There are too many classifications of headaches, hard to remember!

*Only for medical professionals to read for reference.
This article gives a systematic summary of painful cranial neuropathy and other facial pain and other types of headaches.

Clinically, there are many patients with headache as the main complaint.
We must be familiar with the classification and disease spectrum of headache, so that we can consider possible diseases as soon as possible after understanding the patient’s medical history and physical examination, and select appropriate auxiliary examinations to verify the diagnosis.
, Give a reasonable treatment plan.

Earlier we have released: ●The most comprehensive mind map of "Headache Diagnosis and Treatment", hurry up and collect it! ●The most comprehensive mind map of "primary headache", hurry up and collect it! ●Super-full mind map for 4 types of secondary headaches! This is how Daniel remembered it ● 4 mind maps to clear away the knowledge points of secondary headaches, they are worth collecting! The mind map of the third type of headache (painful cranial neuropathy and other facial pain and other types of headache) in the "2018 International Headache Classification Third Edition" is now released.
It is also the final chapter of the classification.
It is not easy to organize.
If you feel the content Useful, please save, favorite, like, share! 1.
Painful cranial neuropathy Painful cranial neuropathy refers to the afferent nerves of the trigeminal nerve, intermediate nerve, glossopharyngeal nerve and vagus nerve, as well as the occipital nerve, which transmits painful stimuli to the central pathway of the brainstem and processes the head The brain area where the neck is nociceptive and painful, the brain can feel the pain in the innervated area.

Pain can take any form.

Neurofacial pain can be classified according to different clinical features and etiology.
According to the syndrome, it can be divided into neuralgia (pain in the distribution area of ​​single or multiple nerves) and neuropathy (abnormal changes in the function or pathology of one or more nerves) ; According to location, it can be divided into central neuropathic pain (pain caused by injury or disease of the central somatosensory nervous system) and peripheral neuropathic pain (pain caused by injury or disease of the peripheral somatosensory nervous system); according to etiology, it is divided into classic (Neurovascular compression), idiopathic (no clear cause found), or secondary (with clear cause).

(Classic neuralgia is caused by secondary neurovascular compression, but because of its wider treatment options and due to different neuropathophysiology, it is separate from other causes).

▌ Pain due to trigeminal nerve injury or pathology Trigeminal neuralgia refers to a disease characterized by repeated, unilateral, transient, and electric shock-like pain, which suddenly stops and is confined to the distribution of one or more branches of the trigeminal nerve , Can be induced by benign stimuli.

It may have no obvious cause or be caused by another disease with a well-diagnosed diagnosis.

In addition, it may be accompanied by persistent, moderate-intensity facial pain.

According to different causes, trigeminal neuralgia is divided into classic trigeminal neuralgia and painful trigeminal neuropathy.

Classical trigeminal neuralgia refers to trigeminal neuralgia caused by neurovascular compression.
According to whether there is facial pain between episodes, it is divided into pure paroxysmal and persistent facial pain.

Persistent facial pain may be caused by peripheral or central sensitization.

Neurovascular compression is mainly located in nerve roots.
Compared with venous compression, arterial compression is more related to clinical symptoms.

Secondary trigeminal neuralgia is often secondary to multiple sclerosis (MS), cerebellopontine angle tumors, arteriovenous malformations, connective tissue diseases, skull malformations, dural arteriovenous fistulas, and hereditary neuropathy or nerve hyperexcitability.

According to statistics, 2%-5% of MS patients have trigeminal neuralgia, and the pain in such patients can sometimes exist on both sides.

Trigeminal neuralgia without obvious abnormalities in electrophysiological examination and MRI is idiopathic trigeminal neuralgia.
According to whether there is facial pain during the interictal period, it is divided into pure paroxysmal and persistent facial pain.

Painful trigeminal neuropathy is caused by another disease and indicative nerve damage.
Facial pain in the distribution area of ​​single or multiple branches of the trigeminal nerve.

Common diseases are caused by acute herpes zoster virus infection, herpes sequelae, trauma, MS, space-occupying diseases or systemic diseases.

The main pain is usually continuous or nearly continuous, and is usually described as a burning pain, squeezing pain, or a stinging pain.

In the distribution area of ​​the trigeminal nerve, there may be anesthesia on the neurological examination, and mechanical hyperalgesia and cold hyperalgesia are common.
The hyperalgesic area is much larger than the punctate trigger area in trigeminal neuralgia.

▌ Pain due to glossopharyngeal nerve injury or pathology The second common type of painful cranial neuropathy is pain due to glossopharyngeal nerve injury or pathology, and is divided into glossopharyngeal neuralgia and painful glossopharyngeal neuropathy.

Glossopharyngeal neuralgia refers to the disease in the distribution area of ​​the glossopharyngeal nerve and the ear and pharyngeal branches of the vagus nerve with unilateral short-term tingling and sudden stop as the sign.
It is in the ear, tongue base, tonsil fossa and/or Pain under the angle of the mandible is usually induced when swallowing, talking, or coughing, and can be relieved and recurred in the form of trigeminal neuralgia.

Glossopharyngeal neuralgia and trigeminal neuralgia can occur at the same time.

Before the onset of glossopharyngeal neuralgia, the affected area can experience discomfort several weeks or even months before the onset of the disease.
Glossopharyngeal neuralgia may radiate to the eyes, nose, chin or shoulders, and even cause weight loss.
In rare cases, the pain may be Accompanied by vagus nerve symptoms, such as cough, hoarseness, syncope, and/or bradycardia.

The classic glossopharyngeal neuralgia can be found by MRI or surgery that the glossopharyngeal nerve root is compressed by blood vessels.

Continuous glossopharyngeal neuralgia is caused by neck trauma, MS, tonsils or local tumors, cerebellopontine angle tumors, and Arnold-Chiari malformations.

Painful glossopharyngeal neuropathy refers to pain in the glossopharyngeal nerve distribution area (back of the tongue, tonsil fossa, pharynx, and/or under the mandibular angle), and pain is usually felt in the ipsilateral ear.

Primary pain is often continuous or nearly continuous, and is usually a burning or pressure sensation, or a tingling sensation.

There may be sensory loss in the back of the tongue and tonsil fossa on the same side, and the pharyngeal reflex may be weakened or disappeared.

Cerebellopontine angle tumors and iatrogenic injuries during surgery can cause painful glossopharyngeal neuropathy.

▌ Pain due to intermediate nerve injury or disease Pain due to intermediate nerve injury or disease is divided into intermediate neuralgia and painful intermediate neuropathy.

Intermediate neuralgia refers to a rare disease characterized by short-term paroxysmal pain in the deep part of the external auditory canal, sometimes radiating to the parietal-occipital region.

In most cases, vascular compression can be found during surgery, and sometimes thickened arachnoid membranes can be seen, but there can be no clear cause, or a complication of herpes zoster, or a complication of MS or tumors (very rare) .

Stimulation of the posterior wall of the external auditory meatus and/or the area around the auricle can be induced.

Painful interneuropathy refers to pain that occurs in the distribution area of ​​the intermediate nerve (external auditory canal, auricle, or mastoid area), and is usually described by patients as continuous or near-continuous dull pain in the deep part of the external auditory canal.

Temporary, paroxysmal pain may be superimposed, but this is not the main type of pain.

There may be sensory loss in the skin of the external auditory canal, auricle, or mastoid area, usually very mild.

Mostly caused by herpes zoster virus infection, facial tumors or geniculate ganglion injury.

▌ Occipital neuralgia Occipital neuralgia occurs in the back of the scalp, unilateral or bilateral paroxysmal tear-like or needle-like pain in the distribution area of ​​the major occipital, minor occipital, and/or third occipital nerve, sometimes accompanied by the affected area Loss of sensation or dullness of sensation, usually accompanied by tenderness of the affected nerve.

The pain caused by occipital neuralgia can radiate to the frontal and orbital regions through the connection of the trigeminal nerve and cervical nerve interneurons in the nucleus of the trigeminal nerve spinal tract.

It must be distinguished from occipital radiating pain caused by atlantoaxial joints or upper neck articular process, and occipital radiating pain induced by neck muscle tenderness.

2.
Other facial pain Other facial pain includes cervical tongue syndrome, painful optic neuritis, headache due to ischemic ophthalmic nerve palsy, Tolosa-Hunt syndrome, trigeminal sympathetic-ocular sympathetic syndrome (Reader's syndrome) ), recurrent painful ophthalmoplegia neuropathy, burning mouth syndrome (BMS), persistent idiopathic facial pain (PIFP), and central neuropathic pain due to MS and post-stroke central pain (CPSP).

Neck tongue syndrome refers to a sharp, sharp tingling in the occiput and/or upper neck on one side due to a sudden head rotation, and abnormal sensation and/or posture in the ipsilateral tongue.

Painful optic neuritis refers to pain in the back of one or both eyes caused by demyelination of the optic nerve, accompanied by central visual disturbance.

Headache due to ischemic ophthalmic artery nerve palsy refers to unilateral forehead and/or periorbital pain caused by ischemic palsy of the ipsilateral III, IV, and/or VI cranial nerve, accompanied by other symptoms and signs.
Such as diplopia.

Tolosa-Hunt syndrome refers to unilateral orbital or periorbital pain, accompanied by cavernous sinus, superior orbital fissure, or orbital inflammatory granuloma caused by the third cranial nerve, one of the fourth and (or) sixth cranial nerves Or multiple cranial nerve palsy.

Hormone therapy is effective.

Trigeminal nerve sympathetic-ocular sympathetic syndrome (Reader's syndrome) refers to persistent unilateral pain located in the distribution area of ​​the trigeminal branch of the eye, sometimes extending to the distribution area of ​​the maxillary branch, accompanied by ipsilateral Horner's syndrome (Horner's syndrome).

Caused by lesions located in the middle cranial fossa or carotid artery.

Recurrent painful ophthalmoplegia neuropathy refers to the recurrent paralysis of one or more cranial nerves (usually the third cranial nerve) innervating the eye muscles, accompanied by ipsilateral headaches.

Burning Mouth Syndrome (BMS) refers to a burning sensation or perversion in the mouth, at least 2 hours a day, repeated attacks for more than 3 months, and no obvious clinical lesions have been found.

The prevalence of menopausal women is high, and some studies suggest that they are comorbid with psychosocial and mental disorders.

Recent laboratory and cranial imaging examinations have found changes in the central and peripheral nervous system.

Persistent idiopathic facial pain (PIFP) refers to persistent facial pain and/or oral pain.
The clinical manifestations are diverse, but they are still recurrent, exceeding 2 hours a day for more than 3 months, and there is no clinical evidence of neurological damage.

Central neuropathic pain refers to unilateral or bilateral head and neck pain, with various manifestations, with or without sensory changes.

Depending on the cause, the pain may be persistent, or gradually relieved and recurring.

Divided into central neuropathic pain due to MS and CPSP.

3.
Other types of headaches.
There are some headaches that do not fit into any existing headache classification because they are described for the first time or simply because there is not enough available data to distinguish the types of headaches.

Uncharacteristic headaches can only be used when information is not available, such as the death of the patient, the inability to communicate, or the inability to obtain useful information.

The following is the full version of the mind map for the third part of painful cranial neuropathy and other facial pain and other types of headaches.

At this point, the 2018 International Headache Classification Standards have all been sorted out.

The key is to learn how to use this classification, instead of memorizing the classification standards from beginning to end, it should be used as a tool that can be reviewed repeatedly.

In clinical practice, there is no need to use classification criteria for obvious migraine or tension-type headache, but it can be used to help diagnose when the diagnosis is not clear.

When doing headache research, this classification standard is indispensable: for any research, whether it is a drug clinical trial, pathophysiology, or biochemical research, each patient in the group must meet the diagnostic criteria of the disease.

This classification standard is hierarchical and should be used to specify how specific diagnosis is needed.

There are 5 layers in total.

First, you should make a rough assessment of the classification of headaches.

For example, is it migraine or tension-type headache, or trigeminal autonomic headache? Then make a more specific diagnosis based on the information obtained.

The degree of specificity depends on the purpose of the diagnosis.

If it is general medicine, the first and second level diagnosis is sufficient.

If it is a headache specialist medical treatment or a headache center, the fourth and fifth levels of diagnosis are more appropriate.

When it is suspected that a patient has multiple headache types or subtypes, it is strongly recommended that the patient use a diagnostic headache diary to record the headache characteristics of each attack.

Studies have shown that headache diaries can improve the accuracy of diagnosis and make more accurate judgments about the therapeutic effects of drugs.

In addition, the headache diary also helps to count the number of attacks of two or more headache types or subtypes.

In clinical practice, more than 90% of headaches are primary headaches.

Secondary headaches account for less than 10% of clinical headaches, and the classification is complicated.
It is difficult to master it.
It is necessary to promptly identify secondary headaches caused by critical illness and keep in mind the early warning symptoms of headaches.

It can also identify pain-relieving cranial neuropathy and other facial pains as well as other types of headaches.

After this period of study, I believe that everyone has an overall understanding of the classification of headaches.
In the later content, we will introduce in detail the diagnostic criteria and treatment options for several common headaches, so stay tuned! References: [1] Headache Classification Committee of the International Headache Society (IHS), The International Classification of Headache Disorders, 3rd edition, Cephalalgia.
2018 Jan;38(1):1-211.
[2] Yu Shengyuan, International Headache Classification Third Edition (beta version) Chinese version,