Extracranial Vascular Responses to Sublingual Nitroglycerin and Oxygen Inhalation in Cluster Headache Patients

SYNOPSIS
Photoplethysmography was used to record pulsations from the superficial temporal artery and supraorbital region in 23 patients suffering from cluster headaches, in 10 patients between bouts of headache, and in 10 control subjects. Pulsations were measured before and after the administration of 0.9 mg nitroglycerin sublingually, at the onset and height of cluster attacks and after inhalation of 100% oxygen for 10 minutes. Following nitroglycerin administration, increases in superficial temporal artery pulsations were greater on the symptomatic than on the nonsymptomatic side in patients who later developed headache. This persisted during cluster headache. By contrast, the increase in superficial temporal artery pulsations was greater on the nonsymptomatic side in patients who did not develop headache. The cause of this paradoxical response is unclear. Inhalation of oxygen produced significantly greater reduction of supraorbital pulsations on the symptomatic side in patients with headache. Decreases in both arterial territories following oxygen inhalation were significantly greater during cluster headache than between bouts or than in control subjects.     

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.     

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.     

MRI of Intracranial Arteries in Nitroglycerin Induced Cluster Headache Attacks

Eight patients with episodic cluster headache, five in active episode, three out of episode, were investigated as to diameters of intracranial arteries before and after nitroglycerin (NG) administration. The diameter of all intracranial carotids were increased about 10 minutes after NG, although more in the patients in episode than in patients out of episode. The dilatation remained for the next 60 minutes in the patients who did not get a cluster headache attack. There was a normalization of the diameters of the internal carotid arteries compared to the initial values, at maximum pain in all patients who got a cluster headache attack. Similar changes were also found in the basilar arteries. The findings support the hypothesis of a constriction of intracranial arteries at maximum pain in cluster headache attacks to stop the pain.     

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.     

Preventive Effects of Hyperbaric Oxygen in Cluster Headache

The effect of a 2-week course of hyperbaric oxygen on both the duration and frequency of cluster headache attacks was tested in four patients suffering from chronic cluster headache with no clear response to pharmacological treatments. Two patients (two courses in one case) dramatically improved while on hyperbaric oxygen treatment, this positive response remaining for 2 and 31 days posttreatment. Case 3 only improved in frequency, while the remaining patient showed no benefit. These findings suggest that daily hyperbaric oxygen treatment can be used as a transient preventive treatment for desperate cluster headache sufferers.     

Assymetry of Temporal Artery Diameters During Spontaneous Attacks of Cluster Headache

Background.— Cluster headache is characterized by strictly unilateral head pain associated with symptoms of cranial autonomic features. Transcranial Doppler studies showed in most studies a bilateral decreased blood flow velocity in the middle cerebral artery.
Objective.— To investigate whether there is a bilateral or unilateral extracranial vasodilation during spontaneous cluster headache attacks.
Design and methods.— In 9 cluster headache patients, we investigated the luminal diameter of the superficial temporal artery with ultrasound on the headache and headache-free side during and outside cluster headache attacks.
Results.— During cluster headache attacks, the diameter of the superficial temporal artery on the painful side was greater, 1.48 mm, than the diameter on the nonheadache site, 1.14 mm ( P  < .01). Outside attacks, median diameters on the 2 sides were quite comparable, 1.34 vs 1.31 mm ( P  = .67).
Conclusions.— What was observed is most likely a general pain-induced arterial vasoconstriction (confer the decrease in diameter on the pain-free side) with an unchanged superficial temporal artery on the pain side because of some vasodilator influence.     

Association of Sustained Oxyhemoglobin Desaturation and Onset of Cluster Headache Attacks

Ten episodic cluster headache patients in their active cluster period, ten patients in remission and five control subjects were monitored for minute to minute changes in oxygen saturation (SaO2) and pulse rate before and after nitroglycerin (NTG) administration. A transient but significant decrease in SaO2 and increase in pulse rate of 25 minutes duration occurred following NTG in all groups. These changes may reflect physiologic hemodynamic effects of NTG as a smooth muscle relaxant. Subsequently, SaO2 levels and pulse rate recovered to baseline values in remission and control groups. In contrast, SaO2 values in the active cluster group decreased further and after an extended period culminated in cluster headache attacks in 10/10 patients. Three major changes, therefore, distinguished active cluster patients from remission and control groups. First, the magnitude of oxygen desaturation increased after the physiological effects of NTG ceased. Second, oxygen desaturation was sustained for an additional 9 to 30 minutes duration. Third, the hypoxemic state culminated in attacks in all cases. Our findings suggest that the active cluster period may be characterized by an impaired mechanism to autoregulate, and thus compensate, for hypoxemia. It is further proposed that persistence of hypoxemia and the cluster attack onset may share a common mechanism, coupling the two events. We suggest that abnormal central and/or peripheral chemoreceptor activity may be responsible for these events.     

MR-Angiography During Spontaneous Attacks of Cluster Headache: A Case Report

SYNOPSIS
Magnetic resonance (MR) angiography was performed during and between two spontaneous and untreatedattacks in a 24-year old male patient with episodic cluster headache. The ipsilateral ophthalmic artery wasobserved to be markedly dilated during both attacks. No changes were seen in the internal carotid artery or any ofthe main arterial branches on either side. A repeat MR-angiography two weeks later when the patient was inclinical remission showed no abnormalities. Our present case report suggests that the MR technique provides auseful method for non-invasive angiography during spontaneous attacks of cluster headache.     

Clinical Subtypes of Cluster Headache and Response to Lithium Therapy

SYNOPSIS
Based on clinical features, a group of 31 patients with cluster headache was divided into 1) episodic cluster headache, with months or years of headache free intervals, 2) chronic cluster headache without significant headache free intervals. Chronic type could be subdivided into primary and secondary varieties, secondary being a transformation from episodic cluster headache. A clinical trial of Lithium Carbonate was undertaken. Blood levels of lithium were determined at regular intervals to monitor the therapeutic dosage. Lithium was found to be an effective prophylactic agent in both episodic and chronic cluster headache patients. The percentage of improvement based on headache index was as follows: 55% of patients had more than 90% improvement; 10% of patients showed 60–90% improvement; 15% of patients showed 25–60% improvement; and 20% no improvement. Effectiveness of lithium was evident in less than a week after the initiation of treatment in those who responded. 55% of patients showed mild side effects such as tremor, nausea, diarrhea, abdominal discomfort and lethargy. Only one patient had serious side effects which needed discontinuation of therapy.The beneficial effect of lithium on cluster headaches appear to be independent of its anti-depressant action. Mechanism of action of lithium in cluster headache is not clear.     

Cerebrospinal Fluid Pressure and Venous Pressure in "Dynamite Headache" and Cluster Headache Attacks

Six patients with episodic cluster headache were investigated as to blood pressure, heart rate, cerebrospinal fluid pressure (Pcsf) and frontal vein pressure (Pvf) during five nitroglycerin (NG) provoked attacks and one spontaneous attack. In a seventh studied patient the NG failed to provoke an attack. The earlier reported decrease of systolic blood pressure and increase of diastolic blood pressure and heart rate after NG administration were also found in these patients. The "dynamite headache" was related to the start and duration of an increase of the cerebrospinal fluid pressure. There was no relationship between the start or the maximum pain of the cluster headache attack and changes in Pcsf or Pvf. On breathing oxygen during a cluster headache attack, there was a decrease of Pcsf but in some patients a temporary increase of Pvf was observed, which possibly indicates that oxygen simultaneously attains constriction of arteries and veins.     

Cluster Headache: The Effect of Low Oxygen Saturation

The present study concerns the possible relationship between hypoxia and the generation of cluster headache attacks. Fifteen controls and 25 cluster headache patients were studied. The patients were allocated into two groups according to cluster headache stage, i.e. cluster or remission period. During the tests, all the subjects were asked to inhale 12% oxygen (88% N2) for 30 min, and the decreasing oxygen saturation (SaO2%) was monitored. Patients in the remission period showed nearly the same decrement of SaO2% as controls. At the end of the test, patients in the bout showed significantly less reduction of SaO2% than the controls. In 5 patients, the test was carried out both in and outside the cluster periods. The tendency to less decrement in oxygen saturation in the cluster phase was as marked with this comparison, but the difference between the groups was not significant, probably partly due to the low number of tests carried out. Only one patient got a typical attack. It seems that hypoxia of this magnitude per se is not the cause of attacks. The different pattern with respect to SaO2% following 12% O2 inhalation in cluster headache may be due to an abnormality in central regulation and/or chemoreceptor sensitivity.     

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.     

Cluster Headache in Two Sisters. Pupillary Response to Phenylephrine and Tyramine

Two sisters with cluster headache were studied with respect to the pupillary responses to instillation into the conjunctival sac of a single drop of a 1% solution of phenylephrine and a 2% solution of tyramine. The changes in pupillary diameters were documented by photographic pupillometry prior to and at 15, 30, 60, and 90 minutes after the instillations.
Of the two sisters, one (case A) was examined during a symptom-free interval, when she had been free from cluster headache attacks for 2 1/2 years. When the cluster headaches recurred, retesting was performed. The other sister (case B) had been free from cluster headaches for 9 years, when she was examined.
The findings indicate hypofunction within the postganglionic sympathetic nerve fibers during a cluster headache period. The hypofunction is bilateral, and thus, can not be a consequence of the unilateral cluster headache attacks. During remissions, tyramine induces a marked mydriasis, particularly on the symptomatic side, tentatively indicating an excessive release of stored monoamines.     

Cluster Headache: The Ventilatory Response to Transient Hypoxia with Pure Nitrogen

SYNOPSIS
To determine whether the carotid body plays a pathogenetic role in cluster headache, 20 cluster headache patients have been studied. Of these, 11 patients were in the interparoxysmal cluster phase, and 9 were in remission. Comparison was made with healthy subjects matched for sex, age, and smoking habits. Transient hypoxia was induced by inhalation of 1–8 breaths of 100% nitrogen (N₂), until the arterial oxygen saturation (SaO₂) decreased to around 80%. Changes in ventilation (tidal volume, inspiratory minute ventilation (V_I ), and end-tidal PCO₂ (P_ETCO₂)), were analyzed breath-by-breath.
Under basal conditions, cluster headache patients had a slightly higher SaO₂ and V_I when compared to controls. P_ETCO ₂ was significantly lower (P < 0.05) during the cluster period as measured by Wilcoxon signed rank test for paired data, and during remission, according to the Student's paired t-test, in comparison with controls. After exposure to N₂, no significant difference was found in the rate of reduction of SaO₂ between any of the groups. A higher absolute increase in V_I , but a relative (%) decrease in V_I at moderate hypoxia were measured, the differences between patients and controls being on the border of the level of significance. Chemoreceptor sensitivity of the carotid body, expressed as the slope of a regression curve obtained by plotting the increase in V_I against the reduction in SaO 2 , showed no statistical difference between the groups. The results do not support the hypothesis of a pathogenetic role for the carotid body in cluster headache.