Cluster-type Headache

Primary Category

Headache & Pain

P-Category

Secondary Category

S-Category

Authors:

Muhammad Umair MD,
Muhammad Roshan Asghar MD,
Junaid Kalia MD

Introduction

Cluster headache is defined as a sudden onset, extremely painful, unilateral headache with at least one autonomic symptom ipsilateral to the headache.

It occurs in cyclical patterns or cluster periods.

The headache frequency ranges from every alternate day up to eight times a day.

It occurs approximately at the same time of the day, most often occurring at night due to circadian periodicity and awakening the patient.

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It is also called trigeminal autonomic cephalgia.

Classification

It is divided into two main types based on the frequency

Episodic Cluster Headache

More common, affecting 85% to 90% of the population.

Daily attacks occur for weeks to months followed by a period of remission lasting months to years.

Typically have 1-2 episodes per year.

Most often occur in spring or autumn seasons.

Chronic Cluster Headache

Affects about 15% to 20% of patients.

Attack lasts more than a year without remission or remission that lasts less than one month.

10% to 20% of these patients will develop drug resistance.

Probable Cluster Headache

Such patients who fulfill all but one criteria of cluster headache.

Epidemiology

Least common type of headache.

Exact prevalence of cluster headache is unknown in the United States.

Cluster headache has a population prevalence of 0.1% and a male/female ratio of 3.5-7:1.

The onset occurs mostly between the ages of 20 to 40 years.

Pathophysiology

Imbalance between the sympathetic and parasympathetic neural system causes cranial-autonomic symptoms.

Hypothalamus has a definitive association with cluster headache as demonstrated by PET scans showing activation of the inferior hypothalamic grey matter during an attack.

Trigeminal autonomic reflex, a part of the parasympathetic system, is also involved leading to conjunctival injection, lacrimation, rhinorrhea, and facial vasodilation.

Calcitonin-gene related-peptide (CGRP) is also involved in cluster headaches.

It may also be caused by sudden release of chemicals; histamine and serotonin.

Defects in central pain pathways and autonomic nervous system lead to dysfunction of supraspinal pain control.

Etiology and Risk Factors

Male gender

Age greater than 30 years

Alcohol consumption, leading to vasodilation

Prior brain surgery or trauma

A family history; first degree relatives are 18 times more likely to be diagnosed

Diagnostic Criteria

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International Classification of Headache Disorders (ICHD-3) Diagnostic Criteria for Cluster Headache

Criteria A:

At least 5 attacks fulfilling the criteria B-D.

Criteria B:

Severe or very severe unilateral orbital, supraorbital and/or temporal pain lasting 15-180 minutes (when untreated).

Criteria C:

Either or both of the following;

At least one of the following symptoms or signs, ipsilateral to the headache:

Conjunctival injection or Lacrimation
Nasal Congestion and/or Rhinorrhea
Eyelid edema
Forehead and facial swelling
Forehead and facial flushing
Sensation of fullness in the ear
Miosis and/or ptosis

A sense of restlessness or agitation

Criteria D:

Attacks have a frequency between one every other day and eight per day for more than half of the time when the disorder is active.

Source: ICHD-3 The International Classification of Headache Disorders 3rd edition. https://ichd-3.org/3-trigeminal-autonomic-cephalalgias/3-1-cluster-headache/

Differential Diagnosis

Migraine

Paroxysmal hemicrania:

Short-Lasting Unilateral Neuralgiform Headaches with Conjunctival Injection and Tearing (SUNCT syndrome)

Tension headache

Trigeminal neuralgia

Evaluation

Refer to

Treatment and Management

Lifestyle Modifications

They can help avoid cluster headaches.

Keeping regular sleep schedule

Alcohol cessation.

Abortive Therapy

Sumatriptan 6 mg, subcutaneous- maximum 2/day.

Intranasal sumatriptan 20 mg or zolmitriptan 5 mg are alternatives.

100% Oxygen via non-rebreather mask at 12-15L/min.

Transitional Therapy

Helps patient through period of crisis while starting preventive therapies

Prednisolone 1mg/kg for 5 -7 days. Tapered over 2 weeks.

Greater Occipital Nerve blocks can also be used

Preventive Therapy

First line

Verapamil, starting dose 240 mg with increments of 80mg

Second line

Lithium and Topiramate.

Less effective options

Gabapentin, Sodium Valproate and Melatonin.

Neuromodulation:

These FDA-approved non invasive devices include

Cefaly, sends signals to the Supraorbital Nerve.

GammaCore, stimulates the Vagus Nerve. Also called as Non-invasive Vagus Nerve Stimulator (nVNS).

Surgery

It is the last resort treatment in patients who don't respond to any medication modality.

Conventional Surgery

The trigeminal nerve- main nerve involved in the pain pathway, is cut behind the ear and around the eye.

Side effects include permanent facial numbness and damage to eyes.

Glycerol Injections

Glycerol injected into the Facial nerve.

It is a safer option as compared to other surgical modalities.

Complications

Absence from work

Absence from school

Poor quality of life

Sleep disturbance

Depression

Anxiety

Aggressive behavior

Suicidal ideations in severe cases

Prognosis

Approximately 25% of the patients will never have a second episode of cluster headaches.

About 15% to 20% of the patients will progress to Chronic Cluster headaches, with around 10% to 20% of them developing drug resistance.

Generally, the cluster headache will resolve in about 15 years time.

Bibliography

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Cluster headache. (n.d.). PubMed. https://pubmed.ncbi.nlm.nih.gov/23939643/

Cluster headache and other TACs: Pathophysiology and neurostimulation options. (n.d.). PubMed. https://pubmed.ncbi.nlm.nih.gov/28128461/

Cluster headache - Symptoms and causes. (2019, June 4). Mayo Clinic. https://www.mayoclinic.org/diseases-conditions/cluster-headache/symptoms-causes/syc-20352080

Cluster headache - Symptoms, causes, and treatment. (2002, March 27). WebMD. https://www.webmd.com/migraines-headaches/cluster-headaches

Cluster headaches: Types, symptoms, and causes. Healthline. https://www.healthline.com/health/cluster-headache

Cluster headache: Background, pathophysiology, etiology. (2021, July 12). Diseases & Conditions - Medscape Reference. https://emedicine.medscape.com/article/1142459-overview

Cluster headache. (2021, September 8). The Migraine Trust. https://migrainetrust.org/understand-migraine/types-of-migraine/other-headache-disorders/cluster-headache/

Cluster headaches. (2017, October 18). nhs.uk. https://www.nhs.uk/conditions/cluster-headaches/

Cluster headaches: Treatment, symptoms, and causes. Medical and health information. https://www.medicalnewstoday.com/articles/172387#treatment

ICHD-3 The International Classification of Headache Disorders 3rd edition. https://ichd-3.org/3-trigeminal-autonomic-cephalalgias/3-1-cluster-headache/

Cluster headache: MedlinePlus medical encyclopedia. (n.d.). MedlinePlus - Health Information from the National Library of Medicine. https://medlineplus.gov/ency/article/000786.htm