Cluster headache attacks and multiple sclerosis

We report the case of a patient who developed typical cluster headache attacks and was diagnosed as having multiple sclerosis (MS) at the same time. The headache attacks resolved after i.v. treatment with methylprednisolone. MR imaging showed a pontine demyelinating lesion involving the trigeminal nerve root inlet area, on the same side as the pain. The association between cluster headache and MS has been rarely described before. This case suggests that in patients with cluster headache neuroimaging is often useful in order to exclude structural lesions.     

Cluster headache--acute and prophylactic therapy

Cluster headache (CH) pain is the most severe of the primary headache syndromes. It is characterized by periodic attacks of strictly unilateral pain associated with ipsilateral cranial autonomic symptoms. The majority of patients have episodic CH, with cluster periods that typically occur in a circannual rhythm, while 10% suffer from the chronic form, with no significant remissions between cluster periods. Sumatriptan injection or oxygen inhalation is the first-line therapy for acute CH attacks, with the majority of patients responding to either treatment. The calcium channel blocker verapamil is the drug of choice for CH prevention. Other drugs that may be used for this purpose include lithium carbonate, topiramate, valproic acid, gabapentin, and baclofen. Transitional prophylaxis, most commonly using corticosteroids, helps to control the attacks at the beginning of a cluster period. Peripheral neural blockade is effective for short-term pain control. Recently, the therapeutic options for refractory CH patients have expanded with the emergence of both peripheral (mostly occipital nerve) and central (hypothalamic) neurostimulation. With the emergence of these novel treatments, the role of ablative surgery in CH has declined.     

Botulinum toxin type-A therapy in cluster headache: an open study

The objective of this open single-centre study was to evaluate the efficacy and tolerability of botulinum toxin type-A (BTX-A) as add-on in the prophylactic treatment of cluster headache (CH). Twelve male patients with episodic (n=3) or chronic (n=9) CH, unresponsive to common prophylactic medications, were treated with a cumulative dose of 50 International Units (IU) BTX-A according to a standardised injection scheme into the ipsilateral pericranial muscles. One patient with chronic CH experienced a total cessation of attacks and in 2 patients attack intensity and frequency improved. In another patient with chronic CH typical attacks were not influenced, but an ipsilateral continuous occipital headache significantly improved. Patients with episodic CH did not benefit from BTX-A treatment. Tolerability was excellent. These findings provide evidence that BTX-A may be beneficial as an add-on prophylactic therapy for a limited number of patients with chronic CH.     

Electrical Stimulation of Sphenopalatine Ganglion for Acute Treatment of Cluster Headaches

(Headache 2010;50:1164‐1174) Introduction.— Cluster headaches (CH) are primary headaches marked by repeated short‐lasting attacks of severe, unilateral head pain and associated autonomic symptoms. Despite aggressive management with medications, oxygen therapy, nerve blocks, as well as various lesioning and neurostimulation therapies, a number of patients are incapacitated and suffering. The sphenopalatine ganglion (SPG) has been implicated in the pathophysiology of CH and has been a target for blocks, lesioning, and other surgical approaches. For this reason, it was selected as a target for an acute neurostimulation study. Methods.— Six patients with refractory chronic CH were treated with short‐term (up to 1 hour) electrical stimulation of the SPG during an acute CH. Headaches were spontaneously present at the time of stimulation or were triggered with agents known to trigger clusters headache in each patient. A standard percutaneous infrazygomatic approach was used to place a needle at the ipsilateral SPG in the pterygopalatine fossa under fluoroscopic guidance. Electrical stimulation was performed using a temporary stimulating electrode. Stimulation was performed at various settings during maximal headache intensity. Results.— Five patients had CH during the initial evaluation. Three returned 3 months later for a second evaluation. There were 18 acute and distinct CH attacks with clinically maximal visual analog scale (VAS) intensity of 8 (out of 10) and above. SPG stimulation resulted in complete resolution of the headache in 11 attacks, partial resolution (>50% VAS reduction) in 3, and minimal to no relief in 4 attacks. Associated autonomic features of CH were resolved in each responder. Pain relief was noted within several minutes of stimulation. Conclusion.— Sphenopalatine ganglion stimulation can be effective in relieving acute severe CH pain and associated autonomic features. Chronic long‐term outcome studies are needed to determine the utility of SPG stimulation for management and prevention of CH.     

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.     

Migraine - Cluster Headache Syndrome

SYNOPSIS
The migraine-cluster headache syndrome is defined as headaches that are predominately of one type but with at least one major timing factor plus three lesser features of the other or five lesser features of the other (no more than one of the opposing features is a weak differential feature). The most important factors distinguishing migraine (M) from cluster headache (CH) are the timing of attacks (random occurrence versus clustering, frequency, duration, hour of onset) and the prodromal phenomena which occur with classical M, but are rarely seen with CH. Somewhat less significant differentiating characteristics are the associated symptoms of ipsilateral eye redness and tearing, nasal congestion and secretion, and partial Horner's syndrome which often accompany CH, in contrast to the symptoms of nausea and vomiting commonly associated with M. Also helpful in establishing the diagnosis are the quality, site and recurrent laterality of the pain, as well as its aggravating and ameliorating factors. Weak features in the differential diagnosis of M and CH are age of onset, sex, family history, and response to therapy.
Patients with M or CH seen at the Headache Unit of Montefiore Medical Center during the past two years were evaluated for the occurrence of the migraine-cluster headache syndrome. Nine (1.0%) of 923 patients with M had 4 or more features of CH and 5 (3.2%) of 154 patients with CH had 4 or 5 characteristics of M. In 4 patients symptoms of M and CH were blended in such a way neither headache predominated.     

Cluster headache with aura

The objective of our study is to report the frequency and characteristics of cluster headache with aura among the population of patients with cluster headache treated in our outpatient neurology clinic. 254 patients were submitted to semi-structured interviews to identify the presence of symptoms similar to the migraine aura. 5 patients who suffered from a cluster headache with aura filled a diary with the characteristics of the pain attacks and the aura. All the patients with either episodic or chronic cluster headache were studied. The pain attacks were associated with symptoms similar to the migraine aura in five patients (2%). These disorders were usually ipsilateral to the pain and consisted of paresthesias of the trigeminal territory, clumsiness of the limbs or visual disturbances. Neither paresis nor hypoesthesia could be proved by clinical examination during the attack. We propose to reserve the name of cluster headache with aura to these cases that show similar symptomatology to the migraine aura. These manifestations suggest the participation of the central nervous system in this type of cluster headache. Received: 16 July 2001, Accepted in revised form: 4 December 2001 Correspondence to E. Martínez-Fernández     

Pathophysiology and treatment of trigeminal autonomic cephalalgias

Trigeminal autonomic cephalalgias (TACs) are a group of primary headache disorders, which are characterized by strictly unilateral pain, together with ipsilateral cranial autonomic symptoms. TACs include cluster headache (CH), paroxysmal hemicrania (PH) and short-lasting unilateral neuralgiform headache attacks with conjunctival injection and tearing (SUNCT syndrome). These diseases all have one thing in common: an activation of trigeminal nociceptive afferentia with a reflex-like activation of cranial autonomic efferentia via the facial nerve. TACs show differences not only in the length and frequency of attacks but also in the response to drug treatment. It is important to recognize and differentiate between these syndromes because they react very well, but very selectively to therapy.     

Neues zur Pathophysiologie und Therapie der trigeminoautonomen Kopfschmerzsyndrome

Trigeminal autonomic cephalalgias (TACs) are a group of primary headache disorders, which are characterized by strictly unilateral pain, together with ipsilateral cranial autonomic symptoms. TACs include cluster headache (CH), paroxysmal hemicrania (PH) and short-lasting unilateral neuralgiform headache attacks with conjunctival injection and tearing (SUNCT syndrome). These diseases all have one thing in common: an activation of trigeminal nociceptive afferentia with a reflex-like activation of cranial autonomic efferentia via the facial nerve. TACs show differences not only in the length and frequency of attacks but also in the response to drug treatment. It is important to recognize and differentiate between these syndromes because they react very well, but very selectively to therapy.     

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.     

Ophthalmologic aspects of headache

Pain around the eye can be caused by local ophthalmic disorders or by disease of other structures sharing trigeminal nerve sensory innervation. In general, most ocular causes for pain also cause the eye to be red, thus alerting the examiner to the focality of the problem. However, conditions like eyestrain, intermittent angleclosure glaucoma or neovascular glaucoma, and low-grade intraocular inflammation can be painful and not be associated with obvious redness. Ocular signs and symptoms also occur with numerous other causes of headache. Double vision in association with periocular pain can result from orbital lesions, isolated cranial neuropathies, and cavernous sinus lesions. Pupillary abnormalities like Horner's syndrome may result from a variety of painful conditions, including cluster headache, parasellar neoplasms or aneurysms, internal carotid dissection or occlusion, and Tolosa-Hunt syndrome. Pain with a dilated and unreactive pupil may reflect a benign condition like Adie's syndrome or ophthalmoplegic migraine, or it may herald the presence of a life-threatening posterior communicating artery aneurysm. Headache and transient visual loss can be manifestations of classic migraine, or be symptoms of ocular hypoperfusion from ipsilateral internal carotid occlusion or increased intracranial pressure from pseudotumor cerebri. In a young patient, head pain with a fixed visual deficit may result from optic neuritis, in an older adult, temporal arteritis may be the culprit. Ophthalmologic aspects of headache thus encompass problems that range from simple and benign to complex and formidable.     

Management of cluster headache in the Emergency Department

Although cluster headache (CH) is considered one of the most distinctive and painful primary headache disorders in clinical practice because of the brevity of each attack, its management is not always ergonomic or possible in the Emergency Department. In case of a previously competent diagnosis, the Emergency Department’s team should send the patient to a headache centre where specialists in the management of CH can handle the pathology in the best way. In our headache centre we treat patients with CH attacks with a hyperbaric chamber, confirming the effectiveness of hyperbaric oxygen in CH patients.     

Cataract in chronic cluster headache: two case reports and review of the literature

Cluster headache (CH) consists of attacks of severe, unilateral orbital/supraorbital/temporal pain, lasting for 15–180 min, occurring once or more times a day, and associated with ipsilateral conjunctival injection, lacrimation and other symptoms. Cataract is clouding of the lens of the eye causing a progressive and painless loss of vision. We describe the cases of two men (not relative, but with the same last name, which originates from north-eastern Italy) that in young adult age, after years of suffering from chronic CH, developed cataract on the same side of the pain attacks. Patient 1 was diagnosed as having cataract 18 years after the onset of episodic (and subsequently chronic) CH. Patient 2 began suffering from chronic CH at the age of 44 years and after 8 years he developed cataract. This is the first report of cataract in patient suffering of CH and occurring in the eye affected by the pain attack. No financial support received.     

Oxygen therapy influences episodic cluster headache and related cutaneous brush and cold allodynia

Cluster headache (CH) is characterized by a series of sudden attacks of short-lasting severe headache pain with ipsilateral autonomic features, including lacrimation, rhinorrhea, localized sweating, eyelid edema, and partial or complete Horner's syndrome. Just like in migraine, brush allodynia has been described for CH and for short lasting unilateral neuralgiform headache with conjunctival injection and tearing (SUNCT) syndrome. Administration of normobaric oxygen is part of the standard therapy for CH attacks. Here, we describe a young male with a first CH attack and the influence of oxygen on pain and concomitant cutaneous allodynia.     

Episodic Cluster Headache: NREM Prevalence of Nocturnal Attacks. Time to Look Beyond Macrostructural Analysis?

(Headache 2010;50:1050‐1054) Background.— A high prevalence of nocturnal sleep‐related attacks is reported in patients with cluster headache (CH). Episodic CH is considered closely related to rapid eye movement (REM) sleep. Objective.— The aim of this study was to analyze the relationships between episodic CH attacks and sleep macrostructure. Methods.— Data were obtained by means of 24‐hour continuous ambulatory polysomnography (PSG) capturing CH attacks in 4 out of 7 episodic CH patients (all males; mean age 38.4 ± 9.2 years) studied. Results.— Eight CH attacks were captured during the PSG monitoring; 5 arose from sleep: 4 from non‐rapid eye movement (NREM) sleep (stage 2 NREM), and 1 from REM sleep. One patient experienced CH attacks during both NREM and REM sleep in the same night. Conclusions.— In the light of previous literature findings, the prevalence of NREM‐related episodic CH attacks observed, and the finding of attacks arising during both REM and NREM sleep in the same subject, suggest that the relationship between CH and sleep stages is heterogeneous, and the existence of a specific macrostructural pattern associated with episodic CH attacks appears to be uncertain. A more comprehensive approach taking into account the microstructure of NREM and REM sleep is expected to provide more in depth information about the pathophysiology of CH, whose complexity might overcome the simplistic dichotomy of REM/NREM staging.     

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.     

Transient side shift of cluster headache attacks after unilateral greater occipital nerve injection

Attacks of cluster headache (CH) are usually side-locked in most, but not all, patients. In a few patients, the side may alternate between or, rarely, within cluster episodes. We observed seven cases in whom the side of CH attacks temporarily shifted immediately or shortly after unilateral injection of the greater occipital nerve (GON) with corticosteroids. In five patients with previously side-locked CH attacks and in two patients with previously side-alternating CH attacks, a side shift for several weeks occurred immediately (N = 6) or shortly (N = 1) after GON injection. We concluded that unilateral GON injections might cause a transient side shift of CH attacks through inhibition of the ipsilateral hypothalamic attack generator causing relative overactivity of the contralateral side. The potential benefit of bilateral GON injection in patients who experienced a side shift after unilateral injection should be formally investigated.     

Extratrigeminal Cluster Headache

SYNOPSIS
Three cases with periodicity and pain profile characteristic of episodic cluster headache, whose headaches were solely confined to the regions of the head and neck outside the trigeminal territory, are reported. Two were females, who had associated nausea and vomiting with severe attacks. The male patient exhibited autonomic symptoms in the eye during the attacks. Alcohol induced headache in one. All three patients responded to anticluster headache therapy.
These cases are illustrative of a wider spectrum of clinical manifestations of cluster headache than was originally recognized. They question the theory that cluster headache may be due to a lesion involving the cavernous sinus. One the other hand, it points to involvement of a more complex pain circuit consisting of upper cervical nerves, posterior fossa innervation, trigeminal system and the autonomic pathways.     

A double-blind placebo-controlled trial of intranasal capsaicin for cluster headache

It has been suggested that treatment of cluster headache (CH) patients with topical capsaicin may desensitize sensory neurons by depleting the nerve terminals of substance P. We attempted to determine whether capsaicin is effective in aborting CH attacks. Patients in acute cluster were randomized to receive either capsaicin or placebo in the ipsilateral nostril for 7 days. Patients recorded the severity of each headache for 15 days. Headaches on days 8–15 of the study were significantly less severe in the capsaicin group vs the placebo group. There was also a significant decrease in headache severity in the capsaicin group on days 8–15 compared to days 1–7, but not in the placebo group. Episodic CH patients appeared to benefit more than chronic CH patients. These results indicate that intranasal capsaicin may provide a new therapeutic option for the treatment of this disease.     

Cluster headache without autonomic symptoms: why is it different?

BACKGROUND: Some patients with otherwise typical cluster headache (CH) have persistent attacks free of cranial autonomic symptoms (CAS). The factors responsible for this atypical presentation are not known. OBJECTIVES: To identify factors associated to the absence of CAS in patients with CH. METHODS: A prospective series of 157 patients with the diagnosis of CH was analyzed, comparing 148 typical CH patients with 9 CH patients without CAS. RESULTS: Patients without CAS reported significantly less intense attacks (P = .003) when compared to those with CAS. There was also a tendency (not reaching statistical significance) for a higher frequency of females and chronic CH among those without CAS. Otherwise, there were no differences between the two groups (in age, duration of illness, follow-up time, attack duration or frequency, nor side or site of pain). A logistic regression analysis showed that only pain intensity could explain the difference between the two groups, since the other explanatory variables were also associated with different intensity of attacks. CONCLUSIONS: These results support the hypothesis that CH without cranial autonomic symptoms represents a milder form of CH.