Prednisone for the treatment of withdrawal headache in patients with medication overuse headache: A randomized, double-blind, placebo-controlled study

Purpose: To investigate the efficacy of prednisone for treatment of withdrawal headache in patients with medication overuse headache (MOH). Patients and methods: In this prospective double-blind, placebo-controlled, parallel designed multicentre trial, 96 consecutive patients with MOH were randomized to withdrawal treatment with either 100 mg prednisone or placebo over 5 days. Patients were enrolled if they met the International Headache Society criteria for MOH and were diagnosed with migraine or episodic tension-type headache as primary headache. Exclusion criteria comprised significant neurological or psychiatric disorders. Withdrawal symptoms, including headache severity and intake of rescue medication, were documented for 14 days after randomization. Results: Patients treated with prednisone did not experience fewer hours of moderate or severe headache than patients receiving placebo. However, patients requested less rescue medication within the first 5 days. Conclusions: During withdrawal in MOH, prednisone reduces rescue medication without decreasing the severity and duration of withdrawal headache

Prednisone for the treatment of withdrawal headache in patients with medication overuse headache: A randomized, double-blind, placebo-controlled study

Purpose: To investigate the efficacy of prednisone for treatment of withdrawal headache in patients with medication overuse headache (MOH). Patients and methods: In this prospective double-blind, placebo-controlled, parallel designed multicentre trial, 96 consecutive patients with MOH were randomized to withdrawal treatment with either 100 mg prednisone or placebo over 5 days. Patients were enrolled if they met the International Headache Society criteria for MOH and were diagnosed with migraine or episodic tension-type headache as primary headache. Exclusion criteria comprised significant neurological or psychiatric disorders. Withdrawal symptoms, including headache severity and intake of rescue medication, were documented for 14 days after randomization. Results: Patients treated with prednisone did not experience fewer hours of moderate or severe headache than patients receiving placebo. However, patients requested less rescue medication within the first 5 days. Conclusions: During withdrawal in MOH, prednisone reduces rescue medication without decreasing the severity and duration of withdrawal headache

Prävalenz von Kopfschmerzen bei Patienten mit einer genetisch determinierten mitochondrialen Erkrankung

Das Auftreten der Migräne wurde bei mitochondrialen Erkrankungen in der Vergangenheit wiederholt berichtet. Zusätzlich wurde aufgrund verschiedener Untersuchungen ein Zusammenhang zwischen dem mitochondrialen Stoffwechsel und der Pathophysiologie der Migräne angenommen. Daraus wurde geschlussfolgert, dass Mutationen im mitochondrialen Genom als Risikofaktor für Migränekopfschmerzen eine Rolle spielen. In der vorliegenden Arbeit wurden 42 Patienten mit einer genetisch determinierten mitochondrialen Erkrankung zur Frage der Häufigkeit von Kopfschmerzen und nach einem Zusammenhang mit Haplotypen der mtDNA (H sowie ACE) untersucht. Kopfschmerzen wurden bei 70% aller Patienten mit genetisch determinierten mitochondrialer Erkrankung festgestellt, wobei der Kopfschmerz vom Spannungstyp von 55%, gefolgt von der Migräne mit und ohne Aura von 38% der Patienten berichtet wurde. Die Mutation A3423G zeigte innerhalb der Gruppe der mitochondrialen Mutationen die höchste Prävalenz mit 88%. Kopfschmerzen bestanden auch sehr häufig bei singulären Deletionen (78%) sowie bei multiplen Deletionen (54%). Entgegen den Angaben in der Literatur wurde der Haplotyp H nur bei wenigen Patienten nachgewiesen (29%). Bei diesen Patienten war der Kopfschmerz vom Spannungstyp (55%) häufiger als die Migräne mit und ohne Aura (33%). Die Verteilung der Haplotypen D/D, D/I und I/I entsprach den Daten der Literatur, auch hier wurde der Kopfschmerz vom Spannungstyp im Vergleich zur Migräne mit und ohne Aura von einer größeren Anzahl von Patienten berichtet. Diese Arbeit zeigt, dass bei Patienten mit mitochondrialen Erkrankungen Kopfschmerzen häufiger auftreten als in der Allgemeinbevölkerung, dies gilt nicht nur für die Mutation A3243G sondern auch für singuläre Deletionen und multiple Deletionen. Entgegen der Annahme in der Literatur hat der Kopfschmerz vom Spannungstyp die höchste Prävalenz. Ein deutlicher Zusammenhang zwischen den Haplotypen und dem Auftreten der Migräne mit und ohne Aura konnte nicht gezeigt werden.vorgelegt von: Torsten KrayaMedizinische Universität Wien, Masterarb., 201

Decreased outlet angle of the superior cerebellar artery as indicator for dolichoectasia in late onset Pompe disease

Abstract Background Lysosomal 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. Methods BA 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. Results The 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. Conclusion Pompe 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

Myalgia in 30 Patients with Suspected Myopathy

Background: In patients with neuromuscular disorder, only little data of myalgia frequency and characterization exists. To date, only a weak correlation between pain intensity and pressure pain threshold has been found, and it remains enigmatic whether high pain intensity levels are equivalent to high pain sensitivity levels in neuromuscular disorders. Methods: 30 sequential patients with suspected neuromuscular disorder and myalgia were analyzed with regard to myalgia characteristics and clinical findings, including symptoms of depression and anxiety and pain- threshold. Results: A neuromuscular disorder was diagnosed in 14/30 patients. Muscular pain fasciculation syndrome (MPFS) without evidence for myopathy or myositis was diagnosed in 10/30 patients and 6/30 patients were diagnosed with pure myalgia without evidence for a neuromuscular disorder (e.g., myopathy, myositis, MPFS, polymyalgia rheumatica). Highest median pain scores were found in patients with pure myalgia and polymyalgia rheumatica. Pressure pain threshold measurement showed a significant difference between patients and controls in the biceps brachii muscle. Conclusion: Only a weak correlation between pain intensity and pressure pain threshold has been suggested, which is concordant with our results. The hypothesis that high pain intensity levels are equivalent to high pain sensitivity levels was not demonstrated

Whole-body muscle MRI of patients with MATR3-associated distal myopathy reveals a distinct pattern of muscular involvement and highlights the value of whole-body examination

Objective!#!MATR3-associated distal myopathy is a rare distal myopathy predominantly affecting lower legs as well as wrist- and finger extensors. Whilst most distal myopathies are clinically and genetically well characterized, diagnosis often remains challenging. Pattern-based magnetic resonance imaging (MRI) approaches offer valuable additional information. However, a consistent pattern of muscular affection is missing for most distal myopathies. Thus, the aim of the present study was to establish a disease-specific pattern of muscular involvement in MATR3-associated distal myopathy using whole-body MRI.!##!Methods!#!15 patients (25-79 years of age, 7 female) with MATR3-associated distal myopathy were subjected to whole-body MRI. The grade of fatty involution for individual muscles was determined using Fischer-Grading. Results were compared to established MRI-patterns of other distal myopathies.!##!Results!#!There was a predominant affection of the distal lower extremities. Lower legs showed a severe fatty infiltration, prominently affecting gastrocnemius and soleus muscle. In thighs, a preferential involvement of semimembranous and biceps femoris muscle was observed. Severe affection of gluteus minimus muscle as well as axial musculature, mainly affecting the thoracic segments, was seen. A sufficient discrimination to other forms of distal myopathy based solely on MRI-findings of the lower extremities was not possible. However, the inclusion of additional body parts seemed to yield specificity.!##!Interpretation!#!Muscle MRI of patients with MATR3-associated distal myopathy revealed a distinct pattern of muscular involvement. The usage of whole-body muscle MRI provided valuable additional findings as compared to regular MRI of the lower extremities to improve distinction from other disease entities

Headache and migraine in mitochondrial disease and its impact on life—results from a cross-sectional, questionnaire-based study

Headache is frequent in patients with mitochondrial disorders. Previous studies point to a higher prevalence of headache in these patients than in the general population. As mitochondrial disorders often present a variety of other symptoms, the question arises how much the presence of headache really influences daily life. We performed a cross-sectional, questionnaire-based study investigation with 61 patients with a genetically proved mitochondrial disease mainly composed of CPEO phenotype. Headache was examined using a standardized questionnaire, and classified according to ICHD-2. Headache-related disability was evaluated by the Headache-Impact-Test-6 (HIT-6). Additionally, depression and anxiety were examined using the Hospital Anxiety and Depression Scale (HADS) and Short-Form-Health Survey (SF-12) was used to investigate the health-related quality of life. Headache was reported by 43/61 (70.5%) of the patients. 35/61 patients (57.4%) described a Tension-type headache (TTH) and 26 patients (42.6%) a migraine. Patients reporting headache had a significantly higher HIT-6 score than those without (mean: 54.47 vs. 38.47, p < 0.001). The HIT-6 score was significantly higher in patients reporting a migraine compared to those with a tension-type headache (mean: 62.13 vs. 46.18, p < 0.001). In the HADS score and in the SF-12 were not significantly influenced by the occurrence of headache. This study confirms the previously reported frequent occurrence of headache in a large cohort of patients with a confirmed mitochondrial disease. Migraine had the greatest impact on daily living, which appeared not to be confounded by depression and anxiety. Thus, we conclude that Migraine may be a substantial contributor for burden of disease in patients with mitochondrial diseases

Additional file 1: of Decreased outlet angle of the superior cerebellar artery as indicator for dolichoectasia in late onset Pompe disease

Figure S1. A case of very prominent vertebrobasilar dolichoectasia. Vertebrobasilar arteries of a 75 year old female LOPD patient. Basilar artery shows to be dilated and elongated with massive cranial and posterior shift of the BA bifurcation height (A coronal, B sagittal view). This LOPD patient experienced a left side thalamic hemorrhage. As consequence of massive BA shift the SUCA outlet angle was not reasonable measurable. Abbr. 1 distal part of basilar artery, 2 bifurcation of basilar artery, 3 posterior cerebral artery, 4 superior cerebellar artery, 5 anterior inferior cerebellar artery, 6 vertebral artery. (PDF 150 kb

The headache registry of the German Migraine and Headache Society (DMKG): baseline data of the first 1,351 patients

Background Although good treatment options exist for many headache disorders, not all patients benefit and disability continues to be large. To design strategies for improving headache care, real-world data observing standard care is necessary. Therefore, the German Migraine and Headache Society (DMKG) has established the DMKG Headache Registry. Here we present methods and baseline data. Methods Accredited German headache centers (clinic-based or private practice) can offer participation to their patients. Patients provide headache history, current headache load (including a mobile headache diary), medication and comorbidities and answer validated questionnaires, prior to their physician appointment. Physicians use these data as the base of their history taking, and add, change or confirm some central information. Before the next visit, patients are asked to update their data. Patients will continuously be included over the next years. Results The present analysis is based on the first 1,351 patients (1110 females, 39.6 ± 12.9 years) with a completed first visit. Most participants had a migraine diagnosis. Participants had 14.4 ± 8.5 headache days and 7.7 ± 6.1 acute medication days per month and 63.9% had a migraine disability assessment (MIDAS) grade 4 (severe disability). 93.6% used at least one acute headache medication, most frequently a triptan (60.0%) or non-opioid analgesic (58.3%). 45.0% used at least one headache preventive medication, most frequently an antidepressant (11.4%, mostly amitriptyline 8.4%) or a CGRP(receptor) antibody (9.8%). Most common causes for discontinuation of preventive medication were lack of effect (54.2%) and side effects (43.3%). Conclusion The DMKG Headache Registry allows to continuously monitor headache care at German headache centers in both a cross-sectional and a longitudinal approach