Background & Aims
Cluster Headache (CH) is a severe disabling headache disorder characterized by extremely painful, short lasting headache attacks, with ipsilateral autonomic symptoms. CH belongs to the group of trigeminal autonomic headaches. The hypothalamus, the trigeminovascular system and the autonomic nervous system are involved in CH pathophysiology. Acute treatment include triptans and oxygen; bridging therapy is performed with oral corticosteroids and/or nerve blocks; preventive treatment include drugs (like verapamil, lithium) and/or neuromodulation (1,2,3).
Cluster-like Headache (CLH) is a rare syndrome in which CH is associated with another neurological disease, such as vascular, neoplastic and inflammatory pathologies (4,5,6,7).
CLH may be resistant to CH treatments and interventional therapies and/or neuromodulation can be required.
Methods
The patient is a 51-years-old man, smoker (1 pack/day), with hypertension and dyslipidemia. Since the age of 34 years he has been affected by episodic CH (according to the ICHD-3 criteria), with about one bout per year.
The attacks are characterized by excruciating pain (lasting 30-60 minutes) in the left orbital and temporal regions, associated with ipsilateral lacrimation, conjunctival injection, ptosis, eyelid edema and rhinorrhea.
The attacks are responsive to subcutaneous sumatriptan, unfortunately with need for multiple injections per day.
Preventive therapies were partially effective or ineffective.
No neurological abnormalities were observed on examination.
Brain MRI showed two cavernous malformations located in the left thalamus and in the left parietal lobe.
Results
From 34 to 39 years of age CLH was responsive to verapamil.
Since the age of 40 CLH has had an incomplete response to therapy with verapamil, lithium, prednisone, melatonin, topiramate, pregabalin, gabapentin, valproic acid, carbamazepine, clonidine, rotigotine, agomelatin, quetiapine, diazepam.
In the last three years the patient has been successfully treated with nerve/ganglion blocks (ipsilateral greater occipital nerve, supraorbital nerve, sphenopalatine ganglion, and ciliary ganglion) and with ipsilateral greater occipital nerve stimulation.
Conclusions
A diagnosis of CLH was made.
Our patients has attacks that perfectly overlap with CH. Since CLH could mimic primary CH, many Authors recommend to investigate all patients with CH with brain MRI (6).
The cavernous malformation located in the left thalamus may have a role in the pathophysiology of CH symptoms in our case.
In our patient subcutaneous administration of sumatriptan is effective for treating attacks.
Unfortunately the patient become progressively resistant to typical CH preventive treatments.
Deep brain stimulation was not considered in our patient due to the increased risk of intracranial bleeding associated with cavernomas.
Nerve/ganglion blocks and/or neuromodulation have proven effective and safe in managing CLH.
References
1.Hoffmann J , May A. Diagnosis, pathophysiology, and management of cluster headache. Lancet Neurol. 2018 Jan;17(1):75-83.
2.Wei DY, Goadsby P. Cluster headache pathophysiology – insights from current and emerging treatments. Nat Rev Neurol. 2021 May;17(5):308-324.
3.Christoph Diener HC, May A. Drug Treatment of Cluster Headache. Drugs. 2022 Jan;82(1):33-42.
4.Giraud P, Jouanneau E, Borson-Chazot F, Lanteri-Minet M, and Chazot G. Cluster-like headache: literature review. J Headache Pain. 2002 Sep; 3(2): 71–78.
5.Mainardi F, Trucco M, Maggioni F, Palestini C, Dainese F, Zanchin G. Cluster-like headache. A comprehensive reappraisal. Cephalalgia. 2010 Apr;30(4):399-412.
6.Edvardsson B. Symptomatic cluster headache: a review of 63 cases. Springerplus. 2014 Feb 3:3:64.
7.Long R, Zhu Y, Wang A. Cluster headache due to structural lesions: A systematic review of published cases.
Presenting Author
Maria Segneri
Poster Authors
Antonio Carnevale
M.D.
Neurology San Filippo Neri Hospital-Rome
Lead Author
Topics
- Specific Pain Conditions/Pain in Specific Populations: Headache