Massimo Leone

..Cluster headache sine headache should be considered as a subtype of the cluster headache syndrome...

..1%). Acute hypothalamic stimulation is not effective in resolving ongoing CH attacks, suggesting that hypothalamic stimulation acts by complex mechanisms in CH prevention...

..After 42 months (left) and 31 months (right) of follow-up, the patient remains crisis free without the need for pharmacological prophylaxis...

..Among these, the novel approach based on hypothalamic deep brain stimulation is one of the most promising...

..The technique is shedding further light on the pathophysiology of the disease, and is also providing clues to functioning of the hypothalamus itself...

..We found only one new first-degree relative with cluster headache (1/40 = 2.5%). The selective interview may be used with confidence as a means of investigating the hereditary component of cluster headache...

..SUNCT is commonly described as drug resistant. Recent studies report that lamotrigin may be the drug of choice for SUNCT...

..The procedures were well tolerated with no significant adverse events. Hypothalamic DBS is an efficacious and safe procedure to relieve otherwise intractable CCH and SUNCT...

..We report the first patient with SUNCT in whom severe intractable pain (70 per day) was well controlled by electrode implant to and continuous stimulation of the posterior inferior hypothalamus. Ann Neurol 2005;57:925-927...

..New insights into mechanisms of head pain have emerged thanks to neuroimaging obtained in experimentally induced headaches, and during peripheral and central neurostimulation...

..We also propose that only completely drug-resistant (as opposed to partially drug-resistant) patients be considered for neurostimulation unless there are other indications...

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..There are no persistent side effects. Hypothalamic stimulation is an effective, safe, and well-tolerated alternative to surgery for chronic patients with drug-refractory CH...

..The high frequency of side shift in chronic cluster headache should be considered when proposing surgical treatment for severe intractable forms of the disease...

..Future studies might determine whether other areas of the pain matrix are suitable targets for neuromodulation in patients with cluster headache who do not respond to hypothalamic modulation...

..Hypothalamic stimulation is an effective, safe and well tolerated treatment for chronic drug-refractory CH. It appears as a valid alternative to destructive surgical modalities, and has the additional advantage of being reversible...

..The rationale, study results and selection criteria for neurostimulation procedures are also summarized, as are the disadvantages of these procedures...

..Infection, transient loss of consciousness, and micturition syncope have been reported, but treatment interruption usually is not required...

..These preliminary results indicate that hypothalamic stimulation is associated with marked reduction of direct costs in the management of complete drug-resistant chronic CH...

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..Hypothalamic stimulation is more invasive and can be performed only in specialist neurosurgical centers. Published experience suggests that about 60% of patients with chronic CH obtain long-term benefit with hypothalamic stimulation...

..We have proposed that only patients suffering from daily neurovascular headaches in the last 1-2 years, with complete drug-resistance should be proposed for invasive procedures...

..The careful description of the surgical steps may contribute to a standardization of the procedure and make the interpretation of results easier even if obtained in series of patients operated on by different authors...

..These data point to a central pathogenesis for chronic CHs...

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..The authors propose a novel physiopathological theory of SIH based on some anatomical considerations about the spinal venous drainage system...

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..Although the outcomes are encouraging, the invasive nature of the technique and the occurrence of rare but major adverse events have suggested a safer peripheral approach with occipital nerve stimulation (ONS)...

..According to us, the refinement of surgical techniques and of metabolic and functional brain neuroradiological investigations will lead to a refinement of the therapeutical strategies in such patients...

..Hydrocephalus and low-intracranial pressure syndrome should be ruled out in patients showing tonsillar herniation in an MRI study and consulting due to daily headache...

..However before a definition of "refractory" chronic migraine can become established, generally accepted diagnostic criteria and treatment guidelines for this condition need to be developed...

..CONCLUSION: Coexistence of trigeminal neuralgia with various TAC forms suggests a pathophysiological relationship between these short-lasting unilateral headaches...

..Hypothalamus implantation is associated with a small risk of intracerebral haemorrhage and must be performed by an expert neurosurgical team, in selected patients...

..The IHS criteria for headache attributed to SIH could not classify the headache in most of our SIH patients. A revision of the IHS criteria for headache attributed to SIH is necessary...

..Hypothalamic activation might play a part in terminating rather than triggering attacks, and might also give rise to a central permissive state, allowing attacks to take place...

..We describe the development and clinical validation of a patient self-administered tool assessing the quality of multiple sclerosis diagnosis disclosure...

..The most relevant clinical features, the expected MRI findings, and the therapeutic options regarding both conditions are discussed...

..Neuroimaging studies are, therefore, essential for suggesting and confirming the diagnosis...

..DBS of the posterior hypothalamus produced a significant and marked reduction of pain bouts in CH patients and in the SUNCT patient. The attempts to treat atypical facial pain in three patients failed...

..Confirmation of these findings, as well as extension to other forms of behavioral and cognitive-behavioral treatment, is required...

..Both the drugs tested here may exert their effect by restoring a normal inhibitory tone to the pain modulating pathways from the trigemino-vascular system, a circuit putatively implicated in CCH...

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..This paper is intended as an update on the technique as well as a comparison in effectiveness to our prior approach...

..Positioning deep brain stimulators in the posterior hypothalamus may offer a tool to better characterise the activity of this part of the brain in humans...

..Finally, we also tested the same procedure in three patients with refractory atypical facial pain, but unfortunately, they did not respond to this treatment...

..All SIH patients suffered from headache. Early recognition of SIH may avoid dangerous worsening due to delayed diagnosis. Orthostatic headache, the main symptom, suggests the diagnosis...

..If, however, sumatriptan is ineffective at that dose it can be increased to a maximum of 100 mg...

..So far other operations have been performed and the results are encouraging in terms of both pain relief and safety. The efficacy of hypothalamic electrical stimulation provides some hints into cluster headache pathophysiology...

..Clinical aspects and different therapeutic strategies for chronic daily headache patients with medication overuse are considered...

..The present study suggests that in the clinical practice the incidence of benign forms among the painful ophthalmoplegias is more elevate than the symptomatic ones and underlines the need of a specific nosography for benign forms...

..We also found that MIDAS and SF-36 were sensitive to clinical changes in a group of 84 patients with chronic migraine and medication overuse who completed the both questionnaires before and after treatment...

..To assess the responsiveness of the three most used patient reported multiple sclerosis (MS) specific questionnaires: the Functional Assessment of MS (FAMS), the MS Impact Scale (MSIS-29) and the 54 item MS Quality of Life (MSQOL-54)...

..Our results may contribute to a better clinical definition of long-lasting headaches...

..The high percentage of migraine cases in the side-locked unilateral group reflects the high proportion of migraine patients in the studied population...

..Excluding the requirement for response to epidural blood patch (criterion D) and considering headaches appearing within 2 h of sitting or standing up would capture more patients...

..We therefore consider it essential to propose criteria for selecting chronic cluster headache patients for hypothalamic deep brain stimulation before this procedure is undertaken at other academic medical centres...

..Minimal changes in the phrasing and content of the items would be sufficient to render the MIDAS specific for the younger population with headache...

..A more precise definition of clinical criteria for cervicogenic headache vs migraine is, therefore, required...

..Considered in the light of other recent reports of familial cluster headache, these cases suggest that cluster headache can have a genetic component...

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..It will be a task for ENT specialists to clarify the real role of very hypothetical primary forms frequently assigned to diagnosis without a correct rationale...

..Further studies are necessary to clarify the effectiveness of transdermal clonidine in the prophylaxis of episodic cluster headache...

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..Second-degree relatives had an eightfold significantly increased risk. The increased familial risk strongly supports the hypothesis that cluster headache has a genetic component in some families...

..These findings suggest that a basal dysfunction in the L-arginine-NO pathway may be involved in the peripheral mechanisms predisposing subjects with neurovascular headaches to individual attacks...

..For most scales the difference was significant (P < 0.0001, Student's t-test, Bonferroni correction). Our findings suggest that cluster headache has marked functional consequences even when appropriate treatments are used...

..We evaluated two families with CH by linkage analysis to 6q23 region and the mutation scanning of the TAR 1, TAR 3, TAR 4, TAR 5, PNR and GPR58 genes by denaturing high liquid chromatography is in progress in 16 familial cases...

..Instead, the data suggest a hitherto unrecognized functional modulation of the pain processing network as the mode of action of hypothalamic deep brain stimulation in cluster headache...

..This review covers the past 3 years, which have seen remarkable progress in understanding the pathophysiology of circadian headache syndromes and have brought exciting news...

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..These results should be considered rather good because, unlike other frequently used techniques, SPG blockade is not invasive and should therefore always be attempted before submitting patients to more invasive surgical approaches...