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.     

Ipsilateral cluster headache and chronic paroxysmal hemicrania: two case reports

We report two patients with ipsilateral attacks of cluster headache and chronic paroxysmal hemicrania. The first patient, a 33-year-old man, started having attacks of chronic cluster headache at the age of 27. At 33, they were replaced by typical attacks of ipsilateral chronic paroxysmal hemicrania which showed a dramatic improvement with indomethacin 150 mg daily. After two days of complete remission, cluster headache attacks reappeared and persisted until verapamil, 360 mg a day, was added to indomethacin. The second patient, a 45-year-old man, first developed attacks of episodic cluster headache at the age of 35. At 44, he experienced ipsilateral typical attacks of chronic paroxysmal hemicrania, and two months later attacks of cluster headache. Under verapamil 240 mg daily, attacks of cluster headache disappeared, but those of chronic paroxysmal hemicrania increased in frequency until indomethacin 150 mg daily was added. These observations suggest a close relationship but not a similarity between cluster headache and chronic paraoxysmal hemicrania, and show the practical therapeutic interest of maintaining this distinction.     

Ocular Blood Flow Changes in Cluster Headache and Chronic Paroxysmal Hemicrania

Intraocular pressure and pulsatile ocular blood flow were recorded during and between attacks in patients suffering from cluster headache (n = 18) or chronic paroxysmal hemicrania (n = 7). Similarities, as well as significant differences, were observed between the two groups of patients, pointing to fundamental differences between the two disorders with regard to pathophysiology. Compared with healthy controls, the cluster headache patients demonstrated low pulsatile ocular blood flow values between attacks, with an increase to normal levels during pain. The chronic paroxysmal hemicrania patients, however, had normal values between attacks with a marked and significant increase to high values during attacks.     

Chronic Paroxysmal Hemicranial: Mechanical Precipitation of Attacks

SYNOPSIS,
Chronic paroxysmal hemicrania (CPH) is a cluster headache variant, characterized by daily, frequent, unilateral, excruciatingly severe but rather short lasting headache attacks, which will not surface during chronic indomethacin medication. Three of five patients with an established diagnosis of CPH were able to precipitate attacks by head flexion (or rotation), whereas this ability was not detected in any of 40 patients with regular cluster headache. Attacks with associated unilateral tearing, conjunctival injection, slight miosis, ptosis and headache may start 4–15 second after beginning of head flexion. Precipitated and spontaneous attacks seem identical both clinically and as far as the immediate increment in corneal indentation pulse (CIP) amplitudes and intraocular pressure are concerned.
There are various alternative explanations for the underlying mechanism, the most plausible of which concerns sympathetic nerve involvement.     

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.     

Chronic paroxysmal hemicrania presenting as otalgia with a sensation of external acoustic meatus obstruction: two cases and a pathophysiologic hypothesis

OBJECTIVE: To describe two cases of chronic paroxysmal hemicrania manifested by otalgia with a sensation of external acoustic meatus obstruction and to suggest that the trigeminal-autonomic reflex is a mechanism for the sensation of ear blockage. BACKGROUND: Maximum pain in chronic paroxysmal hemicrania is most often in the ocular, temporal, maxillary, and frontal regions. It is less often located in the nuchal, occipital, and retro-orbital areas. Review of the literature on chronic paroxysmal hemicrania found no reports of pain primarily localized to the ear and associated with a sensation of external acoustic meatus obstruction. METHODS: The history, physical examination, imaging studies, and successful treatment plan in two patients with otalgia and ear fullness and a subsequent diagnosis of chronic paroxysmal hemicrania are summarized. RESULTS: The first patient was a 42-year-old woman with a 10-year history of unilateral, severe, paroxysmal otalgia occurring five times a day with a duration of 2 to 60 minutes. During an attack, the ear became erythematous and the external acoustic meatus felt obstructed. There were no other associated autonomic signs. The second patient was a 49-year-old woman with a 3-year history of unilateral, severe, paroxysmal otalgia occurring 4 to 15 times a day with a duration of 3 to 10 minutes. During an attack, her ear felt obstructed, and she noted ipsilateral eyelid edema and ptosis. Both patients quickly became pain-free after taking indomethacin and required its continued use to prevent headache recurrence. CONCLUSIONS: Chronic paroxysmal hemicrania may be manifested by otalgia with a sensation of external ear obstruction. When the otalgia is paroxysmal, unilateral, severe, frequent, and associated with autonomic signs, one should consider the diagnosis of chronic paroxysmal hemicrania, especially because of the prompt response to indomethacin. The most important feature to consider when making the diagnosis of chronic paroxysmal hemicrania is the frequent periodicity of discrete, brief attacks of unilateral cephalgia separated by pain-free intervals. It is hypothesized that the sensation of ear obstruction in these patients is due to swelling of the external acoustic meatus mediated through increased blood flow by the trigeminal-autonomic reflex.     

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.     

Simultaneous occurrence of ipsilateral cluster headache and chronic paroxysmal hemicrania: a case report

A 42-year-old man came to our headache unit in October 1995 complaining of recurrent attacks of headache, which had begun in February 1991. Chronic cluster headache was diagnosed, and he was given verapamil, 360 mg per day. The attacks ceased in the following months and verapamil was stopped in March 1996. In May 1997, a recurrence of the attacks required the readministration of verapamil, 360 mg per day. The attacks decreased (one to three per week), but after 2 months the patient reported a worsening in his condition due to the appearance of shorter attacks, which were diagnosed as chronic paroxysmal hemicrania. The administration of indomethacin, 225 mg per day, resulted in the disappearance of the short attacks. The concomitant occurrence of attacks of cluster headache and chronic paroxysmal hemicrania suggests the presence of shared factors in the pathophysiology of the two forms of headache. This hypothesis is supported by previous reports in the literature.     

Prophylactic Treatment of Cluster Headache with Budipine

SYNOPSIS
For two years we have tested the prophylactic efficacy of budipine for the treatment of cluster headache. The open study was carried out on 27 men and 8 women. 23 patients suffered from episodic and 2 from chronic cluster headache. 7 patients had cluster-migraine, and 3 chronic paroxysmal hemicrania. 20 patients were free of cluster headache attacks 2–6 days after beginning daily prophylactic budipine therapy with 15–60 mg. 14 patients showed a markedly decreased frequency of attacks and the intensity of persisting attacks was diminished. One patient reported only a slight improvement. There were no non-responders to budipine therapy. 10 patients showed only mild budipine side effects. Budipine was found very effective for treatment of cluster headache.     

Cluster headache and paroxysmal hemicrania: differential diagnosis

The utility of the differences between cluster headache (CH) and paroxysmal hemicrania (PH) is limited by the considerable overlap of their clinical characteristics. We compared 54 patients with CH and eight patients with PH in terms of demographic features, characteristics of headache attacks, associated autonomic features, temporal forms of disorders, and response to verapamil. According to our results, clinical features that distinguished CH and PH patients were: maximal pain localization, ocular in CH patients and extra-ocular in PH group; mean attack duration was longer and mean attack frequency was lower in CH patients in comparison with PH patients. Conjuctival injection was the only autonomic sign seen more frequently in CH patients. There were more CH patients with episodic and more PH patients with unremitting form of the disorder in examined groups. Although statistical analysis pointed out a significant difference between these clinical features, there was no clinical characteristic that exclusively belonged to one of these headache entities. Demographic characteristics (age, gender, social background), the other headache attack features (nocturnal attacks, interattack tenderness), the other autonomic signs, as well as the response to verapamil did not differ significantly between two groups.     

Chronic cluster-like headache secondary to prolactinoma: uncommon cephalalgia in association with brain tumors

Headache is a common and disabling aspect of pituitary disease. Chronic and episodic migraine are the most common clinical syndromes of headaches related to pituitary tumors, although other types of headache, such as trigeminal autonomic cephalalgias (TACs), can also be present. TACs include short-lasting, unilateral neuralgiform headache attacks with conjunctival injection and tearing; paroxysmal hemicrania; and cluster headache. We report on a patient with a chronic cluster-like headache associated with a macroprolactinoma. Although cabergoline, pregabalin, and corticosteroids were not effective as preventive treatments, high-dose verapamil showed good efficacy. Morphine and octreotide were efficacious as abortive treatments for attacks, but pain was only partially responsive to oxygen and refractory to subcutaneous sumatriptan.     

Cognitive processing in cluster headache

Little is known about specific changes of cognitive processing in cluster headache. Studies on event-related potentials (ERP) suggest that stimulus evaluation is impaired in chronic cluster headache and in episodic cluster headache during the cluster period, but not in the interval between two periods. Patients with chronic paroxysmal hemicrania do not show this impairment. Unlike patients with migraine, patients with cluster headache do not present with a loss of cognitive habituation as measured by ERP. In neuropsychologic evaluations, a reversible decline of memory processing was detected during the cluster attack, but not between two attacks. Long-term observation revealed no progressive cognitive decline in cluster headache patients over the years. With regard to personality changes, a liability susceptibility to anxiety disorders and to hypochondriasis, but not to mood changes, has been described inconsistently. All changes in alterations of cognitive processing in cluster headache are demonstrated to be mild and do not relevantly contribute to the clinical picture of this disease.     

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.     

Behandlung eines atypischen und therapierefraktären Clusterkopfschmerzes mit Topiramat

Cluster headache is the third most frequent type of primary cephalgia only exceeded in frequency by tension-type headache and migraine. Cluster headache is characterized by periodic attacks of unilateral excruciating pain and accompanying autonomic features. It is arguably the most disabling form of primary headache. Therefore, prompt recognition of this disorder is necessary to provide an opportunity for effective treatment. We report on a patient with cluster headache, who complained about an atypical interval of dull headache between the cluster attacks, thus excluding the differential diagnosis of hemicrania continua. The common drugs for acute cluster attacks proved to be ineffective as well as prophylactic treatment with steroids and verapamil. Only administration of topiramate led to relief of pain.     

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.     

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.     

SUNCT syndrome: forehead sweating pattern

The forehead sweating function has been assessed in SUNCT syndrome-a short-lasting, unilateral, neuralgifonn headache syndrome with autonomic phenomena on the symptomatic side (conjunctival injection, lacrimation, etc.). In the three patients (of a total of six) who could be studied during paroxysms, increased evaporation was present on the symptomatic side of the forehead compared to the non-symptomatic side during attacks or to the symptomatic side between attacks. Basal sweating was generally within control limits, so long as the attack frequency was not so high as to influence the interictal level. During attacks precipitated by eating chocolate or sour apple (in the case of one of the patients), forehead sweating was also increased on the symptomatic side. The forehead sweating responses to heating and pilocarpine were without any notable or systematic asymmetries. The forehead sweating pattern in SUNCT syndrome may differ from the patterns in unilateral headaches like cluster headache, on the one hand (in which there is generally an asymmetry during heating and pilocarpine tests), and chronic paroxysmal hemicrania (CPH) and cervicogenic headache, on the other (where there is no systematic increase during attacks).     

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 paroxysmal hemicrania. IX. On the mechanism of attack-related sweating

In eight patients with chronic paroxysmal hemicrania (CPH), forehead sweating was measured after various provocation tests-body heating, exercise, and subcutaneous pilocarpine administration (0.1 mg/kg body weight). Evaporation was measured bilaterally on the forehead with an Evaporimeter (in g/m²/h). This was carried out in a thermo room under standardized conditions. There was no definite deficit in heat-induced or exercise-induced sweating on the symptomatic side of the forehead, contrary to findings in cluster headache. Neither did pilocarpine lead to any marked initial, temporary predominance of sweating on the symptomatic side, which has previously been found in cluster headache. In cluster headache there may be denervation supersensitivity of the sweat glands in the forehead of the symptomatic side. The present study does not therefore provide evidence for supersensitivity phenomena which could explain the homolateral forehead sweating increase seen during attacks in some CPH patients. The localized sweating increase in the forehead during attacks of CPH may possibly be a result of direct sympathetic stimulation.     

Case Report: Chronic Cluster Headache with Unusual High Frequency of Attacks

SYNOPSIS
A case of chronic cluster headache with unusually frequent attacks (up to 30 per day) is discussed. Such numerous attacks are usually observed in chronic paroxysmal hemicrania only. The patient did not respond to indomethacin therapy, but rapid improvement during lithium treatment was observed.