ICD-10 CODE R51
Clinical Syndrome
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 ( Table 3.1 ). 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 ( Fig. 3.1 ). 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 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 |
Signs and Symptoms
During attacks of chronic paroxysmal hemicrania, patients exhibit the following physical findings suggestive of Horner syndrome on the ipsilateral side of the pain:
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Conjunctival and scleral injection
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Lacrimation
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Nasal congestion
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Rhinorrhea
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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.
Testing
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 ( Fig. 3.2 ). 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.
