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Cluster headache (CH) is a subtype of trigeminal autonomic headache known as "suicidal headache" due to the intensity of pain at the time of attack, and presents clinically as very severe pain in the strictly unilateral orbital, supraorbital, and/or temporal regions with pain-side autonomic symptoms and/or restlessness and restlessness
.
The harm of CH and the low accuracy of diagnosis should be paid attention
to the severe pain of CH attacks, prone to anxiety, depression and aggressive behavior, and may have a variety of complications (such as cardiovascular disease and suicidal tendencies, etc.
) , resulting in a great burden of
disease.
At the same time, due to the low prevalence of CH, many physicians lack the understanding of the disease, which often leads to CH patients not being correctly diagnosed
.
Foreign data show that 43% of CH patients have a diagnosis delay of more than 5 years, and relevant studies in China have found that the diagnosis delay of more than 5 years can reach 62.
5%, and the diagnosis delay of more than 10 years is as high as 40.
0%.
In order to improve the standardized diagnosis and treatment level of CH in China and benefit more CH patients, relevant academic associations organized an expert group to formulate the "Guidelines for the Diagnosis and Treatment of Cluster Headache in China", which evaluated the clinical practice and high-quality literature evidence of cluster headache at home and abroad in recent years, and elaborated on the pathogenesis, clinical manifestations, diagnosis and differential diagnosis and treatment of CH, which was published in the Chinese Journal of Pain Medicine
.
It is worth noting that the guide condenses the key points of diagnosis and treatment of CH into a flow chart, so save it quickly!
Figure 1.
CH diagnosis and treatment flow chart [1]
Of course, looking directly at the flow chart will inevitably look a little confused, let's follow the guide to learn the basics first
.
▌The clinical manifestations of CH headache are known for a wide variety and complex manifestations
, and it is difficult to carry out
precise treatment without being familiar with the clinical features of each headache.
The guidelines divide CH into episodic CH (eCH) and chronic CH (cCH), and its temporal characteristics, seizure characteristics, and comorbid characteristics are shown in
the following table.
Table 1.
Clinical features of CH [1]
▌Diagnostic criteria for CH should be combined with diagnostic criteria
(Table 2) and historical features, such as headache severity, headache side, site of attack, Frequency, duration, accompanying symptoms, etc
.
The pre-onset (prodromal) symptoms, accompanying symptoms, and post-onset symptoms in the course of CH should be distinguished to avoid missed diagnosis and misdiagnosis
.
Be particularly alert to the possible presence of persistent background pain
.
In addition, it should be considered comprehensively in combination with the clinical characteristics of CH patients in China, such as: CH patients in China are more common in temporal and orbital pain; Pain is mostly concentrated in the first distribution area of the trigeminal nerve, and the incidence of restlessness and restlessness is low
.
The diagnostic criteria for CH, eCH and cCH are shown in the following table, among which cCH is relatively rare than eCH, but can be converted to
each other.
Table 2.
Diagnostic criteria for CH [1]
▌Differential diagnosis of CH The diagnosis
of CH should first exclude secondary causes and distinguish them from other trigeminal autonomic headaches, such as paroxysmal migraine, Transient unilateral neuralgia-like headache attacks with conjunctival hyperemia and tearing (SUNCT), and primary headaches such as migraine and sleep headache with ipsilateral autonomic symptoms need to be distinguished, as shown in Figure 1
.
1Secondary headache
The etiology of secondary headache is often serious, if neglect is easy to lead to delay, the identification should be particularly cautious, the guidelines give the key points of differentiation between CH and secondary headache from multiple perspectives such as medical history, etiology, examination, and treatment effect feedback (Table 3).
Table 3.
Differentiation of CH from secondary headache[1]2
Most of the episodes of CH are accompanied by trigeminal autonomic symptoms, so it is necessary to further distinguish it from paroxysmal migraine headache, persistent migraine headache, and SUNCT according to the relevant characteristics of headache (Table 4), such as the duration and frequency of headache and response to indomethacin
.
Table 4.
The main clinical features of CH and other trigeminal autonomic headaches[1] Note: PH is paroxysmal migraine; HC is persistent migraine headache3
Other primary headaches Sometimes there can be a certain phenotypic overlap between migraine and CH, which can easily lead to misdiagnosis, and the onset time and degree of sleep headache are similar to CH, which may also lead to misdiagnosis, and the key points of differential diagnosis are shown in
the following table.
Table 5.
Differentiation between CH and primary headache[1]
▌CH treatment of CH is mainly divided into three types: acute treatment, preventive treatment and transitional treatment, and in recent years, some new drugs and neuromodulation technologies have gradually been used in the treatment
of CH
。
1Acute phase treatment
The goal of treatment is to quickly relieve headaches and stop acute headache attacks
as soon as possible.
Commonly used criteria for evaluating the effectiveness of treatment include: (1) painless within 15 minutes; (2) the degree of headache within 30 minutes (from moderate to severe or very severe pain to mild or no pain); (3) pain improvement duration of 60 minutes; (4) There is no need to take the drug
again within 15 minutes of treatment.
Treatment includes the use of triptans, lidocaine, somatostatin and its analogues, oxygen, neuromodulation therapy, etc.
, and the guidelines recommend as shown in the table below
.
Table 6.
Recommended treatment of acute CH attacks in adults[1]2
Prophylactic treatment aims to reduce the frequency and severity of headache attacks during the cluster period and to improve the efficacy
of acute treatment.
Efficacy indicators include reduced frequency of headache attacks, reduced duration of headaches, reduced headache intensity, and improved response to acute treatment
during the cluster period.
Preventive treatment should be considered when CH causes patients to: (1) severely impaired quality of life, work or school (as judged by the patient); (2) frequent headache attacks during the cluster period; (3) The effect of drug treatment in the acute phase is not optimal or the patient cannot tolerate it
.
Transitional treatment, also known as short-term preventive treatment or bridging therapy, because preventive treatment drugs require a certain amount of time and drug dosage to be effective in the therapeutic effect, for patients with high-frequency attacks with a headache frequency of ≥ 2 times a day, transitional therapy can be used when the preventive drug is started or the dose is increased, and the treatment period usually lasts no more than 2 weeks
.
Efficacy indicators include frequency of CH, duration of headache, degree of headache, number of medications administered in the acute phase of the attack, and time of cluster phase
.
The guidelines' treatment methods, doses, and levels of evidence for CH preventive treatment and transitional treatment are shown
in the table below.
Table 7.
CH preventive therapy and transitional therapy are recommended [1]
3Neuromodulation therapy and new drugs
Neuromodulation treatments include sphenopalatine ganglion radiofrequency ablation, sphenopalatine ganglion stimulation, non-invasive vagus nerve stimulation, invasive occipital nerve stimulation, and deep hypothalamic stimulation
.
The primary objective is to use noninvasive or invasive neuromodulation therapy for refractory CH or intolerance to conventional care to reduce the serious adverse effects and disabling effects of headache
in patients.
Novel drugs include CGRP monoclonal antibodies, lysergic acid diethylamine, etc.
, and their safety and efficacy need to be confirmed
by further clinical trials.
▌ The prognosis
of CH differs
in long-term outcomes for patients with eCH and cCH.
eCH and cCH can be converted to each other, eCH is usually easily converted to cCH if it is poorly controlled, and cCH can be converted into eCH
with better prognosis under standardized management.
Studies have shown that late age of onset, male sex, and disease duration of more than 20 years may be key factors affecting the prognosis of
CH.
Questionnaire survey
Dry goods are full, hurry up and collect!
Cluster headache (CH) is a subtype of trigeminal autonomic headache known as "suicidal headache" due to the intensity of pain at the time of attack, and presents clinically as very severe pain in the strictly unilateral orbital, supraorbital, and/or temporal regions with pain-side autonomic symptoms and/or restlessness and restlessness
.
The harm of CH and the low accuracy of diagnosis should be paid attention
to the severe pain of CH attacks, prone to anxiety, depression and aggressive behavior, and may have a variety of complications (such as cardiovascular disease and suicidal tendencies, etc.
) , resulting in a great burden of
disease.
At the same time, due to the low prevalence of CH, many physicians lack the understanding of the disease, which often leads to CH patients not being correctly diagnosed
.
Foreign data show that 43% of CH patients have a diagnosis delay of more than 5 years, and relevant studies in China have found that the diagnosis delay of more than 5 years can reach 62.
5%, and the diagnosis delay of more than 10 years is as high as 40.
0%.
The first Chinese CH diagnosis and treatment guidelines were released
In order to improve the standardized diagnosis and treatment level of CH in China and benefit more CH patients, relevant academic associations organized an expert group to formulate the "Guidelines for the Diagnosis and Treatment of Cluster Headache in China", which evaluated the clinical practice and high-quality literature evidence of cluster headache at home and abroad in recent years, and elaborated on the pathogenesis, clinical manifestations, diagnosis and differential diagnosis and treatment of CH, which was published in the Chinese Journal of Pain Medicine
.
It is worth noting that the guide condenses the key points of diagnosis and treatment of CH into a flow chart, so save it quickly!
Figure 1.
CH diagnosis and treatment flow chart [1]
Of course, looking directly at the flow chart will inevitably look a little confused, let's follow the guide to learn the basics first
.
▌The clinical manifestations of CH headache are known for a wide variety and complex manifestations
, and it is difficult to carry out
precise treatment without being familiar with the clinical features of each headache.
The guidelines divide CH into episodic CH (eCH) and chronic CH (cCH), and its temporal characteristics, seizure characteristics, and comorbid characteristics are shown in
the following table.
Table 1.
Clinical features of CH [1]
▌Diagnostic criteria for CH should be combined with diagnostic criteria
(Table 2) and historical features, such as headache severity, headache side, site of attack, Frequency, duration, accompanying symptoms, etc
.
The pre-onset (prodromal) symptoms, accompanying symptoms, and post-onset symptoms in the course of CH should be distinguished to avoid missed diagnosis and misdiagnosis
.
Be particularly alert to the possible presence of persistent background pain
.
In addition, it should be considered comprehensively in combination with the clinical characteristics of CH patients in China, such as: CH patients in China are more common in temporal and orbital pain; Pain is mostly concentrated in the first distribution area of the trigeminal nerve, and the incidence of restlessness and restlessness is low
.
The diagnostic criteria for CH, eCH and cCH are shown in the following table, among which cCH is relatively rare than eCH, but can be converted to
each other.
Table 2.
Diagnostic criteria for CH [1]
▌Differential diagnosis of CH The diagnosis
of CH should first exclude secondary causes and distinguish them from other trigeminal autonomic headaches, such as paroxysmal migraine, Transient unilateral neuralgia-like headache attacks with conjunctival hyperemia and tearing (SUNCT), and primary headaches such as migraine and sleep headache with ipsilateral autonomic symptoms need to be distinguished, as shown in Figure 1
.
1Secondary headache
The etiology of secondary headache is often serious, if neglect is easy to lead to delay, the identification should be particularly cautious, the guidelines give the key points of differentiation between CH and secondary headache from multiple perspectives such as medical history, etiology, examination, and treatment effect feedback (Table 3).
Table 3.
Differentiation of CH from secondary headache[1]2
Most of the episodes of CH are accompanied by trigeminal autonomic symptoms, so it is necessary to further distinguish it from paroxysmal migraine headache, persistent migraine headache, and SUNCT according to the relevant characteristics of headache (Table 4), such as the duration and frequency of headache and response to indomethacin
.
Table 4.
The main clinical features of CH and other trigeminal autonomic headaches[1] Note: PH is paroxysmal migraine; HC is persistent migraine headache3
Other primary headaches Sometimes there can be a certain phenotypic overlap between migraine and CH, which can easily lead to misdiagnosis, and the onset time and degree of sleep headache are similar to CH, which may also lead to misdiagnosis, and the key points of differential diagnosis are shown in
the following table.
Table 5.
Differentiation between CH and primary headache[1]
▌CH treatment of CH is mainly divided into three types: acute treatment, preventive treatment and transitional treatment, and in recent years, some new drugs and neuromodulation technologies have gradually been used in the treatment
of CH
。
1Acute phase treatment
The goal of treatment is to quickly relieve headaches and stop acute headache attacks
as soon as possible.
Commonly used criteria for evaluating the effectiveness of treatment include: (1) painless within 15 minutes; (2) the degree of headache within 30 minutes (from moderate to severe or very severe pain to mild or no pain); (3) pain improvement duration of 60 minutes; (4) There is no need to take the drug
again within 15 minutes of treatment.
Treatment includes the use of triptans, lidocaine, somatostatin and its analogues, oxygen, neuromodulation therapy, etc.
, and the guidelines recommend as shown in the table below
.
Table 6.
Recommended treatment of acute CH attacks in adults[1]2
Prophylactic treatment aims to reduce the frequency and severity of headache attacks during the cluster period and to improve the efficacy
of acute treatment.
Efficacy indicators include reduced frequency of headache attacks, reduced duration of headaches, reduced headache intensity, and improved response to acute treatment
during the cluster period.
Preventive treatment should be considered when CH causes patients to: (1) severely impaired quality of life, work or school (as judged by the patient); (2) frequent headache attacks during the cluster period; (3) The effect of drug treatment in the acute phase is not optimal or the patient cannot tolerate it
.
Transitional treatment, also known as short-term preventive treatment or bridging therapy, because preventive treatment drugs require a certain amount of time and drug dosage to be effective in the therapeutic effect, for patients with high-frequency attacks with a headache frequency of ≥ 2 times a day, transitional therapy can be used when the preventive drug is started or the dose is increased, and the treatment period usually lasts no more than 2 weeks
.
Efficacy indicators include frequency of CH, duration of headache, degree of headache, number of medications administered in the acute phase of the attack, and time of cluster phase
.
The guidelines' treatment methods, doses, and levels of evidence for CH preventive treatment and transitional treatment are shown
in the table below.
Table 7.
CH preventive therapy and transitional therapy are recommended [1]
3Neuromodulation therapy and new drugs
Neuromodulation treatments include sphenopalatine ganglion radiofrequency ablation, sphenopalatine ganglion stimulation, non-invasive vagus nerve stimulation, invasive occipital nerve stimulation, and deep hypothalamic stimulation
.
The primary objective is to use noninvasive or invasive neuromodulation therapy for refractory CH or intolerance to conventional care to reduce the serious adverse effects and disabling effects of headache
in patients.
Novel drugs include CGRP monoclonal antibodies, lysergic acid diethylamine, etc.
, and their safety and efficacy need to be confirmed
by further clinical trials.
▌ The prognosis
of CH differs
in long-term outcomes for patients with eCH and cCH.
eCH and cCH can be converted to each other, eCH is usually easily converted to cCH if it is poorly controlled, and cCH can be converted into eCH
with better prognosis under standardized management.
Studies have shown that late age of onset, male sex, and disease duration of more than 20 years may be key factors affecting the prognosis of
CH.
small
knot
This guideline is the first clinical practice guide on the diagnosis and treatment of CH in China, which is of great significance
.
However, due to the relative rarity of CH, its clinical evidence is still relatively lacking or insufficient, and some recommendations are weak
.
Therefore, the guidelines still recommend that clinicians should make comprehensive judgments
based on the hospital environment and specific patient conditions, such as disease severity, patient treatment intention, patient response to drugs, and disease progression when using this guideline.
Questionnaire survey
Stroke (commonly known as "stroke") has the characteristics of "four highs" with high morbidity, high disability rate, high mortality rate and high recurrence rate, acute onset, rapid progression and serious consequences, 1 in every 4 people in the world will have a stroke, 1 person will die of stroke every 6 seconds, and 1 person will be disabled due to stroke every 6 seconds, and the patient's family will suffer huge economic losses and physical and mental suffering
.
Reference sources:
[1] Headache and Sensory Disorders Special Committee of Chinese Research Hospital Association, Pain and Sensory Disorders, Neurologist Branch of Chinese Medical Doctor Association.
Chinese expert consensus on the diagnosis and treatment of cluster headache[J].
Chinese Journal of Pain Medicine,2022,(9):641~653.
)
