Putative pathophysiological mechanisms in recurrent hemicrania from aortic dissection: a case report.

BACKGROUND: Transient or permanent neurological symptoms occur in 17-40% of patients with aortic dissection. They can distract from or even mask the underlying life-threatening condition. CASE PRESENTATION: We present the case of a young Caucasian man who consulted for recurrent episodes of stereotyped right-sided sudden-onset severe headache. Upon questioning, he also reported a dull chest pain. Clinical examination and brain magnetic resonance imaging were unremarkable. The concomitant presence of chest pain made us consider aortic dissection. Contrast-enhanced cervico-thoraco-abdominal computerized tomography revealed type A aortic dissection. The patient underwent surgical replacement of the ascending aorta and reported no further episode of headache thereafter. Differential diagnosis of headache in this case includes paroxysmal hemicrania, cluster headache, migraine, trigeminal neuralgia and short lasting unilateral neuralgiform headache with conjunctival injection and tearing. Failure to match diagnostic criteria for any of these primary headache disorders and the resolution of pain episodes following surgery led us to postulate that these new-onset hemicrania episodes were symptomatic of aortic dissection. We hypothesize that aortic wall ischemia could have activated the trigeminovascular system and thereby caused hemicranial pain. Such an effect might be mediated by two different pathways that can be referred to as anatomical and humoral. The humoral hypothesis would posit that ischemia results in synthesis of pro-inflammatory mediators released from the aortic wall into the blood stream, such that they reach the central nervous system and directly stimulate specific receptors. The anatomical hypothesis would imply that pain signals generated by nociceptors in the aortic wall are transferred to the trigeminal ganglion via the cardiac plexus, the first cervical ganglion and the internal carotid nerve such that pain perception is referred to related cranio-cervical dermatomes. CONCLUSION: In cases of isolated headache that does not match key diagnostic criteria for a primary headache entity; a thorough review of systems should be performed to look for symptoms that may indicate symptomatic headache from potentially life-threatening conditions. Neurologists should consider aortic dissection in patients presenting with acute headache and chest pain. Further clinical or experimental studies are required to refute or validate the pathophysiological hypothesis discussed here.

Facial pain.

Facial pain is a debilitating disorder if left untreated. Too often, patients are labeled as having psychopathology when face pain etiology is unclear. These patients are categorized as "atypical," "idiopathic," or "psychogenic." Cases of facial pain involving neuropathic, neurovascular, musculoskeletal, as well as intracranial and extracranial systems will be reviewed. Peripheral and central mechanisms associated with these disorders are used to provide an update of these frequently seen clinical issues.

Cefaleas crónicas diarias: características clínicas y tratamiento / Chronic daily hedaches: clinical characteristics and treatment

El 5% de la población general sufre cefaleas más de 15 días por mes. Dentro de este grupo denominado ¶cefaleas crónicas diarias÷, la mayor parte son migrañas crónicas y si bien una causa común es el abuso de fármacos, se ha podido establecer que estas personas tienen un sistema nervioso más vulnerable a los factores precipitantes de la migraña y que habría una alteración estructural en el funcionamiento neuroquímico cerebral que favorecería el establecimiento de esta situación. Otro hecho importante es la comorbilidad con depresión, pánico, enfermedad bipolar, trastornos obsesivo-compulsivos, síndrome de vejiga irritable, trastornos del sueño o fibromialgia. Al estudiar a estos pacientes, se deben descartar otras posibles causas utilizando los adecuados estudios complementarios. El tratamiento debería contemplar también el manejo de la comorbilidad antes descripta que suele confluir en estos pacientes.(AU)

Five percent of the overall population suffer from headaches over 15 days a month. Within this group called "chronic daily headaches", chronic migraines form the largest part, and despite a common cause of them is medication abuse, it has been possible to establish that people suffering from them have a nervous system which is more vulnerable to the factors that may bring about this situation. Another significant fact is the comorbility with depression, panic, bipolar disorder, obsessive-compulsive disorders, irritable bladder syndrome, sleep disorders or fibromyalgia. When studyng these patients, other possible causes must be dismissed, performing the proper complementary studies. Treatment should also provide for the previously described comorbility that usually converges among these patients.(AU)

Cefaleas crónicas diarias: características clínicas y tratamiento / Chronic daily hedaches: clinical characteristics and treatment

El 5% de la población general sufre cefaleas más de 15 días por mes. Dentro de este grupo denominado “cefaleas crónicas diarias”, la mayor parte son migrañas crónicas y si bien una causa común es el abuso de fármacos, se ha podido establecer que estas personas tienen un sistema nervioso más vulnerable a los factores precipitantes de la migraña y que habría una alteración estructural en el funcionamiento neuroquímico cerebral que favorecería el establecimiento de esta situación. Otro hecho importante es la comorbilidad con depresión, pánico, enfermedad bipolar, trastornos obsesivo-compulsivos, síndrome de vejiga irritable, trastornos del sueño o fibromialgia. Al estudiar a estos pacientes, se deben descartar otras posibles causas utilizando los adecuados estudios complementarios. El tratamiento debería contemplar también el manejo de la comorbilidad antes descripta que suele confluir en estos pacientes.

Five percent of the overall population suffer from headaches over 15 days a month. Within this group called "chronic daily headaches", chronic migraines form the largest part, and despite a common cause of them is medication abuse, it has been possible to establish that people suffering from them have a nervous system which is more vulnerable to the factors that may bring about this situation. Another significant fact is the comorbility with depression, panic, bipolar disorder, obsessive-compulsive disorders, irritable bladder syndrome, sleep disorders or fibromyalgia. When studyng these patients, other possible causes must be dismissed, performing the proper complementary studies. Treatment should also provide for the previously described comorbility that usually converges among these patients.

Functional and structural neuroimaging in trigeminal autonomic cephalalgias.

The trigeminal autonomic cephalalgias (TACs) are a group of primary headache disorders characterized by unilateral trigeminal distribution pain that occurs in association with ipsilateral cranial autonomic features. They include cluster headache, paroxysmal hemicrania, and short-lasting unilateral neuralgiform headache with conjunctival injection and tearing. Until recently, primary headache disorders, including the TACs, were widely considered to be caused by peripheral mechanisms such as vascular changes or neurogenic inflammation. Developments in neuroimaging are revolutionizing our understanding of the pathophysiology of primary headache syndromes. Functional imaging studies have demonstrated hypothalamic activation in all the TACs. Furthermore, neuroimaging studies using voxel-based morphometry and magnetic resonance spectroscopy techniques have demonstrated structural and biochemical alterations, respectively, in the hypothalamus of patients with cluster headache. These studies suggest that the hypothalamus plays a crucial role in the pathophysiology of TACs, thereby supporting the notion that these disorders are primarily due to central rather than peripheral mechanisms.

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.

Chronic paroxysmal hemicrania presenting as recurrent orbital inflammation.

Patients with chronic headache associated with ocular symptoms regularly seek ophthalmologists' opinions. We report an unusual case of chronic paroxysmal hemicrania (CPH), a rare but well-described variant of cluster headache in a female presenting to an eye department with recurrent episodes of severe unilateral periorbital swelling with a chronic history of headaches. Clinical features, review of literature, and therapeutic response to indomethacin helped to establish the diagnosis as CPH. This is the first report of severe orbital inflammation with CPH to our knowledge.

Posterior hypothalamic activation in paroxysmal hemicrania.

OBJECTIVE: 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. METHODS: Seven PH patients were studied using positron emission tomography (PET). Each patient was scanned in three states: (1) acute PH attack-off indomethacin; (2) pain-free-off indomethacin; and (3) pain-free after administration of intramuscular indomethacin 100 mg. The scan images were processed and analyzed using SPM99. RESULTS: The study showed no significant activations during state 1 compared with state 2, but there was relative activation of the pain neuromatrix in both states 1 and 2 compared with state 3. This suggests that there is persistent activation of the pain neuromatrix during acute PH attacks and during interictal pain-free states off indomethacin that is deactivated by the administration of indomethacin. In addition, the untreated PH state was associated with significant activation of the contralateral posterior hypothalamus and contralateral ventral midbrain, which extended over the red nucleus and the substantia nigra. INTERPRETATION: These activated subcortical structures may play a pivotal role in the pathophysiology of this syndrome.