Cranial autonomic symptoms in episodic and chronic migraine: a cross sectional study in Iran

Background Cranial autonomic symptoms are common in migraine, with eye redness and tearing being the most common ones. Their identification can help to avoid misdiagnosis, predict the disease course, and select the appropriate treatment. Methods This was a cross-sectional study of 904 patients who presented with migraine to a headache referral clinic. The participants filled out a questionnaire about their headache characteristics, as well as the presence of cranial autonomic symptoms. A total of 904 patients, 698 women (77.2%) and 206 men (22.8%), were included in the study, with a mean (SD) age of 38.05 (11.76) years. Results About 70% of subjects with chronic migraine and 56.2% of those with episodic migraine reported one or more cranial autonomic symptoms. The two most commonly reported autonomic symptoms were eye redness (36.06%) and tearing (21.02%). Chronic migraine (43.4% vs. 29.5%), unilateral headache (56.8% vs. 48.7%), and blurred vision (20% vs. 14.7%) were significantly more frequent in migraineurs with cranial autonomic symptoms. Headache intensity and frequency in subjects with cranial autonomic symptoms were significantly higher than in those without cranial autonomic symptoms. Conclusion We found higher percentages of cranial autonomic symptoms in patients with unilateral headaches, frequent and severe attacks and blurred vision. A diagnosis of cranial autonomic symptoms accompanying migraine may predict more severe disease and the possibility of evolution into chronic migraine.

A case of cranial autonomic dysfunctions predominant migraine: Migraine sans ache

Pain is the essential part of migraine headaches along with other features, whereas, cranial autonomic symptoms (CAS)/signs are a predominant and essential part of trigeminal autonomic cephalalgias. What if other features of migraine are present but the pain is absent? One such variation in migraine symptomatology is reported in this case, where the pain was not there but CAS/signs were predominant along with other features of migraine. The possible mechanism for such variation, in this case, maybe desensitization of afferent loop (comprised by nociceptors in extracranial and intracranial pain-sensitive structures supplied by the trigeminal nerve) and direct activation of the trigemino-cervical complex and brainstem structures causing dissociation of pain from the rest of the features of migraine. In this case, as the pain part gets completely dissociated from CAS/signs, hence this condition can be termed as “migraine sans ache.” The patient was started on anti-migraine treatment and she responded wonderfully

Cranial autonomic symptoms: prevalence, phenotype and laterality in migraine and two potentially new symptoms

BackgroundWhilst cranial autonomic symptoms (CAS) are typically associated with the trigeminal autonomic cephalalgias (TAC’s), they have also been reported in migraine. Identification and understanding of these symptoms in migraine is important to ensure timely diagnosis and effective management. MethodsMigraineurs seen within our tertiary headache service between 2014-2018 ( n =340): cohort one, and a separate cohort of headache patients seen between 2014-May 2021 reporting voice change and/or throat swelling as CAS were selected ( n =64); cohort two. We examined, as a service evaluation, our records regarding age, sex, diagnosis, headache and CAS frequency and laterality as acquired from the first consultation, during which a detailed headache history is taken by a trained physician. ResultsCohort 1: Mean age 43 (range 14-94, SD 15). The most common diagnosis was chronic migraine (78%). Median monthly headache frequency was 26 days (IQR 15-75). At least one CAS was reported in 74%, with a median of two (IQR 0-3). The most common were nasal congestion (32%), lacrimation (31%) and aural fullness (25%). Most patients reported unilateral headache (80%) and strictly unilateral CAS (64%). There was a positive association between headache and CAS laterality (ꭓ 2 1 = 20.7, P < 0.001), with a positive correlation between baseline headache frequency and number of CAS reported ( r = 0.11, P = 0.047). Cohort two: mean age 49 (range 23-83, SD 14). Diagnoses were chronic migraine (50%), chronic cluster headache (11%), undifferentiated continuous lateralised headache (9%), SUNCT/SUNA (8%), hemicrania continua (8%), episodic migraine (8%), episodic cluster headache (3%) and trigeminal neuropathies (3%). Most (89%) described trigeminal distribution pain; 25% involving all three divisions. Throat swelling was reported by 54, voice change by 17, and both by 7. The most common CAS reported were lacrimation ( n = 47), facial swelling ( n = 45) and rhinorrhoea ( n = 37). There was significant agreement between the co-reporting of throat swelling (ꭓ 2 1 = 7.59, P = 0.013) and voice change (ꭓ 2 1 = 6.49, P = 0.02) with aural fullness. ConclusionsCAS are common in migraine, are associated with increasing headache frequency and tend to co-lateralise with headache. Voice change and throat swelling should be recognized as possible parasympathetically-mediated CAS. They may be co-associated and associated with aural fullness, suggesting a broadly somatotopic endophenotype.

SUNCT/SUNA in Pediatric Age: A Review of Pathophysiology and Therapeutic Options

The International Classification of Headache Disorders, 3rd edition (ICHD3) defines Short-lasting Unilateral Neuralgiform Headache Attacks (SUNHA) as attacks of moderate or severe, strictly unilateral head pain lasting from seconds to minutes, occurring at least once a day and usually associated with prominent lacrimation and redness of the ipsilateral eye. Two subtypes of SUNHA are identified: Short-lasting Unilateral Neuralgiform headache attacks with Conjunctival injection and Tearing (SUNCT) and Short-lasting Unilateral Neuralgiform headache attacks with cranial Autonomic symptoms (SUNA). These pathologies are infrequent in children and difficult to diagnose. The authors reviewed the existing literature on SUNCT and SUNA, especially in the developmental age, which describes the pathophysiology in detail and focuses on the therapeutic options available to date. SUNHA-type headaches must be considered on the one hand, for the possibility of the onset of forms secondary to underlying pathologies even of a neoplastic nature, and on the other hand, for the negative impact they can have on an individual’s quality of life, particularly in young patients. Until now, published cases suggest that no chronic variants occur in childhood and adolescents. In light of this evidence, the authors offer a review that may serve as a source to be drawn upon in the implementation of suitable treatments in children and adolescents suffering from these headaches, focusing on therapies that are non-invasive and as risk-free as possible for pediatric patients.

Cranial autonomic symptoms in episodic migraine: An observational study from headache clinic of a tertiary care center of North India

Introduction: The presence of cranial autonomic symptoms (CAS) is a hallmark of trigeminal autonomic cephalalgia like cluster headache but their presence in migraine is also not uncommon. Like in trigeminal autonomic cephalalgias, the activation of the trigeminal autonomic reflex pathway is thought to be the possible explanation of the presence of cranial autonomic symptoms in migraine also. Previous studies suggested that around half of the patients of migraine suffer from these symptoms. The aim of our study was to observe the frequency of cranial autonomic symptoms in episodic migraine patients along with their clinical and autonomic characteristics. Methods and Materials: Fifty patients of episodic migraine attending the headache clinic of Dr. RMLIMS, Lucknow and fulfilling the diagnostic criteria of International classification of headache disorder third edition beta were randomly selected and enrolled in the study. The detailed interview regarding presence of cranial autonomic symptoms was recorded along with the clinical characteristics, demographic features and autonomic profile of the migraine patients. Results: About three-fourth (72%) of the patients were females. The mean age of study participants was 27.7±8.3 years. A considerable number of patients (54%) had a long duration (5-10 years) of illness and 70% of patients had severe headaches. Photophobia was the most common (88%) associated clinical symptoms while lacrimation was the most common (56%) cranial autonomic symptom in migraine patients. Among 50 patients of migraine 74% of patients were having at least one cranial autonomic symptoms. Conclusion: Cranial autonomic symptoms are common in patients of episodic migraine. More severe headache is more likely to be associated with the development of cranial autonomic symptoms.

Update on Treatment of Cluster Headache

Cluster headache (CH) is characterized by severe unilateral short-lasting headache attacks, accompanying ipsilateral cranial autonomic symptoms or the sense of restlessness and agitation, or both. CH is a highly disabling primary headache disorder but often not optimally treated. High-flow oxygen and parenteral triptans are the most effective treatment to treat an acute CH attack. Transitional treatments include systemic steroid therapy and sub-occipital steroid injection. For preventive therapy, verapamil and lithium are recommended as first-line treatments. Novel treatments have appeared, such as neuromodulation and medication targeting calcitonin gene-related peptide (CGRP) or its receptor. Galcanezumab, the only anti-CGRP receptor monoclonal antibody with proven efficacy for the preventive treatment of episodic CH, represents an important advance for pharmacological treatment of CH. Neuromodulation strategies, such as the non-invasive vagus nerve stimulation and the sphenopalatine ganglion stimulation, have been proven effective in reducing the intensity and frequency of attacks, and also to be safe and well tolerated.

Clinical features of cluster headache without cranial autonomic symptoms: results from a prospective multicentre study

Although cranial autonomic symptoms (CAS) are typical in cluster headache (CH), some individuals with CH show no CAS during their headache attacks. Probable cluster headache (PCH) is a subtype of CH that fulfils all but one criterion of CH. This study aimed to investigate the frequency and clinical features of CH and PCH without CAS in comparison to those with CAS. We analysed data from the Korea Cluster Headache Registry, a prospective multicentre registry involving data from 16 hospitals. Of the 216 participants with CH and 26 with PCH, 19 (8.8%) and 7 (26.9%), respectively, did not have CAS. Participants with CH without CAS exhibited less severe anxiety (General Anxiety Disorder-7 score, median [interquartile range], 2.0 [1.0–6.0] vs 8.0 [3.0–12.0], p = 0.001) and depression (Patient Health Questionnaire-9 score, 3.0 [1.0–7.0] vs 7.0 [3.0–11.0], p = 0.042) than those with CAS. Among participants with PCH, headache intensity was less severe in participants without CAS than in those with CAS (numeric rating scale, 8.0 [7.0–8.0] vs 9.5 [8.0–10.0], p = 0.015). In conclusion, a significant proportion of participants with CH and PCH did not have CAS. Some clinical features of CH and PCH differed based on the presence of CAS.

Comprehensive clinical phenotyping of nitroglycerin infusion induced cluster headache attacks

Background Nitroglycerin administration allows the study of cluster headache attacks in their entirety in a standardised way. Methods A single-blind, placebo-controlled, cross-over study using weight-calculated intravenous nitroglycerin administration at 0.5 µg/kg/min over 20 minutes to study cluster headache attacks, including accompanying non-headache symptoms and cranial autonomic symptoms. Results Thirty-three subjects with cluster headache were included in the study; 24 completed all three study visits. Nitroglycerin-induced attacks developed in 26 out of 33 subjects (79%) receiving unblinded nitroglycerin infusion, and in 19 out of 25 subjects (76%) receiving single-blinded nitroglycerin infusion, compared with one out of 24 subjects (4%) receiving single-blinded placebo infusion. Episodic cluster headache subjects had a shorter latency period to a nitroglycerin-induced attack compared to the chronic cluster headache (CCH) subjects ( U = 15, z = −2.399, p = 0.016). Sixteen of nineteen episodic cluster headache (mean, 84%; 95% confidence interval, 66–100%) and 11 of 14 chronic cluster headache subjects developed a nitroglycerin-induced attack (79%, 54–100%) following the unblinded nitroglycerin infusion. Following the single-blinded nitroglycerin infusion, eight out of 13 episodic cluster headache (62%, 31–92%) and 11 out of 12 chronic cluster headache (92%, 73–100%) subjects developed nitroglycerin-induced attacks. Nitroglycerin induced non-headache symptoms in the majority of subjects receiving it: 91% in the open unblinded nitroglycerin visit and 84% in the single-blinded nitroglycerin visits, compared with 33% in the single-blinded placebo visit. Cranial autonomic symptoms were induced by nitroglycerin infusion, 94% in the open unblinded nitroglycerin visit and 84% in the single-blinded nitroglycerin visit, compared with 17% in the single-blinded placebo visit. Conclusion Intravenous weight-adjusted nitroglycerin administration in both episodic cluster headache in bout and chronic cluster headache is effective and reliable in inducing cluster headache attacks, cranial autonomic symptoms and non-headache symptoms.

Cranial suture headache: An extracranial head pain syndrome originating in the cranial sutures of the skull

Objective: To define a new type of head pain syndrome termed “cranial suture headache” which is a localized headache originating along the cranial suture lines of the skull. Background: Well localized headaches maybe extracranial in origin. As trigeminal nociceptors are localized within the cranial sutures of the skull, these fibrous joints maybe the source of head pain for some patients. Methods: Case series. To diagnose cranial suture headache, the patient’s pain had to be localized to the skull and elicited/mimicked by mild to moderate palpation over one or more distinct cranial suture lines. Results: Ten cases are presented. Most of the patients were women (9/10). The headache started daily from onset in all cases. Range of age of headache onset was 32–64 years. Headache was one sided, unless confined to the midline and typically lacked any migrainous and/or cranial autonomic symptoms. Most cranial suture headaches localized to either the sagittal, coronal or squamosal suture lines. Headache duration prior to diagnosis was on average 8.5 years. Triggering events: three began immediately after head trauma, two had very remote head trauma, one was post infectious, one was post craniotomy, while three patients had no known triggering event. All patients were treatment refractory failing at least three preventive medications. All improved with localized anesthetic injection to the suture line(s) and/or onabotulinum toxin A injection only to the cranial sutures. Discussion: Without the recognition of cranial suture-based pain, patients may have unremitting headaches that can last years to decades. The observation that “cranial suture” headache improves with localized treatment only to the cranial sutures would seem to suggest the extracranial origin of the pain.