SUNCT syndrome

Hans-Christoph Diener MD (Dr. Diener of the University of Essen received honorariums from Addex Pharmaceuticals, Allergan, Almirall, AstraZeneca, Bayer Vital, Boehringer Ingelheim, Bristol-Myers Squibb, CoLucid, Coherex, Menerini, GlaxoSmithKline, Minster, Lilly, MSD, Novartis, Neuroscore, Johnson & Johnson, Pfizer, Sanofi-Aventis, Weber & Weber, and Medtronic for service as an advisor or speaker; financial support from Allergan, Almirall, AstraZeneca, Bayer Vital, GlaxoSmithKline, MSD, and Pfizer for research projects; and honorariums from Amgen as an advisor.)
Shuu-Jiun Wang MD, editor. (Dr. Wang of the National Yang-Ming University School of Medicine and the Neurological Institute, Taipei Veterans General Hospital received consulting fees from Eli Lilly.)
Originally released March 12, 1999; last updated August 5, 2016; expires August 5, 2019

This article includes discussion of SUNCT, short-lasting unilateral neuralgiform headache attacks with conjunctival injection and tearing, SUNA, and short-lasting unilateral neuralgiform headache attacks with cranial autonomic symptoms. The foregoing terms may include synonyms, similar disorders, variations in usage, and abbreviations.

Overview

SUNCT (short-lasting unilateral neuralgiform headache attacks with conjunctival injection and tearing) is one of the rarest of all primary headache syndromes. It is one of the trigeminal autonomic cephalalgias, along with cluster headache and paroxysmal hemicrania. It is marked by frequent, short-lasting attacks of 1-sided headache with associated cranial autonomic symptoms. SUNCT forms the majority subset of SUNA (short-lasting unilateral neuralgiform headache attacks with cranial autonomic symptoms), in which the combination of conjunctival injection and tearing are not required for diagnosis. Reports and series have shown therapeutic benefits with medical treatments. Structural imaging studies report an association with aberrant vessel in contact with the trigeminal nerve, and functional imaging studies report hypothalamic activation. Microvascular decompression, occipital nerve stimulation, posterior hypothalamic region deep brain stimulation, and focal radiation therapy have all been reported to be effective in medically intractable cases.

Key points

 

• SUNCT/SUNA are relatively rare but are very severe forms of primary headache syndromes belonging to a family of trigeminal autonomic cephalalgias.

 

• Functional neuroimaging studies in trigeminal autonomic cephalalgias, including SUNCT, report specific activation of the hypothalamus in association with the headaches.

 

• Structural imaging studies report that a significant proportion of patients have an aberrant vascular loop in contact with the ipsilateral trigeminal nerve, akin to the situation in trigeminal neuralgia.

 

• Transitional treatments include intravenous lidocaine, oral or iv corticosteroids, and greater occipital nerve blocks.

 

• Medical treatment options include lamotrigine, topiramate, gabapentin, pregabalin, duloxetine, and carbamazepine.

 

• In patients who are intractable to medical treatments, the surgical options include trigeminal microvascular decompression, occipital nerve stimulation, and posterior hypothalamic region deep brain stimulation.

Historical note and terminology

The syndrome of short-lasting unilateral neuralgiform headache attacks with conjunctival injection and tearing, or SUNCT, was first described by Sjaastad and colleagues in 1978 (Sjaastad et al 1978). The syndrome was recognized in the revised International Classification of Headache Disorders (ICHD-II) in 2004 and modified in the latest version in 2013 (Headache Classification Committee of the International Headache Society 2004; Headache Classification Committee of the International Headache Society 2013).

Table 1. ICHD-3 Diagnostic Criteria for SUNCT and SUNA

3.3 Short-lasting unilateral neuralgiform headache attacks

 

Description: Attacks of moderate or severe, strictly unilateral head pain lasting seconds to minutes, occurring at least once a day and usually associated with prominent lacrimation and redness of the ipsilateral eye.

Diagnostic criteria:

 

A. At least 20 attacks fulfilling criteria B to D

B. Moderate or severe unilateral head pain, with orbital, supraorbital, temporal and/or other trigeminal distribution, lasting for 1 to 600 seconds and occurring as single stabs, series of stabs or in a sawtooth pattern

C. At least 1 of the following cranial autonomic symptoms or signs, ipsilateral to the pain:

 

1. conjunctival injection and/or lacrimation
2. nasal congestion and/or rhinorrhea
3. eyelid oedema
4. forehead and facial sweating
5. forehead and facial flushing
6. sensation of fullness in the ear
7. miosis and/or ptosis

D. Attacks have a frequency of at least 1 a day for more than half of the time when the disorder is active

E. Not better accounted for by another ICHD-3 diagnosis

3.3.1 Short-lasting unilateral neuralgiform headache attacks with conjunctival injection and tearing (SUNCT)

 

Diagnostic criteria:

   

A. Attacks fulfilling criteria for 3.3 short-lasting unilateral neuralgiform headache attacks

   

B. Both of conjunctival injection and lacrimation (tearing).

3.3.1.1 Episodic SUNCT

 

Description: Attacks of SUNCT occurring in periods lasting from 7 days to 1 year, separated by pain-free periods lasting 1 month or more.

 

Diagnostic criteria:

   

A. Attacks fulfilling criteria for 3.3.1 short-lasting unilateral neuralgiform headache attacks with conjunctival injection and tearing and occurring in bouts

   

B. At least 2 bouts lasting from 7 days to 1 year and separated by pain-free remission periods of >1 month.

3.3.1.2 Chronic SUNCT

 

Description: Attacks of SUNCT occurring for more than 1 year without remission, or with remission periods lasting less than 1 month.

 

Diagnostic criteria:

   

A. Attacks fulfilling criteria for 3.3.1 short-lasting unilateral neuralgiform headache attacks with conjunctival injection and tearing, and criterion B below

   

B. Occurring without a remission period, or with remissions lasting <1 month, for at least 1 year.

3.3.2 Short lasting unilateral neuralgiform headache attacks with cranial autonomic symptoms (SUNA)

 

Diagnostic criteria:

   

A. Attacks fulfilling criteria for 3.3 short-lasting unilateral neuralgiform headache attacks, and criterion B below

   

B. Only one or neither of conjunctival injection and lacrimation (tearing).

3.3.2.1 Episodic SUNA

 

Description: Attacks of SUNA occurring in periods lasting from 7 days to 1 year, separated by pain-free periods lasting at least 1 month.

 

Diagnostic criteria:

   

A. Attacks fulfilling criteria for 3.3.2 short-lasting unilateral neuralgiform headache attacks with cranial autonomic symptoms and occurring in bouts

   

B. B. At least two bouts lasting from 7 days to 1 year and separated by pain-free remission periods of >1 month.

3.3.2.2 Chronic SUNA

 

Description: Attacks of SUNA occurring for more than 1 year without remission, or with remission periods lasting less than 1 month.

 

Diagnostic criteria:

   

A. Attacks fulfilling criteria for 3.3.2 short-lasting unilateral neuralgiform headache attacks with cranial autonomic symptoms, and criterion B below

   

B. Occurring without a remission period, or with remissions lasting <1 month, for at least 1 year.

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