Tears of Pain SUNCT and SUNA A/PROFESSOR ARUN AGGARWAL RPAH PAIN MANAGEMENT CENTRE
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1 Tears of Pain SUNCT and SUNA A/PROFESSOR ARUN AGGARWAL RPAH PAIN MANAGEMENT CENTRE
2 IHS Classification 1989 (updated 2004) Primary Headaches 4 categories Migraine Tension-type Cluster and other trigeminal autonomic cephalalgia Other primary headaches Trigeminal Neuralgia Primary cough, exertional and coital headache
3 IHS Classification 1989 (updated 2004) Secondary Headaches Head and or neck trauma Cranial or cervical vascular disorders Non vascular intracranial disorders Substance or withdrawal Infection Disorders of homeostasis Disorders of cranium, neck, eye, ear, nose, teeth, mouth or other facial or cranial structures Psychiatric disorders
4 Trigeminal Neuralgia Intense, paroxysms of sharp, stabbing pain Lasting few seconds to 2 minutes Pain free between attacks Attacks are sterotyped Precipitated from trigger Light touch of the face (washing, shaving, make-up) Chewing, talking, cold wind No clinical neurological deficit Numbness or weakness Not caused by another disorder Classical TN responds well to Tegretol
5 Trigeminal Automonic Cephalgias Group of Primary Headache with Unilateral trigeminal distribution Associated with ipsilateral photophobia and cranial autonomic symptoms Differ in duration, frequency and response to therapy Cluster Headache longest duration and low frequency Paroxysmal Hemicrania immediate duration and frequency SUNA (major DD for TN) Short lasting unilateral neuralgiform attacks with cranial autonomic symptoms Shortest duration and highest frequency SUNCT Short lasting unilateral neuralgiform attacks with conjunctival injection and tearing +/- other cranial autonomic symptoms Shortest duration and highest frequency
6 CAS symptoms SUNCT vs SUNA vs CH
7 IHS criteria for episodic cluster headache A. At least 5 attacks fulfilling B-D B. Severe unilateral orbital, supra-orbital and/or temporal pain lasting minutes untreated C. Headache associated with at least one of the following signs: Conjunctival injection Lacrimation Nasal congestion Rhinorrhea Forehead and facial sweating Miosis, ptosis Eyelid oedema D. Frequency once every other day to 8 per day
8 Cluster headache Chronic refers to similar attacks but occurring for > 1 year without remission or with remission lasting < 14 days 80-90% are episodic 10-20% are chronic 85% with episodic cluster headaches are males Females > males for chronic Characteristically occur at night
9 Cluster Headache Treatment Acute treatment High-flow (12 L/min) 100% nasal oxygen for minutes Nasal lignocaine Dihydroergotamine via IV or rectal suppository Triptans sc or nasal Nasal capsaicin spray Once controlled Prednisone 50 mg daily, tapered slowly over the following 3 weeks to prevent recurrence Chronic Cluster Headache Verapamil 80 mg bd up to 320 mg bd (Heart block, gum hypertropthy, constipation, SOA) Lithium with or without clonazepam Valproate and baclofen have been tried Methysergide
10 Cluster Headache - New Treatments New combinations of old drugs Treximet - 85 mg sumatriptan and 500 mg naproxen New formulations of old drugs Intranasal zolmitriptran (Zomig) Better tolerated than Imigran (unpleasant taste) Inhaled DHE New acute and preventive treatments Telcagepant - a CGRP (Calcitonin Gene-Related Peptide) receptor antagonist - phase III TRPV 1 (Transient Receptor Potential Vanilloid) receptor antagonist on ion channels Selective for calcium, magnesium and sodium Established treatments for other neurologic conditions Memantine - glutamate receptor antagonist (NMDA) - prevention of refractory migraine Gene therapy Mayo Clinic collecting genomic data on 4,500 patients migraine with and without aura and on gender- and agematched controls
11 Chronic Paraxysmal Hemicrania Severe, unilateral, (orbital, supraorbital, temporal) Shorter lasting mins and more frequent (1-40/day) >5/day lasting 2-30min Generally V1 Females > Males Ipsilateral parasympathetic activation miosis Episodic in 20% lasting for a week per month Chronic with no remission Distincively responds very well to Indomethacin 150 mg or less Also COX-2 and topiramate
12 SUNA Short-lasting Unilateral Neuralgiform headaches Attacks with cranial autonomic symptoms Prominently pain in V1 more than V2, V3 or in teeth Strictly unilateral stabbing Very brief (5-240 secs) occurring at least times a day Conjunctival injection or Tearing only but not both Ipsilateral photophobia, phonophobia c.f. bilateral in migraine Other cranial autonomic symptoms Rhinorrhoea Eyelid oedema Facial sweating / flushing Ear flushing Cutaneous stimulation triggers attacks in 22% No refractory period between attacks High rate of pituitary tumours in both so MRI scan indicated
13 SUNCT Short-lasting Unilateral Neuralgiform Headaches with Conjunctival injection and Tearing? Subset of SUNA or separate Prominently pain in V1 more than V2, V3 or in teeth Strictly unilateral stabbing Very brief (5-240 secs) occurring at least times a day Ipsilateral conjunctival injection and tearing always present Cutaneous stimulation triggers attacks in 74% touching, washing, chewing, eating, talking, shaving No refractory period between attacks Dull ache Men > Female
14
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16 SITE of Attacks SUNCT vs SUNA
17 Attack Triggers
18 SUNCT / SUNA Treatment Responds very well to Indomethacin Lamotrigine 2/3 respond Topiramate 2/5 received 50mg bd improved over 10 days Gabapentin Also responsive to sub-occipital stimulation sub-cutaneous Lignocaine infusion
19 Sub-Occipital Stimulators Electrical stimulation of primary sensory afferents Anti-nociceptive effects PET studies Changes in regional cerebral blood flow Dorsal rostral pons Anterior cingulate cortex Occipital neuralgia Chronic migraine Cluster Headache and SUNCT
20 SUNA / SUNCT SUNA/SUNCT vs TN Prominent V1 distribution of pain Attacks triggered by touch Lack of refractory period between attacks SUNA/SUNCT vs CH Lack of response to O2, Triptans and Verapamil Goadsby experience - overdiagnosis of TN in UK <1 in 100 diagnosed with TN actually have TN
21 Mrs BB 58 yo 3 year hx of left facial pain Sharp, shooting and knife like lasting seconds Pain increased by touching nose and face Dx as TN BUT Conjunctival injection Lacrimation left eye Nasal conjestion SUNCT Indocid 25 mg three times a day Complete resolution of pain
22 SUNCT vs SUNA SUNCT most painful condition ever (worse than childbirth) VRS rating 10/10 84% SUNCT vs 33% SUNA SUNCT - more attacks / day Triggered by touching face 74% SUNCT vs 22% in SUNA Conjunctival Injection and Tearing 100% SUNCT vs 22-44% in SUNA Mean age of onset similar Mean duration of symptoms similar 8-10 years Mean duration for diagnosis similar years
23 Conclusion Trigeminal Autonomic Cephalgias are a group of Primary Headache with Unilateral trigeminal distribution pain Associated with ipsilateral cranial autonomic symptoms Lacrimation, conjuctival injection or nasal symptoms Important to differentiate given selective response to treatment Cluster Headache longest duration and low frequency Paroxysmal Hemicrania immediate duration and frequency SUNA (major DD for TN) Short lasting unilateral neuralgiform attacks with cranial autonomic symptoms Shortest duration and highest frequency SUNCT Short lasting unilateral neuralgiform attacks with conjunctival injection and tearing +/- other cranial autonomic symptoms Shortest duration and highest frequency
24
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