Trigeminal Autonomic Trigeminal Autonomic Cephalalgias Cephalalgias Manjit S Matharu Manjit S Matharu Headache Group, Institute of Neurology Headache Group, Institute of Neurology & & The National Hospital for Neurology and The National Hospital for Neurology and Neurosurgery Neurosurgery London London UK UK Third Biennial Hull-BASH Headache Meeting 23 rd January 2009
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Trigeminal Autonomic Cephalalgias Manjit S Matharu Headache Group, Institute of Neurology & The National Hospital for Neurology and Neurosurgery LondonUK.
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Headache Group, Institute of Neurology &Headache Group, Institute of Neurology &The National Hospital for Neurology and NeurosurgeryThe National Hospital for Neurology and Neurosurgery
London London UKUK
Third Biennial Hull-BASH Headache Meeting 23rd January 2009
• SUNCT (Short-lasting SUNCT (Short-lasting Unilateral Neuralgiform Unilateral Neuralgiform headache with Conjunctival headache with Conjunctival injection and Tearing)injection and Tearing)
• Cluster HeadacheCluster Headache
• Paroxysmal HemicraniaParoxysmal Hemicrania
• SUNCT (Short-lasting SUNCT (Short-lasting Unilateral Neuralgiform Unilateral Neuralgiform headache with Conjunctival headache with Conjunctival injection and Tearing)injection and Tearing)
Trigeminal Autonomic Cephalgias Pituitary and TACs
Trigeminal Autonomic Cephalgias Pituitary and TACs
Levy et al, Brain 2005• 84 pituitary tumour patients with headaches studied• 9% had TACs• Functioning adenomas more likely to cause TACs• Investigate all TAC patients for pituitary tumours?
• Prevalence of pituitary tumours in TACs is unknown• 1 in 10 of the population have an incidental pituitary micro-
adenoma (< 1cm diameter) on routine MRI • 1 in 500 have a macro-adenoma
Levy et al, Brain 2005• 84 pituitary tumour patients with headaches studied• 9% had TACs• Functioning adenomas more likely to cause TACs• Investigate all TAC patients for pituitary tumours?
• Prevalence of pituitary tumours in TACs is unknown• 1 in 10 of the population have an incidental pituitary micro-
adenoma (< 1cm diameter) on routine MRI • 1 in 500 have a macro-adenoma
Trigeminal Autonomic Cephalgias Pituitary and TACs
Trigeminal Autonomic Cephalgias Pituitary and TACs
• Difficult to draw up definitive guidelines from retrospective reviews
• Pituitary imaging should be performed in:– Atypical phenotype/abnormal examination
– Treatment resistant cases
• Do typical cases require neuroimaging? – Increases likelihood of identifying incidental lesion
• Implication of data on pituitary lesions?– Need prospective community based study in CH patients
– Carefully elicit symptoms related to pituitary disease in all TAC patients but only perform MRI scans of the pituitary and a basal pituitary hormone profile in:
• patients with atypical features (including pituitary related symptoms)
• abnormal examination
• poor response to appropriate treatments.
• Difficult to draw up definitive guidelines from retrospective reviews
• Pituitary imaging should be performed in:– Atypical phenotype/abnormal examination
– Treatment resistant cases
• Do typical cases require neuroimaging? – Increases likelihood of identifying incidental lesion
• Implication of data on pituitary lesions?– Need prospective community based study in CH patients
– Carefully elicit symptoms related to pituitary disease in all TAC patients but only perform MRI scans of the pituitary and a basal pituitary hormone profile in:
• patients with atypical features (including pituitary related symptoms)
Cluster Headache Vs Paroxysmal HemicraniaCluster Headache Vs Paroxysmal HemicraniaCluster Headache Vs Paroxysmal HemicraniaCluster Headache Vs Paroxysmal Hemicrania
Trial of Indomethacin if:Trial of Indomethacin if:
1.1. Attack frequency Attack frequency >> 5 daily 5 daily
Cohen et al. Migraine Trust Symposium, September 2006
SUNCT SUNCT TREATMENTSTREATMENTS
SUNCT SUNCT TREATMENTSTREATMENTS
Cohen, Matharu, Goadsby. IHS, 2007
Topiramate in SUNCT
• Cross-over RCT of topiramate 50 bd vs placebo• Primary endpoint was reduction in attack frequency by 50% • Secondary endpoint was reduction in ‘attack load’ • N=5
Results•Beneficial in 2 – one had complete cessation of attacks, and one had a 71% reduction in attack load. •Placebo response in one•Two had no benefit
SUNCT SUNCT TREATMENTSTREATMENTS
SUNCT SUNCT TREATMENTSTREATMENTS
Hypothalamic StimulatorHypothalamic Stimulator
Leone M, Leone M, Ann NeurolAnn Neurol 2005. 2005.
Hemicrania ContinuaHemicrania ContinuaHemicrania ContinuaHemicrania ContinuaPET Study
Matharu et al, Headache 2004
Posterior Hypothalamus Dorsal Rostral Pons
• Primary headaches can be pathophysiologically differentiated on the basis of Primary headaches can be pathophysiologically differentiated on the basis of distinct patterns of brain activationdistinct patterns of brain activation
• Dorsal pontine and hypothalamic activation are markers of migrainous Dorsal pontine and hypothalamic activation are markers of migrainous symptoms and cranial autonomic features, respectivelysymptoms and cranial autonomic features, respectively
•
• These structures that likely play a pivotal role in the pathophysiology of These structures that likely play a pivotal role in the pathophysiology of primary headache syndromesprimary headache syndromes
• Primary headaches can be pathophysiologically differentiated on the basis of Primary headaches can be pathophysiologically differentiated on the basis of distinct patterns of brain activationdistinct patterns of brain activation
• Dorsal pontine and hypothalamic activation are markers of migrainous Dorsal pontine and hypothalamic activation are markers of migrainous symptoms and cranial autonomic features, respectivelysymptoms and cranial autonomic features, respectively
•
• These structures that likely play a pivotal role in the pathophysiology of These structures that likely play a pivotal role in the pathophysiology of primary headache syndromesprimary headache syndromes
Activation pattern in primary headachesActivation pattern in primary headachesActivation pattern in primary headachesActivation pattern in primary headaches
Functional Neuroimaging of Primary HeadachesFunctional Neuroimaging of Primary HeadachesFunctional Neuroimaging of Primary HeadachesFunctional Neuroimaging of Primary Headaches
MigraineMigraine CHCH SUNCTSUNCT PHPH HCHC
Posterior hypothalamus
Dorsal rostral pons
““Pain is a more Pain is a more terrible lord of terrible lord of mankind than mankind than even death itself” even death itself”
Albert SchweitzerAlbert Schweitzer
““Pain is a more Pain is a more terrible lord of terrible lord of mankind than mankind than even death itself” even death itself”