Journal of neurology
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Journal of neurology · Mar 2014
Case ReportsSpinal cord stimulation for gait impairment in spinocerebellar ataxia 7.
The aim of this study is to report on the clinical efficacy of epidural thoracic spinal cord stimulation on gait and balance in a 39-year-old man with genetically confirmed spinocerebellar ataxia 7. A RESUME Medtronic electrode was placed at the epidural T11 level. Spatiotemporal gait assessment using an electronic walkway and static posturography were obtained and analyzed in a blinded manner with and without stimulation. ⋯ Thoracic epidural spinal cord stimulation had a mild but clinically meaningful beneficial effect in improving gait and balance in a patient with SCA-7. The underlying pathophysiologic mechanisms remain to be elucidated. Further experience with spinal cord stimulation in refractory gait disorders is warranted.
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Journal of neurology · Mar 2014
Case ReportsPatients with unruptured intracranial aneurysms at the waiting list for intervention: risk of rupture.
Unruptured intracranial aneurysms are usually not managed on an emergency basis, although for patients, uncertainty and waiting can be stressful. We assessed the incidence of aneurysms rupturing during the initial period of awareness of having an aneurysm. We studied all patients visiting our service with an unruptured intracranial aneurysm between January 2000 and March 2013. ⋯ We compared this incidence with expected incidence (based on size and site for each aneurysm). 398 patients were included; five had aneurysmal rupture during the exposure time. The observed incidence of aneurysmal rupture during exposure time was 47/1,000 patient-years (95 % confidence interval 15-111); the expected incidence was 0.7/1,000. Our data suggest that the risk of aneurysmal rupture early after detection of unruptured aneurysms is higher than expected based on aneurysm characteristics.
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Journal of neurology · Mar 2014
Time intervals from aneurysmal subarachnoid hemorrhage to treatment and factors contributing to delay.
In the management of aneurysmal subarachnoid hemorrhage (aSAH), aneurysm treatment as early as feasible is mandatory to minimize the risk of a rebleed and may thus improve outcome. We assessed the different time intervals from the first symptoms of aSAH to start of aneurysm treatment in an effort to identify which factors contribute mostly to a delay in time to treatment. In 278 aSAH patients, time intervals between the different steps from initial hemorrhage to aneurysm treatment were retrospectively reviewed, and delaying factors were determined. ⋯ Independent factors predicting delay to treatment were primary presentation at a referring hospital and admission to the treatment center later in the day. Delay in treatment was not independently related to poor outcome. The interval to aneurysm treatment might be improved upon by immediate and direct transport to the treatment center combined with optimization of in-hospital logistics, following the 'time-is-brain' concept so successfully adopted in the treatment of ischemic stroke.
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Journal of neurology · Mar 2014
Update on laser-evoked potential findings in fibromyalgia patients in light of clinical and skin biopsy features.
In fibromyalgia (FM), reduced habituation of laser-evoked potentials (LEPs) suggests a dysfunction of pain processing at a central level. In this study, we aimed to further examine the nociceptive pathways at the peripheral to the central level in a large group of FM patients by means of LEPs and skin biopsy, in light of healthy controls findings and main clinical features. One hundred and ninety-nine FM patients and 109 age- and sex-matched controls were submitted to LEPs by the dorsum of the right hand and the skin over the right chest and knee tender point stimulation. ⋯ In the FM group, LEPs habituation was correlated with pain at tender points and bad quality of life. Epidermal fiber density was significantly reduced in FM patients versus controls, and correlated with N2-P2 amplitude by the hand and chest tender-point stimulation. Dysfunction in the nociceptive system at both the central and peripheral levels may concur to explain phenotypical eterogeneity and clinical symptom complexity in fibromyalgia.
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Journal of neurology · Mar 2014
Randomized Controlled TrialPatient subgroup analyses of the treatment effect of subcutaneous interferon β-1a on development of multiple sclerosis in the randomized controlled REFLEX study.
The REFLEX study (NCT00404352) established that subcutaneous (sc) interferon (IFN) β-1a reduced the risks of McDonald MS (2005 criteria) and clinically definite multiple sclerosis (CDMS) in patients with a first clinical demyelinating event suggestive of MS. The aim of this subgroup analysis was to assess the treatment effect of sc IFN β-1a in patient subgroups defined by baseline disease and demographic characteristics (age, sex, use of steroids at the first event, classification of first event as mono- or multifocal, presence/absence of gadolinium-enhancing lesions, count of <9 or ≥9 T2 lesions), and by diagnosis of MS using the revised McDonald 2010 MS criteria. Patients were randomized to the serum-free formulation of IFN β-1a, 44 μg sc three times weekly or once weekly, or placebo, for 24 months or until diagnosis of CDMS. ⋯ McDonald 2010 MS was retrospectively diagnosed in 37.7 % of patients at baseline. Both regimens of sc IFN β-1a significantly reduced the risk versus placebo of McDonald 2005 MS and CDMS, irrespective of McDonald 2010 status at baseline (risk reductions between 29 and 51 %). The effect of sc IFN β-1a was not substantially influenced by baseline patient demographic and disease characteristics, or baseline presence/absence of McDonald 2010 MS.