Different forms of trigeminal autonomic cephalalgias in the same patient: description of a case

The trigeminal autonomic cephalalgias (TACs), including cluster headache, paroxysmal hemicrania and SUNCT, are characterized by the cardinal combination of short-lasting unilateral pain and autonomic phenomena affecting the head. Hemicrania continua (HC) shares many clinical characteristics with TACs, including unilateral pain and ipsilateral autonomic features. Nevertheless, HC is separately classified in the revised International Classification of Headache Disorders (ICHD-II). Here, we describe the case of a 45-year-old man presenting an unusual concurrence of different forms of primary headaches associated with autonomic signs, including subsequently ipsilateral cluster headache, SUNCT and HC. This report supports the theory that common mechanisms could be involved in pathophysiology of different primary headache syndromes.     

Unilateral photophobia or phonophobia in migraine compared with trigeminal autonomic cephalalgias

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, short-lasting 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.     

Trigeminal autonomic cephalalgias: current and future treatments

The trigeminal autonomic cephalgias include cluster headache, paroxysmal hemicrania, and short-lasting unilateral neuralgiform headache attacks with conjunctival injection and tearing (SUNCT). The evidence for the current treatment options for each of these syndromes is considered, including oxygen, sumatriptan, and verapamil in cluster headache, indomethacin in paroxysmal hemicrania, and intravenous lidocaine and lamotrigine in SUNCT. Some treatments such as topiramate have an effect in all of these, as well as in migraine and other pain syndromes. The involvement of the hypothalamus in functional imaging studies implies that this may be a substrate for targeting treatment options in the future.     

Therapie und Prophylaxe von Cluster-Kopfschmerzen und anderen trigemino-autonomen Kopfschmerzen

Following the new IHS classification, cluster headache, paroxysmal hemicrania, and short-lasting unilateral neuralgiform headache with conjunctival injection and tearing (SUNCT syndrome) are included in the classification as trigeminal autonomic cephalgias (TAC). The similarities of these syndromes suggest a considerable shared pathophysiology. These syndromes have in common that they 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 edema. Broadly the management of TAC comprises acute and prophylactic treatment. Some types of trigeminal 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. This review covers the clinical picture and therapeutic options. Although studies following the criteria of evidence-based medicine (EBM) are rare, most patients can be treated sufficiently.     

Osmophobia in primary headaches

This study evaluates osmophobia (defined as an unpleasant perception, during a headache attack, of odours that are non–aversive or even pleasurable outside the attacks) in connection with the diagnosis of primary headaches. We recruited 775 patients from our Headache Centre (566 females, 209 males; age 38±12 years), of whom 477 were migraineurs without aura (MO), 92 with aura (MA), 135 had episodic tension–type headache (ETTH), 44 episodic cluster headache (ECH), 2 chronic paroxysmal hemicrania (CPH) and 25 other primary headaches (OPHs: 12 primary stabbing headaches, 2 primary cough headaches, 3 primary exertional headaches, 2 primary headaches associated with sexual activity, 3 hypnic headaches, 2 primary thunderclap headaches and 1 hemicrania continua). Among them, 43% with MO (205/477), 39% with MA (36/92), and 7% with CH (3/44) reported osmophobia during the attacks; none of the 135 ETTH and 25 OPH patients suffered this symptom. We conclude that osmophobia is a very specific marker to discriminate adequately between migraine (MO and MA) and ETTH; moreover, from this limited series it seems to be a good discriminant also for OPHs, and for CH patients not sharing neurovegetative symptoms with migraine. Therefore, osmophobia should be considered a good candidate as a new criterion for the diagnosis of migraine.     

Chronic daily bilateral headache responsive to indomethacin

Indomethacin is known to be specifically effective for chronic paroxysmal hemicrania, episodic paroxysmal hemicrania, and hemicrania continua. Different forms of idiopathic stabbing headaches have also been responsive to indomethacin, but less consistently than the others. Two cases of indomethacin-responsive headache are reported. One patient presented with what appeared to be new-onset, chronic, daily, bilateral headache aggravated by coughing. Both the chronic daily headache and the exacerbations induced by coughing were suppressed with indomethacin therapy. The second patient experienced hemicrania continua responsive to indomethacin, and the response persisted even when the headache evolved into bilateral continuous pain. There may be other idiopathic primary headache disorders that are peculiarly responsive to indomethacin. When any primary headache disorder does not respond to standard therapy, a brief therapeutic trial of indomethacin is warranted.     

Effective management of ice pick pains, SUNCT, and episodic and chronic paroxysmal hemicrania

Idiopathic stabbing headaches, the SUNCT syndrome, and the paroxysmal hemicranias are a group of primary headache disorders that are characterized by brief, short-lived attacks of head pain, which recur multiple times throughout the day. These syndromes are much less prevelant than other types of primary headaches such as migraine and tension-type headaches but are significantly more disabling. Recognition of these uncommon disorders is important because their management differs from standard headache therapies.     

The “Other” Headaches: Primary Cough,Exertion, Sex,and Primary Stabbing Headaches

Primary cough headache, primary exertional headache, primary sexual headache, and idiopathic stabbing headache are included in “Other Primary Headaches” (Group 4) in the International Classification of Headache Disorders, 2nd edition (ICHD-II). Headaches provoked by cough, exertion, and sex have different age distributions, but they do share some clinical and pathogenic characteristics. The triggering activities frequently involve Valsalva-like maneuvers, which may explain part of the pathogenesis. Primary stabbing headache is common and characterized by ultra-short stabbing headaches. All these headache disorders respond well to indomethacin, and they are commonly comorbid with migraine except for primary cough headache. Of note, some patients with sexual headache had reversible cerebral vasoconstriction syndromes. Recent large-scaled studies have revealed that the ICHD-II criteria of these four headache disorders cannot be completely fulfilled. Further revisions for the ICHD-II criteria are required based on these results of the evidence-based studies.     

Hemicrania continua evolving from episodic paroxysmal hemicrania

A 45-year-old woman, who had been diagnosed in our unit with episodic paroxysmal hemicrania, was seen 2 years later for ipsilateral hemicrania continua in remitting form. Both types of headache had a complete response to indomethacin and did not occur simultaneously. The patient had a previous history of episodic moderate headaches that met criteria for probable migraine without aura and also had a family history of headache. The clinical course in this case suggests a pathogenic relationship between both types of primary headache.     

Chronic Paroxysmal Headache: Two Cases With Cerebrovascular Disease

Paroxysmal headaches often occur in benign headache disorders such as episodic cluster headache, chronic paroxysmal hemicrania (CPH) and episodic paroxysmal hemicrania. We report 2 patients with paroxysmal headaches occurring in association with cerebrovascular disease. The first patient had paroxysmal headaches from an arteriovenous malformation which resolved following embolization. In the second patient, headache followed a cerebral infarction and responded to treatment with indomethacin. We suggest that vascular disease may cause paroxysmal headaches resembling CPH. Patients with an atypical presentation of CPH warrant a neuroimaging procedure.     

Other primary headaches

Most primary headaches are classified into a few categories, such as migraine or muscle contraction headache, and patients suffering from these headaches are common. On the other hand, other primary headaches are very rare. In this section entitled "Other primary headaches", eight headaches, including primary stabbing headache, primary cough headache, primary exertional headache, primary headache associated with sexual activity, hypnic headache, primary thunderclap headache, hemicrania continua, and new daily-persistent headache, are described. Some characteristics of other primary headaches are common in symptomatic headaches, such as subarachnoid hemorrhage or arterial dissection. Therefore, careful evaluations including neuroimaging are necessary to exclude organic diseases.     

Diagnosis and Clinical Features of Trigemino‐Autonomic Headaches

Although severe short‐lasting headaches are rare, they can be considered disabling conditions with a major impact on the quality of life of patients. These headaches can divided broadly in to those associated with autonomic symptoms, so called trigeminal autonomic cephalgias (TACs), and those with few or no autonomic symptoms. The TACs include cluster headache, paroxysmal hemicranias, hemicrania continua, and short‐lasting unilateral neuralgiform headache attacks with cranial autonomic symptoms as well as short‐lasting unilateral neuralgiform headache attacks with conjunctival injection and tearing syndrome. In all of these syndromes, half‐sided head pain and ipsilateral cranial autonomic symptoms such as lacrimation or rhinorrhea are prominent. The paroxysmal hemicranias have, unlike cluster headaches, a very robust response to indomethacin, leading to a notion of indomethacin‐sensitive headaches. The diagnosis of TACs is exclusively a clinical task. Because of the fact that cluster headache is strictly half‐sided, typically involves the region around the eye and temple and often starts in the upper jaw, most patients first consult a dentist or ophthalmologist. No single instrumental examination has yet been able to define, or ensure, the correct diagnosis, or differentiate idiopathic headache syndromes. It is crucial that a trained neurologist sees these patients early so that management can be optimized and unnecessary procedures can be avoided. Although TACS are, in comparison to migraine, quite rare, they are nevertheless clinically very important for the neurologist to consider as they are easy to diagnose and the treatment is very effective in most patients.     

The differential diagnosis of chronic daily headaches: an algorithm-based approach

Chronic daily headaches (CDHs) refers to primary headaches that happen on at least 15 days per month, for 4 or more hours per day, for at least three consecutive months. The differential diagnosis of CDHs is challenging and should proceed in an orderly fashion. The approach begins with a search for "red flags" that suggest the possibility of a secondary headache. If secondary headaches that mimic CDHs are excluded, either on clinical grounds or through investigation, the next step is to classify the headaches based on the duration of attacks. If the attacks last less than 4 hours per day, a trigeminal autonomic cephalalgia (TAC) is likely. TACs include episodic and chronic cluster headache, episodic and chronic paroxysmal hemicrania, SUNCT, and hypnic headache. If the duration is > or =4 h, a CDH is likely and the differential diagnosis encompasses chronic migraine, chronic tension-type headache, new daily persistent headache and hemicrania continua. The clinical approach to diagnosing CDH is the scope of this review.     

Short-lasting headaches in children

Short-lasting headaches have been studied infrequently in children and it is not known if the main categories of primary headaches of this type in adults are applicable to children. We report our experience with a group of 20 children with a brief headache. Two patients had a secondary headache. One patient had a headache with some clinical characteristics of paroxysmal hemicrania. The remaining 17 had a very brief headache. They were in many aspects comparable to others from previous studies on idiopathic stabbing headache in children: no associated symptoms, no other associated headache, frequent family history of migraine. They differed, however, in the younger age of the patients and the more frequent extratrigeminal location of the pain. Extratrigeminal ice-pick pain may be a variant of idiopathic stabbing headache, more prevalent in young children.     

Chronic paroxysmal hemicrania: the first possible bilateral case

There are three headaches syndromes that are typically characterized by strictly unilateral and always same-sided attacks: cluster headache, "cervicogenic" headache, and chronic paroxysmal hemicrania (CPH). In rare cases, cluster headache also occurs bilaterally; "cervicogenic" headaches probably as well. We present a patient with a probable bilateral CPH. To our knowledge no such case has previously been described.     

Kopfschmerzattacken und autonome Symptome

Following the new IHS-classification, cluster headache, paroxysmal hemicrania and SUNCT syndrome are included into the classification as trigemino-autonomic cephalgias (TAC's). Clinically, they share strictly halfsided head pain with autonomic symptoms. The headaches often occur during particular sleep stages and are associated with other chronobiologic factors. Broadly the management of TAC's comprises acute and prophylactic treatment. Paroxysmal hemicrania and hemicrana continua have a very robust response to indomethacin. Acute cluster headache attacks can be treated with inhalation of oxygen or serotonin agonists, whereas verapamil is the drug of choice in the prophylactic treatment. This review covers the clinical picture and therapeutic options. Allthough studies following the criteria of evidence based medicine (EBM) are rare, most patients can be treated sufficiently.     

Chronic daily headache in a paediatric headache centre

The prevalence and the clinical features of chronic daily headache (CDH) were studied in 968 children and adolescents observed during a period of one year in the Headache Centre of the Anna Meyer Paediatric Hospital of Florence. Nine hundred and fortyfour patients (97.52%) had primary headache according to ICHD-II, 24 subjects had secondary headache and 56 patients had CDH (5.93% of primary headaches). The mean age of subjects with CDH was higher than general (13.5 vs. 11.5 years), with a female preponderance (69.6% vs. 30.4%). According to the ICHD-II, headaches were classified as chronic migraine in 10 patients (1.5.2 ICHD-II), chronic tension-type headache in 36 (2.3 ICHD-II), new daily persistent headache in 8 (4.8 ICHD-II) and 2 patients reported mixed pattern (chronic migraine+chronic tension type headache). Medication overuse was not implicated in our patients.     

Short-lasting headache syndromes and treatment options

A number of primary headache syndromes are marked by their short duration of pain. Many of these syndromes have their own unique treatment, so they must be recognized by practicing physicians. In this article, a number of the shortlasting headache disorders are reviewed, including chronic paroxysmal hemicrania, SUNCT syndrome, hypnic headache, exploding head syndrome, primary stabbing headache, and cough headache.     

CLUSTER, NEURALGIAS, AND TACS

A number of primary headache syndromes are marked by their short duration of pain. Many of these syndromes have their own unique treatment, so they must be recognized by practicing physicians. In this article, a number of the short-lasting headache disorders are reviewed, including chronic paroxysmal hemicrania, SUNCT syndrome, hypnic headache, exploding head syndrome, primary stabbing headache, and cough headache.     

Paroxysmale Hemikranie und SUNCT

Paroxysmal hemicrania is experienced as headache attacks with pain and accompanying symptoms similar to those of cluster headaches. Attacks are, however of shorter duration, occur more frequently, affect predominantly women and respond reliably to indomethacin. Paroxysmal hemicrania can also occur secondary to an identifiable cause. To exclude symptomatic, paroxysmal hemicrania, especially with an atypical clinical picture and poor response to indomethacin, a careful diagnostic approach is necessary. The SUNCT syndrome (short-lasting unilateral neuralgiform headache attacks with conjunctival injection and tearing) is characterized by one-sided pain attacks of short duration, much shorter than other trigeminal autonomic cephalgias. Classically, the pain is accompanied by ipsilateral lacrimation and conjunctival injection. Some patients have been described with both cluster headache and trigeminal neuralgia. These patients should receive both diagnoses. It is important to differentiate these headache entities as specific therapy is needed for each to achieve optimal pain relief.