Cluster Headache Flashcards
… headache is a severe primary headache disorder characterised by recurrent unilateral headaches centred on the eye or temporal region.
Cluster headache is a severe primary headache disorder characterised by recurrent unilateral headaches centred on the eye or temporal region.
The lifetime prevalence of … headaches is estimated to be 124 per 100,000 people.
The lifetime prevalence of cluster headaches is estimated to be 124 per 100,000 people.
cluster headaches - onset and gender affected most?
Onset tends to be between the ages of 20-40. Males are affected four times more commonly than females.
The trigeminal autonomic cephalalgias (TACs) are a collection of primary headache disorders characterised by … headache and parasympathetic autonomic features.
The trigeminal autonomic cephalalgias (TACs) are a collection of primary headache disorders characterised by unilateral headache and parasympathetic autonomic features.
TACs are a relatively rare cause of primary headache disorder. There are four distinct forms (cluster headache, paroxysmal hemicrania, short-lasting unilateral neuralgiform headache and hemicrania continua) but they share a number of characteristics:
TACs are a relatively rare cause of primary headache disorder. There are four distinct forms (cluster headache, paroxysmal hemicrania, short-lasting unilateral neuralgiform headache and hemicrania continua) but they share a number of characteristics:
Unilateral pain within the trigeminal distribution
Ipsilateral cranial autonomic features
Paroxysmal hemicrania
Patients with paroxysmal hemicrania (PH) suffer from short (2-30 minutes), severe episodes of unilateral orbital, supraorbital and/or temporal pain. Attacks tend to happen several times over the course of a day.
Ipsilateral autonomic features include conjunctival injection and/or lacrimation, nasal congestion, rhinorrhoea, sweating, miosis/ptosis or eyelid oedema. Alternatively, or in addition, a sense of restlessness or agitation may also be present.
PH resolves completely with the administration of indomethacin (an NSAID).
What is conjunctival injection?
Conjunctival injection or hyperemia is a nonspecific response with enlargement of conjunctival vessels induced by various diseases
Summary of Cluster headache
Cluster headache is a neurological disorder characterized by recurrent, severe headaches on one side of the head, typically around the eye. There are often accompanying autonomic symptoms during the headache such as eye watering, nasal congestion and swelling around the eye, typically confined to the side of the head with the pain. The are typically treated with inhaled oxygen and sumatriptans
Pathology and causes of cluster headache
One sided headache in ophthalmic nerve distribution region with autonomic symptomatology
Hypothalamus involvement - episodic occurrence of cluster attacks
Posterior hypothalamic activation -> secondary trigeminal stimulation -> afferents to nucleus caudalis - projection to thalamus, sensory cortex - pain perception
Hyper-activation of parasympathetic pterygopalatine ganglion -> autonomic symptoms
Cavernous sinus walls inflammation -> decreased venous flow and injury of internal carotid artery sympathetic fibers
Types of cluster headache (2)
Episodic - daily episodes over 6-12 weeks; “clusters”, followed by remission period up to 12 months
Constant - episodes without substantial remission period
Causes of cluster headache and risk factors?
Unknown; possibly genetic causes
Risk factors include male gender, stressful periods, allergic rhinitis, sexual intercourse, tobacco and excessive alcohol use
Complications of cluster headache?
Progresses episodic -> chronic
Signs and symptoms of cluster headache
Cluster headaches are described in the ICHD-3 as:
‘Attacks of severe, strictly unilateral pain which is orbital, supraorbital, temporal or in any combination of these sites, lasting 15-180 minutes and occurring from once every other day to eight times a day. The pain is associated with ipsilateral conjunctival injection, lacrimation, nasal congestion, rhinorrhoea, forehead and facial sweating, miosis, ptosis and/or eyelid oedema, and/or with restlessness or agitation.’
Cluster headaches have been defined in the International Classification of Headache Disorders 3rd edition (ICHD-3).
The following diagnostic criteria are given: (5)
1) At least five attacks fulfilling criteria B-D
2) Severe or very severe unilateral orbital, supraorbital and/or temporal pain lasting 15-180 minutes (when untreated)
3)Either or both of the following:
- at least one of the following symptoms or signs, ipsilateral to the headache:
A) conjunctival injection and/or lacrimation
B) nasal congestion and/or rhinorrhoea
C) eyelid oedema
D) forehead and facial sweating
E) miosis and/or ptosis
- a sense of restlessness or agitation
4) Occurring with a frequency between one every other day and 8 per day
5) Not better accounted for by another ICHD-3 diagnosis.
Cluster headaches can be further classified into two subtypes:
Episodic: refers to patients where multiple attacks occur within periods lasting 7 days to 1 year but with pain-free intervals of at least 3 months between bouts. This is the more common form accounting for 80-90% of cases.
Chronic: attacks occurring over a period of 1 year or longer without a period of remission greater than 3 months. This accounts for around 10-20% of cases.
Differential diagnosis for cluster headache
Other causes of headache, both primary and secondary, should be considered.
Primary = Migraines, Tension-type headache, Other TACs, Other primary headache disorders
Secondary: Trauma Idiopathic intracranial hypertension Subarachnoid haemorrhage Space occupying lesion Giant cell arteritis Infection Drugs and medications Venous sinus thrombosis Malignant hypertension Temporomandibular disorder
Red flags - headache
Severe sudden onset: consider causes like SAH, venous sinus thrombosis, vertebral artery dissection
Progressive or persistent, acute change: consider space-occupying lesions, subdural haematoma
Worse on standing: consider CSF leak
Worse on lying: consider causes of raised ICP; space-occupying lesions, venous sinus thrombosis
Headache characteristics
Severe sudden onset: consider causes like SAH, venous sinus thrombosis, vertebral artery dissection
Progressive or persistent, acute change: consider space-occupying lesions, subdural haematoma
Worse on …: consider CSF leak
Worse on …: consider causes of raised ICP; space-occupying lesions, venous sinus thrombosis
Severe sudden onset: consider causes like SAH, venous sinus thrombosis, vertebral artery dissection
Progressive or persistent, acute change: consider space-occupying lesions, subdural haematoma
Worse on standing: consider CSF leak
Worse on lying: consider causes of raised ICP; space-occupying lesions, venous sinus thrombosis
Precipitating factors in headaches
Recent trauma: consider … … (subacute/chronic)
Triggered by … manoeuvre: consider posterior fossa lesion or Chiari 1 malformation
Recent trauma: consider subdural haematoma (subacute/chronic)
Triggered by Valsalva manoeuvre: consider posterior fossa lesion or Chiari 1 malformation
Associated features in headache - red flag symptoms
Fever, photophobia, neck stiffness: consider …, encephalitis
Papilloedema: consider BIH, … … thrombosis, …-… lesions
Dizziness/vertigo: consider …
Visual changes: consider … … arteritis, glaucoma
Vomiting: consider space-occupying lesions, … … poisoning
Atypical aura: consider sinister cause like …
Fever, photophobia, neck stiffness: consider meningitis, encephalitis
Papilloedema: consider BIH, venous sinus thrombosis, space-occupying lesions
Dizziness/vertigo: consider stroke
Visual changes: consider giant cell arteritis, glaucoma
Vomiting: consider space-occupying lesions, carbon monoxide poisoning
Atypical aura: consider sinister cause like stroke
Patient factors and comorbidities in headache - red flags
Age > …: consider sinister causes such as giant cell arteritis and space-occupying lesions
Age < …: consider evaluation for secondary causes
…: in particular increased risk of malignancy and infection
Active or previous cancer: consider metastatic spread, cancer therapy may increase the risk of infection
Pregnancy: consider causes like pre-eclampsia and venous … …
Age > 50: consider sinister causes such as giant cell arteritis and space-occupying lesions
Age < 10: consider evaluation for secondary causes
Immunodeficiency: in particular increased risk of malignancy and infection
Active or previous cancer: consider metastatic spread, cancer therapy may increase the risk of infection
Pregnancy: consider causes like pre-eclampsia and venous sinus thrombosis
If multiple close contacts present with headache consider …
If multiple close contacts present with headache consider carbon monoxide poisoning
Patients presenting with their first bout of a cluster-like headache should be …
Patients presenting with their first bout of a cluster-like headache should be referred to neurology for further review.
Investigations for cluster headache typically consist of neuroimaging to exclude sinister causes for symptoms. Options include:
MRI brain
CT head
What is often used first-line to terminate acute attacks of cluster headache?
Sumatriptan is often used first-line to terminate acute attacks.
Cluster headache - effect on mental health
The severity and disabling nature of the condition can have profound effects on mental health. People with cluster headaches are at increased risk of depression and suicide. Patients should be regularly screened for mental illnesses and have their suicide risk assessed. Where appropriate referral to specialist services should be arranged.
Identifiable triggers in cluster headache
Some patients have identifiable triggers and where possible patients should avoid these. Both alcohol and smoking can trigger attacks, where appropriate offer cessation support.
Acute management of cluster headache (2)
Triptans: these are 5HT1-receptor agonists that can be taken via the subcutaneous or intranasal route. Subcutaneous or intranasal sumatriptan or intranasal zolmitriptan may be used.
Short burst oxygen therapy: in the absence of contraindications 100% oxygen (12-15L/min) can be administered via a non-rebreather face mask for 15-20 minutes. It is possible to set up oxygen at home.
Traditional analgesic medications like paracetamol, opiates and NSAIDs are not recommended.
Initial option for preventative management of cluster headaches?
Preventative medications can help prevent the number of attacks a patient experiences. The initial option is normally verapamil (a calcium channel blocker).
Other options that may be used under specialist guidance include glucocorticoids, lithium and the monoclonal antibody galcanezumab.
Which calcium channel blocker is usually recommended as preventative management for cluster headaches?
Verapamil
In patients with disease refractory to medical options there are a number of therapies that may aid resolution: (cluster headaches)
(3)
Greater occipital nerve blocks
Deep brain stimulation
Trigeminal nerve compression
