Observations
Chronic Paroxysmal Hemicranial: Mechanical Precipitation of Attacks O. Sjaastad, K. Egge, I. Hørven, K. Kayed, L. Lund-Roland, D. Russell and I. SIørdahl Conradi From the Department of Neurology, Regionsykehuset, University of Trondheim, Trondheim, the Departments of Neurology and Ophthalmology, Rikshospitalet (National Hospital of Norway), Oslo University Hospitals, Oslo and the Department of Neurology, Drammen County Hospital, Drammen, Norway. Reprint requests to: Professor Ottar Sjaastad, Dept. of Neurology, Regionsykehuset, University of Trondheim, 7000 Trondheim, Norway. Accepted for Publication: October 27, 1978 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. (Headache 19:31-36, 1979) Chronic paroxysmal hemicrania (CPH)1 is clinically a cluster headache variant. Neither regular cluster headache nor CPH-patients have hitherto been known to be able to precipitate attacks on their own. Some CPH-patients however, seem to possess this ability. The study of this precipitation mechanism may aid in elucidating the pathogenesis not only of CPH in particular, but also of the cluster headache syndrome, in general. MATERIAL To date, a total of five verified cases of chronic paroxysmal hemicrania are known, of whom three are able to precipitate attacks. Their case reports are herein reported. None of the forty patients with regular cluster headache who were systematically questioned, gave a positive history of similar precipitation mechanisms. CPH-patients gave informed consent to partake in the various studies. CPH-CASES WITH PRECIPITATED ATTACKS Case 1. One previously described patient (Case 2)1 has occasionally, in recent years, been able to precipitate attacks by bending her head forward. Precipitated attacks were similar to spontaneous attacks, but were short lasting and less severe. During established attacks, pain was increased by head rotation to the non-symptomatic (right) side or by head flexion. A distinct tenderness was found midway between the mastoid process and the external occipital protuberance on the symptomatic side. On palpation of this area, pain was felt in the left ocular/temporal region, which stopped immediately at the end of palpation. Pressure over the carotid artery area on the sympathetic side produced a moderate but typical pain in the ocular/temporal region on the same side. This pain persisted for a few second only upon discontinuation of the applied pressure. (Table 1) No definite Horner-like findings presented, clinically. Table 1 Precipitation of Attacks Case No.
Head flexion
1 2 3
+ ++ ++
Precipitation mechanism Head rotation External pressure carotid area, symptomatic side (+) + + + (?)
Case 2. Our most recently discovered case, a 31 year old woman, has been diagnosed as suffering from cerebral palsy. This also applies to her
Table 2 Sequence of Events During Precipitated Attacks Time lag in seconds before: Case No. Mechanism 3 Hd. Flexion Hd. Rotation 2 Hd. Flexion
N Pounding 18 5-15 5 20-30 8 4-30
Tearing 8-15 20-30 -
Conjunctival injection Headache 10-20 10-30 20-30 30-40 7-40
monozygotic twin, who does not suffer from headache. She has had severe rightsided headache attacks for 5 years. The frequency of attacks the first year was one per week. The frequency gradually increased so that during the last 11/2 years she had between 10 and 30 attacks per day. Each attack usually lasts from 1/2 - 10 minutes but are not accompanied by conjunctival injection, tearing, miosis or ptosis. A clear, relative increase in corneal indentation pulse (CIP) amplitudes is present on the symptomatic side during attacks (symptomatic/ asymptomatic side ratio in the free interval: 1.02; during attack: 1.53). Continuous indomethacin treatment relieved attacks completely. There is distinct tenderness between the mastoid process and the external occipital protuberance on the symptomatic side. Pressure applied to this area produces pain similar to that of spontaneous attacks. The pain stops on discontinuation of the external pressure. She is almost invariably able to precipitate typical attacks by head flexion, followed by sudden head extension (Table 1 and 2). There is a pronounced tenderness over the carotid area on the symptomatic side. In 4 experiments, external pressure applied to this area provoked typical attacks, i.e. the head pain continued for 5 - 10 minutes after discontinuation of the pressure (Table 1). Ocular circulation was partly monitored by dynamic tonometry during these experiments. Ocular circulation did not appear to be compromised in the experiments where headache was elicited. Case 3. This patient is a 29 year old female. Her birth was particularly difficult due to a breech delivery. Prior to the development of the present headache pattern, she suffered from uncharacteristic head pain. Otherwise, she had experienced good health. The present headache. The present headache pattern emerged four years ago. The long-term course showed rather marked fluctuation: Moderate and severe attack periods, each of some few weeks duration, alternated continuously. Attack frequency varied accordingly, i.e. from 8 to 30 attacks/24 hours. Most attacks appeared spontaneously without any head bending or rotation. The first indication of an attack was a weak pulsating pain in the right side of the neck (A). Her head then started to nod immediately afterwards, apparently in a pulse-synchronous fashion. Symptomatic side tearing, conjunctival injection and nasal stenosis appeared at this stage. The crescendo pain then started within seconds, pari passu with the fading away of the severe pounding. The pain was excruciatingly severe and strictly unilateral (B) and was felt in the right temporal region, behind the right eye, and in front of and just above the right ear. The maximal pain was eventually felt in the latter area; at the height of attack the pain might have a slight throbbing quality. Spontaneous attacks lasted 5 - 15 minutes. During attack, she preferred the erect or sitting position. With continuous medication, she has now been attack-free for 31/2 years. The indomethacin requirement ranges from 150 mg per day to 25 mg on alternate days. Attacks recur with original severity and frequency when indomethacin placebo is administered in a blind fashion. During the worst periods, there might be slight nausea. There was no associated vomiting, no visual phenomena, phono- or photo-phobia during attacks. She had a feeling of "being in a closed box" during attacks. There was no hearing loss, as observed in other cases of CPH.2 Facial sweating could not be observed, neither during attack nor free intervals. In addition to the aforementioned pain types A and B, she might experience a sudden, brief ( < 1 second duration) pain of similar quality as type B, irradiating from the neck (C). This pain might be precipitated when she suddenly was frightened or immediately upon sudden turning of the head to the right. If she awaited a coming event with great reluctance, she would have few attacks, even in the absence of indomethacin treatment. If she looked forward to a special event, she was bound to get frequent and heavy attacks. Precipitation of attack. The patient was able to precipitate attacks by bending her head maximally forward, both in the sitting and supine positions, or by rotating the head maximally to the symptomatic side. In periods with frequent attacks, bending invariably precipitated attack, while rotation produced attacks somewhat less regularly. To her, spontaneous and
precipitated attacks seemed identical. Bending the whole body forwards did not suffice -, the head itself had to be flexed, but not to the extent that the chin touched the neck. Approximately 5 - 15 seconds after beginning to bend her head, she experienced a pulsating sensation in the nape of the neck (right side). With rotation, the latency period was usually 20 - 30 seconds. The sequence of events was then as with spontaneous attacks (Table 2). Once the attack had begun, it continued irrespective of the head position; the total duration usually did not exceed 10 min. The pain was accentuated by bending the head forwards. Regardless of precipitating mechanism, the severity of attack as well as the latency was dependent upon the frequency of spontaneous attacks. There did not seem to be any refractory period following precipitated attacks. On chronic indomethacin therapy, she had the same strange feeling in the neck on rotating her head, but an attack did not ensue. Experiments to precipitate attacks "externally" were also conducted in phases when the patient was able to precipitate attacks on her own. I)
An immediately occurring pain, similar to the previously described spontaneously occurring type C, could be elicited from a circumscribed tender area on the symptomatic side along the tendon insertions midway between the mastoid process and the external occipital protruberance. A genuine attack, however, could not be elicited in this way.
II)
An attack could not be precipitated by external compression of the internal carotid artery on the symptomatic side. The completeness of compression was controlled by homolateral dynamic tonometry and intraocular pressure monitoring in some experiments.
III)
The right carotid artery area was tender on palpation. Attempts to precipitate attacks by rubbing this area have so far given inconclusive results (Table 1). Usually, attacks could not be precipitated by external pressure applied to the symptomatic side carotid artery area. Abortive attacks were probably produced on 2 occasions (strength around 20 - 25% and duration 20 - 25 seconds), but no full-blown attacks.
On two occasions full-blown attacks began approximately 10 - 20 seconds after discontinuation of pressure to the symptomatic carotid area. The close temporal association makes it likely that there was a cause and effect relationship between the applied stimulus and the development of attack. During an established attack (spontaneous or provoked) ocular pain was clearly enhanced by pressure against the carotid area on the symptomatic side. On several occasions the internal carotid artery was compressed in the upper part of the neck on the symptomatic side during the entire process of bending the head and for some time after onset of pain. Pain attacks of usual strength and duration developed in spite of the compression. Clinical and supplementary test findings. There was a tendency to miosis and ptosis on the symptomatic side during attacks (Table 3 and 4). Local Table 3 Patient No. 3 Various Clinical Parameters During Precipitated Attacks
Between attacks During attacks *Friedenwald's
Corneal temp. (°C) Symp./asymp.side 34.75 34.0 35.0 33.9
Intraoc.press.* (mm Hg) Symp./asymp.side 10.2 9.4 15.9 12.2
Ptosis Symp./asymp.side ++ -
1955 converting table (Friedenwald 1957).
Table 4 Pupillary Width During Several Spontaneous Attacks on Different Days (Measurements in mm). Patient No. 3
Between attacks During attacks *Represents
I
II
III*
Symp./asymp.side 4 4 3.5 4
Symp./asymp.side 4 4 3 4
Symp./asymp.side Identical size 1/2 - 1
Chronic Paroxysmal Hemicranial: Mechanical Precipitation of Attacks O. Sjaastad, K. Egge, I. Hørven, K. Kayed, L. Lund-Roland, D. Russell and I. SIørdahl Conradi From the Department of Neurology, Regionsykehuset, University of Trondheim, Trondheim, the Departments of Neurology and Ophthalmology, Rikshospitalet (National Hospital of Norway), Oslo University Hospitals, Oslo and the Department of Neurology, Drammen County Hospital, Drammen, Norway. Reprint requests to: Professor Ottar Sjaastad, Dept. of Neurology, Regionsykehuset, University of Trondheim, 7000 Trondheim, Norway. Accepted for Publication: October 27, 1978 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. (Headache 19:31-36, 1979) Chronic paroxysmal hemicrania (CPH)1 is clinically a cluster headache variant. Neither regular cluster headache nor CPH-patients have hitherto been known to be able to precipitate attacks on their own. Some CPH-patients however, seem to possess this ability. The study of this precipitation mechanism may aid in elucidating the pathogenesis not only of CPH in particular, but also of the cluster headache syndrome, in general. MATERIAL To date, a total of five verified cases of chronic paroxysmal hemicrania are known, of whom three are able to precipitate attacks. Their case reports are herein reported. None of the forty patients with regular cluster headache who were systematically questioned, gave a positive history of similar precipitation mechanisms. CPH-patients gave informed consent to partake in the various studies. CPH-CASES WITH PRECIPITATED ATTACKS Case 1. One previously described patient (Case 2)1 has occasionally, in recent years, been able to precipitate attacks by bending her head forward. Precipitated attacks were similar to spontaneous attacks, but were short lasting and less severe. During established attacks, pain was increased by head rotation to the non-symptomatic (right) side or by head flexion. A distinct tenderness was found midway between the mastoid process and the external occipital protuberance on the symptomatic side. On palpation of this area, pain was felt in the left ocular/temporal region, which stopped immediately at the end of palpation. Pressure over the carotid artery area on the sympathetic side produced a moderate but typical pain in the ocular/temporal region on the same side. This pain persisted for a few second only upon discontinuation of the applied pressure. (Table 1) No definite Horner-like findings presented, clinically. Table 1 Precipitation of Attacks Case No.
Head flexion
1 2 3
+ ++ ++
Precipitation mechanism Head rotation External pressure carotid area, symptomatic side (+) + + + (?)
Case 2. Our most recently discovered case, a 31 year old woman, has been diagnosed as suffering from cerebral palsy. This also applies to her
Table 2 Sequence of Events During Precipitated Attacks Time lag in seconds before: Case No. Mechanism 3 Hd. Flexion Hd. Rotation 2 Hd. Flexion
N Pounding 18 5-15 5 20-30 8 4-30
Tearing 8-15 20-30 -
Conjunctival injection Headache 10-20 10-30 20-30 30-40 7-40
monozygotic twin, who does not suffer from headache. She has had severe rightsided headache attacks for 5 years. The frequency of attacks the first year was one per week. The frequency gradually increased so that during the last 11/2 years she had between 10 and 30 attacks per day. Each attack usually lasts from 1/2 - 10 minutes but are not accompanied by conjunctival injection, tearing, miosis or ptosis. A clear, relative increase in corneal indentation pulse (CIP) amplitudes is present on the symptomatic side during attacks (symptomatic/ asymptomatic side ratio in the free interval: 1.02; during attack: 1.53). Continuous indomethacin treatment relieved attacks completely. There is distinct tenderness between the mastoid process and the external occipital protuberance on the symptomatic side. Pressure applied to this area produces pain similar to that of spontaneous attacks. The pain stops on discontinuation of the external pressure. She is almost invariably able to precipitate typical attacks by head flexion, followed by sudden head extension (Table 1 and 2). There is a pronounced tenderness over the carotid area on the symptomatic side. In 4 experiments, external pressure applied to this area provoked typical attacks, i.e. the head pain continued for 5 - 10 minutes after discontinuation of the pressure (Table 1). Ocular circulation was partly monitored by dynamic tonometry during these experiments. Ocular circulation did not appear to be compromised in the experiments where headache was elicited. Case 3. This patient is a 29 year old female. Her birth was particularly difficult due to a breech delivery. Prior to the development of the present headache pattern, she suffered from uncharacteristic head pain. Otherwise, she had experienced good health. The present headache. The present headache pattern emerged four years ago. The long-term course showed rather marked fluctuation: Moderate and severe attack periods, each of some few weeks duration, alternated continuously. Attack frequency varied accordingly, i.e. from 8 to 30 attacks/24 hours. Most attacks appeared spontaneously without any head bending or rotation. The first indication of an attack was a weak pulsating pain in the right side of the neck (A). Her head then started to nod immediately afterwards, apparently in a pulse-synchronous fashion. Symptomatic side tearing, conjunctival injection and nasal stenosis appeared at this stage. The crescendo pain then started within seconds, pari passu with the fading away of the severe pounding. The pain was excruciatingly severe and strictly unilateral (B) and was felt in the right temporal region, behind the right eye, and in front of and just above the right ear. The maximal pain was eventually felt in the latter area; at the height of attack the pain might have a slight throbbing quality. Spontaneous attacks lasted 5 - 15 minutes. During attack, she preferred the erect or sitting position. With continuous medication, she has now been attack-free for 31/2 years. The indomethacin requirement ranges from 150 mg per day to 25 mg on alternate days. Attacks recur with original severity and frequency when indomethacin placebo is administered in a blind fashion. During the worst periods, there might be slight nausea. There was no associated vomiting, no visual phenomena, phono- or photo-phobia during attacks. She had a feeling of "being in a closed box" during attacks. There was no hearing loss, as observed in other cases of CPH.2 Facial sweating could not be observed, neither during attack nor free intervals. In addition to the aforementioned pain types A and B, she might experience a sudden, brief ( < 1 second duration) pain of similar quality as type B, irradiating from the neck (C). This pain might be precipitated when she suddenly was frightened or immediately upon sudden turning of the head to the right. If she awaited a coming event with great reluctance, she would have few attacks, even in the absence of indomethacin treatment. If she looked forward to a special event, she was bound to get frequent and heavy attacks. Precipitation of attack. The patient was able to precipitate attacks by bending her head maximally forward, both in the sitting and supine positions, or by rotating the head maximally to the symptomatic side. In periods with frequent attacks, bending invariably precipitated attack, while rotation produced attacks somewhat less regularly. To her, spontaneous and
precipitated attacks seemed identical. Bending the whole body forwards did not suffice -, the head itself had to be flexed, but not to the extent that the chin touched the neck. Approximately 5 - 15 seconds after beginning to bend her head, she experienced a pulsating sensation in the nape of the neck (right side). With rotation, the latency period was usually 20 - 30 seconds. The sequence of events was then as with spontaneous attacks (Table 2). Once the attack had begun, it continued irrespective of the head position; the total duration usually did not exceed 10 min. The pain was accentuated by bending the head forwards. Regardless of precipitating mechanism, the severity of attack as well as the latency was dependent upon the frequency of spontaneous attacks. There did not seem to be any refractory period following precipitated attacks. On chronic indomethacin therapy, she had the same strange feeling in the neck on rotating her head, but an attack did not ensue. Experiments to precipitate attacks "externally" were also conducted in phases when the patient was able to precipitate attacks on her own. I)
An immediately occurring pain, similar to the previously described spontaneously occurring type C, could be elicited from a circumscribed tender area on the symptomatic side along the tendon insertions midway between the mastoid process and the external occipital protruberance. A genuine attack, however, could not be elicited in this way.
II)
An attack could not be precipitated by external compression of the internal carotid artery on the symptomatic side. The completeness of compression was controlled by homolateral dynamic tonometry and intraocular pressure monitoring in some experiments.
III)
The right carotid artery area was tender on palpation. Attempts to precipitate attacks by rubbing this area have so far given inconclusive results (Table 1). Usually, attacks could not be precipitated by external pressure applied to the symptomatic side carotid artery area. Abortive attacks were probably produced on 2 occasions (strength around 20 - 25% and duration 20 - 25 seconds), but no full-blown attacks.
On two occasions full-blown attacks began approximately 10 - 20 seconds after discontinuation of pressure to the symptomatic carotid area. The close temporal association makes it likely that there was a cause and effect relationship between the applied stimulus and the development of attack. During an established attack (spontaneous or provoked) ocular pain was clearly enhanced by pressure against the carotid area on the symptomatic side. On several occasions the internal carotid artery was compressed in the upper part of the neck on the symptomatic side during the entire process of bending the head and for some time after onset of pain. Pain attacks of usual strength and duration developed in spite of the compression. Clinical and supplementary test findings. There was a tendency to miosis and ptosis on the symptomatic side during attacks (Table 3 and 4). Local Table 3 Patient No. 3 Various Clinical Parameters During Precipitated Attacks
Between attacks During attacks *Friedenwald's
Corneal temp. (°C) Symp./asymp.side 34.75 34.0 35.0 33.9
Intraoc.press.* (mm Hg) Symp./asymp.side 10.2 9.4 15.9 12.2
Ptosis Symp./asymp.side ++ -
1955 converting table (Friedenwald 1957).
Table 4 Pupillary Width During Several Spontaneous Attacks on Different Days (Measurements in mm). Patient No. 3
Between attacks During attacks *Represents
I
II
III*
Symp./asymp.side 4 4 3.5 4
Symp./asymp.side 4 4 3 4
Symp./asymp.side Identical size 1/2 - 1
