Annals of neurology
-
Annals of neurology · Apr 2006
Comparative StudyImaging brain damage in first-degree relatives of sporadic and familial multiple sclerosis.
Our objective was to assess brain damage in first-degree relatives of patients with sporadic and familial multiple sclerosis (MS). ⋯ Focal brain abnormalities indistinguishable from those of MS occur in asymptomatic first-degree relatives of MS patients. These are twice more frequent in fMS than in sMS but do not lead to the widespread tissue damage commonly found in MS patients. Although there is a genetic susceptibility to develop brain abnormalities suggestive of focal demyelination in first-degree relatives of MS patients, other factors are probably critical for the development of a diffuse, clinically relevant, pathology.
-
Annals of neurology · Apr 2006
Comparative StudySuppression of cortical spreading depression in migraine prophylaxis.
Topiramate, valproate, propranolol, amitriptyline, and methysergide have been widely prescribed for migraine prophylaxis, but their mechanism or site of action is uncertain. Cortical spreading depression (CSD) has been implicated in migraine and as a headache trigger and can be evoked in experimental animals by electrical or chemical stimulation. We hypothesized that migraine prophylactic agents suppress CSD as a common mechanism of action. ⋯ Our data suggest that CSD provides a common therapeutic target for widely prescribed migraine prophylactic drugs. Assessing CSD threshold may prove useful for developing new prophylactic drugs and improving upon existing ones.
-
Annals of neurology · Mar 2006
Case Reports Comparative StudySporadic onset of erythermalgia: a gain-of-function mutation in Nav1.7.
Inherited erythermalgia (erythromelalgia) is an autosomal dominant disorder in which patients experience severe burning pain in the extremities, in response to mild thermal stimuli and exercise. Although mutations in sodium channel Na(v)1.7 have been shown to underlie erythermalgia in several multigeneration families with the disease that have been investigated to date, the molecular basis of erythermalgia in sporadic cases is enigmatic. We investigated the role of Na(v)1.7 in a sporadic case of erythermalgia in a Chinese family. ⋯ Founder mutations in Na(v)1.7, which can confer hyperexcitability on peripheral sensory neurons, can underlie sporadic erythermalgia.
-
Annals of neurology · Mar 2006
Comparative Study Clinical TrialPosterior 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. ⋯ These activated subcortical structures may play a pivotal role in the pathophysiology of this syndrome.