predominantly in the posterior circulation. Dilative arteriopathy was found in three patients; two of them showed vertebrobasilar dolichoectasia. Despite of the dilative arteriopathy, the MFV was normal, indicating increased CBF and dilated resistance vessels. RI of all examined arteries and CVR of MCA were normal.Conclusion: The data suggest that dilation of small and large cerebral arteries is a common feature in adults with Pompe disease. Increased CBF might be the consequence of dilated resistance vessels. However, dysfunction of resistance vessels was rarely found.Synopsis: In adults with Pompe disease, dilation of small and large cerebral arteries is a common feature and might be associated with increased cerebral blood flow.

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BackgroundLysosomal α-glucosidase deficiency (Pompe disease) not only leads to glycogen accumulation in skeletal muscle, but also in the cerebral arteries. Dolichoectasia of the basilar artery (BA) has been frequently reported. Therefore progression of BA dolichoectasia in late onset Pompe patients (LOPD) was studied.MethodsBA length, diameter and volume, and cerebral lesions were analysed by MRI/TOF-MR angiography or CT/CT angiography in 20 LOPD patients and 40 controls matching in age, sex- and cardiovascular risk factors. The height of BA bifurcation was assessed semi-quantitatively using the Smoker’s criteria and quantitatively by measuring the outlet angle of the superior cerebellar artery (SUCA). Nine patients were followed over 5 years.ResultsThe height of the BA bifurcation was abnormal in 12/20 (60%) LOPD patients and in 12/40 (30%) matched controls. The SUCA outlet angle was reduced in LOPD patients compared to controls (127 ± 33° vs. 156 ± 32°, p = 0.0024). The diameter, length and volume of the BA were significantly increased in LOPD patients compared to controls. 12/20 (60%) LOPD patients and 27/40 (68%) controls presented white matter lesions. During 5 years 2/9 LOPD patients developed an abnormal height of BA bifurcation according to the Smoker’s criteria and in all patients the SUCA outlet angle decreased (138 ± 34° vs. 128 ± 32°, p = 0.019). One patient with prominent basilar dolichoectasia experienced a thalamic hemorrhage.ConclusionPompe disease is associated with BA dilation, elongation and elevated bifurcation height of the BA which might result in cerebrovascular complications. The SUCA outlet angle seems to be useful for monitoring the progression of BA dolichoectasia.Electronic supplementary materialThe online version of this article (10.1186/s13023-018-0794-6) contains supplementary material, which is available to authorized users.

Background Numerous but inconclusive findings have sparked an ongoing debate about whether the arteries of migraine patients undergo vascular alterations. The outlet angle of the superior cerebellar artery (SUCA) and the lateral displacement of basilar arteries are good surrogate parameters for determining elongation of the vertebrobasilar arteries. Methods We retrospectively determined the SUCA outlet angle and the lateral displacement of the basilar artery in 63 patients with migraine (30.6 ± 8.9 years, 84% women, 16% chronic migraine, 60% migraine with aura) and compared these with 126 age- and sex-matched control subjects. Results In patients with migraine, the SUCA outlet angle was lower (159 ± 26° vs. 169 ± 29°, p = 0.020) and the lateral displacement of the basilar artery was greater (3.7 ± 2.7 mm vs. 2.8 ± 2.4 mm, p = 0.020) than in the control subjects. Age, gender, migraine characteristics and presence of any cardiovascular risk factors did not affect the SUCA outlet angle or lateral displacement of the basilar artery. Conclusion Migraine patients exhibited a lower SUCA outlet angle and greater lateral displacement of the basilar arteries. Both may be attributable to the elongation of the vertebrobasilar arteries, which is an indication of arterial wall pathology in migraine.

Allergies against nail varnish and its remover and against artificial nails should be included in the differential diagnosis of skin allergies, even when patients have additional type IV sensitizations against common allergens. As the causative allergen can be easily avoided, knowledge of the particular problems associated with these allergies is of a great practical significance.