Headaches with (ipsilateral) autonomic symptoms

Abstract. Primary short-lasting headaches broadly divide themselves into those associated with autonomic symptoms, so called trigemino-autonomic cephalgias (TACs), and those with little autonomic syndromes. The trigeminoautonomic cephalgias include cluster headache and paroxysmal hemicranias, in which head pain and cranial autonomic symptoms are prominent. The most striking feature of cluster headache is the circadian and circannual periodicity of the attacks. Inheritance may play a role in some families. The attacks are of extreme intensity, of short duration, occur unilaterally, and are accompanied by symptoms of autonomic dysfunction. Medical treatment includes both acute therapy aimed at aborting individual attacks and prophylactic therapy aimed at preventing recurrent attacks during the cluster period. Some types of trigemino-autonomic headaches, such as paroxysmal hemicrania and hemicrania continua have, unlike cluster headaches, a very robust response to indomethacin, leading to a consideration of indomethacin-sensitive headaches.     

Trigeminal autonomic cephalgias: diagnosis, therapy, atypical forms and pathophysiology

Trigeminal autonomic cephalgias; cluster headache, paroxysmal hemicrania, hemicrania continua and SUNCT (short-lasting unilateral neuralgiform headache attacks with conjuctival injection and tearing) syndrome are characterized by unilateral trigeminal distribution of pain and accompanying ipsilateral autonomic symptoms. Other than cluster headache, all of these syndromes have been described within last twenty years, and are found relatively less frequent and less known forms. Diagnosis of paroxysmal hemicrania and hemicrania continua, especially atypical forms, essentially depends on indomethacin responsiveness. For SUNCT syndrome, there is not such a drug which provides a practical approach to both diagnosis and therapy and its diagnosis depends on clinical features. So far, case reports from various countries helped us realize the existence of secondary forms of these syndromes and the necessity of imaging techniques, especially for recently described autonomic cephalgias.     

Update on the diagnosis and management of Trigemino-Autonomic Headaches

Severe shortlasting headaches are rare but very disabling conditions with a major impact on the patients’ quality of life. Following the IHS criteria, these headaches broadly divide themselves into those associated with autonomic symptoms, so called trigeminal autonomic cephalgias (TACs), and those with few autonomic symptoms. The trigeminal-autonomic cephalgias include cluster headache, paroxysmal hemicranias, and a syndrome called SUNCT (short lasting unilateral neuralgic cephalgias with conjunctival injection and tearing). In all of these syndromes, hemispheric head pain and cranial autonomic symptoms are prominent. The paroxysmal hemicranias have, unlike cluster headaches, a very robust response to indomethacin, leading to a notion of indomethacin-sensitive headaches. Although TACs are, in comparison with migraine, quite rare, it is nevertheless very important to consider the clinical factor that they are easy to diagnose and the treatment is very effective in most patients. Received in revised form: 11 May 2006     

Rare nocturnal headaches

PURPOSE OF REVIEW: This review describes rare headaches that can occur at night or during sleep, with a focus on cluster headaches, paroxysmal hemicrania, short-lasting unilateral neuralgiform headache attacks with conjunctival injection and tearing, hypnic headache and exploding head syndrome. RECENT FINDINGS: It is known that cluster headaches and hypnic headache are associated with rapid eye movement sleep, as illustrated by recent polysomnographic studies. Functional imaging studies have documented hypothalamic activation that is likely to be of relevance to circadian rhythms. These headache syndromes have been shown to respond to melatonin and lithium therapy, both of which have an indirect impact on the sleep-wake cycle. SUMMARY: There is growing evidence that cluster headache and hypnic headache are chronobiological disorders.     

Trigemino-autonomic cephalalgias. Report of two cases

Trigemino-autonomic cephalalgias (TAC) constitute a rare group of primary headache conditions associated with unilateral fluctuating head pain and autonomic symptoms. These syndromes, including cluster headache, hemicrania continua, paroxysmal hemicrania and SUNCT, are much less prevalent than migraine and tension-type headache. The pathogenesis of TAC is unknown. Similar neuropeptide changes seen in all TAC syndromes suggest a shared underlying pathophysiology in these headaches. Some of them respond to the treatment with indomethacin and sumatriptan. Two patients suffering from one of the trigemino-autonomic cephalalgias are presented in the paper.     

Pathophysiology of cluster headache: a trigeminal autonomic cephalgia

Cluster headache is a form of primary neurovascular headache with the following features: severe unilateral, commonly retro-orbital, pain accompanied by restlessness or agitation, and cranial (parasympathetic) autonomic symptoms, such as lacrimation or conjunctival injection. It occurs in attacks typically of less than 3 h in length and in bouts (clusters) of a few months during which the patient has one or two attacks per day. The individual attack involves activation of the trigeminal-autonomic reflex; thus, such headaches can be broadly classified with the other trigeminal-autonomic cephalgias, such as paroxysmal hemicrania and the syndrome of short-lasting unilateral neuralgiform headache attacks with conjunctival injection and tearing. Observations of circadian biological changes and neuroendocrine disturbances have suggested a pivotal role for the hypothalamus in cluster headache. Functional neuroimaging with PET and anatomical imaging with voxel-based morphometry have identified the posterior hypothalamic grey matter as the key area for the basic defect in cluster headache.     

Idiopathic facial pain other than vascular pain

Cluster Headache associates a severe pain generally unilateral and autonomic symptoms with a remarkable periodicity. In the first part we tried to explain the conception of physiopathology of these short lasting headache syndromes and in the second part we described the clinical features. The short lasting primary headaches are divided into two groups: those with marked autonomic activation which comprise chronic and episodic paroxysmal hemicrania, short lasting unilateral neuralgiform headache with conjunctival injection and tearing (SUNCT syndrome) and cluster headache. The second group includes two entities, one without autonomic activation: Hypnic Headache and one with mild autonomic features: Hemicrania Continua. The paroxysmal hemicranias are characterized by attack frequency ranges from 15 to 20 per day of short lasting attacks of unilateral pain that typically last 2 to 10 minutes, the severe pain is associated with autonomic symptoms and responds to treatment with indomethacin. The SUNCT syndrome has a less severe pain but marked autonomic activation during attacks, this syndrome is actually resistant to proposed therapy. The Hypnic Headache and the Hemicrania Continua have yet less severe pain with very mild or without autonomic features.     

Trigeminal autonomic cephalgias due to structural lesions: a review of 31 cases

Trigeminal autonomic cephalgias (TACs) include cluster headache, paroxysmal hemicrania, and short-lasting unilateral neuralgiform headache with conjunctival injection and tearing. Associated structural lesions may be found, but a causal relationship is often difficult to establish. We sought to identify clinical predictors of underlying structural abnormalities by reviewing previously described and new TAC and TAC-like cases associated with a structural lesion. We found that even typical TACs can be caused by an underlying lesion. Clinical warning signs and symptoms are relatively rare. We recommend neuroimaging in all patients with a TAC or TAC-like syndrome.     

Epidemiology of unilateral headaches

Headache in general represents a major public health problem with a magnitude that has not been fully acknowledged until recently. While the percentage of the global adult population with migraine is approximately 10%, other forms of unilateral headaches such as cluster headache, paroxysmal hemicrania, hemicrania continua and short-lasting unilateral neuralgiform headache with conjunctival injection and tearing (SUNCT) syndrome are very rare. These headaches cause disability, suffering and loss of quality of life that easily compares to that of other chronic disorders. Continuous scientific research works towards a better understanding of these disorders and has accomplished a greater insight into the underlying pathophysiological mechanisms. This has led to improved treatment strategies in primary patient care that may prevent prolonged suffering of affected patients in the future.     

Trigeminal autonomic cephalgias (TACs)

The concept of a group of headaches whose pathophysiological focus revolves around the trigeminal-autonomic reflex fills a useful gap in characterising a number of primary headache syndromes. Broadly, these syndromes involve activation of trigeminovascular nociceptive pathways with reflex cranial autonomic activation. Clinically, this physiology predicts pain with some combination of lacrimation, conjunctival injection, nasal congestion, or eyelid oedema. Several of the primary neurovascular headaches, notably cluster headache, paroxysmal hemicrania and short-lasting neuralgiform pain with conjunctival injection and tearing (SUNCT), seem to immediately fit this classification. This physiology also explains why some patients with migraine present cranial autonomic features, and the concept is thus broadly useful for clinicians seeking a pathophysiological understanding of the primary neurovascular headaches. Given the known pathophysiology one can place the various treatments aimed at preventing these headaches or indeed treating the acute attacks, into context.     

Trigeminal autonomic cephalalgias: diagnostic and therapeutic developments

PURPOSE OF REVIEW: The review was designed to summarize recent research relevant to the trigeminal autonomic cephalalgias, which include cluster headache, paroxysmal hemicrania, short-lasting unilateral neuralgiform headache attacks with conjunctival injection and tearing/cranial autonomic features (SUNCT/SUNA). Hemicrania continua is included for completeness. RECENT FINDINGS: Cluster headache has the longest attack duration and relatively low attack frequency. Paroxysmal hemicrania has intermediate duration and intermediate attack frequency. Short-lasting unilateral neuralgiform headache attacks with conjunctival injection and tearing have the shortest attack duration and the highest attack frequency. Hemicrania continua has a continuous pain with exacerbations that can include cranial autonomic symptoms as part of the phenotype. The importance of diagnosing these syndromes resides in their excellent but highly selective response to treatment. SUMMARY: Considerable advances have been made in the diagnostic and therapeutic approaches to trigeminal autonomic cephalalgias and these are important for neurologists to consider.     

Paroxysmal hemicrania, SUNCT, and hemicrania continua

The trigeminal autonomic cephalalgias (TACs) are a group of primary headache disorders characterized by unilateral pain in the somatic distribution of the trigeminal nerve and ipsilateral autonomic signs, which reflect activation of the cranial parasympathetic pathway. The group includes cluster headache, paroxysmal hemicrania, and short-lasting unilateral neuralgiform headache with conjunctival injection and tearing. Hemicrania continua was previously classified as one of the TACs. Despite their common elements, individually these headaches differ with respect to attack duration, frequency, and response to indomethacin.     

Episodic paroxysmal hemicrania: two new cases and a literature review.

Episodic paroxysmal hemicrania (EPH) is a rare disorder characterized by discrete bouts of hemicranial headache separated by headache-free remissions. Although EPH resembles episodic cluster headache in the location and quality of pain as well as the pattern of associated autonomic features, it is distinguished by the greater frequency and shorter duration of individual headaches. Differentiation of these disorders is important because EPH almost invariably responds to treatment with indomethacin but not to standard cluster headache therapy.     

Chronic paroxysmal hemicrania and hemicrania continua responding to topiramate: two case reports

Chronic paroxysmal hemicrania (CPH) is a rare primary headache syndrome, which is classified along with cluster headache and short-lasting unilateral neuralgiform headache attacks with conjunctival injection and tearing as a trigeminal autonomic cephalalgia (TACs). Hemicrania continua (HC) was previously classified as one of the TACs, but in the recent second classification of the International Headache Society this disorder was moved to the group of other primary headaches. Both CPH and HC are characterised by moderate to excruciating pain requiring pharmacological treatment; furthermore, both conditions are characterised by an absolute response to indomethacin, which represents one of the current diagnostic criteria for these two syndromes. Unfortunately, in about one-fourth of cases treatment with indomethacin may cause adverse events, mostly gastrointestinal. We report one subject with CPH and another with HC intolerant to indomethacin, who responded remarkably well to topiramate.     

Trigeminal autonomic cephalalgias: Diagnosis and treatment

The trigeminal autonomic cephalalgias (TACs) are a group of primary headache disorders characterized by unilateral head pain that occurs in association with ipsilateral cranial autonomic features. The TACs include cluster headache, paroxysmal hemicrania, and short-lasting unilateral neuralgiform headache attacks with conjunctival injection and tearing (SUNCT) and its close relative short-lasting unilateral neuralgiform headache attacks with cranial autonomic symptoms (SUNA). These syndromes cause patients considerable disability and certainly very significant suffering. They are distinguished by the frequency of attacks of pain, the length of the attacks, and very characteristic responses to medical therapy, such that the diagnosis can usually be made clinically, which is important because it completely dictates therapy. The management of TACs can be very rewarding for physicians and highly beneficial to patients.     

Indomethacin-responsive headaches in children and adolescents

Headache is a common symptom in childhood and adolescence. Effective therapy for this symptom is based on the specific headache syndrome. This article presents examples of the four recognized Indomethacin-responsive headache syndromes encountered in pediatrics including exertional headache, cyclic-cluster migraine, chronic paroxysmal hemicrania, and hemicrania continua. Although uncommon conditions, successful treatment depends on recognition of these indomethacin-responsive headache syndromes.     

Neurostimulation in primary headache syndromes

Neurostimulation therapy involves the use of peripheral or central nerve electrical stimulation approaches for the treatment of medically intractable headache. Currently, for peripheral stimulation the main approach is that of occipital nerve stimulation, while for central stimulation deep-brain approaches with the target of the region of the posterior hypothalamic gray matter have been explored. Target conditions include migraine and the trigeminal autonomic cephalalgias: cluster headache, paroxysmal hemicrania and short-lasting unilateral neuralgiform headache attacks with conjunctival injection and tearing/cranial autonomic features (SUNCT/SUNA), as well as hemicrania continua. The initial results are encouraging and given the very significant disability of medically intractable primary headaches, this is a very promising area for patients and physicians alike.     

Diagnostics of cervicogenic headache

Cervicogenic headache (CH) is a clearly defined headache syndrome and can usually be differentiated from other unilateral headaches like cluster headache, chronic paroxysmal hemicrania (CPH) and hemicrania continua by a thorough history and neurological examination. Combinations of CH with other headaches like migraine, tension headache, combination headache and drug induced headache, that occur quite frequently, can complicate the diagnosis. In our own material of 5520 headache patients the prevalence of CH was 13.8% (763). Four hundred and thirty of these patients suffered from CH in combination with one or more other headaches.