Chronic paroxysmal hemicrania

Editor-In-Chief: Prab R Tumpati, MD
Obesity, Sleep & Internal medicine
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Chronic paroxysmal hemicrania
Synonyms	N/A
Pronounce	N/A
Specialty	N/A
Symptoms	Severe unilateral headache, lacrimation, rhinorrhea, ptosis, miosis
Complications	N/A
Onset	Typically in adulthood
Duration	2 to 30 minutes per attack
Types	N/A
Causes	Unknown, possibly related to trigeminal autonomic cephalalgias
Risks	More common in females
Diagnosis	Clinical evaluation, response to indomethacin
Differential diagnosis	Cluster headache, migraine, SUNCT syndrome
Prevention	N/A
Treatment	Indomethacin
Medication	Indomethacin
Prognosis	Good with treatment
Frequency	Rare
Deaths	None directly attributed

Chronic paroxysmal hemicrania (CPH) is a rare type of headache disorder that is characterized by frequent, short-lasting attacks of severe unilateral pain. It is classified under trigeminal autonomic cephalalgias, a group of primary headache disorders. The condition is notable for its excellent response to the non-steroidal anti-inflammatory drug indomethacin. The pathophysiology of CPH is not fully understood, but it is believed to involve the trigeminal nerve and autonomic nervous system. The facial nerve may also play a role, as depicted in the image provided. Diagnosis is primarily clinical, based on the characteristic symptoms and the patient's response to indomethacin. Differential diagnosis includes other headache disorders such as cluster headache and migraine. CPH is more common in females and typically begins in adulthood. The prognosis is generally good with appropriate treatment, and the condition is considered rare.== Chronic Paroxysmal Hemicrania ==

Diagram of the cranial nerves, including the facial nerve (VII) which may be involved in headache disorders.

Chronic Paroxysmal Hemicrania (CPH) is a rare primary headache disorder characterized by frequent, short-lasting, and severe unilateral headaches. It is classified under the group of trigeminal autonomic cephalalgias (TACs), which also includes cluster headaches and SUNCT syndrome.

Clinical Features

CPH is marked by attacks of severe pain that are strictly one-sided, typically around the eye, temple, or forehead. These attacks last from 2 to 30 minutes and can occur more than five times a day. The pain is often accompanied by autonomic symptoms such as conjunctival injection, lacrimation, nasal congestion, or rhinorrhea on the affected side.

Pathophysiology

The exact pathophysiology of CPH is not fully understood, but it is believed to involve dysfunction of the hypothalamus and the trigeminal nerve system. The involvement of the facial nerve (cranial nerve VII) is also considered due to the autonomic symptoms observed during attacks.

Diagnosis

Diagnosis of CPH is primarily clinical, based on the characteristic pattern of headache attacks and response to treatment. It is important to differentiate CPH from other TACs and secondary causes of headache. Neuroimaging may be performed to rule out structural lesions.

Treatment

CPH is uniquely responsive to indomethacin, a nonsteroidal anti-inflammatory drug (NSAID). Indomethacin provides complete relief of symptoms in most patients, which is a key diagnostic criterion. Other treatments, such as verapamil or topiramate, are generally ineffective.

Prognosis

With appropriate treatment, the prognosis for individuals with CPH is excellent. Indomethacin effectively controls the attacks, allowing patients to lead normal lives. However, long-term use of indomethacin may be associated with gastrointestinal side effects, necessitating regular monitoring.

See Also

• Cluster headache
• Trigeminal neuralgia
• SUNCT syndrome
• Indomethacin