Trigeminal Autonomic Cephalalgias: Paroxysmal Hemicrania, SUNCT/SUNA, and Hemicrania Continua

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.

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Aura in trigeminal autonomic cephalalgia is probably mediated by comorbid migraine with aura

Cephalalgia ◽

10.1177/03331024211030499 ◽

2021 ◽

pp. 033310242110304

Author(s):

Kuan-Po Peng ◽

Marlene Schellong ◽

Arne May

Keyword(s):

Cluster Headache ◽

Migraine With Aura ◽

Migraine Without Aura ◽

Telephone Interview ◽

Medical Center ◽

Temporal Correlation ◽

Hemicrania Continua ◽

Paroxysmal Hemicrania ◽

Trigeminal Autonomic Cephalalgias ◽

Trigeminal Autonomic Cephalalgia

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.

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The Promising Effect of Nerve Decompression in Trigeminal Autonomic Cephalalgias: Report of Case Series

Frontiers in Neurology ◽

10.3389/fneur.2021.678749 ◽

2021 ◽

Vol 12 ◽

Author(s):

Mansoureh Togha ◽

Ali Totonchi ◽

Hojjat Molaei ◽

Hossein Ansari

Keyword(s):

Drug Therapy ◽

Nerve Stimulation ◽

Pain Control ◽

Treatment Options ◽

Case Series ◽

Hemicrania Continua ◽

Paroxysmal Hemicrania ◽

Trigeminal Autonomic Cephalalgias ◽

Chronic Paroxysmal Hemicrania ◽

Nerve Surgery

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.

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A case of post-traumatic LASH syndrome responsive to indomethacin and melatonin (a man with a unique triad of indomethacin-responsive trigeminal autonomic cephalalgias)

Cephalalgia ◽

10.1177/0333102414544980 ◽

2014 ◽

Vol 35 (5) ◽

pp. 453-456 ◽

Cited By ~ 6

Author(s):

Todd D Rozen ◽

Jennifer L Beams

Keyword(s):

Motor Vehicle ◽

Motor Vehicle Accident ◽

Hemicrania Continua ◽

Paroxysmal Hemicrania ◽

Secondary Case ◽

Single Individual ◽

Trigeminal Autonomic Cephalalgias ◽

Vehicle Accident ◽

Time Period ◽

Post Traumatic

Objective The objective of this article is to present the first post-traumatic/secondary case of LASH syndrome and the first melatonin-responsive case of LASH. Methods We present a case report. Results A 44-year-old man developed three distinct headache syndromes in progression over a 2.5-year time period after a motor vehicle accident. He initially had paroxysmal hemicrania, which he experienced for 15 months, then transitioned to hemicrania continua for 3.5 months, then LASH syndrome, which he endured until he was treated with indomethacin and became pain free. Then after an inability to taper off indomethacin he was placed on melatonin and achieved a pain-free state. Conclusion This is the first post-traumatic/secondary case of LASH syndrome, the first male patient to be documented with LASH, and the first LASH case to show complete alleviation with melatonin. This patient’s unique case history provides another example of how multiple trigeminal autonomic cephalalgias can occur in a single individual. This may be one of the first cases of three distinct trigeminal autonomic cephalalgias developing after trauma.

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Patent foramen ovale in trigeminal autonomic cephalalgias and hemicrania continua: a non-specific pathophysiological occurrence?

Arquivos de Neuro-Psiquiatria ◽

10.1590/s0004-282x2010000400028 ◽

2010 ◽

Vol 68 (4) ◽

pp. 627-631 ◽

Cited By ~ 4

Author(s):

Vanise Amaral ◽

Gabriel R. de Freitas ◽

Bruno C.B Rodrigues ◽

Daniel de H Christoph ◽

Carlos A. de Pinho ◽

...

Keyword(s):

Cluster Headache ◽

Transcranial Doppler ◽

Epworth Sleepiness Scale ◽

Patent Foramen Ovale ◽

Hemicrania Continua ◽

Paroxysmal Hemicrania ◽

Trigeminal Autonomic Cephalalgias ◽

Foramen Ovale ◽

Trigger Factors ◽

Individual Susceptibility

Patent foramen ovale (PFO), a relatively common abnormality in adults, has been associated with migraine. Few studies also linked PFO with cluster headache (CH). To verify whether right-to-left shunt (RLS) is related to headaches other than migraine and CH, we used transcranial Doppler following microbubbles injection to detect shunts in 24 CH, 7 paroxysmal hemicrania (PH), one SUNCT, two hemicrania continua (HC) patients; and 34 matched controls. RLS was significantly more frequent in CH than in controls (54% vs. 25%, p=0.03), particularly above the age of 50. In the HC+PH+SUNCT group, RLS was found in 6 patients and in 2 controls (p=0.08). Smoking as well as the Epworth Sleepiness Scale correlated significantly with CH, smoking being more frequent in patients with RLS. PFO may be non-specifically related to trigeminal autonomic cephalalgias and HC. The headache phenotype in PFO patients probably depends on individual susceptibility to circulating trigger factors.

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Concomitant occurrence of different trigeminal autonomic cephalalgias: A case series and review of the literature

Cephalalgia ◽

10.1177/0333102413506127 ◽

2013 ◽

Vol 34 (3) ◽

pp. 231-235 ◽

Cited By ~ 10

Author(s):

Andreas Totzeck ◽

Hans-Christoph Diener ◽

Charly Gaul

Keyword(s):

Cluster Headache ◽

Clinical Presentation ◽

Case Series ◽

Primary Headache ◽

Hemicrania Continua ◽

Paroxysmal Hemicrania ◽

Trigeminal Autonomic Cephalalgias ◽

Headache Disorders ◽

Primary Headache Disorders

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.

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Chronic Paroxysmal Hemicrania, Hemicrania Continua and Sunct Syndrome in Association with Other Pathologies: A Review

Cephalalgia ◽

10.1111/j.1468-2982.2003.00646.x ◽

2004 ◽

Vol 24 (3) ◽

pp. 173-184 ◽

Cited By ~ 63

Author(s):

M Trucco ◽

F Mainardi ◽

F Maggioni ◽

R Badino ◽

G Zanchin

Keyword(s):

Causal Relationship ◽

Diagnostic Criteria ◽

Case Reports ◽

International Headache Society ◽

Hemicrania Continua ◽

Paroxysmal Hemicrania ◽

Trigeminal Autonomic Cephalalgias ◽

Sunct Syndrome ◽

Associated Lesions ◽

Chronic Paroxysmal Hemicrania

We present a review of 22 cases of headache mimicking chronic paroxysmal hemicrania (CPH) (17 female and five male; F : M ratio 3.4), nine cases mimicking hemicrania continua (HC) (seven female and two male) and seven cases mimicking SUNCT syndrome (five male and two female) found in association with other pathologies published from 1980 up to the present. All case reports were discussed with respect to diagnostic criteria proposed by International Headache Society (IHS) for CPH, by Goadsby and Lipton for HC and SUNCT, and evaluated to identify a possible causal relationship between the pathology and the onset of headache. The aim of the present review was to evaluate if the presence of associated lesions and their location could help elucidate the pathogenesis of trigeminal autonomic cephalalgias (TACs).

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Dose, Efficacy and Tolerability of Long-Term Indomethacin Treatment of Chronic Paroxysmal Hemicrania and Hemicrania Continua

Cephalalgia ◽

10.1046/j.1468-2982.2001.00287.x ◽

2001 ◽

Vol 21 (9) ◽

pp. 906-910 ◽

Cited By ~ 61

Author(s):

JA Pareja ◽

AB Caminero ◽

E Franco ◽

JL Casado ◽

J Pascual ◽

...

Keyword(s):

Hemicrania Continua ◽

Paroxysmal Hemicrania ◽

Chronic Paroxysmal Hemicrania ◽

Indomethacin Treatment

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Chronic paroxysmal hemicrania, hemicrania continua and SUNCT: the fate of the three first described cases

The Journal of Headache and Pain ◽

10.1007/s10194-006-0303-5 ◽

2006 ◽

Vol 7 (3) ◽

pp. 151-156 ◽

Cited By ~ 16

Author(s):

Ottar Sjaastad

Keyword(s):

Hemicrania Continua ◽

Paroxysmal Hemicrania ◽

Chronic Paroxysmal Hemicrania

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Hemicrania continua

Oxford Textbook of Headache Syndromes ◽

10.1093/med/9780198724322.003.0021 ◽

2020 ◽

pp. 203-207

Author(s):

Johan Lim ◽

Joost Haan

Keyword(s):

Age Of Onset ◽

Primary Headache ◽

Hemicrania Continua ◽

Moderate Intensity ◽

Trigeminal Autonomic Cephalalgias ◽

The Third ◽

The Mean ◽

Autonomic Reflex ◽

Patients Experience

Hemicrania continua is an uncommon primary headache characterized by continuous, unilateral cranial pain of moderate intensity, more painful exacerbations with cranial autonomic features, and an absolute response to indomethacin. It is considered one of the trigeminal autonomic cephalalgias. Activation of the trigeminal–autonomic reflex and the contralateral posterior hypothalamic grey is thought to play an important role in its pathophysiology. The mean age of onset is in the third decade and there is a female preponderance of 2:1. Hemicrania continua can be divided into a remitting and an unremitting type; most patients suffer from the unremitting type. Any part of the head or neck can be affected, and pain is mainly described as throbbing. Many patients experience migrainous features during exacerbations. Physical and supplementary investigations are mostly normal. Other trigeminal autonomic cephalalgias and migraine are the main differential diagnostic alternatives for consideration.

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