Headache Master School Japan-Osaka 2016 II. Management of refractory headaches Case Presentation 2. SUNCT/SUNA: concept, management and prognosis Noboru Imai(Department of Neurology, Japanese Red Cross Shizuoka Hospital) October 23th, 2016; Osaka International House Foundation
The trigeminal autonomic cephalalgias (TACs) 3.1 Cluster Headache 3.2 Paroxysmal Hemicrania 3.3 Short-lasting unilateral neuralgiform headache attacks (SUNCT/SUNA) 3.3.1 Short-lasting unilateral neuralgiform headache attacks with conjunctival injection and tearing (SUNCT) 3.3.2 Short lasting unilateral neuralgiform headache attacks with cranial autonomic symptoms (SUNA) 3.4 Hemicrania Continua 3.5 Probable TAC IHS. ICH-3beta. Cephalalgia 2013
The pathogenesis of TACs May A. Lancet 2005; 366: 843-855
Posterior hypothalamic activation of TACs Cluster headache (PET) Paroxysmal hemicrania (PET) SUNCT (fmri) Hemicrania continua (PET) May et al., Lancet 1998; 351:275-278 Matharu et al., Ann Neurol 2006; 59:535-545 May et al., Ann Neurol 1999; 46:791-793 Matharu et al., Headache 2004; 44:747-761
SUNCT/SUNA A. At least 20 attacks fulfilling criteria B D B. Moderate or severe unilateral head pain, with orbital, supraorbital, temporal, and/or other trigeminal pain Distribution: lasting for 1 600 seconds and occurring as single stabs, a series of stabs, or in a saw-tooth pattern C. At least one 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 edema 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 one a day for more than half the time when the disorder is active E. Not better accounted for by another ICHD-3 diagnosis.
3.3.1 SUNCT Diagnostic criteria: A. Attacks fulfilling criteria for 3.3 Short-lasting unilateral neuralgiform headache attacks B. Both of conjunctival injection and lacrimation (tearing).
3.3.2 SUNA 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).
Case 1: 27-year-old man Moderate or severe left-sided headaches lasting 30 120 min with ipsilateral lacrimation, nasal congestion and rhinorrhoea First episode: 5 months ago The frequency of attacks: 1 or 2 times per day Persistent background pain One month pain-free period Triggered by neck exercise, wasabi
Past history Acute treatment: Oral Triptans, NSAIDs, OTCs ; not effective Oxygen; moderate pain reduction within 15 to 30 minutes Preventive treatment: Carbamazepine; not effective Family history None On examination General and Neurological examination normal Imaging MRI normal
Treatment Acute treatment subcutaneous sumatriptan (6 mg) ; not effective Preventive treatment Oral steroid, verapamil, lithium, lomerizine, indomethacin; not effective Topiramate;,the frequency was reduced to half, but not effective 2 months later
Obtaining a good history Headache episodes consist of a series of sawtooth pattern attacks! 10 0 Lamotrigine: suppressed attacks up to 200mg daily
Case 2: a 5-year-old girl Severe, unilateral (mainly left-sided) temporal headaches lasting 20 90 s with ipsilateral lacrimation, nasal congestion and rhinorrhoea, facial flushing First episode at 16 months old The frequency of moderate or severe headache attacks gradually increased 84 to 10 20 times per day Triggered by hitting her head or body, taking a bath, experiencing a temperature change, or sleeping Alleviated by carbamazepine
Genetic analysis of the pedigree Familial pedigree Affected individuals Unaffected individuals I-1 I-2 (76y) II-1* (56y) II-2 (50y) II-3 (51y) III-1 (32y) III-2* (29y) III-3* (41y) III-4* (24y) III-5* (22y) IV-1* (12y) IV-2* (6y) IV-3* (5y) IV-4* (1y) A novel SCN9A mutation in this family suggested that the family suffers from a variant of paroxysmal extreme pain disorder. : Position of the mutation (NM_002977: c.5218g>c, p.val1740leu) Electropherograms of the heterozygous SCN9A mutation. A G/C change is represented as an S according to the IUPAC code.
Functional study The subtraction ictal SPECT co-registered to MRI (SISCOM) A A B C A) Transverse, B) sagittal, and C) coronal images.
Clinical features of TACs Cluster headache Paroxysmal hemicrania SUNCT/SUNA Hemicrania Continua Sex F<M F=M F=M F>M Pain Type Stabbing, boring Throbbing, boring, Burning, stabbing, Throbbing, ache, stabbing sharp sharp, pressure Mild to moderate Severity Excruciating Excruciating background with Moderate to severe severe exacerbations Duration and frequency 15 180 mins, 1 8/day 2 30 mins, 5 40/day 1 600 s, 1 200/day Continuous Autonomic features Yes Yes Yes Yes with exacerbations Indomethacin effect No Complete resolution No Complete resolution First choice abortive agent Injectable or nasal sumatriptan Oxygen None None None First choice preventative agents Verapamil Prednisone Indomethacin Lamotrigine Indomethacin Cohen et al., Brain 2006; 129:2746-2760 ;Cittadini et al., Brain 2008; 131:1142-1155 Miller et al., Curr Pain Headache Rep 2014; 18: 438
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