Cluster Headache

台北榮總神經內科 傅中玲醫師 

Cluster headaches are among the most painful of all headaches. The signature is a pattern of periodic cycles of headache attacks, which may be one of the following: Episodic (occurring regularly for weeks to months, followed by long pain-free periods), or Chronic (occurring without sustained breaks).

Cluster symptoms tend to occur during spring, autumn or both and they most often occur at night. Over half of cluster headache patients experience warning symptoms, long before an attack. They are called prodromal, or premonitory, symptoms. They include the following: strange tingling sensations around the eye, nose, or neck, nasal congestion or runny nose, excessive tearing, feeling restless or depressed. When the actual attack occurs, symptoms typically escalate rapidly (within about 15 minutes) to intense levels. The pain is typically described as severe, stabbing or boring. It is virtually always on one side, although it may occur on the opposite side in other attacks or even within the same attack. Pain does not worsen with movement (as it often does with migraine headaches). Other symptoms include the following: excessive tearing, feelings of intense restlessness and agitation, facial sweating, and nausea.

 Headache attacks tend to occur with great regularity at the same time of day. About 75% occur between 9 at night and 10 in the morning. Peaks have also been reported between 1 PM and 3 PM. A single cluster attack is usually brief but extremely painful, lasting about one to three hours. During an active cycle, sufferers can experience these attacks as infrequently as one every other day to several attacks a day. Cycles of such daily or near daily attacks typically occur over the course of a week to a year--most often in spring and autumn. Usually a patient has one or two cycles per year that each last one to three months.

 Evidence now strongly suggests that abnormalities in the hypothalamus, a complex structure located deep in the brain, may play a major role in cluster headaches. Advanced imaging techniques have revealed that a specific area in the hypothalamus is asymmetrical in these patients and is activated during a cluster headache attack. The most important nervous cluster is the suprachiasmatic nuclei (SCN), which appears to help coordinate the body's activities (sleep/wake) with the environment (dark/light). Some studies support the idea that some failure in this biologic pacemaker may impair the pain control system and cause these terrible attacks.

 Lifestyle factors, including smoking, alcohol abuse, and stress (in particular stressful work situations), appear to play a very strong role in this headache. Alcohol, in fact, can trigger an attack. A particularly high association exists between smoking and cluster headaches. Quitting smoking, however, is not associated with any fewer or less severe attacks.

 The most effective treatments for a cluster attack are the following: oxygen inhalation and triptan drugs (injections of sumatriptan). Relief can occur in five to 10 minutes. Prevention of attacks during a cluster cycle is extremely important. The following are the most commonly used preventive agents: calcium-channel blockers (most often used for preventing cluster headaches), corticosteroids, lithium and antiepileptic medications.

 Surgical intervention may be considered for patients with chronic cluster headaches that do not respond to treatments at all or when they have not gone into remission for at least a year. To date, surgery has limited success and can have distressing side effects. Deep brain electrical stimulation is showing promise, however.  

Pathophysiology of Tension-type Headache

新光醫院神經科 陳威宏醫師 

Tension type headache (TTH) is the most frequent primary headache, with a lifetime prevalence of 74% in general population. Despite its tremendous socioeconomic impact in modern society little is known about the underlying pathophysiology and treatment. For decades, it has been debated if the pain in TTH originates from myofascial tissues or from central mechanisms in the brain. The increased tenderness in TTH represents the activation of peripheral nociceptors.  Decreased pain, thermal and electrical thresholds have been reported in chronic TTH patients, which probably represents a central misinterpretation of the incoming signals. Nitric oxide (NO) might play a role in the pathophysiology of chronic TTH. It has been demonstrated that NO synthetase inhibitor reduces headache and muscle hardness, whereas the NO donor glycerol trinitrate causes headache in patients with chronic TTH. Myofascial factors and peripheral sensitization of nociceptors play an important role in the episodic form, and central sensitization has been demonstrated in the chronic form. As chronic tension-type headache usually evolves from the episodic form, prevention and reversal of this central sensitization may be an important target for future pathophysiological studies and drug development. 

Nonpharmacologic Treatments for Primary Chronic Headache

高雄長庚神經內科  李連輝醫師 

As the mechanisms of chronic headache are so complicated with multiple factors. Edogeneous and exogeneous factors, environmental and genetic predisportions. So the treatment of chronic headache need to be multyiplicity: acute and prophylactic with pharmacologic and nonpharmacologic treatments.

I.  Classification and causes of primary chronic hedacahe

II. Pathogenesis of chronic headache(multiple factors)

III.Treatments of Chronic headache: 1)Acute treatment 2) Prophylactics treatments 3)  Pharmacologic treatments 4) Nonpharmacologic treatments

IV.Nonpharmacologic treamnents:

  1. Migraine:

1)      Common provacational triggers for migraine

2)      Evidence- based guideline of nonpharmacologic treatments in migraine

       2. Tension headache:

1)      Conventional nonpharmacologic treatments: Psychologic, physical treatments

2)      Unconventional nonpharmacologic treatments: Local therapy, manipulation of the neck, acupuncture, hypnosis, others

V.  Demonstration of nonpharmacologic treatments(digital)

1)      Biofeedback 2) Relaxation 3) Walking 4) Jogging 5) Taichi chikon(with tree)

VI.  Strategy of chronic headache treatments in future

1)      Pharmacoliogic and nonpharnacologic treatments

2)      Team work for treat chronic headache patients

3)      Research

Migrainous Infarction

台北榮總神經內科 國立陽明大學醫學院醫學系神經科 王署君醫師 

The diagnosis of migrainous infarction is based on the association of the abrupt onset of a neurologic deficit during a migraine attack with evidence of cerebral infarction on neuroimaging. Other causes of stroke must be excluded. Strict criteria for the diagnosis of migrainous infarction must be applied because migraine is common and patients with migraine may suffer from other causes of stroke. The diagnosis of migrainous infarction should be made only when patients with an established history of migraine suffer a cerebral infarction during a typical migraine attack (Rothrock et al 1988).

The diagnostic criteria of migrainous infarction were just revised by the International Classification of Headache Disorders, second edition, as follows (coded as 1.5.4): (1) the present attack in a patient with 1.2 migraine with aura is typical of previous attacks except that 1 or more aura symptoms persists for more than 60 minutes; (2) neuroimaging demonstrates ischemic infarction in a relevant area; (3) the headache is not attributable to another disorder (Headache Classification Subcommittee of the International Headache Society, 2004).

The exact cause of migrainous infarction is still not certain. Based on studies using cerebral angiography during attacks, the most important underlying mechanism for the stroke is believed to be carotid or vertebral arterial spasm resulting in a critical degree of cerebral hypoperfusion (Featherstone 1986; Rothrock et al 1988; Sanin and Mathew 1993). However, this may not always be true, so other factors should be considered. The incidence of migrainous infarction is rare, according to the strict diagnosis proposed by International Headache Society. Henrich and colleagues reported that the incidence rate of first migrainous infarction was 3.36 per 100,000 per year (Henrich et al 1986). However, in the absence of other stroke risk factors, this estimate was reduced to 1.44 per 100,000 people per year. In a recent study of the Barcelona Stroke Registry, Arboix and colleagues reported that the group of patients with migrainous infarction accounted for only 0.6% of all first-ever acute strokes, 0.8% of ischemic strokes, 12.8% of ischemic strokes of unusual etiology, and 13.7% of ischemic strokes in young adults 45 years of age or younger (Arboix et al 2003). Because distinct diagnostic criteria are lacking, migrainous infarction should be considered a diagnosis by exclusion.

The diagnosis demands a well established history of migraine and exclusion of other conditions that cause stroke. Computed tomography, MRI (including diffusion-weighted image, perfusion-weighted image, magnetic resonance spectroscopy, and MRA), cerebral arteriography, transcranial Doppler evaluations, and transesophageal echocardiography evaluations should be performed when possible. Lumbar puncture and blood studies to help exclude vasculitis should also be carried out. Prophylactic treatment of migrainous infarction in patients with prolonged aura includes platelet antiaggregants or calcium channel blockers. Aspirin administration should be considered as a prophylactic treatment against migrainous infarction. Patients who have anticardiolipin syndrome may need anticoagulants. Calcium channel blockers are recommended for patients at risk for migrainous infarction. Nimodipine reduces cerebral vasoconstriction, and several studies have demonstrated the potential effectiveness of calcium channel blockers in the prophylactic treatment of migraine headaches (Meyer 1985).Ergotamine, triptans, and serotonergic medications may initiate or worsen intracranial vasospasm and dysautoregulation in patients with migrainous infarction. These drugs should be withheld in patients with prolonged aura. Peripheral beta-blockers, such as propranolol, should also be withheld, since they may worsen intracranial vasoconstriction.

In general, the long-term prognosis in patients with migrainous infarction is good. Milhaud and colleagues, in a prospective stroke registry, found that the outcome at 1 month was favorable in more than 70% of migraineurs with ischemic stroke (Milhaud et al 2001). Arboix and colleagues reported the mean length of hospital stay of 9 consecutive patients with migrainous infarction was 9.75±6.2 days. No patients died during hospital stay, and 67% were symptom-free at discharge (Arboix et al 2003).

 Cases  Demonstration

活水神經內科診所  王博仁醫師 

The classification and diagnostic criteria of Migraine with aura, Persistent aura without infarction and Migrainous infarction have some changes in ICHD-II. There are 6 subforms of Migraine with aura. The identification of the symptom of motor weakness is the first step in their differentiation. The past history of Migraine with aura is essential in diagnosis of Persistent aura without infarction and Migrainous infarction. Similar aura, but lasting more than one week, is mandatory in Persistent aura without infarction. On the other hand, when the similar aura symptom persisted more than 60 minutes with a relevant positive finding in neuroimaging, then we can therefore consider the diagnosis of Migrainous infarction. Clinical cases were demonstrated for the audience to practice these criteria.