SUNCT/SUNA in children and adolescents: Application of ICHD-3 criteria and treatment response: Case series of 13 SUNCT/SUNA pediatric cases

Cephalalgia ◽  
2020 ◽  
pp. 033310242095452
Author(s):  
Sharoon Qaiser ◽  
Andrew D Hershey ◽  
Joanne Kacperski

Introduction Trigeminal autonomic cephalalgias (TACs) are characterized by paroxysmal attacks of unilateral primary headaches associated with ipsilateral craniofacial autonomic symptoms. In this pediatric case series, 13 cases of short-lasting unilateral neuralgiform headache attacks with conjunctival injection and tearing (SUNCT)/short-lasting unilateral neuralgiform headache with autonomic symptoms (SUNA), including children ages 3–18 years, are discussed. This paper reviews the application of International Classification of Headache Disorders, 3rd edition (ICHD-3) criteria especially in children presenting with SUNCT or SUNA. This is the largest pediatric case series of SUNCT/SUNA reported in the literature. Background Trigeminal autonomic cephalalgias are rare in children and adolescents, with SUNCT/SUNA having the least reported cases. We will discuss the application of ICHD-3 criteria to diagnose SUNCT/SUNA in children and review overlapping cases and their response to different treatment options including indomethacin, which is typically reserved for specific subtypes of TACs; for example, paroxysmal hemicrania. Conclusion This case series presents a unique opportunity to aid in the diagnosis and treatment of similar pediatric cases in the future. It helps us to broaden the ICHD-3 criteria to diagnose and treat different overlapping trigeminal autonomic cephalalgia cases in children.

2021 ◽  
Vol 12 ◽  
Author(s):  
Mansoureh Togha ◽  
Ali Totonchi ◽  
Hojjat Molaei ◽  
Hossein Ansari

Trigeminal Autonomic Cephalalgias (TAC) are excruciating headaches with limited treatment options. The chronic forms of TACs, including chronic cluster, chronic paroxysmal hemicrania, and hemicrania continua, are disabling conditions. In addition to drug therapy, there are some studies regarding nerve blocking and nerve stimulation with acceptable results. Here we report four cases of decompression nerve surgery with promising results on pain control in these difficult to treat headaches.


Cephalalgia ◽  
2014 ◽  
Vol 35 (3) ◽  
pp. 234-239 ◽  
Author(s):  
MAS Ahmed ◽  
Sarah Donaldson ◽  
Francis Akor ◽  
Denise Cahill ◽  
Raed Akilani

Background Although olfactory hallucination (OH) has been reported in patients with primary headaches, olfactory aura has not been recognised by the International Classification of Headache Disorders (ICHD-2). In this study, we examined the frequency and characteristics of OH among children and adolescents with primary headaches. Methods 839 neurologically normal patients with primary headaches (537 migraine) were eligible for the assessment of olfactory hallucination. Headache diagnosis was based on the ICHD. Data were prospectively collected during clinic sessions and using headache diaries. Results Olfactory hallucination was reported exclusively during headache attacks by 21/839 (2.5%) patients, all of whom had migraine. The prevalence of olfactory hallucination was 3.9% among migraineurs (6.5% among those with migraine aura). Olfactory hallucination shortly followed the onset of headaches and lasted from 15 to 50 minutes. Of those with MA, 10 patients had visual aura; two had somatosensory aura; one had motor aura; and two had a combination of visual and somatosensory aura. Using the ICHD-2, both OH and migraine aura occurred in the same headache attacks. In 12/15 patients, OH occurred simultaneously with migraine aura, whereas in 3/12 patients, it preceded aura. Conclusion Our findings show that olfactory hallucination occurs in migraine and it has similarities to migraine aura.


Cephalalgia ◽  
2013 ◽  
Vol 34 (3) ◽  
pp. 231-235 ◽  
Author(s):  
Andreas Totzeck ◽  
Hans-Christoph Diener ◽  
Charly Gaul

Introduction The trigeminal autonomic cephalalgias (TACs) subsume four primary headache disorders. Hemicrania continua is increasingly regarded as an additional TAC. In rare cases patients may present with two different TACs or a TAC and hemicrania continua. Cases We report four patients with two different TACs or one TAC and hemicrania continua. Two patients presented with cluster headache and paroxysmal hemicrania, one patient with cluster headache and hemicrania continua, and one patient suffered from cluster headache and SUNCT. Discussion While the International Classification of Headache Disorders (ICHD-II) proposes specific diagnostic criteria, the variability of clinical presentation may make clear diagnosis difficult. All patients fulfilled the ICHD-II criteria. The manifestation of two different TACs or hemicrania continua in one patient is uncommon but possible and should be taken into account especially when chronic headache patients present with changing headache symptoms.


Author(s):  
Thijs H. Dirkx ◽  
Peter J. Koehler

The trigeminal autonomic cephalalgias (TACs), including cluster headache, paroxysmal hemicrania, SUNCT (short-lasting unilateral neuralgiform headache attacks with conjunctival injection and tearing), SUNA (with cranial autonomic symptoms), and hemicrania continua, belong to the primary headaches. They are characterized by severe unilateral headache attacks in association with ipsilateral cranial autonomic features. Cluster headache is the most frequent of the TACs. The other TACs are rare, but epidemiological data are scarce and variable. The various types of TAC are distinguished not only by differences in attack frequency and duration, but also by differences with respect to treatment response. The typical headache syndromes, fulfilling the International Classification of Headache Disorders-3 criteria, have also been described in association with other disorders and imaging is required to exclude intracranial pathology in all newly diagnosed patients.


Cephalalgia ◽  
2008 ◽  
Vol 28 (6) ◽  
pp. 626-630 ◽  
Author(s):  
P Irimia ◽  
E Cittadini ◽  
K Paemeleire ◽  
AS Cohen ◽  
PJ Goadsby

Our objective was to compare the presence of self-reported unilateral photophobia or phonophobia, or both, during headache attacks comparing patients with trigeminal autonomic cephalalgias (TACs)—including cluster headache, shortlasting unilateral neuralgiform headache attacks with conjunctival injection and tearing (SUNCT) and paroxysmal hemicrania—or hemicrania continua, and other headache types. We conducted a prospective study in patients attending a referral out-patient clinic over 5 months and those admitted for an intramuscular indomethacin test. Two hundred and six patients were included. In episodic migraine patients, two of 54 (4%) reported unilateral photophobia or phonophobia, or both. In chronic migraine patients, six of 48 (13%) complained of unilateral photophobia or phonophobia, or both, whereas none of the 24 patients with medication-overuse headache reported these unilateral symptoms, although these patients all had clinical symptoms suggesting the diagnosis of migraine. Only three of 22 patients (14%) suffering from new daily persistent headache (NDPH) experienced unilateral photophobia or phonophobia. In chronic cluster headache 10 of 21 patients (48%) had unilateral photophobia or phonophobia, or both, and this symptom appeared in four of five patients (80%) with episodic cluster headache. Unilateral photophobia or phonophobia, or both, were reported by six of 11 patients (55%) with hemicrania continua, five of nine (56%) with SUNCT, and four of six (67%) with chronic paroxysmal hemicrania. Unilateral phonophobia or photophobia, or both, are more frequent in TACs and hemicrania continua than in migraine and NDPH. The presence of these unilateral symptoms may be clinically useful in the differential diagnosis of primary headaches.


2016 ◽  
Vol 16 (4) ◽  
pp. 180-186
Author(s):  
Michał Modestowicz ◽  
◽  
Wojciech Kozubski ◽  

Trigeminal autonomic cephalalgias are a group of primary headache disorders presenting as unilateral pain in the somatic distribution of the trigeminal nerve, associated with ipsilateral cranial autonomic symptoms. This clinicopathologic group includes cluster headache, paroxysmal hemicrania, hemicrania continua and short-lasting unilateral neuralgiform headache attacks with conjunctival injection and tearing/cranial autonomic features, which differ mainly as regards the duration and frequency of pain as well as response to treatment. These disorders are not as rare as they were thought to be and due to the severity of the pain can substantially affect the patients’ quality of life. Many other forms of primary headaches, such as migraine, trigeminal neuralgia and primary stabbing headache, as well as secondary headaches, particularly those caused by pituitary, posterior fossa, orbital, paranasal sinus and vascular pathology, need to be carefully considered in the diagnosis of trigeminal autonomic cephalalgias. Research in this field, particularly using functional neuroimaging, has resulted in a much better understanding of these disorders. Dysfunction in the nociceptive modulatory pathways in brain’s pain matrix is currently thought to produce a permissive state for the occurrence of a trigeminal autonomic cephalalgia attack, with posterior hypothalamus serving as a terminator rather than the generator of the attack. The current treatment strategies include medical and surgical approaches; of the latter, neuromodulation techniques, particularly deep brain stimulation of posterior hypothalamus, have proven to be particularly effective and promising.


Children ◽  
2021 ◽  
Vol 8 (2) ◽  
pp. 101
Author(s):  
Maximilian David Mauritz ◽  
Anna Enninger ◽  
Christine Wamsler ◽  
Julia Wager ◽  
Boris Zernikow

Paroxysmal Hemicrania is a rare form of primary headache in children and adolescents, belonging to the group of trigeminal autonomic cephalalgias. Patients suffer from severe, short-lasting unilateral headaches accompanied by symptoms of the autonomic system on the same side of the head. The short duration of attacks distinguishes Paroxysmal Hemicrania from other trigeminal autonomic cephalalgias. Indomethacin is the treatment of choice, and its effectiveness provides a unique diagnostic criterion. However, the long-term outcomes in children are highly underreported. In this case-series, n = 8 patients diagnosed with Paroxysmal Hemicrania were contacted via telephone 3.1 to 10.7 years after initial presentation. A standardized interview was conducted. n = 6 patients were headache-free and no longer took indomethacin for 5.4 ± 3.4 years. The mean treatment period in these patients was 2.2 ± 1.9 years. Weaning attempts were undertaken after 1.7 ± 1.3 months; in n = 3 patients, more than one weaning attempt was necessary. n = 2 patients were still taking indomethacin (4.5 and 4.9 years, respectively). Both unsuccessfully tried to reduce the indomethacin treatment (two and six times, respectively). Adverse effects appeared in n = 6 (75%) patients and led to a discontinuation of therapy in n = 2 patients. Our long-term follow-up suggests that in a substantial proportion of pediatric patients, discontinuing indomethacin therapy is possible without the recurrence of Paroxysmal Hemicrania.


2021 ◽  
pp. 100-108
Author(s):  
N. V. Vashchenko ◽  
A. M. Uzhakhov ◽  
Ju. E. Azimova

Trigeminal autonomic cephalalgias (TACs) are rare but are the most intense primary headaches that severely limit patients’ ability to work and be socially active. This article reviews the modern classification of TACs, based on the International Classification of Headache Disorders-3, and the key differences between TAC types, as well as the pathophysiological mechanisms – the role of the trigeminovascular system, autonomic nervous system, hypothalamus and vagus nerve – and their relation to circadian rhythms. The sleep disturbances that can occur in patients with TACs, exacerbating the course of the disease, and the role of melatonin, hypothalamus and suprachiasmatic nucleus in these conditions are also discussed. In addition, current therapies for cluster headache are described, which include acute therapy and prophylactic therapy, with recommendations regarding the timing of prophylactic therapy discontinuation. The review also includes the available data on melatonin as well as new therapies such as CGRP monoclonal antibodies and neuromodulation, which includes the two most promising techniques: non-invasive vagus nerve stimulation and sphenopalatine ganglion microstimulation. Furthermore, the authors present the clinical case of a patient with chronic cluster headache, which was significantly reduced in frequency and intensity when melatonin was added to the therapy.


Cephalalgia ◽  
2021 ◽  
pp. 033310242110304
Author(s):  
Kuan-Po Peng ◽  
Marlene Schellong ◽  
Arne May

Objective The presence of aura is rare in cluster headache, and even rarer in other trigeminal autonomic cephalalgias. We hypothesized that the presence of aura in patients with trigeminal autonomic cephalalgias is frequently an epiphenomenon and mediated by comorbid migraine with aura. Methods The study retrospectively reviewed 480 patients with trigeminal autonomic cephalalgia in a tertiary medical center for 10 years. Phenotypes and temporal correlation of aura with headache were analyzed. Trigeminal autonomic cephalalgia patients with aura were further followed up in a structured telephone interview. Results Seventeen patients with aura (3.5%) were identified from 480 patients with trigeminal autonomic cephalalgia, including nine with cluster headache, one with paroxysmal hemicrania, three with hemicrania continua, and four with probable trigeminal autonomic cephalalgia. Compared to trigeminal autonomic cephalalgia patients without aura, trigeminal autonomic cephalalgia patients with aura were more likely to have a concomitant diagnosis of migraine with aura (odds ratio [OR] = 109.0, 95% CI 30.9–383.0, p < 0.001); whereas the risk of migraine without aura remains similar between both groups (OR = 1.10, 95% CI = 0.14–8.59, p = 0.931). Aura was more frequently accompanied with migraine-like attacks, but not trigeminal autonomic cephalalgia attacks. Interpretation In most patients with trigeminal autonomic cephalalgia, the presence of aura is mediated by the comorbidity of migraine with aura. Aura directly related to trigeminal autonomic cephalalgia attack may exist but remains rare. Our results suggest that aura may not be involved in the pathophysiology of trigeminal autonomic cephalalgia.


2008 ◽  
Vol 2 (3) ◽  
pp. 201-211 ◽  
Author(s):  
Carlijn de Roos ◽  
Ad de Jongh

Given the limited number of reported cases in literature, it might be concluded that it is rare to develop a choking phobia in childhood. However, it appears as though confusion in terminology and the time lapse between the onset of the disorder and treatment often results in the diagnosis being missed. In this article, we discuss a review of the clinical symptoms, differential diagnosis, comorbidity, etiology, and treatment options for choking phobia. We present a case series, describing the successful EMDR treatment of choking phobia for 4 children and adolescents, with positive outcomes achieved in 1 or 2 sessions. In addition, a detailed transcript is presented of a 15-year-old girl with a choking phobia related to an incident that occurred 5 years previously. The rapid elimination of symptoms in all 4 cases indicates that EMDR can be an effective treatment for choking phobias resulting from previous disturbing events. Randomized research on this promising intervention is strongly suggested.


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