Primary headache attributed to external compression or traction to the head : a narrative review

Background: The aim of this review is to synthesize the existing knowledge regarding headaches attributed to external physical stimuli, as classified by the ICHD-3 (Group 4.6). Two forms can be distinguished in this group: (1) headache attributed to external compression and (2) headache attributed to external traction. Methods: A comprehensive literature review was conducted using the Medline (PubMed) database and other relevant academic sources. All English-language articles were subjected to a relevance assessment. Results: The prevalence of the two types of headache varies considerably, with a higher incidence observed in women or in the presence of predisposing factors (e.g., work with compulsory helmets or long hair). An external-compression headache is typically described as a pressing sensation, whereas an external-traction headache is characterized by a sensation of pulling. The headaches typically persist for less than an hour after the stimulus has ceased, and the intensity is typically reported as mild to moderate. Apart from avoiding the trigger, which is not always possible, effective therapies have not been established. Conclusion: Both types of headaches are relatively common, yet they remain underrepresented in the scientific literature. Furthermore, there is a dearth of comprehensive overviews. If the triggering factor cannot be eliminated, both types of headaches can interfere with daily living and working

Phänotypen und Genotypen distaler Myopathien

Distalen Myopathien sind eine Gruppe von sehr seltenen hereditären Muskelerkrankungen. Die Klassifikation beruht auf dem Erkrankungsbeginn, Phänotyp, Vererbungsmodus, EMG-Veränderungen sowie histologische Kriterien. Für viele Formen wurden die ursächlichen Gendefekte mittlerweile identifiziert. Im Rahmen dieser Arbeit werden neue Ergebnisse zu Phänotyp, Diagnostik und Pathophysiologie von distalen Myopathien dargestellt. Dies umfasst eigene Untersuchungen zur Epidemiologie der Distalen Myopathien, insbesondere zur Distale Myopathie bei Matrin3-Mutation mit nasaler Sprache und Schwäche der Rachenmuskulatur (VCPDM), wobei es gelang eine der weltweit größten Gruppen sowohl klinisch als auch histologisch zu dokumentieren. Neue Aspekte waren hier eine Beteiligung der axiale, proximalen sowie der Atemmuskulatur. Weiterhin wurde andere seltene Distale Myopathien oder Myopathien mit distalem Phänotyp dargestellt.Distal myopathies are a group of very rare hereditary Muscle disease. The classification is based on the onset of the disease, phenotype, mode of inheritance, EMG changes and histological criteria. For many forms, the causative genetic defects have now been identified. In the context of this work new results on phenotype, diagnostics and pathophysiology of distal myopathies are presented. This includes own investigations on the epidemiology of distal myopathies, in particular on distal myopathy in Matrin3 mutation with vocal cord and pharyngeal muscle weakness (VCPDM), whereby one of the world's largest groups was successfully documented both clinically and histologically. New aspects were the involvement of the axial, proximal and respiratory muscles. Furthermore other rare distal myopathies or myopathies with distal phenotype were presented

Cardiac manifestations in adult MELAS syndrome (mitochondrial encephalomyopathy with lactic acidosis and stroke-like episodes syndrome) : a cross-sectional study

Backround: Cardiac involvement has been reported in different mitochondrial geno- and phenotypes, including mitochondrial myopathy, encephalopathy, lactic acidosis and stroke-like (MELAS) syndrome. However, cardiac manifestations are diverse and not well described. Methods: We prospectively examined cardiac manifestations in 11 adult patients with MELAS syndrome harboring the MTTL1 m.3243A < G-mutation using patient records, cardiac MRI (1.5 Tesla), echocardiography, electrocardiogram (ECG), laboratory tests of cardiac markers (CK, CK-MB, Trop I, BNP), and clinical severity (NMDAS = Newcastle Mitochondrial Disease Scale). Results: Among 11 consecutive patients with MELAS syndrome (73% male, mean age 37.5 ± 10.6 years) cardiac manifestations were found in nine (82%). Pathology was mainly detected using MRI (9 of 11, 82%). Six patients showed diffuse late enhancement in the left ventricle, one a left ventricular ejection fraction (LVEF) below 30%, two with a LVEF in the range of 40–50% in the cardiac MRI, and another five patients presenting diastolic dysfunction as defined by echocardiography. Only one patient with late enhancement on MRI also showed a conduction block in the ECG. There was no correlation between the cardiac manifestations and the NMDAS score or heteroplasmy grade. Conclusions: Cardiac involvement in MELAS syndrome harboring the MTTL1 m.3243A > G mutation mostly entails cardiomyopathy, which was particularly evident in the cardiac MRI. Only one patient (1/11, 9.1%) had conduction defects. Thus, cardiac testing including cardiac MRI, echocardiography and ECG seems to be important for prognosis of MELAS patients

Myalgia in 30 patients with suspected myopathy

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 demonstratedPublikationsfond ML

Vertebrobasilar artery elongation in migraine : a retrospective cross-sectional study

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

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.Publikationsfond ML

Increased blood flow velocity in middle cerebral artery and headache upon ingestion of ice water

Introduction: “Headache attributed to ingestion or inhalation of a cold stimulus” (HICS) is one of the most common primary headache disorders. Little is known about the pathophysiology of HICS and other headache disorders. The aim of this study was to analyze mean flow velocity (MFV) and cerebrovascular resistance (RI) in both middle cerebral arteries (MCA) upon ingestion of ice water. Methods: The MFV and RI in both MCAs was continuously measured by transcranial sonography. HICS was induced by drinking 200 ml of ice water. Results: In all volunteers, the ingestion of ice water led to a decrease in RI, which was accompanied by an increase in MFV. In volunteers with induced HICS, MFV were significantly higher compared to volunteers that did not experience HICS. In volunteers with HICS, MFV increased even more significantly when lacrimation occurred compared to volunteers in which it did not. In volunteers without induced HICS, MFV was higher in those volunteers with a positive history of HICS than in those with a negative HICS history. Conclusion: This study revealed a raised MFV upon ingestion of ice water. Volunteers with a provoked case of HICS had a higher MFV than volunteers without HICS. The increase in MFV was even higher when the headache was accompanied by lacrimation. This may indicate an involvement of the trigeminal-parasympathetic vasodilator reflex.Publikationsfond ML

Erenumab escalation in migraine - double dose without additional benefit : a retrospective experience

Background: Erenumab is a monoclonal antibody specifically targeting the CGRP-receptor. Several studies showed efficacy and safety in patients with migraine. Less is known regarding dosage increase, especially in a difficult to treat patients. The aim of the study is to evaluate the increased dosage under real world conditions with particular focus on 70 mg non-responders. Methods: In a retrospective analysis, patients treated in tertiary headache centers (Halle or Jena, Germany) receiving 70 mg erenumab for at least 3 months with a dosage increase to 140 mg were analyzed. Data were evaluated regarding headache days, intake of acute medication, previous prophylaxis, and medication overuse. Baseline and all treatment intervals were determined as three-month periods. Results: Datasets of 52 migraine patients (90.4% women) aged between 22 and 78 years (mean 50.4 years, SD 12.1 years) were analyzed. At baseline (mean headache-days 15.67 ± 6.37) 51.9% met criteria for chronic migraine and 56% were currently overusing acute medication. While therapy with 70 mg showed significant improvement in headache days and 50% response, further improvement was not achieved for therapy escalation to 140 mg. The same applies to the secondary endpoints and covers the entire study population as well as the subgroups of chronic and episodic migraine. The 50% response of the 70 mg non-responders for escalation was only 5.14%. Conclusions: In this difficult-to-treat patient cohort we reconfirmed the effectiveness of erenumab, but could not detect any additional benefit for a dosage escalation from 70 mg to 140 mg erenumab

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

Minimal important difference of the Migraine Disability Assessment (MIDAS): Longitudinal data from the DMKG Headache Registry

Background: The Migraine Disability Assessment (MIDAS) is widely used. However, there are limited data on how much a reduction in the MIDAS score indicates a change that matters to the patient. Methods: Data from the DMKG (i.e. German Migraine and Headache Society) Headache Registry were used to determine the minimal important difference (MID) of the MIDAS, using the Patient Global Impression of Change (PGIC) as anchor and applying average change and receiver operating characteristic curve methods. Results: In total, 1218 adult migraine patients (85.6% female, 40.2 ± 12.8 years, baseline MIDAS 44.2 ± 47.4, follow-up MIDAS 36.5 ± 45.3) were included. For patients with baseline MIDAS >20 (MIDAS grade IV, n = 757), different methods using PGIC “somewhat improved” as anchor yielded percent change MIDs of the MIDAS between −29.4% and −33.2%. For baseline MIDAS between 6 and 20 (grades II and III, n = 334), using PGIC “much improved” as anchor, difference change MIDs were between −3.5 and −4.5 points. Conclusions: Based on the above results, we estimated the MID of the MIDAS at −30% for patients with a baseline MIDAS >20, and at −4 points for those with a baseline MIDAS of 6–20, for a tertiary headache care population. Trial Registration: The DMKG Headache Registry is registered with the German Clinical Trials Register (DRKS 00021081)