Cluster-type Headache

Muhammad Umair, MBBS(1), Muhammad Roshan Asghar, MBBS(1), Junaid Kalia(2)
  1. Shaikh Khalifa Bin Zayed Al-Nahyan Medical and Dental College, Lahore
  1. Founder, AINeuroCare.com


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

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

Bibliography

  1. Diagnosis, pathophysiology, and management of cluster headache. (n.d.). PubMed. https://pubmed.ncbi.nlm.nih.gov/29174963/
  1. Cluster headache. (n.d.). PubMed. https://pubmed.ncbi.nlm.nih.gov/23939643/
  1. Cluster headache and other TACs: Pathophysiology and neurostimulation options. (n.d.). PubMed. https://pubmed.ncbi.nlm.nih.gov/28128461/
  1. Cluster headache - Symptoms and causes. (2019, June 4). Mayo Clinic. https://www.mayoclinic.org/diseases-conditions/cluster-headache/symptoms-causes/syc-20352080
  1. Cluster headache - Symptoms, causes, and treatment. (2002, March 27). WebMD. https://www.webmd.com/migraines-headaches/cluster-headaches
  1. Cluster headaches: Types, symptoms, and causes. Healthline. https://www.healthline.com/health/cluster-headache
  1. Cluster headache: Background, pathophysiology, etiology. (2021, July 12). Diseases & Conditions - Medscape Reference. https://emedicine.medscape.com/article/1142459-overview
  1. Cluster headache. (2021, September 8). The Migraine Trust. https://migrainetrust.org/understand-migraine/types-of-migraine/other-headache-disorders/cluster-headache/
  1. Cluster headaches. (2017, October 18). nhs.uk. https://www.nhs.uk/conditions/cluster-headaches/
  1. Cluster headaches: Treatment, symptoms, and causes. Medical and health information. https://www.medicalnewstoday.com/articles/172387#treatment
  1. ICHD-3 The International Classification of Headache Disorders 3rd edition. https://ichd-3.org/3-trigeminal-autonomic-cephalalgias/3-1-cluster-headache/
  1. Cluster headache: MedlinePlus medical encyclopedia. (n.d.). MedlinePlus - Health Information from the National Library of Medicine. https://medlineplus.gov/ency/article/000786.htm
 
A Living Pocketbook of Neurology
A Living Pocketbook of Neurology
Applied, Concise, Practical, Up-to-date, Mobile-friendly, peer-reviewed & Open-Access Pocketbook of Neurology and related clinical specialties
Unleash the Digital Healer in You! >> 🌐 https://junaidkalia.com <<
Unleash the Digital Healer in You! >> 🌐 https://junaidkalia.com <<
Learn Digital Health, AI-in-Healthcare, ValueBased Care & More!
🎯 Follow Junaid Kalia MD [Editor-in-Chief]
"If anyone saved a life, it would be as if he saved the life of all mankind"
"If anyone saved a life, it would be as if he saved the life of all mankind"
An AINeuroCare Academy Project - Coaching Clinicians in Digital Healthcare
💡 Learn! 🎥 YouTube 💌 Newsletter 🎓Academy 📝 Blog
👨‍⚕️ Connect! 👥 Linkedin 👋 Facebook 🕊️ Twitter 📸 Instagram

Disclaimer: For Education purposes only. You must NOT rely on the information on this website as an alternative to medical advice from your healthcare provider

CC BY-NC-ND - Attribution-NonCommercial-NoDerivs - AINeuroCare PLLC ©️