cluster headache is prone to disability due to its severe pain during attack, and the diagnosis accuracy rate in my country is currently low, and the treatment of
is not standardized. In order to improve the standardized diagnosis and treatment of cluster headaches in my country and benefit more patients with cluster headaches, the China Medical Association Neurologists Branch and the China Research Hospital Association Headache and Sensory Disorder Committee have evaluated the clinical practice and high-quality literature evidence of cluster headaches at home and abroad in recent years, and elaborated on the pathogenesis, clinical manifestations, diagnosis, differential diagnosis, and treatment of cluster headaches. Come and learn together. The pathogenesis of
~. The pathogenesis of
CH is not fully defined. It is currently believed that it is mainly due to the synchronous abnormal activities of three important components: trigeminal nerve vascular pathway, trigeminal nerve -automatic nerve reflex, and the hypothalamus.
clinical characteristics
CH is mainly divided into episodic CH (eCH) and chronic CH (cCH).
(I) Time characteristics
CH attacks often have a certain seasonal rhythm, which is manifested as easy to occur when seasons alternate, such as frequent occurrence in spring and autumn and less frequent occurrence in winter. At the same time, CH attacks have circadian rhythmic characteristics, and most patients have relatively fixed headache attack times every day, so they are known as "alarm clock headache".
(II) Attack characteristics
1. Inducement
During the cluster period, drinking, weather changes, odor stimulation, emotional factors, mental stress, lack of sleep, drugs ( histamine , nitroglycerin ), etc. can all induce attacks. The most common causes are drinking, weather changes and lack of sleep.
2. Pre-attack (prodromal) symptoms
CH 10 to 20 minutes before the onset of the attack, prodromal symptoms such as headache side discomfort, craniocerebral autonomic neurological symptoms (the most common are head and face discomfort, neck stiffness, anxiety, depression, photophobia, etc.).
3. Avatar symptoms
Avatar symptoms occasionally appear in CH, among which visual avatars are the most common type in patients with CH.
4. Characteristics of headache
CH is manifested as severe or extremely severe pain in the orbit, supraorbital and/or temporal area on the unilateral orbital. When the pain is severe, it can affect the forehead, top, occipital or face. It is often manifested as sharp pain, pulsating pain, squeeze pain or explosive pain, which can be sudden and sudden. It is an important feature of
The headache site is always fixed to one side (the Asian population has a higher frequency of pain on the right side), but some patients also experience other changes in the headache side between different cluster periods or within the same cluster period.
The symptoms of ipsilateral autonomic nerves during headache are important characteristics of CH. More than 90% of CH patients are accompanied by at least one of the following symptoms: conjunctival congestion , tears, nasal congestion, runny nose, eyelid swelling, ptosis, pupil shrinkage, facial sweating and flushing, etc.
(III) Co-morbid
CH patients are often accompanied by brain dysfunction diseases such as depression and sleep disorders.
Diagnosis and differential diagnosis
(1) Diagnosis of CH in
➤ The diagnostic criteria for CH in ICHD-3 are as follows:
1. Meet more than 5 episodes of 2 to 4;
2. Severe or extremely severe pain occurring in unilateral orbit, supraorbital and/or temporal areas. If the pain is not treated, it will last for 15 to 180 minutes;
3. When a headache occurs, it meets at least 1 of the following 2 items:
(1) At least 1 of the following symptoms or signs (and the same side of the headache): ① Conjunctiva congestion and/or tears; ② Nasal congestion and/or runny nose; ③ Eyelial edema; ④ Pupils shrink and/or ptosis; (2) Frequency of irritability or restlessness;
4. The frequency of attack once a day to 8 times a day;
5. It cannot be better explained by other diagnoses in ICHD-3.
➤Occurrence CH: The cluster period lasts for 7 days to 1 year, and the headache remission period lasts for at least 3 months.
diagnostic criteria: ① Onset meets the CH diagnostic criteria and occurs during the cluster period; ② At least 2 cluster periods last for 7 days to 1 year (untreated), and the headache remission period is ≥3 months.
➤Chronic CH: There is no remission period for at least 1 year or the remission period is less than 3 months.
diagnostic criteria: ① onset meets the CH diagnostic criteria and meets the standards ②; ② There is no remission period or remission period is less than 3 months for at least 1 year.
(2) Differential diagnosis of
CH. The diagnosis of secondary causes must be ruled out first and distinguished from other trigeminal autonomic headaches, such as paroxysmal migraine headaches, short-term unilateral neuralgia-like headaches with conjunctival congestion and tears. At the same time, it is necessary to distinguish primary headaches such as migraine , sleep headaches, etc. with ipsilateral autonomic nerve symptoms. The CH diagnosis and treatment flow chart is shown in Figure 1.
Figure 1CH diagnosis and treatment flow chart
1. Secondary headache: Some craniocerebral organic damage manifestations can be similar to CH. Symptoms alone cannot be distinguished, and a comprehensive diagnostic evaluation is required.
2. Other trigeminal autonomic headaches: Most of the trigeminal autonomic headaches are associated with trigeminal autonomic headaches, so they need to be further identified based on the relevant characteristics of the headache, such as the duration, frequency of the headache and the response to indomethacin. The duration of different types of headaches may overlap, so comprehensive analysis is required in combination with other characteristics. The main clinical features of CH and other trigeminal autoneurotic headaches are shown in Table 1.
Table 1 The main clinical features of CH and other trigeminal nerve autonomic headaches
3. Other primary headaches:
(1) Migraine: There may sometimes be a certain phenotypic overlap between migraine and CH, which can easily lead to misdiagnosis. If the migraine attack is strictly unilateral, or coexist with the ipsilateral cranial autonomic neurological symptoms, or if the cluster-like headache attack has different headaches, aura symptoms, accompanied by photophobia, nausea or vomiting, the two need to be carefully identified.
When the following conditions occur, this headache is more supported: ① The headache lasts for a long time without treatment; ② Daily physical activities will aggravate the headache or avoid daily activities due to headaches (clinically, many migraine patients choose to stay in bed instead of continuing to move or work when headaches occur. On the contrary, CH patients are often agitated when headaches occur); ③ The severity of the pain varies. Migraines are often moderate and severe pain, while CH is often extremely severe pain. But when there is a very obvious periodic attack, headaches are more supportive of CH.
(2) Sleeping headache: Sleeping headache only occurs during sleep and often occurs between 1:00 and 3:00 in the morning, so it can be confused with CH. Sleeping headaches often occur frequently, reaching 10 times a month, lasting for more than 3 months, often causing patients to wake up from sleep. After waking up with pain, the headache lasts for more than 15 minutes, which can last for up to 4 hours; it usually manifests as mild or moderate pain, and one in five patients has severe headaches. The difference between the former is that female patients dominate, and the ratio between women and men is 1.7:1; the onset age is too old, often after the age of 50; the pain is mostly bilateral rather than unilateral; the lack of autonomic neurological symptoms; and sleep headaches occur during strict sleep, while CH can also occur during the day; in terms of treatment, the similarity with CH is that sleep headaches are also effective for the treatment of lithium carbonate, but taking caffeine , indomethacin and flucinrizine hydrochloride before going to bed may be effective, which is the difference from CH. The treatment of
for treating
CH is divided into three types: acute phase treatment, preventive treatment and transitional treatment. In recent years, some new drugs and neuroregulatory technologies have also been gradually used in the treatment of CH.
(I) Acute phase treatment
1. Treatment purpose: quickly relieve headaches and terminate acute phase headache attacks as soon as possible.
2. Commonly used criteria for evaluating the effectiveness of treatment: ① painless within 15 minutes; ② The degree of headache within 30 minutes (converted from moderate to severe or extremely severe pain to mild or non-pain); ③ Pain improvement lasts for 60 minutes; ④ No need to take medicine again within 15 minutes of treatment.
3. Recommended and evaluated in acute phase (see Table 2).
Table 2 Recommended treatment for acute CH attacks in adults
(II) Prophylactic treatment
1. Treatment purpose: The purpose of prophylactic treatment is to reduce the frequency of headache attacks during the cluster period, reduce the degree of attacks, and improve the efficacy of acute treatment.
2. Effectiveness indicators of preventive treatment: ① The frequency of headache attacks decreased during the cluster period; ② The duration of headache decreased; ③ The degree of headache was reduced and the response to acute treatment was better.
3. Indications for preventive treatment
Preventive treatment should be considered when CH causes the patient to experience the following situations: ① The patient's quality of life, work or academic performance is seriously damaged (based on the patient's own judgment); ② Frequent headaches occur during the cluster period; ③ The acute phase of drug treatment is poor or the patient cannot tolerate it.
4. Prophylactic therapeutic drug
(1) Verapamil : is currently considered a first-line therapeutic drug for preventive treatment of CH. Studies have shown that verapamil 360mg can effectively reduce the frequency of daily attacks, with the maximum therapeutic dose of 960mg per day. The dosing time of verapamil should be 1.5 times the previous cluster period, and the best efficacy can be achieved 2 to 3 weeks after the medication. The incidence of heart block caused by verapamil is relatively high. Electrocardiogram should be performed before and after increasing the dose during treatment, and heart rate and blood pressure should be closely monitored during medication.
(2) Lithium salt : For patients who fail to treat verapamil, cannot obtain verapamil, or cannot use verapamil due to adverse reactions, lithium salt can be used as a second-line drug for preventive treatment. However, long-term use can lead to renal insufficiency and hypothyroidism.
(3) Melatonin: The correlation between hypothalamus and CH and the circadian rhythm of CH onset both support the feasibility of melatonin treatment.
(4) Other drugs: topiramate has insufficient evidence for preventive treatment of CH and is only used when verapamil or lithium salt treatment fails or there is no medicine. Common adverse reactions include cognitive impairment , sensory abnormalities, speech disorders, etc., and are prohibited for kidney stones patients. Other drugs include warfarin, dimethyl ergotrine and sodium hydroxybutyrate .
(III) Transitional treatment
1. Purpose and indications of transitional treatment: Since preventive treatment drugs require a certain amount of time and drug dosage to effectively play a therapeutic role, for patients with high-frequency attacks with a frequency of ≥2 times a day, transitional treatment can be used when the preventive drugs are started or the dose is increased, and the treatment cycle usually lasts no more than 2 weeks.
2. Effectiveness indicators of transitional treatment: ①Frequency of CH; ②Duration of headache; ③Degree of headache; ④Number of medication used in the acute stage of attack; ⑤Cluster period time.
3. Drug evaluation and recommendation for transitional treatment (see Table 3).
Table 3 Recommended CH preventive and transitional treatment
(IV) Neurological regulation treatment
1. Treatment purpose: When is ineffective for drug treatment, or is intolerant to conventional treatment, non-invasive or invasive neuroregulatory treatment can be used to reduce the serious adverse effects of headache on patients and disability.
2. Commonly used treatment methods for nerve regulation: ① Sphinopal palatine ganglion radiofrequency ablation; sphinopal palatine ganglion stimulation; ③ Non-invasive vagus nerve stimulation; ④ Invasive occipital nerve stimulation; ⑤ Deep hypothalamic stimulation.
(V) Related studies of the new therapeutic drug
show that CGRP monoclonal antibody and diethylamine lysergic acid have certain efficacy in the preventive treatment of CH, but more evidence is still needed for long-term efficacy and safety. The long-term outcomes of patients with
prognosis
eCH and cCH differed. eCH and cCH can convert each other. If the control is poor, eCH is usually easily converted to cCH, while cCH can convert to eCH with a better prognosis under standardized management. Studies have shown that late onset age, male, and disease course of more than 20 years may be key factors affecting the prognosis of CH.
Summary
In view of the high disability and high disease burden of CH, it is necessary to form a standardized diagnosis and treatment system based on its pathophysiology and disease characteristics, and improve its clinical management quality through diagnosis, differential diagnosis and treatment.However, since cluster headaches are relatively rare, their clinical evidence is still relatively lacking or insufficient, and some recommendations are weakly recommended. Therefore, when using this guide, clinicians need to make comprehensive judgments based on the hospital environment and specific patient conditions, such as the severity of the disease, the patient's willingness to treat, the patient's response to drugs, and the progress of the disease.
Yimaitong is compiled from: Neurologists Branch of the Chinese Medical Association, Chinese Research Hospital Association (CRHA, Chinese Research Hospital Association). "Guidelines for the Diagnosis and Treatment of Cluster Headaches in China". Chinese Journal of Pain Medicine. 2022, 28(9):641-653.
