Lawrence C. Newman 1 2 MD
Peter Goadsby 3 MD
Richard B. Lipton 1 MD
1 Department of Neurology, Albert Einstein College of Medicine, Bronx (LCN, RBL)
2 Headache Institute, St. Luke's-Roosevelt Hospital Center, New York (LCN), New York
3 Institute of Neurology, The National Hospital for Neurology and Neurosurgery, London, United Kingdom (PG)
Address reprint requests to
Lawrence C. Newman, MD
The Headache Institute
St. Luke's-Roosevelt Hospital Center
1000 Tenth Avenue
New York, NY 10019
CLUSTER HEADACHE
Cluster headache is a well-described syndrome characterized by discrete
bouts of excruciatingly severe unilateral headaches associated with
ipsilateral cranial autonomic features. Although relatively uncommon,
this disorder is important to physicians treating patients with
headache because the sufferers tend to be desperate, often
misdiagnosed, and often mismanaged.
Epidemiology
Cluster headache is a rare disorder affecting approximately 0.1% of the
population.[18] [28] The disorder affects men more often than women
with the sex ratio ranging from 3.5 to 6.7:1.[47] [52] The
male-to-female ratio, based on the year of onset of the disorder, has
decreased from 6.2:1 in the 1960s to 2.1:1 in the 1990s.[52] More
recent evidence suggests that cluster, similar to migraine, may have a
familial predisposition; Russell et al[87] reported a 14-fold risk of
cluster headache among first-degree relatives with the disorder.
Clinical Features
The International Headache Society (IHS) divides cluster headache into
two subtypes, episodic and chronic cluster.[40] The chronic form is sub
classified further into primary and secondary variants; the distinction
is based on the presence or absence of prior episodic cluster attacks.
Most patients suffer from episodic cluster headaches in which attacks
recur for weeks to months at a time (the cluster period), then are
separated by months or years of pain freedom (the remission period).
The IHS mandates that episodic cluster consists of headache attacks
that occur in periods lasting 1 week to 1 year and are separated by
pain-free periods lasting 14 days or more. When remission periods last
less than 14 days or when attacks recur without remission for more than
1 year, the designation chronic cluster is given. Approximately 10% of
sufferers never experience remissions. The chronic subtype may evolve
from an initially episodic course (secondary chronic cluster) or may
begin de novo (primary chronic).
Cluster headache is so named because attacks occur in groups or
clusters separated by periods of pain-free remissions. The mean age of
onset is 28 to 30 years, although attacks may begin during childhood or
in later life. During the cluster period, attacks may recur once per
week, or eight attacks per day may occur.[40] [116] Most patients
experience one to two attacks daily.[27] The individual attacks usually
are brief, lasting 30 to 180 minutes each (mean, 45 minutes). Attacks
tend to recur at the same time each day and are notable in that they
often occur at night, awakening the patient from a sound sleep. A
seasonal preponderance occurs, with cluster phase often occurring in
the spring or autumn months.
Attacks of cluster are excruciatingly severe; unilateral (usually
without side-shift); and of maximal intensity in, around, or behind the
eye. The pain may radiate into the ipsilateral temple, neck, jaw, or
upper teeth and frequently is misdiagnosed as sinus or dental disease.
Rarely the pain may occur on the opposite side of the head in a
subsequent cluster cycle; rarer still, in some patients, the pain
alternates sides within a cycle. Even less common are patients in whom
the pain of cluster occurs in the neck, the occiput, or other sites
outside the trigeminal territory.[88] [112] The pain typically is
described as boring or stabbing and has been likened to a hot poker in
the eye. The onset of pain usually is rapid, peaking within 5 to 10
minutes. Although migraine sufferers prefer to lie in a darkened, quiet
room, sufferers of cluster headache are restless; lying down often
exacerbates the pain. Commonly, patients pace to and fro during an
attack, sit upright in a chair holding their heads in their hands, or
bang their heads against a wall. The pain paroxysms are so severe that
many patients are driven to desperate measures, including suicide.
During acute attacks of pain, sufferers experience one or more
autonomic features ipsilateral to the headache. These autonomic signs
and symptoms include ptosis (droopy eyelid), eyelid edema (swollen
eyelid), miosis (constricted pupil), lacrimation (teary eye),
conjunctival injection (bloodshot eye), forehead and facial sweating,
nasal congestion, and rhinorrhea (runny nose). These accompanying
features are brief, lasting only for the duration of the acute pain.
Rarely a partial Horner syndrome (ptosis and miosis) may persist
between attacks. Patients with typical cluster headaches preceded by
visual auras have been described.[90]
Treatment
The treatment of cluster headache consists of abortive and prophylactic
treatments. Abortive agents are employed to shorten the individual
attack, whereas preventive agents are used to shorten or abolish the
cycles. Acute treatments include inhalation of 100% oxygen at 8 to 10
L/min for 10 to 15 minutes through a nonrebreathing facemask;
sumatriptan, 6 mg subcutaneous injections or 20 mg intranasally; and
intravenous, intramuscular, or subcutaneous injections of
dihydroergotamine, 0.5 to 1.0 mg, at headache onset.
Prophylactic medications useful in preventing attacks during cluster
cycles include verapamil, sodium valproate, lithium carbonate,
methysergide, and ergotamine tartrate. For refractory patients, a
combination of two or more drugs may be required. The general
principles guiding the use of these agents for cluster suggest that the
medications be initiated early in the cluster period, then continued
for approximately 2 weeks longer than the typical cycle, at which point
they should be tapered gradually. Because a period of 2 weeks usually
is required for most of these agents to take effect, often they are
begun simultaneously with a prednisone taper, beginning at 60 mg/d,
then tapered slowly during 10 to 14 days. Prednisone is helpful in that
it rapidly induces a headache remission and keeps the patient
headache-free, usually within 1 to 2 days.
Verapamil is the preventive medication of choice for the episodic and
chronic forms of the disorder. Typical doses range from 120 to 480 mg
daily, although higher doses occasionally are employed, particularly in
chronic cluster headache. Verapamil may be combined with ergotamine,
lithium, sodium valproate, or methysergide.
Sodium valproate has been reported to be effective in an open study in
the management of cluster headache in doses ranging from 250 to 1000 mg
daily.[41] Liver function and platelets should be monitored before
initiating therapy and periodically during the course of treatment.
Ergotamine tartrate, 3 to 4 mg/d in divided doses, occasionally is
useful in the treatment of these headaches. Ergotamine taken before
bedtime usually prevents nighttime attacks. The concurrent use of
sumatriptan and dihydroergotamine is contraindicated.
Lithium carbonate, 300 mg twice a day, is more effective for the
treatment of chronic than episodic cluster headache.[53] Lithium levels
should be maintained between 0.4 and 0.8 mEq/L.
Methysergide is effective in approximately 65% of patients with
episodic cluster,[16] but because of the potential for fibrotic
complications, it should not be used for longer than 6 months at a
time. The daily dose is usually 2 mg 3 times a day, but higher doses
occasionally are needed. The medication should not be used routinely
with ergotamine tartrate, dihydroergotamine, or sumatriptan.
Headaches Sharing Some Clinical Features With Cluster Headache
The cluster headache variants discussed in this article consist of a
diverse group of relatively uncommon headache disorders. Some of these
headaches differ from cluster headache primarily in their frequency,
duration, or treatment response. These disorders consist of paroxysmal
hemicranias, hemicrania continua (HC), and SUNCT syndrome
(short-lasting unilateral neuralgiform headaches with conjunctival
injection and tearing). A different group of cluster variants is
characterized by their clinical presentations, which share features of
cluster headache and another primary headache disorder, the cluster-tic
and cluster-migraine syndromes. The hypnic headache syndrome does not
share the cranial autonomic features of cluster headache, but because
it may be misdiagnosed as cluster resulting from the pattern of
short-lived nocturnal attacks it also is described in this article.
PAROXYSMAL HEMICRANIAS
The paroxysmal hemicranias are a group of rare, benign headache
disorders that clinically resemble cluster headache, fail to remit with
standard anticluster therapies, and respond spectacularly to
indomethacin. Chronic paroxysmal hemicrania (CPH) first was described
in 1974,[99] was named in 1976,[100] and appears in the IHS
classification.[40] The first cases of CPH were characterized by
multiple, short-lived, unilateral attacks that occurred on a daily
basis for years without remission.[2] [86] [93] [99] [100] [106] [113]
With time, it became clear that not all patients experienced this
chronic unremitting course; some patients reported a remitting pattern
with discrete headache phases separated by prolonged periods of
pain-free remissions.[5] [7] [15] [30] [32] [48] [61] [67] [113] [114]
Kudrow et al[48] named this pattern episodic paroxysmal hemicrania
(EPH). Other patients have experienced an initially episodic course
that over time evolved into the chronic nonrelenting phase.[2] [25]
The nomenclature for the various forms of the paroxysmal hemicranias
has been controversial. The IHS recognizes only CPH. One nomenclature
system uses CPH for the entire category and subclassifies the following
forms: (1) chronic from onset, (2) chronic evolved from the remitting
form, and (3) nonchronic/remitting.[2] The authors prefer CPH, EPH, and
CPH evolved from EPH to describe these various syndromes.[5] [48] [61]
[63] These terms are useful in that they are analogous to the IHS
nomenclature of cluster headache. Proposed changes to the IHS
classification system regarding the paroxysmal hemicranias are listed
in the accompanying box.
| Box 1. Suggested Criteria for Paroxysmal Hemicranias Chronic paroxysmal hemicrania 1. At least 30 attacks fulfilling B-E 2. Attacks of severe unilateral orbital, supraorbital, or temporal pain always on the same side lasting 2-45 minutes 3. Attack frequency >5 a day for more than half the time (periods with lower frequency may occur) 4. Pain is associated with at least one of the following signs and symptoms on the pain side: 1. Conjunctival injection 2. Lacrimation 3. Nasal congestion 4. Rhinorrhea 5. Ptosis 6. Eyelid edema 5. At least one of the following: 1. There is no suggestion of one of the disorders listed in groups 5-11 2. Such a disorder is suggested but excluded by appropriate investigations 3. Such a disorder is present, but the first headache attacks do not occur in close temporal relation to the disorder Note: Most cases respond rapidly and absolutely to indomethacin (usually in doses of 150 mg/d or less). Episodic paroxysmal hemicrania 1. At least 30 attacks fulfilling B-F 2. Attacks of severe unilateral orbital, or temporal pain, or both, that always is unilateral and lasts 1-30 minutes C. Attack frequency of 3 or more a day 3. Clear intervals between bouts of attacks that may last months to years 4. Pain is associated with at least one of the following signs and symptoms on the pain side: 1. Conjunctival injection 2. Lacrimation 3. Nasal congestion 4. Rhinorrhea 5. Ptosis 6. Eyelid edema 5. At least one of the following: 1. There is no suggestion of one of the disorders listed in groups 5-11 2. Such a disorder is suggested but excluded by appropriate investigations 3. Such a disorder is present, but the first headache attacks do not occur in close temporal relation to the disorder Note: Most cases respond rapidly and absolutely to indomethacin (usually in doses of 150 mg/d or less). From Goadsby PJ, Lipton RB: A review of paroxysmal hemicranias, SUNCT syndrome and other short-lasting headaches with autonomic feature, including new cases. Brain 120:193-209, 1997; with permission. |
Epidemiology
There have been more than 100 published case reports of paroxysmal
hemicranias. Initially, these disorders were believed to be rare;
however, the number of diagnosed cases is surely much higher because
these disorders no longer are considered uncommon enough to warrant
publication. Clinically, it appears that CPH is more common than EPH;
only 19 well-documented cases of EPH appear in English
publications.[24] [32] [67]
In contrast to cluster headache, CPH shows a female preponderance, with
a sex ratio of approximately 2:1, whereas there is no gender preference
noted for EPH. The age of onset ranges from 3 to 81 years, with a mean
of approximately 33 years.[2] [10] [35] [46] [98] No family history of
CPH or EPH was found in any of the reported cases. A documented family
history of migraine was evident in 21% of reported cases,[63] whereas
only one prior report documented a positive family history of cluster
headaches.[2]
Clinical Features
Because the clinical profiles of EPH and CPH are essentially identical,
these entities are discussed together. The pain is strictly unilateral
and without side-shift in most sufferers. Bilateral pain was reported
in one patient,[83] and in three patients, the headache showed
side-shift.[2] The maximal pain is experienced in the ocular, temporal,
maxillary, and frontal regions; nuchal and retro-orbital pain has been
described less often. At times, the pain has been reported to radiate
into the ipsilateral shoulder and arm. Strictly occipital pain has been
reported in single reports of CPH[91] and EPH.[24] Rarely, toothache or
otalgia is the presenting symptom.[6] [21]
The pain usually is characterized as a throbbing, boring, pulsatile, or
stabbing sensation that ranges from moderate to excruciating in
severity. Occasionally a mild discomfort at the usual site of pain is
present interictally.[2] During acute attacks, sufferers usually prefer
to sit quietly or lie in bed in the fetal position[115] ; only in rare
reports do the patients assume the pacing activity usually seen in
cluster.
In CPH, attacks recur 1 to 40 times daily. Most patients experience 15
or more attacks per day.[35] [63] [98] There is, however, a marked
variability in attack frequency. Mild attacks recur 2 to 14 times
daily; severe attacks recur 6 to 40 times daily.[63] Individual
headaches usually last 2 to 25 minutes (range, 2 to 120 minutes).[7]
[35] [63] [86] [93] In EPH, the daily attack frequency is 2 to 30
times, with attacks lasting 3 to 30 minutes each. The headache phase
lasts 2 weeks to 4.5 months; remissions last 1 to 36 months.[24] [61]
[63] [67] As with cluster headaches, attacks of CPH and EPH recur
throughout the day and night; nocturnal attacks have been reported to
occur in association with rapid eye movement (REM) sleep.[45] [67] [86]
[98] [100]
Attacks may be triggered by bending or rotating the head,[2] [103]
pressing on the transverse process of C4-5, C2 root, or greater
occipital nerve.[102] [104] During acute attacks, one or more
ipsilateral autonomic symptoms usually are present. Approximately 60%
of sufferers report ipsilateral lacrimation. Homolateral ptosis (33%),
conjunctival injection (36%), nasal congestion (42%), and rhinorrhea
(36%) may accompany the headaches,[2] [35] [93] and ipsilateral miosis
has been reported occasionally. [26] [82] Increased forehead sweating
has been reported to occur in some patients on the symptomatic side
during acute headache episodes, as has generalized sweating.[94] [104]
In contrast to cluster headache, Horner's syndrome does not appear to
accompany attacks of CPH or EPH.[23] [91] [92] Although the IHS
criteria require at least one ipsilateral autonomic feature during
attacks, patients with otherwise typical CPH without autonomic symptoms
have been described.[8] [68] CPH has been reported to occur in
association with other primary headache disorders, such as
migraine,[73] cluster headache, [13] [43] [79] [117] and trigeminal
neuralgia.[12] [36] [121] CPH is recognized in children.
Cases of symptomatic paroxysmal hemicrania have been reported as well.
Disorders that have mimicked paroxysmal hemicranias include aneurysms
within the circle of Willis[57] ; arteriovenous malformations and
cerebrovascular accidents[10] [62] ; collagen vascular disease[57] ;
Pancoast tumor[22] ; tumors of the frontal lobe, [57] sella
turcica,[29] [118] and cavernous sinus[108] ; intracranial
hypertension[39] ; and thrombocythemia. [50] A single posttraumatic
case of CPH with typical migrainous aura has been described.[54]
Treatment
Indomethacin is the treatment of choice for paroxysmal hemicranias and
has been deemed the sine qua non for establishing the diagnosis.
Therapy usually is initiated at a dose of 25 mg 3 times a day and
increased to 50 mg 3 times a day in 1 week if there is no response or
only partial benefit. Headache resolution usually is prompt, occurring
1 to 2 days after the effective dose has been established, although it
may take 2 weeks. Maintenance with doses ranging from 25 to 100 mg
daily usually suffices; however, at times doses of 300 mg/d are
required.
Dosage adjustments occasionally are necessary to treat the clinical
fluctuations that sometimes are seen in these disorders; nighttime
dosing with sustained-release indomethacin often prevents nocturnal
exacerbations. During the active headache cycle, patients report that
skipping or delaying doses of indomethacin may result in the prompt
re-emergence of headaches. In patients suffering from EPH, indomethacin
is given for slightly longer than the usual headache period, then
tapered gradually. In patients with CPH, long-term treatment usually is
required, although long-lasting remissions have been reported after
cessation of indomethacin in rare patients with CPH.[95]
Patients requiring continuous, high dosages of indomethacin may have
underlying pathology and need careful diagnostic evaluation.[108]
Gastrointestinal side effects secondary to indomethacin may be treated
with antacids, misoprostol, or histamine H2 receptor antagonists, and
these agents always should be considered for patients who require
long-term treatment.
Although the IHS criteria require indomethacin response as a diagnostic
criterion, other agents have been reported to be of some benefit.
Acetylsalicylic acid has been shown to be efficacious during the early
phases of CPH in some patients[2] and possibly during the childhood
phase of the disorder.[46] Partial success has been reported with
verapamil,[14] [89] steroids,[37] and naproxen.[2] A piroxicam
derivative was reported to be efficacious in some patients with CPH who
also were responsive to indomethacin therapy,[97] and acetazolamide
therapy successfully treated a patient previously unresponsive to
indomethacin.[119] Sumatriptan has been reported to be beneficial in a
patient suffering from bilateral CPH,[39] although its usefulness in
the typical unilateral cases has not been shown.[17] [32] Successful
treatment with the cyclooxygenase 2 inhibitor celecoxib was
reported.[55] In patients refractory to indomethacin therapy, the
diagnosis should be reconsidered.
HEMICRANIA CONTINUA
HC is another rare primary headache syndrome that responds to treatment
with indomethacin. The disorder initially was described by Sjaastad and
Spierings[107] in two patients with continuous headaches from onset.
Subsequent reports described patients in whom episodes of prolonged
pain were separated by pain-free remissions.[44] [64] [72] [109] In
some patients, an initially episodic course evolved into the continuous
form. As with paroxysmal hemicrania, the nomenclature for HC is
controversial. Sjaastad and Antonaci[96] proposed that the
classification of HC should be separated into remitting and unremitting
forms. The unremitting form may be subclassified further as unremitting
from onset and unremitting evolved from remitting. The authors have
proposed nomenclature analogous to cluster headache: a remitting form
with distinct headache phases separated by pain-free remissions, an
unremitting form that evolved from the remitting form, and an
unremitting form with continuous headache persisting for years.[32]
[64] HC is not included currently in the IHS classification scheme.
Proposed criteria are listed in Box 1.
Epidemiology
HC, similar to paroxysmal hemicrania, is probably an under recognized
disorder. In headache subspecialty practices, HC is a common cause of
refractory, unilateral, chronic daily headache. The disorder shows a
marked female preponderance, with a female-to-male ratio of
approximately 2:1. The age of onset ranges from 11 to 58 years (mean,
34 years).
Clinical Features
Most patients describe strictly unilateral pain, without side-shift.
Two patients reported bilateral radiation of predominantly unilateral
pain; one patient described bilateral pain; and in one patient with the
remitting form, the pain alternated sides from attack phase to attack
phase. The maximal pain is experienced in the ocular, temporal, and
maxillary regions. Occasionally the pain may radiate into the
ipsilateral occiput, nuchal, and retro-orbital areas.
The pain usually is described as a steady ache or throbbing pain.
Superimposed on the continuous baseline low-level discomfort, most
patients report exacerbations of more intense pain lasting 20 minutes
to several days. Although significantly more intense than the usual
background discomfort, the painful exacerbations never reach the level
experienced by cluster headache sufferers. These exacerbations may
occur at any time of the day or night and frequently awaken the patient
from sleep. Exacerbations of pain often are accompanied by nausea;
photophobia; and the autonomic features of cluster headache, including
ipsilateral ptosis, conjunctival injection, lacrimation, and nasal
congestion. When present, these associated features usually are much
less pronounced than when seen in cluster headaches. Painful
exacerbations are associated with a sensation of ocular discomfort,
often likened to a foreign body in the eye (typically reported as sand
or hair). Concurrent idiopathic (primary) stabbing headaches (jabs and
jolts) are reported by many patients, occasionally occurring only in
association with the painful exacerbations. During exacerbations of
pain, patients assume the pacing activity usually seen with cluster
headaches.
Although it is not a true cluster variant, HC may be mistaken for
cluster if the physician focuses on the painful flare-ups with
associated autonomic features. A careful history should reveal the
presence of the continuous, low-level baseline discomfort in addition
to the more disabling exacerbations. The autonomic features of HC, when
present, tend to be much less pronounced than those of cluster.
Organic mimics of HC have been reported to occur in association with a
mesenchymal tumor involving the sphenoid bone, clinoid process, and
skull base.[32] Rarely the diagnosis of HC is masked by a concurrent
medication rebound headache. In these instances, discontinuation of the
overused analgesic is not associated with headache cessation, and the
diagnosis of HC is made by exclusion.[120]
Treatment
Similar to paroxysmal hemicrania, HC is responsive to indomethacin. The
treatment strategies detailed in the preceding section apply to this
disorder as well. Other agents that may have partial success in the
treatment of HC include naproxen and paracetamol, paracetamol in
combination with caffeine, ibuprofen, piroxicam, and rofecoxib.[32]
[80] Kuritzky[49] reported four patients who met the clinical criteria
for HC but failed to respond to treatment with indomethacin or other
agents. Kuritzky[49] termed this condition indomethacin-resistant HC.
SUNCT SYNDROME
The SUNCT syndrome is one of the rarest of the unusual primary headache
disorders and has the most dramatic and variable clinical presentation.
The syndrome first was described in 1978 and characterized more fully
in 1989.[101] [105] Currently, 26 sufferers have been reported. The
disorder has a male predominance (18 men, 8 women) with a sex ratio of
2.25:1.[4] [9] [32] [56] [76] [77] [84] Suggested clinical criteria for
the diagnosis of SUNCT syndrome are listed in the accompanying box.
| Box 2. Suggested Criteria for SUNCT 1. At least 30 attacks fulfilling B-E 2. Attacks of unilateral moderately severe orbital or temporal stabbing or throbbing pain lasting 15-120 seconds 3. Attack frequency 3-100 a day 4. Pain is associated with at least one of the following signs and symptoms of the affected side with feature 1 being most often present and prominent: 1. Conjunctival injection 2. Lacrimation 3. Nasal congestion 4. Rhinorrhea 5. Ptosis 6. Eyelid edema 5. At least one of the following: There is no suggestion of one of the disorders listed in groups 5-11 Such a disorder is suggested but excluded by appropriate investigations Such a disorder is present, but the first headache attacks do not occur in close temporal relation to the disorder From Goadsby PJ, Lipton RB: A review of paroxysmal hemicranias, SUNCT syndrome and other short-lasting headaches with autonomic feature, including new cases. Brain 120:193-209, 1997; with permission. |
Clinical Features
SUNCT syndrome is characterized by brief headache episodes that recur
multiple times per day. The age of onset ranges from 23 to 77 years
(mean, 51 years).[77] The pain usually is maximal in the orbital and
periorbital regions and may radiate to the ipsilateral forehead,
temple, nose, cheek, and palate.[77] Attacks typically are unilateral;
in three patients, however, pain was experienced simultaneously on the
opposite side.[77] The pain quality usually is described as burning,
stabbing, or electric-like. Paroxysms begin and end abruptly, reaching
maximum intensity within 2 to 3 seconds.[77] Individual headache
attacks last 5 to 250 seconds each (mean, 49 seconds),[75] although
attacks lasting 2 hours each have been described.[70] Some patients
describe a dull interictal discomfort that persists between acute
episodes,[70] although most patients report being totally pain-free
between attacks. Four patients experienced a status like pattern, in
which painful paroxysms persisted 1 to 3 days.[69]
The temporal pattern is quite variable, with symptomatic periods
alternating with periods of pain-free remissions occurring in an
erratic fashion. Symptomatic periods generally last a few days to
several months and occur once or twice yearly. Remissions range from 1
week to 7 years but usually are of a few months' duration.[77] During
the symptomatic phase, daily attacks recur 6 to 77 times (mean, 28
times) [75] ; however, tremendous variability occurs among different
patients and within the same patient. Attacks may be as infrequent as
once per day or less to more than 30 attacks per hour.[75] [77] In one
patient, attacks recurred in a repetitive and overlapping fashion for 1
to 3 hours at a time, twice daily.[74] Although attacks recur
throughout the day, a bimodal distribution with increased attack
frequency occurring in the morning and afternoon and evening hours has
been described.[75] Nocturnal attacks were experienced by 12
patients.[77]
The acute attacks of headache in SUNCT syndrome are accompanied by a
variety of associated symptoms, the most prominent of which are
ipsilateral conjunctival injection and lacrimation. Ipsilateral nasal
congestion, rhinorrhea, and eyelid edema are reported less commonly. In
some patients, the accompanying autonomic phenomena were bilateral,
although more pronounced on the side of the headache.[77] The
associated tearing and conjunctival injection usually begin 1 to 2
seconds after the acute episodes of pain and may persist for a few
seconds longer than the painful episodes.[77] In two patients, the
associated symptoms remained 30 to 60 seconds after headache
resolution,[3] [105] and in one patient, eyelid edema persisted for 5
to 10 minutes.[38] Rhinorrhea, when present, is delayed, occurring
relatively late during the course of the headache.[77]
Many patients can precipitate acute attacks by touching certain trigger
zones within the territory of V1-3. Other precipitating maneuvers
include touching the hair, forehead, face, nose, and lip on the
symptomatic side. Washing, shaving, eating, chewing, tooth brushing,
talking, and coughing were reported as headache triggers.[77]
Mechanical movements of the neck can precipitate attacks, although some
patients could lessen or abort attacks by rotating their neck
continuously.[77] [105] In contrast to trigeminal neuralgia, most
patients have no refractory period. Secondary causes of SUNCT syndrome
have been reported in four patients and include homolateral
cerebellopontine angle arteriovenous malformations,[11] [20] brainstem
cavernous hemangioma,[59] and a posterior fossa lesion in a patient
with acquired immunodeficiency syndrome.[32]
Treatment
SUNCT syndrome has proved to be refractory to a variety of therapeutic
approaches. Medications typically employed in the treatment of
migraine, cluster, and other short-lived headache syndromes are
ineffectual, as are anesthetic blockades.[71] Carbamazepine was
reported to have a possibly beneficial effect in 5 of 18 patients.[77]
Azathioprine, oral sumatriptan, prednisone, valproate, and nifedipine
were shown to be mildly efficacious in single reports.[19] [31] [38]
[71] Lamotrigine has been reported to abolish the syndrome and may be
the treatment of choice.[19] Verapamil and omeprazole were reported to
worsen the condition.[71]
CLUSTER-MIGRAINE AND CLUSTER-TIC SYNDROMES
Occasionally, patients describe headaches that have the features of
cluster headache and another primary headache disorder. Recognition of
these syndromes is important because the therapeutic options are
different from the standard treatment of the separate disorders.
The cluster-migraine syndrome is diagnosed when elements of migraine
headache occur simultaneously in patients suffering with cluster
headache. Solomon and Kappa[111] instituted arbitrary diagnostic
criteria to establish the diagnosis of this uncommon syndrome.
According to their criteria, the diagnosis of cluster-migraine syndrome
was given to patients who had symptoms of one headache predominantly
(either migraine or cluster), but in whom four or five features of the
other headache also were present. Patients who experienced cluster
headache with nausea, vomiting, photophobia, or phonophobia would
receive this diagnosis, as would patients with migraine who experienced
ipsilateral autonomic features. The criteria employed by Solomon and
Kappa[111] were not precise; the required number of associated symptoms
was picked arbitrarily. Other clinicians have reported patients with
two distinct headache disorders. Graham[34] described patients who
suffered from recurrent bouts of migraine headaches that recurred daily
for days or weeks at a time, then entered a period of pain-free
remissions. It is important to recognize the cluster-migraine syndrome
because of the unique treatment strategies that need to be implemented.
Patients suffering from this syndrome have been reported to respond to
inhalation of 100% oxygen as an abortive strategy for acute attacks and
lithium carbonate as prevention. This combination would not be expected
to be helpful in patients suffering from typical migraine.
Alternatively, beta-blockers occasionally may help in this syndrome,
whereas these agents would not be useful in the treatment of cluster
headaches.
The diagnosis of cluster-tic syndrome is given to patients in whom
cluster headache and trigeminal neuralgia coexist.[1] [110] This rare
condition is characterized by repetitive volleys of excruciating
lancinating pains that usually are described with trigeminal neuralgia,
but in this syndrome they occur superimposed on the typical features of
cluster headache. These tic like pains commonly are precipitated by
light cutaneous or mucous membrane stimulation as in typical trigeminal
neuralgia. Concurrently the patient also suffers from the typical
clinical features of cluster headache. Often the two pain syndromes
occur independently of each other for months or years before their
simultaneous occurrence. In general, the cluster-tic syndrome is
difficult to treat and may require a combination of therapies.
Carbamazepine has been reported to abolish the tic like pains, whereas
standard anticluster agents are required to treat the features of
cluster. Solomon et al[110] described four patients with this syndrome
who underwent microvascular decompression of the trigeminal nerve;
compression of the root entry zone by a blood vessel was noted in all
four patients. In two of the three patients in whom there was facial
nerve exploration, an arterial loop was found at the root entry zone of
the facial nerve. Postoperatively, all patients experienced relief of
the neuralgia component of the syndrome; the features of cluster were
not altered by the surgery, however.
HYPNIC HEADACHE SYNDROME
The hypnic headache syndrome is a rare primary headache disorder that
first was described by Raskin in 1988,[85] then by Newman et al in
1990.[65] The initial description of the syndrome consisted of a
bilateral, throbbing headache without associated autonomic features
lasting 15 to 60 minutes, recurring one to three times nightly, often
during REM sleep. At the time of this writing, 37 patients with the
disorder have been described. The disorder has a female predominance
(26 women, 11 men), with a sex ratio of 2.36:1.[25] [32] [33] [42] [60]
[65] [66] [81] [85] Although most patients are elderly, the age of
onset ranges from 40 to 82 years (mean, 66 years; median, 66 years).
Headaches occur at a consistent time each night, usually between 1:00
and 3:00 A.M., and rarely may occur during a daytime nap.[25] The
headaches begin abruptly, are diffuse and throbbing, and spontaneously
resolve in 15 to 180 minutes. In 10 patients (27%), the headache was
hemicranial.[25] [33] [42] [60] No associated autonomic symptoms
accompany the pain, but nausea, photophobia, and phonophobia rarely may
be present.
Treatment
All patients have been treated successfully with bedtime doses of
lithium carbonate, 300 to 600 mg, although many patients could not
tolerate the side effects. Bedtime doses of caffeine (5 patients);
flunarizine (2 patients); indomethacin (1 patient); atenolol (1
patient); and combination of ergotamine tartrate, belladonna, and
caffeine (1 patient) have been reported to treat this syndrome
(Morales-Asin et al, 1998; Dodick et al, 1998; Ivanez et al, 1998). One
patient treated with prednisone, vincristine, and cyclophosphamide for
an unrelated illness experienced headache resolution (Newman et al,
1991). The accompanying box lists suggested criteria for hypnic
headache.
|
Box 3. Suggested Criteria for Hypnic Headache * Note: A rapid clinical response to lithium at bedtime usually is expected. |
SUMMARY
The disorders described in this article are relatively rare, but
probably are more common than previously thought. Because these
disorders cause significant pain and disability and treatment response
differs from that of migraine, tension-type, and cluster headaches,
recognition is essential. Table 1 lists the important clinical features
of these syndromes and contrasts them with cluster headache, the
disorder for which they are often confused.
TABLE 1 -- CLINICAL FEATURES
| Chronic Paroxysmal Hemicrania | Episodic Paroxysmal Hemicrania | SUNCT | Hypnic | Cluster | |
| Sex (F:M) | 2:1 | 1:1 | 1:2 | 2:1 | 1:8 |
| Pain quality | Throbbing, boring, stabbing | Throbbing, boring, stabbing | Burning, stabbing, electric | Throbbing | Stabbing, boring |
| Pain severity | Very severe | Very severe | Moderate | Moderate | Very severe |
| Site of maximal pain | Orbit, temple | Orbit, temple | Periorbital | Diffuse, rarely hemicranial | Orbit, temple |
| Attacks per day | 1-40 | 2-30 | 6-77 | 1-3 | 1 every second day-8 |
| Attacks duration | 2-25 minutes | 3-30 minutes | 5-250 seconds | 30-60 minutes | 15-180 minutes |
| Autonomic features | Present | Present | Present | Absent | Present |
| Triggers | Alcohol, bending head, pressing C4-5, C2, occipital nerve | Alcohol, bending head, pressing C4-5, C2, occipital nerve | V1-3 trigger points, neck movements | Sleep | Alcohol |
| Nocturnal attacks | Yes | Yes | Yes | Yes | Yes |
| Treatment | Indomethacin | Indomethacin | None known | Lithium, caffeine | Verapamil, lithium, methysergide |
SUNCT = Short-lasting unilateral neuralgiform headaches with conjunctival injection and tearing.
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*Addendum: These criteria were proposed before the reports of 10 patients with unilateral hypnic headaches.
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