Chronic paroxysmal hemicrania

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.

Related Pages

• Cluster headache
• Trigeminal neuralgia
• SUNCT syndrome
• Indomethacin