Table of Contents
- Introduction
- Classification
- Episodic Cluster Headache
- Chronic Cluster Headache
- Probable Cluster Headache
- Epidemiology
- Pathophysiology
- Etiology and Risk Factors
- Diagnostic Criteria
- Differential Diagnosis
- Evaluation
- Treatment and Management
- Lifestyle Modifications
- Abortive Therapy
- Transitional Therapy
- Preventive Therapy
- First line
- Second line
- Neuromodulation:
- Surgery
- Conventional Surgery
- Glycerol Injections
- Complications
- Prognosis
- Further Reading
- Bibliography
Primary Category
Headache & Pain
P-Category
Secondary Category
S-Category
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.
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
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.
Further Reading
- Cluster headache - StatPearls - NCBI bookshelf. (2020, June 30). National Center for Biotechnology Information. https://www.ncbi.nlm.nih.gov/books/NBK544241/
- Cluster headache: Background, pathophysiology, etiology. (2021, July 12). Diseases & Conditions - Medscape Reference. https://emedicine.medscape.com/article/1142459-overview
Bibliography
- Diagnosis, pathophysiology, and management of cluster headache. (n.d.). PubMed. https://pubmed.ncbi.nlm.nih.gov/29174963/
- 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
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