SUNCT/SUNA in Pediatric Age: A Review of Pathophysiology and Therapeutic Options

Abstract

The International Classification of Headache Disorders, 3rd edition (ICHD3) defines Short-lasting Unilateral Neuralgiform Headache Attacks (SUNHA) as attacks of moderate or severe, strictly unilateral head pain lasting from seconds to minutes, occurring at least once a day and usually associated with prominent lacrimation and redness of the ipsilateral eye. Two subtypes of SUNHA are identified: Short-lasting Unilateral Neuralgiform headache attacks with Conjunctival injection and Tearing (SUNCT) and Short-lasting Unilateral Neuralgiform headache attacks with cranial Autonomic symptoms (SUNA). These pathologies are infrequent in children and difficult to diagnose. The authors reviewed the existing literature on SUNCT and SUNA, especially in the developmental age, which describes the pathophysiology in detail and focuses on the therapeutic options available to date. SUNHA-type headaches must be considered on the one hand, for the possibility of the onset of forms secondary to underlying pathologies even of a neoplastic nature, and on the other hand, for the negative impact they can have on an individual’s quality of life, particularly in young patients. Until now, published cases suggest that no chronic variants occur in childhood and adolescents. In light of this evidence, the authors offer a review that may serve as a source to be drawn upon in the implementation of suitable treatments in children and adolescents suffering from these headaches, focusing on therapies that are non-invasive and as risk-free as possible for pediatric patients.

Missed pituitary microadenoma during endoscopic transsphenoidal surgery for short-lasting unilateral neuralgiform headache attacks with conjunctival injection and tearing with symptom relief: illustrative case

“…SUNCT is generally medically refractory, which compels the search for alternative treatment paradigms. 5 Though direct PPG block with lidocaine has been reported to acutely resolve symptoms in a patient with SUNCT, 12 a systematic review on the use of PPG blockade for headache syndromes did not demonstrate a cure for the two SUNCT cases that were included. 13 Pulsed radiofrequency of the PPG has been explored as a possible treatment for SUNCT, and this resulted in symptom resolution in seven out of nine cases.…”

“…1 The most widely accepted theory is that an abnormality in the posterior hypothalamus precipitates activation of the trigeminalautonomic reflex. [4][5][6] Functional imaging and brain stimulation studies have identified activation of the posterior hypothalamic gray matter in TACs, which in turn leads to heightened sensitivity to noxious stimuli via the pain-modulating system of the trigemino-hypothalamic tract and the trigeminovascular system. [4][5][6] These systems then stimulate internal trigger areas in the hypothalamus and trigeminal nucleus caudalis, which activate the superior salivatory nucleus.…”

“…[4][5][6] Functional imaging and brain stimulation studies have identified activation of the posterior hypothalamic gray matter in TACs, which in turn leads to heightened sensitivity to noxious stimuli via the pain-modulating system of the trigemino-hypothalamic tract and the trigeminovascular system. [4][5][6] These systems then stimulate internal trigger areas in the hypothalamus and trigeminal nucleus caudalis, which activate the superior salivatory nucleus. In turn, they send parasympathetic fibers through the facial (greater superficial petrosal nerve) and pterygopalatine ganglion (PPG; also known as the sphenopalatine ganglion).…”

Cigarette smoking history (personal and secondary childhood exposure) in non-cluster headache trigeminal autonomic cephalalgias: A clinic based study

“…For PH the largest number of reported cases in a single study with a documented tobacco exposure history was 74 patients (lacked sex distribution), SUNCT and SUNA 20 patients and for HC a study of 34 patients (no sex distribution was noted) (14)(15)(16). All pediatric and adolescent investigations had no personal or secondary cigarette smoke exposure data (17)(18)(19). None of the original case descriptions for each of these unique conditions mentioned a smoking history (20)(21)(22).…”

New Advances in Neuropsychiatric Disorders of Childhood and Adolescence

“…Cesaroni et al, in their review, analyzed the literature evidence about the key features of therapeutic options, during the developmental age, of Short-lasting Unilateral Neuralgiform headache attacks with Conjunctival injection and Tearing (SUNCT) and Short-lasting Unilateral Neuralgiform headache attacks with cranial Autonomic symptoms (SUNA), rare primary headache syndromes belonging to the group of Trigeminal Autonomic Cephalalgias. Thanks to a review of 60 articles, the authors described the pathophysiology of SUNCT and SUNA and analyzed the therapeutic options available to date, especially underlining less-invasive and risk-free therapies as possible treatment options for pediatric patients [6].…”