M. S. Matharu

Posterior hypothalamic activation in paroxysmal hemicrania.

Paroxysmal hemicrania (PH) is a severe, strictly unilateral headache that lasts 2 to 30 minutes, occurs more than five times daily, is associated with trigeminal autonomic symptoms, and is exquisitely responsive to indomethacin. The purpose of the study was to determine the brain structures active in PH.

Medication-overuse headache in patients with cluster headache

Objective: Medication-overuse headache (MOH) in cluster headache (CH) patients is incompletely described, perhaps because of the relatively low prevalence of CH. Methods: The authors describe a retrospective series of 17 patients (13 men, 4 women) with CH who developed MOH in association with overuse of a wide range of monotherapies or varying combinations of simple analgesics (n = 9), caffeine (n = 1), opioids (n = 10), ergotamine (n = 3), and triptans (n = 14). The series includes both episodic (n = 7) and chronic (n = 10) CH patients. Results: A specific triptan-overuse headache diagnosis was made in 3 patients, an opioid-overuse headache diagnosis was made in 1 patient, and an ergotamine-overuse headache diagnosis was made in 1 patient. In approximately half of the patients (n = 8), the MOH phenotype was a bilateral, dull, and featureless daily headache. In the other 9 patients, the MOH was characterized by at least one associated feature, most commonly nausea (n = 6), exacerbation with head movement (n = 5), or throbbing character of the pain (n = 5). The common denominator in 15 patients was a personal or family history, or both, of migraine. The 2 other patients gave a family history of unspecified headaches. Medication withdrawal was attempted and successful in 13 patients. Conclusions: Medication-overuse headache is a previously underrecognized and treatable problem associated with cluster headache (CH). CH patients should be carefully monitored, especially those with a personal or family history of migraine.

Paroxysmal hemicrania: a prospective clinical study of 31 cases

Paroxysmal hemicrania is a rare syndrome characterized by repeated attacks of strictly unilateral, severe, short-lasting pain occurring with cranial autonomic features. The hallmarks of this syndrome are the relatively short attacks and the exquisite response to indometacin. We describe the phenotype of this condition in a series of 31 patients. The mean duration of attack was 17 min. The mean attack frequency was 11. The distribution of the pain was orbital and temporal in 77% of the patients, retro-orbital in 61%, frontal in 55%, occipital in 42%; although pain was also reported in the vertex, second division of trigeminal nerve, neck, nose, jaw, parietal region, ear, teeth, eyebrow, shoulder (ipsilateral and bilateral), arm and third division of trigeminal nerve. Of the cohort, 87% had lacrimation, 68% had conjunctival injection, 58% rhinorrhoea, 54% nasal congestion, ptosis and facial flushing. Other cranial autonomic features include eyelid oedema, forehead/facial sweating, sense of aural fullness and periaural swelling, miosis, mydriasis and swelling of the cheek. The majority of the patients (80%) were agitated or restless, or both, with the pain and 26% were aggressive. All patients had positive placebo control indometacin test (100-200 mg intramuscularly), or a positive oral indometacin trial or both. We suggest the International Headache Society criteria be revised to remove specification of attack site, and to include the full range of cranial autonomic features. Currently, the sine qua non for paroxysmal hemicrania is a response to indometacin. Since there is no reliable clinical marker of that response we recommend an indometacin test, either orally or by injection for any patient with lateralized discrete attacks of head pain with associated cranial autonomic symptoms.

No change in the structure of the brain in migraine: A voxel-based morphometric study

Migraine is a common, disabling form of primary neurovascular headache. For most of the twentieth century it was regarded as a vascular headache whose primary pathophysiology lay in the cranial vasculature. Functional brain imaging using positron emission tomography has demonstrated activation of the rostral brain stem in acute migraine. Voxel‐based morphometry is a new fully automated whole brain technique that is sensitive to subtle macroscopic and mesoscopic structural differences between groups of subjects. In this study 11 patients suffering from migraine with aura (10 females, one male: 23–52 years, mean 31); 11 controls (10 females, one male: 23–52, mean 31); 17 patients with migraine without aura (16 females, one male: 24–57, mean 34); 17 controls (16 females, one male: 24–57, mean 34) were imaged with high resolution volumetric magnetic resonance imaging. There was no significant difference in global grey or white matter volumes between either patients with migraine and controls, or patients with aura and without aura. This study did not show any global or regional macroscopic structural difference between patients with migraine and controls, with migraine sufferers taken as homogenous groups. If structural changes are to be found, other methods of phenotyping migraine, such as by genotype or perhaps treatment response, may be required to resolve completely whether there is some subtle structural change in the brain of patients with migraine.

TRIGEMINAL AUTONOMIC CEPHALGIAS

The trigeminal autonomic cephalgias (TACs) are a group of primary headache disorders characterised by unilateral trigeminal distribution pain that occurs in association with prominent ipsilateral cranial autonomic features.1 The group comprises cluster headache, paroxysmal hemicrania, hemicrania continua, and short lasting unilateral neuralgiform headache attacks with conjunctival injection and tearing (SUNCT syndrome). Firstly, they must be differentiated from secondary TACs, and other short lasting primary headaches (table 1), and then from each other. The concept of a short lasting headache is naturally somewhat artificial in terms of defining “short”—that said, a typical attack time is less than four hours, in contrast with the major differential diagnosis of migraine in which attacks are usually longer.2 The differentiation between TACs is essential since the treatments are very different. Hemicrania continua is a continuous headache and should be considered in the differential diagnosis of relatively long lasting chronic daily headache (see page ii2).3 View this table: Table 1 Primary short lasting headaches1 The TACs are relatively rare, which is likely to be why they are poorly recognised in primary care. TACs will thus be referred to neurologists eventually, offering an excellent opportunity to diagnose and treat these patients. It is noteworthy that each of the TACs has been seen in paediatric practice. The importance of recognising these syndromes is underscored by their excellent but highly selective response to treatment. Cluster headache (CH) is a strictly unilateral headache that occurs in association with cranial autonomic features and, in most patients, has a striking circannual and circadian periodicity. It is an excruciating syndrome and is probably one of the most painful conditions known to humans. Female patients describe each attack as being worse than childbirth. ### Epidemiology The prevalence of CH is estimated to be 0.1%,4 approximately the same as that of multiple sclerosis in the UK. The …

Prolactinomas, dopamine agonists and headache: two case reports.

Headache is a common problem in patients with pituitary tumours. Small pituitary lesions can cause debilitating headache, suggesting that the size of the pituitary tumour may not be the only causal factor in pituitary‐related headache. We present two cases of prolactinoma‐associated headache. The first case has a clinical diagnosis of short‐lasting unilateral headache attacks with conjunctival injection and tearing (SUNCT). The second case has a clinical diagnosis of hemicrania continua and idiopathic stabbing headache. In each case, the administration of dopamine agonists has led to an exacerbation of symptoms. We review the relevant literature to understand the pathophysiological implications of these cases.

SUNCT syndrome: Prolonged attacks, refractoriness and response to topiramate

Short-lasting unilateral neuralgiform headache with conjunctival injection and tearing (SUNCT) syndrome is a rare form of strictly unilateral headache accompanied by cranial autonomic features. The pain is abrupt in onset, of short duration, stabbing or burning in quality, and accompanied by marked ipsilateral cranial autonomic features, usually lacrimation and conjunctival injection.1 The natural history of the condition is poorly understood. Generally, a clustering pattern is reported, with active phases erratically alternating with remissions. During symptomatic periods, the frequency of attacks varies from less than 1 per day to more than 30 per hour. Functional MRI in spontaneous attacks has demonstrated activation of the ipsilateral posterior hypothalamic gray.2 We describe a patient with SUNCT syndrome with previously unreported responsiveness to topiramate, who has atypical relatively long-lasting pain paroxysms and refractoriness after attacks. A 63-year-old medical practitioner presented with a 3-year history of headache. He described strictly right-sided episodes of severe, shooting or burning pain centered on the temple and forehead with occasional radiation to the orbit. The pain usually lasted 30 to 45 seconds but could range from 2 seconds to 1 minute. He had up to 15 …

SUNCT syndrome secondary to prolactinoma

Short-lasting unilateral neuralgiform headache attacks with conjunctival injection and tearing (SUNCT) syndrome is a rare form of primary headache disorder, although secondary causes, particularly posterior fossa abnormalities, are well known. We report a case of SUNCT syndrome secondary to a prolactinoma. Administration of dopamine agonists led to complete resolution of the SUNCT attacks. This case, together with other similar case reports in the literature, highlight the importance of excluding a diagnosis of pituitary adenoma in all suspected cases of SUNCT syndrome, especially as the headache can precede more classical pituitary symptoms by a considerable period of time. Clinicians managing patients with suspected SUNCT syndrome should elicit a history of symptoms associated with pituitary neoplasms, perform a magnetic resonance imaging scan of the brain and pituitary, and screen for serum hormonal abnormalities.

Symptomatic trigeminal autonomic cephalalgias.

Background:The trigeminal autonomic cephalalgias (TACs) are a group of primary headache syndromes characterized by strictly unilateral head pain that occurs in association with ipsilateral cranial autonomic features. The group includes cluster headache, paroxysmal hemicrania, and short lasting unilateral neuralgiform headache attacks with conjunctival injection and tearing. These syndromes differ in attack duration and frequency as well as the response to therapy. Most of the cases of these syndromes are primary headaches, though numerous symptomatic cases have been described albeit that it is difficult to establish a causal relationship with the underlying pathology in most cases. Review Summary:We reviewed the literature to identify the cases of symptomatic TACs that were likely to be secondary to the reported underlying lesion. We also attempted to identify any clinical features that may be pointers for distinguishing these cases from primary cases and thereby inform the diagnostic workup of these disorders. Conclusion:Forty cases of symptomatic TACs were identified. These symptomatic headaches were associated with atypical phenotypes, abnormal examination, and poor treatment response though a significant minority had a typical presentation. A relatively high proportion of all TACs were secondary to pituitary tumors. It is difficult to draw up guidelines for the diagnostic workup required on the basis of this small retrospective case series. It remains unclear whether every TAC patient requires neuroimaging, though if it is considered then magnetic resonance imaging is the preferred modality. In addition, we suggest that all TAC patients should be carefully assessed for pituitary disease related symptoms but further investigations with magnetic resonance imaging of the pituitary gland and pituitary hormonal profile should only be undertaken in patients with atypical features, abnormal examination, or those resistant to the appropriate medical treatment.

Benign cough headache

Benign cough headache is an uncommon primary headache disorder marked by short- lasting attacks of pain triggered by coughing. Magnetic resonance imaging of the brain is required to assure that the cough headache is truly benign. The aetiology of the pain is unclear, but is probably associated with the brief increased intracranial pressure that attends coughing. We have reviewed the clinical features, aetiology, differential diagnosis, management, and prognosis of benign cough headache.