Cluster Headache as the Index Event in MS: A Case Report

We report a 42‐year‐old woman who presented with cluster headache (CH) in association with other neurological symptoms as the index event of new onset multiple sclerosis (MS). Her initial symptoms were left‐sided headache with ipsilateral lacrimation and nasal congestion associated with ipsilateral facial numbness. A subsequent similar headache attack was also associated with ipsilateral arm ataxia and gait ataxia. She had many additional short headache attacks without focal neurological symptoms. Her cluster‐like headache attacks have not recurred since intiation of dimethyl fumarate. Our patient illustrates that cluster‐like headache attacks can occur as a first symptom of MS, in our patient in association with other neurological symptoms. A striking finding in our patient was a large demyelinating lesion in the brachium pontis ipsilateral to the headaches, although additional supratentorial demyelinating lesions were also present. Although CH associated with MS is rare, our patient and the two other reported patients with MS and CH with similar ipsilateral brachium pontis lesions suggest that the lesions in this location may have played a role in the generation of the cluster‐like attacks.     

Nuchal Features of Cluster Headache

Nuchal symptoms were found in the majority of 100 consecutive patients with cluster headache. In 10%, pain was experienced in the neck with the initial typical orbitotemporal pain; in 37%, pain radiated from the orbit or temple to the ipsilateral side of the neck. Sometimes, neck pain heralded the onset of the attack by a few minutes. During an attack, neck stiffness was reported in 40% and tenderness in 29%. Movement of the neck, especially flexion, precipitated cluster headache in 9% of patients. This was particularly true of patients with chronic cluster headache. Neck movement aggravated the headache in 16 of 100 patients and an equal number reported amelioration of pain by neck movement, especially extension. The nuchal features did not necessarily accompany every attack and were usually overshadowed by the severity of the typical headache. Nevertheless, symptoms referable to the neck occur more commonly than is generally appreciated.     

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.     

Subchronic Cluster Headache

SYNOPSIS
A subgroup of 33 patients were distinguished from 59 other patients with episodic cluster headache by shorterremission periods, specifically of less than six months duration. This subgroup (subchronic cluster headache)was compared to episodic cluster headache patients and an additional 29 patients with chronic cluster headache,for differences in several clinical parameters.Results of this study demonstrated that the subchronic group was clinically similar to the chronic group andsignificantly different from episodic cluster headache patients. Thus, sub-chronic cluster headache wascharacterized by an increased frequency of treatment-resistance, headache attacks, drug abuse, hypochondriasisand prolonged cluster periods.Results of this study suggests that subchronic cluster headache, as defined by remission duration of sixmonths or less, may be a clinically relevant class of cluster headache. Distinction of this subgroup from thebroader "episodic" category may permit more accurate prognostic and therapeutic capabilities.     

Cluster Headache: Response to Chlorpromazine

SYNOPSIS
Chlorpromazine (CPZ), in doses ranging from 75–700 mg per day, gave full headache relief in twelve of thirteen (93%) of patients with the cluster headache syndrome. For nine, withdrawal of CPZ within two to three weeks was tolerated without recurrence of symptoms. After a period of extended remission, four of these found CPZ equally effective treatment for subsequent attacks. Three others received sustained relief from headache over six to eight months on maintenance CPZ at 75 mg per day although the cluster was not terminated. The apparent effectiveness of CPZ in this limited series is greater than that of other medications currently employed in the treatment of cluster headaches.     

Cluster Headache with Aura

Aura was not recognized as a clinical symptom of cluster headache until fairly recently, but studies now have indicated that upwards of 20% of patients with cluster headache may have aura, the same percentage of migraine sufferers who have aura. This paper looks at the epidemiology of cluster headache with aura, suggests possible roles of cortical spreading depression in cluster headache pathogenesis, and looks at the clinical/diagnostic implications of aura in cluster headache sufferers.     

Role of Sphenopalatine Ganglion Neuroablation in the Management of Cluster Headache

Cluster headache is a primary neurovascular headache. It is a strictly unilateral head pain that is associated with cranial autonomic symptoms and usually follows circadian and circannual patterns. Chronic cluster headache, which accounts for about 10% to 15% of patients with cluster headache, lacks the circadian pattern and is often resistant to pharmacological management. The sphenopalatine ganglion (SPG), located in the pterygopalatine fossa, is involved in the pathophysiology of cluster headache and has been a target for blocks and other surgical approaches. Percutaneous radiofrequency ablation of the SPG was shown to have encouraging results in those patients with intractable cluster headaches.     

2. Cluster Headache

Cluster headache is a strictly unilateral headache that is associated with ipsilateral cranial autonomic symptoms and usually has a circadian and circannual pattern. Prevalence is estimated at 0.5 to 1.0/1,000. The diagnosis of cluster headache is made based on the patient's case history. There are two main clinical patterns of cluster headache: the episodic and the chronic. Episodic is the most common pattern of cluster headache. It occurs in periods lasting 7 days to 1 year and is separated by at least a 1-month pain-free interval. The attacks in the chronic form occur for more than 1 year without remission periods or with remission periods lasting less than 1 month.
Conservative therapy consists of abortive and preventative remedies. Ergotamines and sumatriptan injections, sublingual ergotamine tartrate administration, and oxygen inhalation are effective abortive therapies. Verapamil is an effective and the safest prophylactic remedy. When pharmacological and oxygen therapies fail, interventional pain treatment may be considered. The effectiveness of radiofrequency treatment of the ganglion pterygopalatinum and of occipital nerve stimulation is only evaluated in observational studies, resulting in a 2 C+ recommendation.
In conclusion, the primary treatment is medication. Radiofrequency treatment of the ganglion pterygopalatinum should be considered in patients who are resistant to conservative pain therapy. In patients with cluster headache refractory to all other treatments, occipital nerve stimulation may be considered, preferably within the context of a clinical study.     

Recurrence of Cluster Headache After Carotid Thrombendarterectomy

SYNOPSIS
The pathophysiology of cluster headache is largely unknown. One important contributing factor may be an abnormal intracranial-extracranial hemodynamic state. A male patient suffered from chronic left-sided cluster headache for about 15 years. After the institution of lithium therapy the symptoms abated. He was completely free from cluster headache for more than 20 years, until the first postoperative day after a thrombo-endarterectomy for symptomatic 70% carotid stenosis.
This case report indicates the importance of abrupt carotid hemodynamic changes along with dysfunction of the cephalic sympathetic nervous system in the initiation of cluster headache.     

Corneal Sensitivity in Cluster Headache

SYNOPSIS
Cluster headache pain is thought to arise from vasodilatation, but a neural origin of the pain has never been completely excluded. Some patients with cluster headache have complained of subjective diminution of sensation over the ipsilateral forehead. This is hard to quantitate and is often variable. Besides, ablation of trigeminal sensory pathways has been re sorted to for relief of pain in some intractable cases.
The present study was undertaken to quantitatively assess the sensitivity of the cornea in cluster patients. Fifteen cluster headache patients were the subjects of this study. Measurement of the corneal sensitivity was undertaken using a Cochet-Bonnet Aesthesiometer. There was no difference between the affected and the unaffected eye. There was no difference in the sensitivity when testing was done during the cluster period and later on when the patients were in remission. It is concluded from this study that there is no objective evidence for involvement of trigeminal sensory pathways in patients with cluster headache.     

Dexamethasone Suppression Test in Cluster Headache

SYNOPSIS
The dexamethasone suppression test was performed on eleven patients with cluster headache. Interest in such investigation of the hypothalamo-pituitary-adrenal axis in cluster headache arose from both a certain analogy with manic-depressive disorders and recent data on abnormal patterns of cortisol secretion in cluster headache. Investigation results were normal in all patients, showing that the hypothalamo-pituitary-adrenal dysfunction reported in depressions is not a biological feature of cluster headache.     

Ciliospinal Reflex Response in Cluster Headache

The ciliospinal reflex response is mainly mediated by second- and third-order sympathetic nerves to the dilatator muscle of the iris. As the pupillary response to various pharmacological agents indicates a sympathetic dysfunction in patients with cluster headache, the ciliospinal reflex was studied in 25 patients. Five of these patients with cluster headache exhibited a Horner-like syndrome (miosis, ptosis) on the symptomatic side. The pupillary responses to phenylephrine and tyramine showed that the Horner-like syndrome was due to postganglionic sympathetic nerve dysfunction. Their ciliospinal reflex response on the symptomatic side was significantly less than in controls and in other patients with cluster headache, lacking a Horner-like syndrome. This also applied to the nonsymptomatic side compared to the majority of cluster headache patients without any clinical evidence of sympathetic nerve dysfunction.
These findings seem to delineate those patients with a Horner-like syndrome as a subgroup, distinctly separated from the majority of cluster headache patients. Furthermore, the findings indicate that the Horner-like syndrome is not a consequence of repeated attacks of headache over many years, but is a manifestation of bilateral cephalic sympathetic dysfunction being more marked on the symptomatic side.
In 18 (72%) of our 25 patients, an asymmetric and lower ciliospinal reflex response on the symptomatic side was seen. In 3 (12%) patients, there was no difference in the response. In 4 patients (16%), the incorrect side was indicated by an asymmetric reflex response. Two of these patients (8%) had suffered from cluster headache on alternating sides.
In summary, the findings support the concept that dysfunction of the sympathetic nervous system, whether peripheral or central, is involved in the pathophysiology of cluster headache.     

Percutaneous Radiofrequency Trigeminal Gangliorhizolysis in Intractable Cluster Headache

SYNOPSIS
Recent interest in the possible role of substance P and other vaso-active polypeptides of the trigeminal vascular system, in the pathogenesis of vascular headache, has lad to a reconsideration of the value of ablative procedures on the trigeminal nerve in the control of chronic, medically intractable cluster headache. Twenty-seven patients with disabling intractable chronic cluster headache underwent radiofrequency trigeminal gangliorhizolysis. Indications included total resistance to prophylactic treatment, narcotic dependency, hypercorticism, and contra-indications to ergotamine and methysergide as a result of severe ischemic heart disease. The procedure had to be repeated in 6 patients, once in 4 and twice in 2. The average follow-up period was 28 months with a range of 6 to 63 months. Excellent results were obtained in 15 patients, very good in 2, good in 3, fair in 1 and poor in 6. Complications were anesthesia dolorosa, stabbing pain over the vertex, ice-pick like pain over the ipsilateral eye, transient corneal infection, transient diplopia, localized dermatosis, and recurrent stye. Complications were mild and transient in the majority of the patients, and the benefits from the surgery far outweighed the discomfort from the complications. The reasons for poor results in some patients were analyzed.
It is concluded that radiofrequency trigeminal gangliorhizolysis is a reasonable alternative in patients with chronic cluster headache 1) who are totally resistant to medical treatment, 2) with a history of strictly unilateral headache, and 3) with stable personality profile and low addiction proneness.     

Metabolic Abnormality in Cluster Headache

SYNOPSIS
Cluster headache has a number of distinct features in the periodicity and frequency of headache and associated phenomena such as, ipsilateral rhinorrhoea and lacrimation. In some cases, however, it may be difficult to confirm diagnosis and apply the appropriate treatment. Lithium is beneficial in cluster headache but would exacerbate migraine. Hence there is a need for diagnostic methods for migraine or migraine subtypes and cluster headache. We have found that one readily measured parameter, erythrocyte choline, is significantly depressed in cluster headache patients both during and between attacks and this may therefore offer an aid to diagnosis. Further analysis of the pathways giving rise to this abnormality may give insight to the aetiology of cluster headache, as discussed below.     

Response of Cluster Headache to Kudzu

Objective.— To describe the self-treatment of cluster headache with kudzu.
Background.— Many cluster headache patients take over-the-counter (OTC) kudzu extract in the belief that it helps their cluster attacks. Kudzu's actual efficacy has not been studied.
Methods.— A database of cluster headache patients was questioned about their use of various alternative remedies to treat their cluster headache. Of 235 patients identified, 16 had used kudzu, consented to interviews, and provided medical records.
Results.— In total, 11 (69%) experienced decreased intensity of attacks, 9 (56%) decreased frequency, and 5 (31%) decreased duration, with minimal side effects.
Conclusion.— Anecdotal evidence suggests that a component in OTC products labeled as kudzu may prove useful in managing cluster headache. This hypothesis should be tested with a randomized clinical trial.     

增加副鼻窦CT扫描对诊断不明原因头痛的临床价值

目的：探讨增加副鼻窦ＣＴ扫描对诊断不明原因头痛的临床价值。材料与方法,对８０例颅脑ＣＴ扫描结果阴性的头痛患者增加副鼻窦螺旋ＣＴ扫描。并作模拟内窥镜,结果：发现副鼻窦炎患者２３例,其中上颌窦炎合并筛窦炎与鼻甲肥大患者９例,同时合并蝶窦炎与额窦炎患者２例,单纯鼻甲肥大患者１２例,鼻中隔偏曲６例,上颌窦囊肿１例,副鼻窦肿瘤１例。结论颅脑与副鼻窦联合ＣＴ扫描,可提高不明原因头痛的确诊率,有利于及时发现副鼻     

Prednisone Therapy for Cluster Headache

SYNOPSIS
Prednisone in peak doses of 10-80 mg/day was employed for therapy in 19 patients with cluster headache. Overall, 14 of the 19 patients (73%) had greater than 50% relief and 11 (58%) had 100% relief from headache. The remaining 5 patients had less than 50% improvement. Of the subjects who were greater than 50% improved, 12 received peak doses of prednisone of 40 mg. or more, one received 30 mg. and one received 10 mg. The subjects with less than 50% improvement received peak prednisone doses of 10, 30, 40, 60 and 80 mg. Recurrence of cluster headache was noted in 79% of patients when the prednisone dose was tapered below 10–20 mg./d. Prednisone appears effective in relief of cluster headache but recurrence can be expected as the dose is reduced.     

Gamma Knife Treatment of Refractory Cluster Headache

Four men and two women were treated for refractory cluster headache by gamma knife radiosurgery of the trigeminal nerve root entry zone. The maximum dose of radiation was 70 Gy to the isocenter. Of five patients treated who had refractory chronic cluster headache and one with refractory episodic cluster headache, four had relief judged excellent. Of the two remaining patients with refractory chronic cluster headache, one had relief judged good and the other fair. Five of the six patients treated had relief within a few days to a week following gamma knife radiosurgery. Three with chronic cluster headache had remissions allowing cessation of all preventive and abortive medication. Although one patient experienced complete relief of chronic cluster headache, he continued to have migraine requiring medication. None of the patients treated developed significant postradiation side effects during a follow-up period of 8 to 14 months. The authors conclude that gamma knife radiosurgery of the trigeminal nerve affords great promise in the management of chronic and refractory cluster headache. The technique seemingly carries negligible short- and long- term risk.     

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.     

Lisuride in Cluster Headache

SYNOPSIS
Ten patients with cluster headache of different types, treated previously with several kinds of drugs, received lisuride hydrogen maleate in doses varying from 2X0.025 mg to 16X0.025 mg.
The results obtained with lisuride were quite satisfactory as compared to results with other drugs, and there were no side-effects in this small group of patients.
Comparatively to migraine, which requires low dosages of lisuride, cluster headache seems to require a higher dosage of the drug for better results.