Occipital Nerve Stimulation for Refractory Chronic Cluster Headache: A Cost-Effectiveness Study.

INTRODUCTION: Occipital nerve stimulation (ONS) is proposed to treat refractory chronic cluster headache (rCCH), but its cost-effectiveness has not been evaluated, limiting its diffusion and reimbursement. MATERIALS AND METHODS: We performed a before-and-after economic study, from data collected prospectively in a nation-wide registry. We compared the cost-effectiveness of ONS associated with conventional treatment (intervention and postintervention period) to conventional treatment alone (preintervention period) in the same patients. The analysis was conducted on 76 rCCH patients from the French healthcare perspective at three months, then one year by extrapolation. Because of the impact of the disease on patient activity, indirect cost, such as sick leave and disability leave, was assessed second. RESULTS: The average total cost for three months was €7602 higher for the ONS strategy compared to conventional strategy with a gain of 0.07 quality-adjusted life-years (QALY), the incremental cost-effectiveness ratio (ICER) was then €109,676/QALY gained. The average extrapolated total cost for one year was €1344 lower for the ONS strategy (p = 0.5444) with a gain of 0.28 QALY (p < 0.0001), the ICER was then €-4846/QALY gained. The scatter plot of the probabilistic bootstrapping had 80% of the replications in the bottom right-hand quadrant, indicating that the ONS strategy is dominant. The average indirect cost for three months was €377 lower for the ONS strategy (p = 0.1261). DISCUSSION: This ONS cost-effectiveness study highlighted the limitations of a short-time horizon in an economic study that may lead the healthcare authorities to reject an innovative strategy, which is actually cost-effective. One-year extrapolation was the proposed solution to obtain results on which healthcare authorities can base their decisions. CONCLUSION: Considering the burden of rCCH and the efficacy and safety of ONS, the demonstration that ONS is dominant should help its diffusion, validation, and reimbursement by health authorities in this severely disabled population.

Long-Term Efficacy of Occipital Nerve Stimulation for Medically Intractable Cluster Headache.

BACKGROUND: Occipital nerve stimulation (ONS) has been proposed to treat refractory chronic cluster headache (rCCH) but its efficacy has only been showed in small short-term series. OBJECTIVE: To evaluate ONS long-term efficacy in rCCH. METHODS: We studied 105 patients with rCCH, treated by ONS within a multicenter ONS prospective registry. Efficacy was evaluated by frequency, intensity of pain attacks, quality of life (QoL) EuroQol 5 dimensions (EQ5D), functional (Headache Impact Test-6, Migraine Disability Assessment) and emotional (Hospital Anxiety Depression Scale [HAD]) impacts, and medication consumption. RESULTS: At last follow-up (mean 43.8 mo), attack frequency was reduced >50% in 69% of the patients. Mean weekly attack frequency decreased from 22.5 at baseline to 9.9 (P < .001) after ONS. Preventive and abortive medications were significantly decreased. Functional impact, anxiety, and QoL significantly improved after ONS. In excellent responders (59% of the patients), attack frequency decreased by 80% and QoL (EQ5D visual analog scale) dramatically improved from 37.8/100 to 73.2/100. When comparing baseline and 1-yr and last follow-up outcomes, efficacy was sustained over time. In multivariable analysis, low preoperative HAD-depression score was correlated to a higher risk of ONS failure. During the follow-up, 67 patients experienced at least one complication, 29 requiring an additional surgery: infection (6%), lead migration (12%) or fracture (4.5%), hardware dysfunction (8.2%), and local pain (20%). CONCLUSION: Our results showed that long-term efficacy of ONS in CCH was maintained over time. In responders, ONS induced a major reduction of functional and emotional headache-related impacts and a dramatic improvement of QoL. These results obtained in real-life conditions support its use and dissemination in rCCH patients.

Isolated peripheral-type facial palsy due to contralateral precentral gyrus cavernoma hemorrhage.

Facial palsy is a condition frequently encountered in neurological daily practice. Typically, telling apart peripheral facial palsy (PFP) from central facial palsy is easy, and depends on the presence of the upper face involvement. However weakness of eye closure can be seen also in cases of central facial palsies, making the diagnosis more difficult. When the facial involvement is isolated, that constitutes a crucial diagnosis challenge because it can be misdiagnosed as Bell's palsy, particularly in young patients. However, subtle clinical findings such as a predominant involvement of the lower face and the presence of a dissociation of emotional and volitional facial movements points towards a central etiology as in our patient. We report here a case of isolated peripheral-type facial palsy due to acute bleeding of supratentorial cavernoma. To our knowledge, such case has not yet been reported.

Is there a timing for sensitivity to acute cerebral ischemia in migraine patients?

BACKGROUND: Migraine may be a factor of increased cerebral sensitivity to ischemia. Previous studies were conducted within 6 to 72 after stroke onset. We aimed to determine if an accelerated infarct growth exists in migraine patients within the first 4.5 h. METHOD: A retrospective case-control study was conducted where all patients admitted for acute stroke started <4.5 h before and who underwent perfusion CT were assessed. The hypoperfusion and necrosis volumes on initial CT perfusion were analyzed, as well as the final infarct volume on MRI performed within 72 h after admission. A no-mismatch pattern was defined as a ratio necrosis/hypoperfusion volume > 83%. RESULTS: 24 patients with personal history of migraine were identified, 8 of them with aura. The control cohort included 51 patients. No difference was found between groups in terms of demographics, initial severity or outcome or presumed cause of stroke. Mean time to CT scan was 125 min in migraine patients and 127 min in the control group. A no-mismatch pattern was equally found in migraine patients and controls, even after adjustment for age, sex and presence of proximal occlusion (p = .22). The final infarct volume was also similar in both groups. CONCLUSIONS: Migraine patients did not display more no-mismatch pattern than controls within the 4.5 h of stroke onset. This deviates from previous studies and may be due to our earlier time from stroke onset to CT scan. A history of migraine may lead to malignant progression of ischemia but occurring only after several hours.

Trigeminal Neuralgia and Charcot-Marie-Tooth Disease: An Intriguing Association. Lessons From a Large Family Case Report and Review of Literature.

We report a case of familial trigeminal neuralgia (TN) and Charcot-Marie-Tooth disease (CMT) caused by an identified MPZ mutation with a review of previous cases described in the literature. BACKGROUND: The association of TN in CMT patients has previously been reported in a few cases. The pathophysiological link can be detailed with recent use of genetic analysis in CMT. METHODS: We report a large family including 7 members affected by CMT, 4 of whom also presented with TN. We then performed a literature review of literature by search of Pubmed from 1950 to September 2018, using the search terms "trigeminal neuralgia" and "Charcot-Marie-Tooth" and the references of relevant articles. RESULTS: Overall, we found 29 previously published TN cases in 12 CMT families. Among them, only 7 families (69%) included several affected members, suggesting that not all mutations involved in CMT predispose to TN. TN in this context seems to present with specific characteristics, including earlier age of onset, bilateral presentation, and poor tolerance to preventive treatments with gait disturbance exacerbated by the underlying neuropathy. CONCLUSION: This report of familial TN in CMT with identified MPZ mutation highlighted specific characteristics of this association. Considered as a rare association in the literature, it may be underestimated and the clinician should be aware of its specific pattern, including earlier age of onset, bilateral presentation, and poor tolerance to preventive treatments.

Atypical MRI presentation of posterior reversible encephalopathy syndrome with predominant brainstem involvement.

We report here the case of a 80-year-old woman for the assessment of an acute confusional state since 2 days asssociated with diffuse and gradual headache. Brain MRI disclosed isolated hyperintense signal on fluid-attenuated inversion recovery sequence involving the medulla and the right inferior cerebellar peduncle with moderate swelling, consistent with vasogenic oedema, without abnormalities on diffusion-weighted imaging sequence. PRES diagnosis was suspected and antihypertensive therapy was introduced to achieve a blood pressure goal < 140/90 mmHg, allowing a quickly favourable clinical course. Three months later, brain MRI demonstrated a complete clearance of the abnormalities, confirming the PRES diagnosis. Atypical MRI findings are possible with involvement of basal ganglia, brainstem or cerebellum, but in these cases, oedema is most of the time accompanied by the classical parieto-occipital region involvement. Vasogenic oedema strictly unilateral or involving exclusively the brainstem or the cerebellum are very rare and should prompt suspicion for an alternative diagnosis. That constitutes a crucial diagnosis challenge in neurology.

In-hospital delays to stroke thrombolysis: Out of hours versus regular hours and reduction in treatment times through the creation of a 24/7 mobile thrombolysis team.

BACKGROUND: The main aim of this study was to evaluate the impact of the implementation of a mobile thrombolysis team (MTT) on time to thrombolysis treatment depending on patient admission time: regular hours (RH) or out of hours (OH). METHODS: 504 consecutive patients treated with IV tPA or with combined IV tPA and mechanical thrombectomy for acute ischemic stroke were retrospectively included between 1st January 2013 and 31st December 2017. Three sub-periods were identified: 2013-2014, 2015-2016, and 2017 during which patients were treated with the usual care (UC), by the MTT or with UC according to their time of admission, or by the MTT, in the three time periods respectively. We compared in-hospital delays according to patient admission time. RESULTS: In 2013-2014, 133 patients were included. Both median door-to-needle (DTN) and imaging to needle (ITN) times were shorter for patients admitted during RH than OH, respectively 75 min versus 85 min and 52 min versus 57 min (P < 0.05), and the proportion of patients with DTN ≤ 60 min was 23% versus 9% (P < 0.05), respectively. In 2015-2016, 223 patients were included. DTN and ITN times were shorter for patients admitted during RH and treated by the MTT than during OH with UC, respectively 54 min versus 78 min and 24 min versus 47 min (P < 0.001), and the proportion of patients with DTN ≤ 60 min was 64% versus 21% (P < 0.001), respectively. In 2017, there was no difference concerning in-hospital delays regardless of patient admission time (P > 0.05). DISCUSSION: DTN time was significantly longer for patients admitted OH. We suggest that the implementation of an around-the-clock MTT would allow a reduction of in-hospital delays and similar times to thrombolysis treatment regardless of admission time.

Predictive Factors of Headache Resolution After Chiari Type 1 Malformation Surgery.

BACKGROUND: Headache is the main and often isolated symptom of patients with Chiari type 1 malformation (CM1). Classically described as occipital and exacerbated by cough, headaches may be poorly characterized, making it difficult to establish CM1 as the underlying cause. Current guidelines for surgical posterior fossa decompression are undefined. The challenge is to distinguish headaches related to CM1 from headaches coincidentally coexisting with CM1. We aimed to determine predictive factors of headache resolution after surgery and applied to our cohort the Chiari Severity Index, a recently developed predictive prognostic score. METHODS: This retrospective study enrolled 49 patients with CM1 and preoperative headache. Standardized telephone interviews regarding headaches before and after surgery were conducted by the same neurologist; magnetic resonance imaging morphometric analyses were performed by an independent neuroradiologist. Headache resolution was defined as ≥50% reduction in frequency of headache days. RESULTS: Preoperative factors of headache resolution after multivariate analysis were attack duration <5 minutes (P = 0.001), triggering by Valsalva maneuvers (P = 0.003), severe intensity of attack (P = 0.05), occipital location (P = 0.05), and greater number of headache days per month (P = 0.04). These characteristics are part of International Headache Society diagnostic criteria for headache attributed to CM1. No radiologic predictive factor was demonstrated. Postoperative improvement was inversely correlated with Chiari Severity Index. CONCLUSIONS: This study confirms the relevance of International Headache Society criteria to identify headaches related to CM1. We propose their systematic use in a preoperative questionnaire.

Imaging Characteristics of Venous Parenchymal Abnormalities.

BACKGROUND AND PURPOSE: There are few published data on the patterns of parenchymal imaging abnormalities in a context of cerebral venous thrombosis (CVT). The objectives of the present study were to describe the patterns of parenchymal lesions associated with CVT and to determine the lesion sites. METHODS: We included 44 consecutively hospitalized patients with CVT and parenchymal lesions on magnetic resonance imaging. The diagnosis of CVT had been confirmed by magnetic resonance imaging/magnetic resonance venography. Magnetic resonance imaging patterns for CVT were retrospectively analyzed with regard to the lesion's type, shape, and site. RESULTS: The most frequent stroke subtype was hemorrhagic ischemia (in 56.8% of cases), followed by intracerebral hematoma (in 22.72% of cases) and nonhemorrhagic ischemia (in 20.45% of cases). Although there were no significant differences between these 3 groups with regard to the clinical and radiological characteristics, we observed a nonsignificant trend (P=0.08) toward a shorter time interval between hospital admission and magnetic resonance imaging for nonhemorrhagic stroke. The CVT parenchymal abnormalities were centered on 6 main foci and were related to the site of venous occlusion: (1) the inferior parietal lobule (n=20; 44.5%), associated mainly with occlusion of the transverse sinus (n=10) or pure cortical veins (n=10); (2) the inferior and posterior temporal regions (n=10; 22.75%), associated mainly with occlusion of the transverse sinus (n=9); (3) the parasagittal frontal region (n=6; 13.6%), associated mainly with occlusion of the superior sagittal sinus (n=4) or the transverse sinus (n=4); (4) the thalamus (n=5; 11.3%) associated with occlusion of the straight sinus (n=5); (5) the cerebellar hemisphere (n=2; 4.5%), associated in both cases with occlusion of the transverse sinus; and (6) the deep hemispheric regions (n=3; 6.8%), associated with occlusion of the superior sagittal sinus in all cases. CONCLUSIONS: Parenchymal lesions caused by CVT display specific anatomic patterns, which is mainly determined by the site of venous occlusion.

Occipital nerve stimulation improves the quality of life in medically-intractable chronic cluster headache: Results of an observational prospective study.

Background Occipital nerve stimulation (ONS) has been proposed to treat chronic medically-intractable cluster headache (iCCH) in small series of cases without evaluation of its functional and emotional impacts. Methods We report the multidimensional outcome of a large observational study of iCCH patients, treated by ONS within a nationwide multidisciplinary network ( https://clinicaltrials.gov NCT01842763), with a one-year follow-up. Prospective evaluation was performed before surgery, then three and 12 months after. Results One year after ONS, the attack frequency per week was decreased >30% in 64% and >50% in 59% of the 44 patients. Mean (Standard Deviation) weekly attack frequency decreased from 21.5 (16.3) to 10.7 (13.8) ( p = 0.0002). About 70% of the patients responded to ONS, 47.8% being excellent responders. Prophylactic treatments could be decreased in 40% of patients. Functional (HIT-6 and MIDAS scales) and emotional (HAD scale) impacts were significantly improved, as well as the health-related quality of life (EQ-5D). The mean (SD) EQ-5D visual analogic scale score increased from 35.2 (23.6) to 51.9 (25.7) ( p = 0.0037). Surgical minor complications were observed in 33% of the patients. Conclusion ONS significantly reduced the attack frequency per week, as well as the functional and emotional headache impacts in iCCH patients, and dramatically improved the health-related quality of life of responders.

Bath-related thunderclap headache: Case report of a male patient.

Bath-related thunderclap headache (BRTH) is a rare entity, closed to reversible cerebral vasoconstriction syndrome. It is only described in middle-aged women and mainly Asiatic ethnic origins. Role of estrogen is consequently discussed. We report here a case of a 36-year-old man, admitted for five episodes of thunderclap headaches, triggered by hot shower. This is the first male case of BRTH, opposing only a hormonal hypothesis. Furthermore, this African patient consolidates the non-exclusivity of this affection to Asian ethnic origins.

[Cluster headache differential diagnosis]. / Diagnostic différentiel de l'AVF.

Cluster headache is characterized by disabling stereotyped headache. Early diagnosis allows appropriate treatment, unfortunately diagnostic errors are frequent. The main differential diagnoses are other primary or essential headaches. Migraine, more frequent and whose diagnosis is carried by excess, trigeminal neuralgia or other trigemino-autonomic cephalgia. Vascular or tumoral underlying condition can mimic cluster headache, neck and brain imaging is recommended, ideally MRI.