Cluster Headache

A cluster phenotype requires recurrent severe or very severe unilateral orbital, supraorbital, and/or temporal pain lasting 15-180 minutes when untreated. Attacks may occur from one every other day to eight per day; a typical bout consists of recurrent attacks over weeks to months. Cranial autonomic features supporting the diagnosis include conjunctival injection or lacrimation, nasal congestion or rhinorrhea, and eyelid swelling. cks nice org uk+2cks nice org ukassessment: Scenario: Headache - diagnosis - CKS - NICEnice org ukHeadaches in over 12s: diagnosis and managementcks nice org ukClinical features | Diagnosis | Headache - cluster - CKS - NICE

Do not regard a convincing autonomic phenotype as sufficient to exclude secondary disease. Structural lesions can cause trigeminal autonomic cephalalgias and TAC-like syndromes; obtain neuroimaging in all patients with a TAC or TAC-like presentation. For a first cluster bout, discuss neuroimaging with a headache-experienced clinician or neurologist. JAMA+1JAMATrigeminal Autonomic Cephalgias Due to Structural Lesionsnice org ukHeadaches in over 12s: diagnosis and management

Escalate urgently when the painful eye is red and visual symptoms include misty vision or halos: acute narrow-angle glaucoma is a key mimic and is suggested by a semidilated pupil, whereas cluster headache is associated with miosis. A suspected serious secondary headache or red-flag presentation warrants specialist admission or referral rather than outpatient cluster-directed treatment alone. cks nice org uk+1cks nice org ukHeadache - cluster | Health topics A to Z | CKS - NICEnice org ukHeadaches in over 12s: diagnosis and management

Prescribe 100% oxygen at a flow of at least 12 L/min by non-rebreathing mask with reservoir bag for acute attacks. Arrange both home and ambulatory oxygen so the patient can treat attacks at onset rather than depend on emergency care. The European Academy of Neurology gives a strong recommendation for 100% oxygen at at least 12 L/min for 15 minutes. Wiley+1WileyEuropean Academy of Neurology guidelines on the ...nice org ukHeadaches in over 12s: diagnosis and management

Use subcutaneous sumatriptan or a nasal triptan when oxygen is unavailable, inadequate, impractical, or when the patient needs a pharmacologic abortive option. American Headache Society guidance identifies subcutaneous sumatriptan, zolmitriptan nasal spray, and high-flow oxygen as established acute treatments; NICE similarly recommends oxygen and/or a subcutaneous or nasal triptan. Wiley+1WileyTreatment of Cluster Headache: The American Headache ...nice org ukHeadaches in over 12s: diagnosis and management

Do not select slow oral rescue strategies as the primary attack plan when attacks are brief and rapidly escalating. Other acute options discussed in reviews include octreotide, lidocaine, ergotamine, and dihydroergotamine, but injectable sumatriptan and inhaled oxygen are viewed as first-line therapies. NEJMNEJMAcute and Preventive Treatments for Cluster Headache | NEJM Clinician

Separate treatment into acute, transitional, and maintenance phases. Acute therapy terminates individual attacks; transitional therapy is used to reduce attack frequency while a maintenance preventive takes effect; the maintenance goal is sustained suppression of further attacks. ScienceDirect+1ScienceDirectWorld neurology updates: Other primary headache disorder – TreatmentScienceDirectDiagnostic protocols and newer treatment modalities for cluster headache - ScienceDirect

For interim suppression during an active bout, use a short course of high-dose oral corticosteroids or consider a local anesthetic/corticosteroid injection around the greater occipital nerve on the pain side. These strategies are specifically described as interim preventives to reduce attack frequency. ScienceDirectScienceDirectWorld neurology updates: Other primary headache disorder – Treatment

Use verapamil as the main medium- to long-term preventive option. When verapamil is not tolerated, ineffective, or insufficient, alternatives discussed for preventive treatment include lithium, topiramate, melatonin, baclofen, valproic acid, and botulinum-based approaches; evidence and selection vary across these options. NEJM+1NEJMAcute and Preventive Treatments for Cluster Headache | NEJM ClinicianScienceDirectWorld neurology updates: Other primary headache disorder – Treatment

For episodic cluster headache, galcanezumab is FDA-approved. This distinction matters: randomized anti-CGRP monoclonal antibody trials have not met prevention endpoints in chronic cluster headache, so do not extrapolate the episodic approval to chronic disease as an established effective strategy. ScienceDirectScienceDirectLong-term safety, tolerability, and efficacy of eptinezumab in chronic cluster headache (CHRONICLE): an open-label safety trial

Refer drug-resistant chronic cluster headache to a headache center capable of neuromodulation assessment. Hypothalamic stimulation has been reported effective in many patients with chronic, several-times-daily attacks refractory to medication, and occipital nerve neurostimulation has shown promise in chronic drug-resistant disease. NatureNatureCluster headache: pharmacological treatment and neurostimulation | Nature Reviews Neurology

At follow-up, record attacks per day, time to treatment, attack duration, response to oxygen, response to triptan, and emergence of a pain-free interval. This distinguishes an episodic bout entering remission from chronic disease, defined by pain-free periods shorter than 1 month over 12 months. nice org uknice org ukHeadaches in over 12s: diagnosis and management

Reassess the diagnostic branch when the clinical pattern changes, new neurologic or ocular findings emerge, or treatment response becomes discordant with the established syndrome. Because structural lesions can present as TACs or TAC-like syndromes, an atypical or evolving course should not be managed as uncomplicated primary cluster headache without imaging review. JAMAJAMATrigeminal Autonomic Cephalgias Due to Structural Lesions

Refer early for specialist-directed preventive selection when attacks remain frequent despite a complete acute plan, when corticosteroid transition or greater occipital nerve injection is being considered, and when medication-refractory chronic disease raises neuromodulation candidacy. Nature+2NatureCluster headache: pharmacological treatment and neurostimulation | Nature Reviews NeurologyScienceDirectWorld neurology updates: Other primary headache disorder – Treatmentcks nice org ukHeadache - cluster | Health topics A to Z | CKS - NICE