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.     

Chronic paroxysmal hemicrania

Pupillometric studies were carried out in eight patients with chronic paroxysmal hemicrania (CPH) and in age- and sex-matched controls in the basal condition and after instillation of 2% tyramine (CPH, n = 5; controls, n = 17), 1% OH-amphetamine (CPH, n = 6; controls, n = 12), and 1% phenylephrine (CPH, n = 6; controls, n = 17). The pupil on the symptomatic and non-symptomatic sides in CPH patients was significantly smaller in the basal condition than in controls, particularly on the symptomatic side. The mydriatic responses to pharmacologic stimulation were essentially similar on the symptomatic and non-symptomatic sides. An evaporimetric study of the forehead sweat glands, using the body heating and pilocarpine tests, was also carried out in these patients and in age- and sex-matched controls. "Early", "intermediate", and "late" measurements demonstrated symmetry of forehead sweating. The findings for both methods of examination thus contrast with those in cluster headache patients. Pupillometric and forehead sweating patterns therefore suggest differences in the pathogenesis of the two headache entities. These tests may be used to distinguish CPH and cluster headache clinically.     

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.     

Thermographic and pupillary asymmetry in chronic paroxysmal hemicrania. A case study

Facial temperature was measured thermographically and pupillary diameter recorded photographically during and between episodes of headache and during spontaneous remission of headache in a patient with chronic paroxysmal hemicrania (CPH). Heat loss from the orbit, nose, cheek and temple was 0.75-1.5 degrees C greater on the symptomatic side during headache, and 0.25-0.75 degrees C greater between headache episodes. Heat loss from these regions of the face was symmetrical during remission of headache. Extensive rhinorrhoea, and slight ptosis and miosis were observed during the active phase of CPH. These findings, which are similar to those previously reported in cluster headache, suggest that CPH is associated with an ocular sympathetic deficit and with overactivity in the greater superficial petrosal nerve.     

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).     

Chronic Paroxysmal Hemicrania (CPH) and Hemicrania Continua: Transition From One Stage to Another

SYNOPSIS
Two female patients, one suffering from CPH in non-remitting form, and the other from hemicrania continua, in the pre-chronic (non-continuous) stage are described. Both were followed through transitions to other stages: the CPH patient was followed from the non-remitting stage to a long-lasting remission, and lastly back to another chronic stage. Indomethacin was effective in all the symptomatic stages. The hemicrania continua patient was followed from the non-continuous to the continuous stage. Indomethacin was effective in both stages. These observations provide further evidence that the non-chronic and chronic ("remitting" and "non-remitting") stages of CPH belong together. The same seems to apply to the two stages of hemicrania continua, the "continuous" and the "non-continuous" stages.     

"Hemicrania Episodica"-A New Type of Headache or a Pre-Chronic Stage of Hemicrania Continua?

For seventeen years, a young man suffered from headaches of one to three days duration occurring once every six or seven days and totally disappearing between episodes. These were strictly unilateral (always on the left side), and were absolutely responsive to indomethacin. They differ clinically from the other two indomethacin-responsive headaches described up to now: Hemicrania Continua (HC) and Chronic Paroxysmal Hemicrania (CPH). He has a sister suffering from HC. Similar to HC, but unlike CPH, topical tyramine in our patient's eyes resulted in anisocoria before, but not after, indomethacin treatment. Our case differs from HC, however, in its time pattern; it could either be a pre-chronic stage of HC or a new type of headache. It seems unlikely that a time pattern that has remained unchanged for 17 years will become continuous, as in HC, in the future, and thus it is possible that our patient represents a case of a new type of headache that we propose to name "Hemicrania Episodica." In any case, it seems probable that this type of headache, although clinically different, may share a common pathogenic basis with HC.     

Autonomic disorders in cluster headache, with special reference to salivation, nasal secretion and tearing

Attacks of cluster headache are often associated with symptoms of an autonomic nature. A test battery allowing quantitation of salivation, nasal secretion and tearing has been employed. Fourteen patients examined under basal conditions hardly differed from a group of controls (N = 20). After stimulation with pilocarpine the patients responded like the controls. During attacks we found minimal bilateral salivation, but an increase of tearing and nasal secretion, mostly on the symptomatic side. These results correspond with those found in CPH. The finding of the minimal salivation is consistent with the notion that the headache attacks are associated with increased sympathico-tonus. The results may suggest that the complexity of the innervation pattern of the different secretory organs examined is more marked than hitherto known.     

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.     

Cluster headache: Forehead sweating pattern during heating and pilocarpine tests

Thirty-one patients with cluster headache were examined with regard to their forehead sweating pattern, by means of the Evaporimeter. Sweating was stimulated in two different ways: by body heating and by parenterally administered pilocarpine. The resulting increase in evaporation was frequently measured at different positions on both sides of the forehead, and the possibility of variations in the pattern related to the passage of time was specifically scrutinized. Some typical patterns emerged. The previously reported, marked asymmetries of response (deficient heat-induced sweating and pilocarpine supersensitivity of the symptomatic side) at the medial positions in the forehead were confirmed. However, the asymmetries invariably faded to some extent with the passage of time. Patients with cluster headache show gross similarities with, but also some minor differences from, the sweat pattern of patients with brain stem lesions causing a Horner's syndrome. A subdivision of the material into groups in accordance with the pupillometric pattern after sympathomimetic stimulation made it clear that the cases of definite evaporimetric asymmetries ("typical reactions") belonged to the group with a typical pupillometric pattern. These results suggest that from an "autonomic" point of view, subpopulations may exist within the clinical entity of cluster headache.     

Chronic paroxysmal hemicrania: Heart rate changes and ECG rhythm disturbances. A computerized analysis of 24 h ambulatory ECG recordings

Ambulatory ECG recordings have been carried out in five patients suffering from CPH. During the study a total of 105 attacks occurred. Contrary to findings in cluster headache, no typical pattern of heart rate change was found in association with attacks of CPH. A striking finding in all patients, however, was that there were often large and rapid variations in heart rate which could be observed "before", "during" or "after" the attacks. One patient developed bradycardia and sino-atrial block and another bundle branch block together with episodes of atrial fibrillation in association with attacks.     

Respiratory Sinus Arrhythmia in Cluster Headache Syndrome

SYNOPSIS
Respiratory sinus arrhythmia is regarded as indicative of cardiac vagal integrity. A ratio of the longest R-R interval to the shortest R-R interval during deep breathing test (E:I ratio) was calculated in controls (n=49), cluster headache (n=33) and CPH (n=4) patients, E:I ratio decreased with age but was not dependent upon sex or upon smoking habits. Furthermore, there were no significant differences as regards E:I ratio between cluster headache patients in and outside a bout, or between patients with right-sided and left-sided headache. However, the E:I ratio was found to be significantly lower in the cluster headache group as such, when compared with controls, but the number of patients disclosing pathological or borderline results was small, 2 and 2, respectively. This may indicate that a putative vagal dysfunction in cluster headache is usually less marked than in patients with e.g. diabetic autonomic neuropathy. Significant attack-related change in the E:I ratio were detected in all individual patients though these changes were not of a uniform nature from individual to individual. E:I ratios were rather high in 3 out of 4 CPH patients examined. However, the number of patients in this group is too small to allow definite statements about the difference between CPH end cluster headache with regard to E:I ratios. There was no significant difference between E:I ratios outside and during a mild, short, mechanically precipitated attack in a single CPH patient.     

"Hemicrania continua": A new clinical entity or a further development from cluster headache?

A case of "hemicrania continua" after cluster headache in the same subject is described. Indomethacin exerted an absolute, persistent effect on the present headache. Even though our data are insufficient to demonstrate a causal relation between the two forms of headache, they do suggest this real possibility.     

The sweating anomaly in cluster headache

We describe a patient with a typical history of cluster headache for more than 18 years. During the first approximately 10 years of his disease, the pain was right-sided, and pupillometric and evaporimetric measurements indicated a sympathetic deficiency on this same side. However, for the next >6 years, his pain was consistently left-sided, although the signs of sympathetic dysfunction still were more marked on the right side. This was also true for the findings obtained during the interictal period and for the heating test performed within an attack. The implications of this interesting case are discussed. The view that two separate lines of symptom production lead to the pain and the autonomic phenomena seems to be supported by this case history. The cluster headache syndrome may also be a bilateral disorder, with only the weight of balance pointing one way or the other. Finally, the autonomic test results of this patient could reflect an autonomic "scar" in the previous headache side.     

Chronic paroxysmal hemicrania—Case report of the second known definite occurrence in a male

This paper describes the second definite case of chronic paroxysmal hemicrania (CPH) in a male. The patient fits all diagnostic criteria for definite CPH: Daily attacks, maximum daily frequency greater than fifteen attacks and striking improvement from Indomethacin.     

"Hemicrania continua". An indomethacin responsive headache. II. Autonomic function studies

Various autonomic parameters have been studied in two patients with "hemicrania continua", a newly described unilateral headache which is aborted by indomethacin. Striking findings were made on pupillometry: In both patients, isocoria was present when untreated. Bilateral instillation of tyramine in the conjunctival sac resulted in a late appearing anisocoria, with the smaller pupil on the symptomatic side. Indomethacin medication corrected this anomaly. These findings add further evidence to our firm belief that "hemicrania continua" differs fundamentally from chronic paroxysmal hemicrania, where such pupillometric changes are not found. There thus seem to be at least two different types of hemicranias with an absolute indomethacin effect.     

Parenteral indomethacin (the INDOTEST) in cluster headache

The interval between indomethacin administration and clinical response may be extremely relevant in the assessment of chronic paroxysmal hemicrania (CPH) and other unilateral headache disorders like cluster headache (CH), with which CPH can be confounded. Indomethacin is inactive in CH; however, in some anecdotal reports in recent years, doubt has been cast on the ineffectiveness of indomethacin in CH. In this study, we have re-assessed the effect of indomethacin treatment in a group of 18 patients with episodic CH (three females and 15 males). From the day 8 of the active period, indomethacin 100 mg i.m. was administered every 12 h, for 2 consecutive days, in an open fashion. The mean daily attack frequency before the test (1.6 +/- 0.6) was not statistically different from that on day 1 (2.1 +/- 0.9) and day 2 (1.9 +/- 0.8) after indomethacin administration. The mean interval between indomethacin injection and the following attack (day 1 and day 2) was 4.6 + 1.1 h. We did not observe any refractory period in any patient after indomethacin. Since the 'expected' attack occurred when there theoretically could have been a protective effect after indomethacin administration, it can be reasonably assumed that there is no such protective effect. The use of a test dose of 100 mg i.m. indomethacin (INDOTEST) appears to provide a clear-cut answer in this situation. It may be a useful tool for a proper clinical assessment of unilateral headache with relatively short-lasting attacks when problems of classification arise. A correct diagnosis of CPH or CH is important, since a CPH diagnosis may imply a lifelong treatment with a potentially noxious drug.     

Cluster headache: pupillometric patterns as a function of the degree of anisocoria

Fifty-three patients with cluster headache, mean age 42.6 years, were examined by means of pupillometry. Pharmacological stimulation was carried out by the instillation of eye drops; the sympathomimetic agents hydroxyamphetamine (a norepinephrine releaser) and phenylephrine (an agent acting directly on the postsynaptic receptors) were applied. Pupillary dilatation was measured at set time intervals, comparing the responses of the symptomatic and non-symptomatic sides. The material was divided into groups according to the degree of basal anisocoria. The subgroup with the most pronounced basal miosis of the symptomatic side demonstrated a uniform pattern of deficient symptomatic side dilatation after OH-amphetamine and supersensitivity to phenylephrine. The other groups demonstrated the same general pattern, but to a far lesser degree. In cluster headache, the extent of nonresponsiveness to OH-amphetamine and of phenylephrine supersensitivity on the symptomatic side thus, at least partly, seems to be a function of the degree of anisocoria. The response pattern in cluster headache seems to differ from that of 3rd neuron Horner's syndrome with an anisocoria of the same extent.     

Neuropeptide changes in a case of chronic paroxysmal hemicrania—evidence for trigemino-parasympathetic activation

Chronic paroxysmal hemicrania (CPH) is a rare headache syndrome of short-lasting attacks of pain, characterized clinically by trigemino-parasympathetic activation. The features of the headache are severe attacks of pain that generally last no more than minutes in association with autonomic activation, such as lacrimation or rhinorrhea. We report a patient fulfilling International Headache Society guidelines for the diagnosis of CPH in whom levels of calcitonin gene-related peptide (CGRP) and vasoactive intestinal polypeptide (VIP) were elevated in the cranial circulation during attacks. Moreover, successful treatment of the problem with indomethacin leads to normalization of the levels of both CGRP and VIP. Given that similar neuropeptide changes are seen in cluster headache the data suggest a shared underlying pathophysiology between CPH and cluster headache.     

Chronic paroxysmal hemicrania presenting as recurrent orbital inflammation

Patients with chronic headache associated with ocular symptoms regularly seek ophthalmologists' opinions. We report an unusual case of chronic paroxysmal hemicrania (CPH), a rare but well-described variant of cluster headache in a female presenting to an eye department with recurrent episodes of severe unilateral periorbital swelling with a chronic history of headaches. Clinical features, review of literature, and therapeutic response to indomethacin helped to establish the diagnosis as CPH. This is the first report of severe orbital inflammation with CPH to our knowledge.