Hello everyone!

As I work at the university, I’m lucky to get an access to some of the published medical journals. During one of my searches, I found a through article which I think is very interesting – Cluster-Like Headache: literature review (J Headache Pain (2002) 3:71–78). Basically it lists many causes for symptoms identical to CH. The CH has disappeared after treating the underlying cause. The authors went through 68 different cases presented by the medical literature between 1980 and 2001.

I strongly believe in finding out about the disease we suffer by ourselves. I also think that a lot of the doctors are so overworked that they won’t have time for the kind of information search that we do. We have the great opportunity to inform our doctors and also the possibility to demand for better care based on these findings. There’s always an underlying cause, but it often remains hidden because of various reasons, e.g. the lack of information by the doctors, the lack of initiative or motivation by the doctors, the lack of funds for the research involved and so on. I believe that many CHs might be because of these causes.

The highlights of the article are here – forgive me for my typos and perhaps some missing literature citations. I went through the article with a yellow marker:

Diagnostic criteria for cluster-like headache:
IHS criteria for cluster headache are met
Physical examination during and outside the attacks is normal
A lesion is located in a site where TVS (trigeminovascular system) can be activated
Headache is cured after treatment of the second disease without recurrence

...orbital burning sensation, moderate intensity of pain and absence of periodicity are frequently depicted in CLH [31, 39]. ...attacks exceeding 180 minutes rule out diagnosis of CLH [25]. ...several cases prove that, even in the presence of IHS criteria for CH, patient examination was abnormal [13, 25]. Clinical examination should be as complete as possible and not limited to face and cranial nerve explorations.


Associated facial diseases are mainly eye lesions, but can also concern facial sinuses and teeth. Orbital exenteration was reported in 6 cases [19], one case being due to cancer and the others to traumatic lesions of the eye.

Another example involving the ocular region in cases of CH is that induced by cutaneous viral infection. Sacquegna et al. [21] reported cases of CLH three months after herpes zoster opthalmicus on the same side as previous viral infection.

Maxillary sinusitis can also be associated with CLH. Takashima et al. [22] described CLH revealing a maxillary sinus infection without any of the usual disorders associated with this disease. ...the authors suggested that the trigeminal and autonomic fibers had been injured by infection.

...an impacted superior wisdom tooth was described as such by Romoli and Cudia [23]. The particularity of this observation is that dental extraction led to complete relief of CLH symptoms that had been present for 15 years. The tooth was within the pterygopalatine fossa, suggesting abnormal pressure or even lesion of the nervus intermedius fibers, the fifth nerve and the sympathetic postganglionic fibers of the superior cervical ganglion.


Cavernous sinus and its vicinity remains one of the most frequent sites where lesions are detected and published for CLH.

Tfelt-Hensen et al. [26], Greve and Mai [13] and Porta-Etassam et al. [14] described several, well documented cases. In these observations, pituitary prolactinomas or growth-hormone producing adenomas were always macroadenomas with suprasellar and laterosellar infiltrations.

In the vicinity of pituitary gland, Hannertz [27] described a parasellar meningioma developing in the left lateral sinus narrowing the cavernous sinus veins. CLH was first episodic, then chronic and later transformed into painful homolateral opthalmoplegia. For 37 years, symptoms were strictly compatible with CH, confirmed by three independent clinicians. ...in this case, as in the previous ones, treatment of meningioma was associated with definitive relief of CLH symptoms prompting Hannertz to suspect a direct link between the two diseases [27].

Trigeminal neurinoma with large extension of the disease into the left cavernous sinus is another etiology for a laterosellar pathology [16]. CLH was typical and systematically located on the same side as main tumor development. One of the interesting points is the extension of the lesion to involve the cisternal part of the trigeminal nerve, the trigeminal ganglion and also contact with the intracavernous portion of the internal carotid.

...Hannertz clearly demonstrated by orbital phlebography that venous drainage in the cavernous sinus was impaired by the tumor.

Infection of the sphenoidal sinus has also been exceptionally reported as a cause of CLH. ...Zanchin et al. [31] described a woman who suffered from fungal sphenoidal sinusitis, first associated with typical CLH but without the usual symptoms associated with sinus disease. The sinusitis due to an aspergilloma was treated with surgery and daily intraconazole. ...the authors hypothesized that CLH symptoms were due to permanent stimulation of the sensory fibers originating from the posterior ethmoidal nerve. Autonomic fibers depending on the sphenopalatine ganglion were also injured by inflammatory cells.


Arteriovenous malformation (AVM), vascular loops, vertebral or carotid aneurysms, arterial dissection or carotid thrombendarterectomy have been reported in cases of CLH. ...in cases of AVM, most observations were not actually CLH as previously defined as the pain was not typical and autonomic symptoms were constantly lacking [32, 33].

Gawen et al. [34] reported a case of CLH localized on the same side as the artery implicated in AVM. When right anterior cerebral artery supplied the AVM, CLH was on the right and then moved to the left side after endovascular embolization. This change was associated with a change in the vessel involved in the vascular malformation. ...onset of CLH was at late adolescence whereas diagnosis of the AVM was made at 28 years of age following subarachnoid hemorrage. ...the authors registered an impairment of blood flow in the vessel supplying the AVM by transcranial Doppler sonography. ...they hypothesized that there was an episodic sensitization of the whole cerebral vasculature due to the modification of blood flow.

...a case where symptoms of typical chronic refractory CH were relieved after surgical decompression of the nervus intermedius.

In eight selected cases of severe chronic CH, the authors conducted a prospective study searching for the presence of an arterial loop. This was found in six cases and treated by microvascular decompression of the nervus intermedius or the trigeminal main sensory root with limited postoperative complications. ...in cases of chronic CH, the presence of a vascular arterial loop should be investigated radiologically but does not indicate symptoms that might suggest their presence.

Vertebral or carotid artery aneurysms are associated in rare cases with CLH [13, 36].

Greve and Mai [13] observed a left aneurysm of the communicating artery which was responsible for subarachnoid hemorrhage. This aneurysm was disclosed after a long pain-free period of typical CLH.

Arterial dissection is one of the less common causes of CLH. Cremer et al. [37] reported a case in which CLH occurred withing two days of a fall responsible for right vertebral dissection. At first, oxygen, sumatriptan, methysergide and valproate worked well in curing or preventing the attacks. ...the usual periodicity and remission observed in CH, as well as the constant efficacy of prophylactic drugs, were rapidly lacking. ...the author suggested a complex central mechanism for attacks implicating other structures such as those located in the cavernous sinus.

Carotid thrombendarterectomy has been described exceptionally in associationg with CLH. ...a patient with a 22-year remission period experienced attacks on the day after carotid thrombendarterectomy. CLH was similar in all ways to that suffered two decades before and was located on the same side as the arterial vascular surgery. ...the authors postulated that CLH was possibly due to a sudden modification of carotid blood flow associated with transitory impairment of autonomic fibers of the face.


De Angelis and Payne [39] reported a leptomeningeal lymphoma in which CLH accompanied the meningeal localization of the disease.

...other examples of tumoral diseases associated with CLH have been published. (...)  In most, clinical examination disclosed abnormalities requiring radiological tests [15, 17].

Midline intracranial calcification with extension into the third ventricle is another isolated example of a disorder showing CLH symptoms [40]. CH was typical in early years and diagnosed as chronic. ...onset of disease was at the age of 60 years and CH seemed to be first chronic and then episodic.

Multiple sclerosis (MS) is a rare etiology of CLH because there are frequently neurological signs associated with pain. (...) Leandri et al. [41] reported a case in which CLh was the first sign of MS. The description adheres exactly to the IHS criteria and preventive therapy was also effective. A demyelination plaque was found by MRI in the pons at the trigeminal root entry zone.

Subclavian steal phenomenon was reported by Piovesan et al. [42] as a cause of CLH. (...) Safenous vein bypass cured abnormal blood circulation and, 3 years later, no CLH was noted. The physiopathological mechanism suggested by the authors is based on hypoxia and increased blood flow in the internal carotid arteries [42]. This would provoke venous engorgement in the cavernous sinus and therefore CLH.

Used literature:

13. Greve E, Mai J (1988) Clusterheadache
like headaches: a symptomatic
feature? A report of three
patients with intracranial pathologic
findings. Cephalalgia 8:75–82

14. Porta-Etassam J, Ramos-Carrasco A,
Berbel-Garcia A, Martinez-Salio A,
Benito-Leon J (2001) Clusterlike
headache as first manifestation of prolactinoma.
Headache 41:723–725

15. Kuritzky J (1984) Cluster headache
like pain caused by an upper cervical
meningioma. Cephalalgia 4:185–186

16. Masson C, Lehericy S, Guillaume B,
Masson M (1995) Cluster-like
headache in a patient with a trigeminal
neurinoma. Headache 35:48–49

17. Taji J, Sas K, Szok D, Vörös E, Vecsei
L (1996) Clusterlike headache as the
first sign of brain metastases of lung
cancer. Headache 36:259–260

19. Evers S, Soros P, Brilla R, Gerding H,
Husstedt IW (1997) Cluster headache
after orbital exenteration. Cephalalgia
17:680–682

21. Sacquegna T, D’Allesandro R, Cortelli
P, De Carolis P, Baltrati A (1982)
Cluster headache after herpes zoster
ophthalmicus. Arch Neurol 39:384

22. Takeshima T, Nishikawa S, Takahashi
K (1988) Cluster headache like symptoms
due to sinusitis: evidence for a
neuronal pathogenesis of cluster
headache syndrome. Headache
28:207–208

23. Romoli M, Cudia G (1988) Cluster
headache due to an impacted superior
wisdom tooth: case report. Headache
28:135–136

25. Milos P, Havelius U, Hindfelt B (1996)
Clusterlike headache in a patient with
pituitary adenoma, with review of literature.
Headache 36:184–188

27. Hannertz J (1989) A case of parasellar
meningioma mimicking cluster
headache. Cephalalgia 9:265–269

31. Zanchin G, Rossi P, Licanadro AM,
Fortunato M, Maggioni F (1995)
Cluster headache, a case of sphenoidal
aspergilloma. Headache
35:494–497

32. Testa D, Frediani F, Bussone G (1988)
Cluster headache like syndrome due to
arteriovenous malformation. Headache
28:36–38

33. Hindfelt B, Olivecrona H (1991)
Cerebral arteriovenous malformation
and cluster like headache. Headache
31:514–517

34. Gawel MJ, Willinsky RA, Krajewski
(1989) A reversal of cluster headache
side following treatment of arteriovenous
malformation. Headache
29:453–454

36. West P, Todman D (1991) Chronic
cluster headache associated with vertebral
artery aneurysm. Headache
31:210–212

37. Cremer PD, Halmagyi GM, Goadsby
PJ (1995) Secondary cluster headache
responsive to sumatriptan. J Neurol
Neurosurg Psychiatry 59:633–634

39. De Angelis LM, Payne R (1987)
Lymphomatous meningitis presenting
as atypical cluster headache. Pain
30:211–216

40. Narbone MC, D’Amico D, Di Maria F,
Arena MG, Longo M (1991) Clusterlike
headache and a median intracranial
calcified lesion: case report.
Headache 31:684–685

41. Leandri M, Cruccu G, Gottlieb A
(1999) Cluster headache-like pain in
multiple sclerosis. Cephalalgia
19:732–734

42. Piovesan EJ, Lange MC, Werneck LC,
Kowacs PA, Engelhorn AL (2001)
Cluster-like headache. A case secondary
to the subclavian steal phenomenon.
Cephalalgia 21:850–851


My best regards and PFDAN for everyone,
(let's keep the doctors busy!),
Rosa

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the more you know
thebb

Couple of more examples of misleading Dx:


Can J Ophthalmol. 2008 Aug;43(4):432-4.
Characteristics of patients presenting with ocular pain.

Wolfe S, Van Stavern G.

Kresge Eye Institute, Wayne State University, Detroit, MI, USA. swolfe@med.wayne.edu

BACKGROUND: The purpose of this study was to assess the utility of neuroimaging in patients with normal neuro-ophthalmic examinations referred to a neuro-ophthalmology clinic for complaints of chronic isolated eye pain. We were also interested in the prevalence of primary headache syndromes in these patients, particularly medication overuse headache (MOH). METHODS: Charts of 125 consecutive patients referred to a neuro-ophthalmology clinic with some form of head or facial pain were reviewed to find patients meeting the criteria of having complaints of chronic eye with normal neuro-ophthalmic examinations. Excluded were patients with primary complaint other than eye pain. A single examiner performed all examinations in a standardized fashion. Each patient was queried about the use of prescription and over-the-counter analgesics. RESULTS: Seventy-three percent of patients had some form of primary headache syndrome, and 21% of patients had some component of probable MOH. Another 4.5% had a presumed component of medication overuse. None of those who received imaging had any results explaining the ocular pain. INTERPRETATION: PRIMARY HEADACHE IS PRESENT IN MOST PATIENTS WITH CHRONIC, ISOLATED OCULAR PAIN. MOH IS COMMON AND PROBABLY UNDERRECOGNIZED IN THIS POPULATION. IT NEEDS TO BE ADDRESSED IF THE PATIENT IS EVER TO BE PAIN FREE. NEUROIMAGING HAS VERY LOW YIELD IN THIS GROUP OF SUBSPECIALTY REFERRAL PATIENTS.

Publication Types:
Research Support, Non-U.S. Gov't

Neurol Sci. 2008 Oct;29(5):331-5. Epub 2008 Oct 21.
Symptomatic cluster-like headache triggered by forehead lipoma: a case report and review of the literature.

Cologno D, Buzzi MG, Cicinelli P, Caltagirone C, Spalletta G.

Clinical Neurophysiology, Department of Neuroscience, Azienda Ospedaliero-Universitaria "Ospedali Riuniti", Foggia, Italy.

We describe the case of a patient with symptomatic strictly unilateral paroxysmal headache mimicking cluster headache related to an ipsilateral forehead lipoma. Interestingly, immediately after the surgical excision of the lipoma pain attacks disappeared with no recurrence during a follow-up period of 18 months. Like other descriptions of cluster-like headaches secondary to extracranial lesions, this case report focuses on the hypothetical role of a peripheral trigger factor for trigeminal autonomic cephalgias (TACs). To our knowledge, this is the first well-described cluster-like headache case secondary to an extracerebral lipoma. This case offered the opportunity to discuss the possible pathophysiological mechanisms underlying probable TACs and the relationship with peripheral extracerebral activation of the trigeminal-autonomic reflex.

PMID: 18941935 [PubMed - in process]

Thats why its so important to get a MRI , it rules out most if not all of these other possible causes.