Epidemiology of NMOSD in Catalonia: Influence of the new 2015 criteria in incidence and prevalence estimates.

BACKGROUND: Population-based studies on neuromyelitis optica spectrum disorders (NMOSD) are limited, and it is unclear whether the rates have changed with the implementation of the new 2015 criteria. OBJECTIVES: To estimate the incidence and prevalence of NMOSD in Catalonia (Spain), using both the 2006 and the 2015 criteria. METHODS: In this clinic-based retrospective study, patients diagnosed with NMOSD between 2006 and 2015 were identified using multiple sources, including direct contact to all Catalan hospitals, identification of cases through the Catalan Health Surveillance System, and registry of antibodies to aquaporin-4 (AQP4-IgG) and myelin oligodendrocyte glycoprotein (MOG-IgG) in a reference laboratory. The incidence rate was calculated for the period 1 January 2006-1 January 2016 and prevalence for the date 1 January 2016. RESULTS: We identified 74 patients (by the 2015 criteria). Most patients were Caucasian (81%), and female (76%) with a median age at disease onset of 42 years (range, 10-76 years). In total, 54 (73%) patients were positive for AQP4-IgG, 11 (15%) double-seronegative, and 9 (12%) MOG-IgG-positive. Rates of incidence and prevalence (0.63/1,000,000 person-years and 0.89/100,000, respectively) were 1.5-fold higher than those reported by the 2006 criteria. Lowest rates were seen in children and elder people and highest in women and middle-aged people (40-59 years). The female predominance was lost in incident AQP4-IgG-seronegative children and AQP4-IgG-positive elder people. MOG-IgG and double-seronegativity contributed similarly but did not influence the long-term outcome. CONCLUSION: The new criteria increase the estimates, but NMOSD remains as a rare disease. The differences in age- and sex-specific estimates highlight the importance of the serologic classification.

Motor Cortex Plasticity during Unilateral Finger Movement with Mirror Visual Feedback.

Plasticity is one of the most important physiological mechanisms underlying motor recovery from brain lesions. Rehabilitation methods, such as mirror visual feedback therapy, which are based on multisensory integration of motor, cognitive, and perceptual processes, are considered effective methods to induce cortical reorganization. The present study investigated 3 different types of visual feedback (direct, mirrored, and blocked visual feedback: DVF, MVF, and BVF, resp.) on M1 cortex excitability and intracortical inhibition/facilitation at rest and during phasic unimanual motor task in 11 healthy individuals. The excitability of the ipsilateral M1 cortex and the intracortical facilitation increased during motor task performance in the DVF and MVF but not in the BVF condition. In addition, MVF induced cortical disinhibition of the ipsilateral hemisphere to the index finger performing the motor task, which was greater when compared to the BVF and restricted to the homologue first dorsal interosseous muscle. The visual feedback is relevant to M1 cortex excitability modulation but the MVF plays a crucial role in promoting changes in intracortical inhibition in comparison to BVF. Altogether, it can be concluded that a combination of motor training with MVF therapy may induce more robust neuroplastic changes through multisensory integration that is relevant to motor rehabilitation.

Effectiveness of transcranial direct current stimulation and visual illusion on neuropathic pain in spinal cord injury.

The aim of this study was to evaluate the analgesic effect of transcranial direct current stimulation of the motor cortex and techniques of visual illusion, applied isolated or combined, in patients with neuropathic pain following spinal cord injury. In a sham controlled, double-blind, parallel group design, 39 patients were randomized into four groups receiving transcranial direct current stimulation with walking visual illusion or with control illusion and sham stimulation with visual illusion or with control illusion. For transcranial direct current stimulation, the anode was placed over the primary motor cortex. Each patient received ten treatment sessions during two consecutive weeks. Clinical assessment was performed before, after the last day of treatment, after 2 and 4 weeks follow-up and after 12 weeks. Clinical assessment included overall pain intensity perception, Neuropathic Pain Symptom Inventory and Brief Pain Inventory. The combination of transcranial direct current stimulation and visual illusion reduced the intensity of neuropathic pain significantly more than any of the single interventions. Patients receiving transcranial direct current stimulation and visual illusion experienced a significant improvement in all pain subtypes, while patients in the transcranial direct current stimulation group showed improvement in continuous and paroxysmal pain, and those in the visual illusion group improved only in continuous pain and dysaesthesias. At 12 weeks after treatment, the combined treatment group still presented significant improvement on the overall pain intensity perception, whereas no improvements were reported in the other three groups. Our results demonstrate that transcranial direct current stimulation and visual illusion can be effective in the management of neuropathic pain following spinal cord injury, with minimal side effects and with good tolerability.

Interferon beta in secondary progressive multiple sclerosis : daily clinical practice.

BACKGROUND AND OBJECTIVE: Observational studies may provide additional information about the behaviour of different drugs in the post-marketing period. We present the data from a cohort of secondary progressive multiple sclerosis (SPMS) patients treated with interferon beta (IFNbeta-1b) at our MS clinic. METHODS: This was an independent, open-label, non-randomised, observational study. Within the period 1998 to 2005, all patients with SPMS who started therapy with IFNbeta-1b at our centre were studied. Each patient was included in a follow-up protocol collecting demographic and baseline clinical data. RESULTS: We studied 146 SPMS patients with a median follow-up of 60 months. Over the total study period, 62.2% of patients had confirmed progression. The analysis of the time to con- firmed progression showed that patients with two or more relapses in the 2 years before IFNbeta initiation, had a higher risk of disability increase than those patients with less than two relapses (p = 0.002). Multiple regression analysis showed disease activity in terms of relapses as the only factor to predict increase of disability during the follow-up period. A significant proportion of patients (36%) stopped treatment during the follow-up period. IFNbeta was safe, although some unexpected adverse events were observed. CONCLUSIONS: A higher disease activity before the beginning of treatment with IFNbeta in SPMS patients with a given EDSS rank could identify those with faster disability progression after treatment initiation.

Carotenoid composition of strawberry tree (Arbutus unedo L.) fruits.

The carotenoid composition of strawberry tree (Arbutus unedo) fruits has been characterised in detail and quantified for the first time. According to the total carotenoid content (over 340 µg/g dw), mature strawberry tree berries can be classified as fruits with very high carotenoid content (>20 µg/g dw). (all-E)-Violaxanthin and 9Z-violaxanthin were found to be the major carotenoid pigments, accounting for more than 60%, responsible for the bright colour of the flesh of ripe fruits. In addition other 5,6-epoxide carotenoids, such as (all-E)-neoxanthin, (9'Z)-neoxanthin (all-E)-antheraxanthin and lutein 5,6-epoxide, together with (all-E)-lutein, (all-E)-zeaxanthin and (all-E)-ß-carotene were found at high levels (>5-20 µg/g dw). The LC-MS (APCI+) analysis of the xanthophyll fraction in their native state (direct extract) revealed that most of them (>90%) were totally esterified with saturated fatty acids (capric, lauric, myristic, palmitic and stearic). Monoesters, homodiesters and heterodiesters of (all-E)-violaxanthin and 9Z-violaxanthin were the major pigments.

Cortical Excitability During Passive Action Observation in Hospitalized Adults With Subacute Moderate to Severe Traumatic Brain Injury: A Preliminary TMS Study.

Studies indicate that motor functions in patients with traumatic brain injury (TBI) can be improved with action observation. It has been hypothesized that this clinical practice relies on modulation of motor cortical excitability elicited by passive action observation in patients with TBI, a phenomenon shown thus far only in normal controls. The purpose of this work was to test this hypothesis and characterize the modulation of motor cortex excitability during passive action observation in patients with subacute moderate to severe TBI. We measured motor evoked potentials induced by single-pulse transcranial magnetic stimulation to the left primary motor cortex and recorded from the contralateral first dorsal interosseus while 20 participants observed videos of static and moving right index finger. Results were compared with those of 20 age-and gender-matched healthy controls. As expected, greater excitability was elicited during moving than static stimuli in healthy subjects. However, this was not observed in patients with TBI. Modulation of motor excitability during action observation is impaired in patients with TBI depending on motor dysfunction, lesion site, and number of days postinjury. These preliminary results suggest a strategy to identify patients in whom action observation might be a valuable neurorehabilitative strategy.

Identification and quantitative analysis of carotenoids and their esters from sarsaparilla (Smilax aspera L.) berries.

The carotenoid composition of sarsaparilla ( Smilax aspera L.) berries has been analyzed for the first time. Lycopene was found to be the main carotenoid (242.44 µg/g fresh wt) in the pulp, followed by ß-carotene (65.76 µg/g fresh wt) and ß-cryptoxanthin (42.14 µg/g fresh wt; including the free and esterified forms). Other minor carotenoids were lycophyll (13.70 µg/g fresh wt), zeaxanthin (8.56 µg/g fresh wt; including the free and esterified forms), lutein (0.94 µg/g fresh wt), and antheraxanthin (0.58 µg/g fresh wt). ß-Cryptoxanthin and zeaxanthin were present in free and esterified forms. ß-Cryptoxanthin was mainly esterified with saturated fatty acids (capric, lauric, myristic, palmitic, and stearic), although a low amount of ß-cryptoxanthin oleate was also detected. In the case of zeaxanthin, only a monoester with myristic acid (zeaxanthin monomyristate) was identified. The diverse carotenoid profile, some with provitamin A activity, together with the relatively high content, up to 375 µg/g fresh wt, makes sarsaparilla berries a potential source of carotenoids for the food, animal feed, and pharmaceutical industries.

Reduction of spasticity with repetitive transcranial magnetic stimulation in patients with spinal cord injury.

OBJECTIVE: Spasticity with increased tone and spasms is frequent in patients after spinal cord injury (SCI). Damage to descending corticospinal pathways that normally exert spinal segmental control is thought to play an important causal role in spasticity. The authors examined whether the modulation of excitability of the primary motor cortex with high-frequency repetitive transcranial magnetic stimulation (rTMS) could modify lower limb spasticity in patients with incomplete SCI. METHODS: Patients were assessed by the Modified Ashworth Scale, Visual Analogue Scale, and the Spinal Cord Injury Spasticity Evaluation Tool (SCI-SET) and neurophysiologically with measures of corticospinal and segmental excitability by the H(max)/M(max), T reflex, and withdrawal reflex. Fifteen patients received 5 days of daily sessions of active (n = 14) or sham (n = 7) rTMS to the leg motor area (20 trains of 40 pulses at 20 Hz and an intensity of 90% of resting motor threshold for the biceps brachii muscle). RESULT: A significant clinical improvement in lower limb spasticity was observed in patients following active rTMS but not after sham stimulation.This improvement lasted for at least 1 week following the intervention. Neurophysiological studies did not change. CONCLUSIONS: High-frequency rTMS over the leg motor area can improve aspects of spasticity in patients with incomplete SCI.

Defining the response to interferon-beta in relapsing-remitting multiple sclerosis patients.

OBJECTIVE: Many patients with multiple sclerosis (MS) are currently receiving treatment with interferon (IFN)-beta. Early identification of nonresponder patients is crucial to try different therapeutic approaches. We investigated various criteria of treatment response to assess which criterion better identifies patients with a poor response. METHODS: We studied relapsing-remitting MS (RRMS) patients treated with IFN-beta and followed them up for at least 2 years. Expanded Disability Status Score was scored every 3 months and relapses were recorded. We analyzed various criteria based on relapses, disability progression, or both. RESULTS: Three hundred ninety-three patients were included. After 2 years of treatment, we observed a proportion of nonresponders, ranging from 7 to 49% depending on the stringency of the criteria used. Criteria based in disability progression had higher sensitivity, specificity, and accuracy. The hazard ratio for the development of marked disability after 6 years of treatment was 39.6 (95% confidence interval, 16.6-94.4) among the patients who fulfilled the criterion based only in disability progression. INTERPRETATION: Criteria of response to IFN-beta therapy in RRMS using disability progression are more clinically relevant than those based only in relapse rate. This finding may be important for the counseling and care of RRMS patients treated with IFN-beta.

Is optic neuritis more benign than other first attacks in multiple sclerosis?

Optic neuritis presentations are thought to have a better prognosis. The aim of our study was to compare conversion to multiple sclerosis on the different topographies of CISs. We prospectively evaluated 320 patients with CISs (123 with optic neuritis, 78 with brainstem syndromes, 89 with spinal cord syndromes, and 30 with other topographies) who were observed for a median of 39 months. Patients underwent brain MRI within 3 months of their first attack and again 12 months later. Conversion to multiple sclerosis determined either clinically or by MRI was evaluated according to topography. Baseline MRI was normal in 49.2% of patients with optic neuritis compared with 24% in brainstem syndromes, 24% in spinal cord syndromes, and 18.5% in other syndromes. Optic neuritis behaved differently from the other CISs for lower conversion to clinically definite multiple sclerosis and smaller proportion of patients fulfilling MRI dissemination in space, time, or both. Nevertheless, when only patients with abnormal cranial MRI results at baseline were selected, no differences for clinical or MRI conversion were found. Optic neuritis has a smaller risk for conversion to multiple sclerosis. Nevertheless, MRI at baseline, not CIS topography, appears to be the crucial issue at multiple sclerosis presentation.

Pronóstico de la polineuropatía secundaria a porfiria aguda intermitente / Prognosis of polyneuropathy associated to intermittent acute porphyria

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