Chronic Paroxysmal Hemicrania

Chronic Paroxysmal Hemicrania

Chronic paroxysmal hemicrania, which is also known as Sjaastad syndrome, shares many characteristics of its more common analogue, cluster headache, but has several important differences.

Similar to cluster headache, chronic paroxysmal hemicrania is a severe, episodic, unilateral headache that affects the periorbital and retroorbital regions. In contrast to cluster headache, which occurs 10 times more commonly in men, chronic paroxysmal hemicrania occurs primarily in women.

The duration of pain associated with chronic paroxysmal hemicrania is shorter than that of cluster headache, lasting 5 to 45 minutes. This pain does not follow the chronobiological pattern seen in patients with cluster headache.

Patients with chronic paroxysmal hemicrania usually experience more than five attacks per day. Chronic paroxysmal hemicrania uniformly responds to indomethacin, whereas cluster headache does not.

Comparison of Cluster Headache and Chronic Paroxysmal Hemicrania

Comparison Factors	Cluster Headache	Chronic Paroxysmal Hemicrania
Gender predominance	Male	Female
Response to indomethacin	Negative	Positive
Chronobiological pattern	Positive	Negative
Alcohol trigger	Positive	Negative
Length of attacks	Longer	Shorter
Horner syndrome	Present	Present

What are the Symptoms of Chronic Paroxysmal Hemicrania

During attacks of chronic paroxysmal hemicrania, patients exhibit the following physical findings suggestive of Horner syndrome on the ipsilateral side of the pain:

• • Conjunctival and scleral injection
• • Lacrimation
• • Nasal congestion
• • Rhinorrhea
• • Ptosis of the eyelid

As in cluster headache, the patient may become agitated during attacks, rather than seeking dark and quiet as does the patient with migraine. In contrast to cluster headache, alcohol consumption does not seem to trigger attacks of chronic paroxysmal hemicrania.

Between attacks, the neurological examination of a patient with chronic paroxysmal hemicrania should be normal.

How is Chronic Paroxysmal Hemicrania diagnosed?

Magnetic resonance imaging (MRI) of the brain provides the best information regarding the cranial vault and its contents.

MRI is highly accurate and helps to identify abnormalities that may put the patient at risk for neurological disasters secondary to intracranial and brainstem pathological conditions, including tumors and demyelinating disease.

Magnetic resonance angiography (MRA) also may be useful in identifying aneurysms, which may be responsible for the patient’s neurological findings. In patients who cannot undergo MRI, such as a patient with a pacemaker, computed tomography (CT) is a reasonable second choice.

Radionuclide bone scanning and plain radiography are indicated if fracture or bony abnormality such as metastatic disease is considered in the differential diagnosis.

Screening laboratory tests consisting of complete blood cell count, erythrocyte sedimentation rate, and automated blood chemistry testing should be performed if the diagnosis of chronic paroxysmal hemicrania is in question. Intraocular pressure should be measured if glaucoma is suspected.

Differential Diagnosis

Chronic paroxysmal hemicrania is a clinical diagnosis supported by a combination of clinical history, abnormal physical examination during attacks, radiography, and MRI.

Pain syndromes that may mimic chronic paroxysmal hemicrania include cluster headache, trigeminal neuralgia involving the first division of the trigeminal nerve, demyelinating disease, and ice pick headache. Trigeminal neuralgia involving the first division of the trigeminal nerve is uncommon and is characterized by trigger areas and tic-like movements.

Demyelinating disease is generally associated with other neurological findings, including optic neuritis and other motor and sensory abnormalities.

The pain of cluster headache lasts much longer than the pain of chronic paroxysmal hemicrania, and cluster headache has a male predominance, a chronobiological pattern of attacks, and a lack of response to treatment with indomethacin.

Treatment

Chronic paroxysmal hemicrania uniformly responds to treatment with indomethacin. Failure to respond to indomethacin puts the diagnosis of chronic paroxysmal hemicrania in question.

A starting dose of 25 mg daily for 2 days and titrating to 25 mg three times per day is a reasonable treatment approach. This dose may be carefully increased up to 150 mg per day. Indomethacin must be used carefully, if at all, in patients with peptic ulcer disease or impaired renal function.

Anecdotal reports of a positive response to cyclooxygenase-2 (COX-2) inhibitors in the treatment of chronic paroxysmal hemicrania have been noted in the headache literature. Underlying sleep disturbance and depression are best treated with a tricyclic antidepressant compound, such as nortriptyline, which can be started at a single bedtime dose of 25 mg.

Complications

Failure to diagnose chronic paroxysmal hemicrania correctly may put the patient at risk if intracranial pathological conditions or demyelinating disease that may mimic the clinical presentation of chronic paroxysmal hemicrania is overlooked.

MRI is indicated in all patients thought to have chronic paroxysmal hemicrania. Failure to diagnose glaucoma, which may cause intermittent ocular pain, may result in permanent loss of sight.

Clinical Pearls

Chronic paroxysmal hemicrania is classified as a trigeminal autonomic cephalgia. The trigeminal autonomic cephalgias are a group of distinct headache syndromes that share a number of common and often overlapping clinical characteristics.

The diagnosis of chronic paroxysmal hemicrania is made by obtaining a thorough, targeted headache history. Between attacks, patients with chronic paroxysmal hemicrania should have a normal neurological examination.

If the neurological examination is abnormal between attacks, the diagnosis of chronic paroxysmal hemicrania should be discarded and a careful search for the cause of the patient’s neurological findings should be undertaken.

Characteristics of the Trigeminal Autonomic Cephalgias

• Unilateral

• Short duration

• High frequency

• Orbital, periorbital, or temporal

• Associated autonomic symptoms

• Lacrimation

• Conjunctival injection

• Nasal congestion

• Ptosis

• Eyelid edema

Characteristics of the Trigeminal Autonomic Cephalgias

• Unilateral

• Short duration

• High frequency

• Orbital, periorbital, or temporal

• Associated autonomic symptoms

• Lacrimation

• Conjunctival injection

• Nasal congestion

• Ptosis

• Eyelid edema