Review Article. Trigeminal Autonomic Cephalalgias. Peter J. Goadsby, MD, PhD.

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Review Article. Trigeminal Autonomic Cephalalgias. Peter J. Goadsby, MD, PhD. Purpose of Review: This article covers the clinical manifestations and differential diagnosis of the trigeminal autonomic cephalalgias TACs.

Recent Findings: TACs comprise a subgroup of primary headache disorders pres- enting with lateralized, often severe, pain accompanied by cranial autonomic fea- tures. Lateralization of symptoms and signs is the key feature differentiating the TACs and migraine. When diagnosing a TAC, it is appropriate to consider underlying pituitary or pituitary region pathology. Cluster headache responds to oxygen and parenteral triptans, with verapamil having the most success for prevention.

Paroxysmal hemicrania responds to indomethacin. Hemicrania continua re- sponds to indomethacin. Summary: TACs are a unique group of primary headache syndromes with indi- vidual features and specific responses to treatment that make their identification crucial for optimum management. Continuum Lifelong Learning Neurol ;18 4 — Address correspondence to Dr Peter J. Relationship Disclosure:.

Dr Goadsby has provided headache expert testimony. Dr Goadsby reports no disclosure. They also respond to different treatments. Cluster headache is characterized by at- tacks of longer duration with relatively low attack frequency. PH is interme- diate in duration and frequency of at- tacks.

In HC, the pain is continuous with frequent exacerbations that may in- clude cranial autonomic symptoms. TACs need to be distinguished from other types of short-lasting headache Table It is important to diagnose. Copyright American Academy of Neurology. Unauthorized reproduction of this article is prohibited. TABLE Cranial Autonomic Symptoms, Sinus Headache, and the Trigeminal Autonomic Cephalalgias The diagnosis of sinus headache, im- plying an erroneous infectious etiol- ogy and necessity for antimicrobials , is common in patients referred to neu- rologists, particularly those consulting headache specialists.

Cranial autonomic symptoms are cardinal features of the TACs and the symptoms for which clus- ter headache is best known. These in- clude conjunctival injection, lacrimation, nasal congestion, and rhinorrhea. They can be elicited by inducing pain in the first division of the trigeminal nerve in volunteer s 4 and are also common in migraine. Case A. A year-old man presented with a 9-year history of troublesome headaches.

As a teenager, he had headache with nausea, during which he preferred to retire to a darkened room. He reported daily headache for the past 9 years. Some part of every day the headache was worse for 2 to 3 hours. The pain was bilateral, although greater on the left. During exacerbations the pain throbbed, and photophobia localized to the left and generalized phonophobia were present.

Head movement had no effect on the pain. He also took thyroxine 2 g daily. Family history of headache included his mother, who associated her headaches with her periods and drinking red wine. On examination he was euthyroid. General and neurologic examinations were normal. Consider the differential diagnosis at this point. The case will continue on page August However, the reason for the symptom is cranial parasympa- thetic activation related to pain rather than nasal congestion due to local in- flammation.

The trigeminal autonomic reflex, which causes parasympathetic activation triggered by nociceptive tri- geminal activation, consists of an af- ferent arc in the trigeminal nerve, most prominently in the first division; a re- flex connection to the parasympathetic superior salivatory nucleus in the pons; and an efferent arc in the facial seventh cranial nerve.

The efferent arc passes through the geniculate ganglion, syn- apsing mainly in the pterygopalatine sphenopalatine ganglion and proj- ecting through the greater superficial petrosal nerve. This well-described physiology ex- plains why so many headaches are misdiagnosed and provides a logical basis on which to rediagnose and cor- rectly manage these patients.

Lateralization An important feature of TAC attacks is lateralization of the symptoms and signs. In the TAC popula- tion, as many as 10 times that number report photophobia or phonophobia, or both, ipsilateral to the pain. In migraine, cranial autonomic symptoms are more often bilateral, less prominent, and var- iable in presentation. Role of the Pituitary Gland. A spectrum of pathology is reported.

Understanding the trigeminal auto-. In a cohort of 84 patients with pituitary. Activity in the region of. MRI brain imaging. Given that TACs are rare and lifelong,. These studies may have im- portant therapeutic implications. Photophobia or phonophobia is ipsilateral to the headache in trigeminal autonomic cephalalgias and remains bilateral in migraine even when the pain is lateralized.

In trigeminal autonomic cephalalgias, cranial autonomic symptoms, such as lacrimation, conjunctival injection, and nasal congestion, tend to be lateralized to the side of pain, prominent, and consistent between one attack and another.

In migraine, cranial autonomic symptoms are often bilateral, mild, and do not always parallel the severity of attacks. Pituitary gland pathology may accompany trigeminal autonomic cephalalgias.

Differential Diagnosis of Trigeminal Autonomic Cephalalgias. It is perhaps the most agonizing afflic- tion. Of the more than patients with cluster headache I have seen, not a single one has experienced more se-. Core features of cluster headache are its circadian periodicity in terms of active and inactive periods over time and the lateralization of the pain Table Most patients with cluster headache. Cluster Headache. Diagnostic Criteria:. At least five attacks fulfilling criteria B through D.

Headache is accompanied by at least one of the following:. Ipsilateral eyelid edema. Ipsilateral forehead and facial sweating. A sense of restlessness or agitation. Attacks have a frequency from one every other day to eight per day.

Not attributed to another disorder. Episodic Cluster Headache. Description: Occurs in periods lasting 7 days to 1 year sep arated by pain-free periods lasting 1 month or more. Attacks fulfilling criteria A through E of cluster headache above. At least two cluster periods lasting from 7 to days and separated by pain-free remissions of Q 1 month. Chronic Cluster Headache Description: Attacks occur for more than 1 year without remission or with remissions lasting less than 1 month.

Attacks recur over 9 1 year without remission periods or with remission periods of G 1 month. They usually experience episodes of one or two attacks of relatively short duration lasting 15 to minutes with unilat- eral pain which occur daily for 8 to 10 weeks yearly.

Patients are generally asymptomatic between attacks. During attacks, patients move unceasingly, pacing, rocking, or even rubbing their head for relief. This is in contrast to patients with migraines, who prefer to remain still.

The pain tends to be retro-orbital or temporal , boring in quality, and intense. Associated features include ipsilateral symptoms of cranial. Clus- ter headache is thought to involve neurons in or around the central pacemaker regions of the posterior hy- pothalamic gray matter Figure This serves three purposes: 1 the clinical phenotype suggests the likely secondary causes that need to be considered and the studies needed, particularly to exclude pituitary gland Y related disorders; 2 the diagnosis gives clarity to the pa- tient and allows the physician to draw on available literature to comment on natural history; and 3 the correct di- agnosis determines specific therapy that will likely be effective in these conditions.

Management The management of cluster headache requires the use of both acute and preventive agents. Preven- tive treatments can be lifesaving for patients with chronic cluster headache. Preventive treatments. The options for preventive treatment in cluster head- ache depend on the episode length Table Patients with short cluster periods require rapid-acting agents that may not be needed for prolonged periods.

Those with long cluster pe- riods or chronic cluster headache re- quire safe, effective medicines that can be administered long-term. Verapamil is widely considered to be the first-line preventive treatment in prolonged epi- sodes, and certainly in chronic cluster headache.

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Trigeminal Autonomic Cephalalgias: Review Article

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