Pause for thought. Dr Jane Anderson Consultant Neurologist

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1 Pause for thought Dr Jane Anderson Consultant Neurologist

2 Which is the top cause of years lived with disability worldwide? 1. COPD 2. Low Back pain 3. Diabetes 4. Migraine with medication overuse headache 5. Major Depression

3 Global Burden of Disease Study 2013_Lancet 2015 Migraine with MOH

4 Is it 1. Primary cardiac presentation 2. Secondary cardiac presentation

5 If secondary is it 1. Cardiac migraine 2. Subarachnoid hameorrhage 3. Phaeochromocytoma 4. Cluster headache

6 Thunderclap headache Peaks within minutes -80% within 1 st minute 1 1 o v 2 o : clinically cannot differentiate1 Primary TCH diagnosis of exclusion SAH CT/LP earlier or CTA later Venous Sinus Thrombosis raised CSF OP, CTV Arterial Dissection focal Neuro signs, MRA Pituitary Apoplexy Spontaneous Intracranial Hypotension 3 rd ventricle colloid cyst Reversible cerebral vasoconstriction syndrome (RCVS)

7 Sub-arachnoid haemorrhage (SAH) Leakage of blood into CSF space meningeal irritation: 85% Saccular Aneurysm, 10% perimensephalic, 5% other Headache in isolation in up to 50-70% Thunderclap HA: Peaks within a minute and lasts at least an hour Worst Ever with: Vomiting in 70% ( vs 42% in benign) Meningism; photophobia LOC: typically transient at onset (26-50%) Transient focal neurology (33%) Subhyaloid haemorrhage (17%) Delirium (16%) Epileptic seizures (6-9%) Sudden death (10%) medscape

8 SAH workup Sensitivity of CT/CSF <6hrs <12hrs <24hrs <1wk <2wk 3wk 4wk CT >98% 95% 90-93% 50% poor poor Defer to 100% 100% ~100% ~100% 70% 40% 12hrs CSF spectrophotometry for xanthochromia Timeline of Xanthochromia in SAH: Vermeulen, Van Gin, JNNP 1990; 53; & JNNP 1989; 52; Timeline of CT in SAH: Van Ginjn, Van Dongen, Neuroradiol : Further Ix guided by history Algorithm adapted from Schwedt et al 2006

9 SAH Delayed or overlooked diagnosis in 1 o care occurred in 44% (32/75) In 2 o care occurred in 13% (49/383)

10 SAH a missed diagnosis 1in 20 missed in A&E: often because diagnosis not considered Beware of distractors Instantaneous headaches only in 50% Focusing on hypertension and arrhythmia 1 in 10 SAH may present with a fit 1-2% present with isolated acute confusion LP is traumatic With delay in diagnosis 25% die before reaching hospital 25% die within hospital

11 clinical presentation Recurrent Episodes since 2013 Initially every 2-3months and over 2 days premonitory: change in mood, sweating severe headaches strictly side-locked & right unilateral focussed in orbit & temple stabbing intense pain hit with baseball peak pain in minutes lasts 20min-3hrs unilateral conjunctival tearing, rhinorrhoea, facial sweating, right eyelid oedema motor agitation typical late evening attack 1-8 attacks/day post-dromal: depressed and drained for hrs

12 clinical presentation 49 yr paroxysmal severe unilateral headache short duration (<3hrs) with autonomic features including cardiovascular Cluster Headache

13 Investigations:- Several normal blood investigations including routine pituitary profile Renin/aldosterone blood & urinary catecholamines Normal imaging studies: MRI with pituitary views CTA with fine cuts high res CT adrenals/abdomen (*) Normal endocrinology work up

14 Autonomic focus:- cranial autonomic symptoms occur in both migraine and TAC laterality & dominance are typical of TAC s Lai et al, jnnp 2008

15 Autonomic focus:- cardiovascular autonomic control?trigemino-cardiac reflex Cardio-inhibitory centre with decelemotor function HR Lang et al 1991, J Anaesth

16 Autonomic focus:- cardiovascular autonomic control in cluster evidence of systemic autonomic dysfunction ictally parasympathetic tone may be increased post hypothalamus hub: regulating autonomic response Barloese, Headache, 2015

17 Headache Jan;8(4): Jacobson LB. Cluster headache: a rare cause of bradycardia. Headache Jul;30(8): Attanasio A1 et al Sinus bradycardia, junctional rhythm and blood pressure increase during repeated cluster headache attacks. Can J Cardiol Nov;20(13): Erdinler I 1 et al Asystole associated with cluster headache. A 43-year-old man with asystole and syncope occurring during cluster headache attacks is reported. The asystole and syncope attacks disappeared completely following prophylactic therapy with methysergide maleate.

18 Medical Treatment Abortive O2 s/c sumatriptan Preventative Verapamil topiramate Transitional Therapy Steroids GON blocks Manjit Matharu

19 Randomised, controlled, double blind studies in cluster headache * * * * *P<0.05 Time= 15min 15 min 30 min 30 min N= Cohen et al, JAMA 2009; van Vliet J et al, Neurology 2003; Cittadini E et al. Arch Neurol 2006; Ekbom K et al. Acta Neurol Scand Manjit Matharu

20 Horizon therapies& NICE Triptans Botulinum toxin TA /2012 ONS IPG /2013 TMS IPG /2014 SPG IPG /2015 VNS IPG /2016 Cefaly IPG /2016

21 MoA Non-invasive Vagal Nerve Stimulator (nvns) Afferent arm of vagus 80% Stimulation of vagus afferents stimulates inhibitory nucleus tractus solitarus & reduced activity in the trigeminal nucleus caudalis

22

23 several hrs Increasing attack duration secs Motor restlessness Migraine CH PH SUNCT SUNA Trigeminal Neuralgia 1 Increasing attack frequency 100 Not side-locked side-locked

24 Thank you for listening

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