Translation and validation of the arabic version of the revised 15‐item myasthenia gravis quality‐of‐life questionnaire

Introduction: We sought to translate, culturally adapt, and assess the Arabic version of the 15‐item myasthenia gravis quality‐of‐life revised scale (MGQOL15R). Methods: We assessed reliability with Cronbach α; reproducibility with intraclass correlation coefficient (ICC); validity with Spearman correlations for myasthenia gravis (MG)‐specific activities of daily living (MG‐ADL), MG composite (MGC) score, and MG manual muscle test (MG‐MMT) and with MGQOL15R in patients with different disease severity through the Kruskal–Wallis test; and sensitivity to change with Wilcoxon signed‐rank test. Results: In 65 enrolled patients, the mean MGQOL15R score was 10.84 ± 8.11 (α = 0.94, ICC = 0.95). The correlation coefficients between MGQOL15R and MGC, MG‐ADL, and MG‐MMT scores were 0.75, 0.75, and 0.74, respectively (P < 0.001). MGQOL15R scores were significantly higher (worse) in patients with more severe disease at baseline and significantly lower (better) in improved patients at follow‐up. Discussion: The Arabic version of MGQOL15R is valid, reliable, stable, and sensitive to changes. Muscle Nerve 57 : 581–585, 2018     

Correlation of single‐breath count test and neck flexor muscle strength with spirometry in myasthenia gravis

Introduction: Although formal spirometry is the gold standard for monitoring respiratory function in patients with myasthenia gravis (MG), such testing is often delayed or unavailable. There is a need for a simple bedside test that can accurately measure respiratory function. Methods: We conducted a prospective, cross‐sectional, single‐blind study in adults with acetylcholine receptor antibody positive MG. Participants performed the single breath count test (SBCT) and underwent manual muscle strength testing, and a respiratory therapist performed spirometry blinded to SBCT and strength results. Results : Thirty‐one patients, aged 57 ± 19 years participated. SBCT showed significant correlations with forced vital capacity (FVC), negative inspiratory force, and neck flexor strength (P < 0.01). FVC showed significant correlation with neck flexor strength (P = 0.02) but no correlation with shoulder abductor strength. Conclusions: These data suggest that the SBCT and neck flexor strength testing are valuable tools for bedside assessment of respiratory function in MG patients. Muscle Nerve 53 : 134–136, 2016     

Cardiac involvement in Dutch patients with sarcoglycanopathy: A cross‐sectional cohort and follow‐up study

Introduction: The aim of this study is to describe the frequency, nature, severity, and progression of cardiac abnormalities in a cohort of Dutch sarcoglycanopathy patients. Methods: In this cross‐sectional cohort study, patients were interviewed using a standardized questionnaire and assigned a functional score. Electrocardiography (ECG), echocardiography, and 24‐h ECG were performed. Results: Twenty‐four patients with sarcoglycanopathy had a median age of 25 years (range, 8–59 years). Beta blockers were used by 13%, and 17% used angiotensin‐converting enzyme inhibitors. ECG abnormalities were present in 5 (21%), and 4 (17%) fulfilled the criteria for dilated cardiomyopathy (DCM). There were no significant differences in median age or severity of disease between patients with or without DCM. Eleven patients were examined earlier. Median follow‐up time was 10 years. Two of the 11 patients (18%) developed DCM during follow‐up. Conclusions: Seventeen percent of the patients with sarcoglycanopathy were found to have dilated cardiomyopathy. We recommend biannual cardiac monitoring, including ECG and echocardiography. Muscle Nerve 50 : 909–913, 2014     

Impact of apathy on health‐related quality of life in recently diagnosed Parkinson's disease: The ANIMO study

The impact of apathy on health‐related quality of life (HRQOL) in recently diagnosed Parkinson's disease (PD) has not been systematically investigated. The objective of this cross‐sectional survey (ANIMO study) was to examine the contribution of apathy to HRQOL in a Spanish sample of recently diagnosed PD patients. PD patients, diagnosed within 2 years of inclusion, were recruited at 102 outpatient clinics in 82 communities throughout Spain. Apathy was quantified using the Lille Apathy Rating Scale and HRQOL with the EuroQol‐5D questionnaire. A mean EuroQol‐5D index score of 0.89 obtained from population references in Spain was used as the cutoff for this study. The relationship between apathy and the dichotomized EuroQol‐5D index score (<0.89 [lower HRQOL] vs ≥0.89 [reference]) was examined using multiple logistic regression analysis, adjusting for sociodemographic and clinical variables. We consecutively recruited 557 patients (60.3% men) with a mean age of 68.8 ± 9.7 years. Apathy was diagnosed in 291 (52.2%) and was related to problems in each of the EuroQoL dimensions. Apathetic PD patients showed EuroQol‐5D index scores significantly lower than those without apathy (0.64 vs 0.83). In an adjusted model, apathetic PD patients were 2.49 times more likely to have lower HRQOL than nonapathetic patients (odds ratio, 2.49; 95% confidence interval, 1.49–4.15, P < 0.01). Apathy is very common in those with recently diagnosed PD and is one of the major clinical determinants of HRQOL in this disease. It should be one of the primary concerns among clinicians who provide treatment to individuals affected by PD. © 2011 Movement Disorder Society     

Mortality in myasthenia gravis: A nationwide population–based follow‐up study in Denmark

Introduction: In previous studies of myasthenia gravis (MG), increased mortality has been reported. The aim of this study was to estimate mortality in patients with acetylcholine receptor antibody–positive (AChR‐Ab–seropositive) MG in a nationwide population–based, long‐term follow‐up study. Methods: All AChR‐Ab–seropositive MG patients, diagnosed between 1985 and 2005, were identified. Defined by age at diagnosis (≤50 or >50 years), patients were classified as having early‐ or late‐onset MG. For comparison, 10 non‐MG individuals from the general population were matched with each patient. All patients and controls were followed until January 1, 2009. Mortality rates and estimated mortality rate ratios (MRRs) were calculated. Results: Of 702 AChR‐Ab–seropositive MG patients, 302 died during follow‐up. Overall mortality was higher for patients with MG (MRR = 1.41, range 1.24–1.60). In late‐onset women and men, the MRRs were 1.64 (1.36–1.99) and 1.22 (1.02–1.46), respectively. Total MRR was highest during the first 5 years after diagnosis. Conclusions: MG diagnosis is still associated with increased mortality. Muscle Nerve 53 : 73–77, 2016     

International clinimetric evaluation of the MG‐QOL15, resulting in slight revision and subsequent validation of the MG‐QOL15r

Introduction: The MG‐QOL15 is a validated, health‐related quality of life (HRQOL) measure for myasthenia gravis (MG). Widespread use of the scale gave us the opportunity to further analyze its clinimetric properties. Methods: We first performed Rasch analysis on >1,300 15‐item Myasthenia Gravis Quality of Life scale (MG‐QOL15) completed surveys. Results were discussed during a conference call with specialists and biostatisticians. We decided to revise 3 items and prospectively evaluate the revised scale (MG‐QOL15r) using either 3, 4, or 5 responses. Rasch analysis was then performed on >1,300 MG‐QOL15r scales. Results: The MGQOL15r performed slightly better than the MG‐QOL15. The 3‐response option MG‐QOL15r demonstrated better clinimetric properties than the 4‐ or 5‐option scales. Relative distributions of item and person location estimates showed good coverage of disease severity. Conclusions: The MG‐QOL15r is now the preferred HRQOL instrument for MG because of improved clinimetrics and ease of use. This revision does not negate previous studies or interpretations of results using the MG‐QOL15. Muscle Nerve 54 : 1015–1022, 2016     

One‐year follow‐up study of neuropathic pain in chronic inflammatory demyelinating polyradiculoneuropathy

We sought to gather information about frequency and features of neuropathic pain (NeP) in chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) patients and to investigate course of NeP during 1‐year follow‐up. Study included 105 patients diagnosed with CIDP. Patients with diabetes (N = 26) were excluded. NeP was diagnosed by the official guidelines and painDETECT questionnaire (PD‐Q). Medical Research Council Sum Score (MRC‐SS), INCAT disability and sensory scores, and Beck Depression Inventory were also measured. PD‐Q showed presence of NeP in 16 (20%) of 79 CIDP patients and their mean pain was moderate (5.1 ± 3.0 of 10). Diagnostic delay in CIDP patients with NeP was prolonged compared to CIDP patients without NeP (21 ± 28 vs 9 ± 12 months, P < .05). Slowly progressive course of the disease was more frequent in patients with NeP (81% vs 52%, P < .05). Patients with NeP had worse INCAT sensory score (P < .01), INCAT disability score (P < .05), MRC‐SS, as well as worse disease outcome at time of testing (P < .05). Depression was more common in patients with NeP (69% vs 17%, P < .01). During 1‐year follow‐up, majority of our CIDP patients had good control of NeP with gabapentinoids or amitriptyline. NeP was common in our cohort of non‐diabetic CIDP patients. It was associated with worse functional disability, worse sensory deficit, and depression. Special attention should be paid to CIDP patients with NeP because they request additional symptomatic therapy that appeared efficacious in our cohort.     

Tremor severity and age: A cross‐sectional,population‐based study of 2,524 young and midlife normal adults

Mild action tremor occurs in most normal people. Yet this tremor mainly has been studied within the context of advanced age rather than among the vast bulk of adults who are not elderly. Whether this tremor worsens during young and middle age is unknown. Using cross‐sectional data from a large population‐based study of young and midlife normal adults (age range, 18–60 years), we assessed whether increasing age is associated with more severe action tremor. Two thousand five hundred and twenty‐four adults in Araihazar, Bangladesh, drew an Archimedes spiral with each hand. Tremor in spirals was rated (0–3) by a blinded neurologist, and a spiral score (range, 0–6) was assigned. Spiral score was correlated with age (r = 0.06, P = .004). With each advancing decade, the spiral score increased (P = .002) so that the spiral score in participants in the highest age group (age 60) was approximately twice that of participants in the youngest age group (age 18–19); P = .003. In the regression model that adjusted for potential confounders (sex, cigarettes, medications, asthma inhalers, and tea and betel nut use), spiral score was associated with age (P = .0045). In this cross‐sectional, population‐based study of more than 2500 young and midlife normal adults, there was a clear association between age and tremor severity. Although the magnitude of the correlation coefficient was modest, tremor severity was higher with each passing decade. These data suggest that age‐dependent increase in tremor amplitude is not restricted to older people but occurs in all adult age groups. © 2011 Movement Disorder Society     

A randomized,double‐blind,placebo‐controlled phase II study of eculizumab in patients with refractory generalized myasthenia gravis

Introduction: Complement activation at the neuromuscular junction is a primary cause of acetylcholine receptor loss and failure of neuromuscular transmission in myasthenia gravis (MG). Eculizumab, a humanized monoclonal antibody, blocks the formation of terminal complement complex by specifically preventing the enzymatic cleavage of complement 5 (C5). Methods: This study was a randomized, double‐blind, placebo‐controlled, crossover trial involving 14 patients with severe, refractory generalized MG (gMG). Results: Six of 7 patients treated with eculizumab for 16 weeks (86%) achieved the primary endpoint of a 3‐point reduction in the quantitative myasthenia gravis (QMG) score. Examining both treatment periods, the overall change in mean QMG total score was significantly different between eculizumab and placebo (P = 0.0144). After assessing data obtained from all visits, the overall change in mean QMG total score from baseline was found to be significantly different between eculizumab and placebo (P < 0.0001). Eculizumab was well tolerated. Conclusion: The data suggest that eculizumab may have a role in treating severe, refractory MG. Muscle Nerve, 2013     

Prevalence and clinical aspects of immigrants with myasthenia gravis in northern Europe

Introduction: Multiethnic studies can provide etiological clues toward the genetic and environmental influence of a disease. The aim of this study was to determine prevalence and clinical features of myasthenia gravis (MG) in immigrants compared with native patients in 2 population‐based cohorts. Methods: This cross‐sectional study included 843 MG patients (375 from Norway and 468 from the Netherlands). Ethnic background was defined by questionnaires. Results: Among the participating MG patients, 163 of 843 (19.3%) were first or second generation immigrants, mainly from Europe, Asia, and South America. No marked prevalence differences were found between immigrants and native ethnic groups. MG with muscle specific kinase antibodies and MG with thymoma were more frequent in Asian MG immigrants compared with other ethnic groups (8% vs. 0–4%; P < 0.001 and 21% vs. 6–10%; P < 0.001), respectively. Conclusions: Our findings indicate that Asian immigrant MG patients carry genetic factors or environmental/lifestyle factors which contribute to their specific phenotype, even after migration. Muscle Nerve 55 : 819–827, 2017     

ORIGINAL ARTICLE: Investigation of responders and non‐responders to long‐term donepezil treatment

Background: Donepezil is effective in maintaining the cognitive function of patients with mild to moderate Alzheimer's disease (AD). However, not all patients respond to donepezil. In the present study, we examined the clinical features of responders and non‐responders to long‐term donepezil treatment. Methods: The present retrospective study was performed on 95 AD outpatients who had been taking donepezil for ≥2 years. All subjects underwent periodic examinations of cognitive function, namely Mini‐Mental State Examination (MMSE) and Rorschach Cognitive Index (RCI), as well as clinical evaluations using the Clinical Dementia Rating (CDR) scale. Patients were divided into three groups as follows: (i) the ‘maintained’ group (MG), in which the global CDR score was maintained over the ≥2 years of treatment; (ii) the ‘declined’ group (DeG), in which the global CDR score increased one rank over the treatment period; and (iii) the ‘obvious and rapid decline’ group (ORDeG), in which the global CDR score increased two ranks early during the treatment period. Clinical features, treatment outcome, the time lag between a caregiver's recognition of the onset of dementia and the start of treatment, behavioral and psychological symptoms of dementia (BPSD), and cognitive functions were compared between the three groups. Results: Patients in the ORDeG (i.e. non‐responders) were significantly younger and had a longer time lag between the onset of dementia and the start of treatment than patients in the MG (P < 0.05). Of note, patients in the ORDeG had a longer period of executive dysfunction before treatment started than patients in the MG (P < 0.001). Evaluation of cognitive function revealed that mean changes from baseline on the MMSE and RCI were significantly lower for patients in the ORDeG compared with the MG at 8 and 4 months, respectively (P < 0.001 and P < 0.05, respectively). Conclusion: Donezepil non‐responders are likely to be younger and to have a longer time lag between the onset of dementia and the start of treatment, in particular a longer duration of executive dysfunction. Furthermore, the non‐responders do not demonstrate maintenance of cognitive functions in the short term. Thus, the early diagnosis of dementia and prompt initiation of donepezil treatment is indicated for a good outcome. To this end, it is important to educate people to recognize a deterioration of executive function in daily living.     

3,4‐Diaminopyridine is more effective than placebo in a randomized,double‐blind,cross‐over drug study in LEMS

The purpose of this study was to investigate the clinical and electrophysiological efficacy of 3,4‐diaminopyridine (DAP) in patients with Lambert–Eaton myasthenic syndrome (LEMS) in a randomized, double‐blind, cross‐over drug trial. The diagnosis of LEMS was made based on the combination of fluctuating muscle weakness, diminished or absent reflexes, and more than 60% increment of the compound muscle action potential (CMAP) amplitude after brief exercise or 50‐HZ stimulation on a repetitive nerve stimulation (RNS) test. Evaluations were done at baseline, with placebo, and with 3,4‐DAP (up to 75–80 mg/day). Assignment of placebo or 3,4‐DAP was done in a double‐blinded manner. Measurements included subjective symptoms score, objective clinical measurements [LEMS classification, muscle strength score, quantitative myasthenia gravis (QMG) score] and RNS test and single‐fiber electromyography (SFEMG). The differences between placebo and baseline values (placebo change) were compared with the differences between 3,4‐DAP and baseline or placebo values (DAP change). Seven patients with LEMS (QMG score >9) participated in the study. One patient had major side‐effects with 3,4‐DAP and withdrew from the study. Statistically significant efficacy was noted with DAP change (N = 13) compared with placebo change (N = 7) according to the subjective symptoms score (P = 0.01), LEMS classification (P < 0.001), muscle strength score (P < 0.006), QMG score (P = 0.02), and CMAP (P = 0.03). For long‐term treatment, 2 patients preferred 3,4‐DAP, 1 chose guanidine hydrochloride, 1 preferred pyridostigmine, and 2 chose no treatment. A randomized, double‐blind, cross‐over drug trial of 3,4‐DAP showed significant efficacy over placebo in patients with LEMS. As a long‐term treatment, however, not all patients preferred this drug. Muscle Nerve, 2009     

Medial gastrocnemius specific force of adult men with spastic cerebral palsy

Introduction: Muscle weakness determines functional impairment in spastic cerebral palsy (SCP). Measurement of specific force (SF) allows for strength comparison with unimpaired populations (controls) accounting for neural (activation and coactivation), architectural (fascicle length and pennation angle), and structural differences (moment arm length). Methods: Medial gastrocnemius (MG) SF (and its determinants) was assessed in both paretic and non‐paretic legs of 11 men with SCP and 11 age‐matched controls during plantarflexion maximal voluntary isometric contraction (MVIC). Results: SCP fascicles were 28% longer than control fascicles (P < 0.05). Pennation angle of SCP patients was 41% smaller than in controls. The physiological cross‐sectional area of SCP MG patients was 47% smaller than in controls (P < 0.05). There was no difference in SF between controls and SCP patients. Conclusions: Weakness in SCP is primarily attributable to deficits in agonist activation and muscle size; consequently, SF measured in the MG is similar between SCP and controls. Muscle Nerve 56 : 298–306, 2017     

Specific T‐cell proliferation to myelin peptides in relapsing‐remitting multiple sclerosis

Background: The identification of major immunogenic peptides in multiple sclerosis (MS) is of great importance for the development of antigen‐specific therapies. Cellular reactivity against a selected mix of seven myelin peptides was evaluated in vitro. The evolution of this reactivity over time and its correlation with clinical variables was also analysed. Material and methods: Forty‐two patients with MS, 15 with other demyelinating diseases and 40 healthy donors (HD) were studied. Cell proliferation was measured by 3[H] thymidine incorporation into samples obtained at 0, 3, 6 and 12 months of MS patient follow‐up. Results: A positive reaction to the peptide mix was detected in 31 of the 42 patients (74%), 12 of the 40 HD (30%) and 6 of the 15 (40%) patients with other demyelinating diseases. Patients with positive proliferation had greater disability (EDSS score, 3 [1–5.5] vs. 1.0[1–2], P = 0.021), higher number of relapses (7 ± 4.1 vs. 3 ± 1.2, P < 0.001) and shorter time since the last relapse (9 ± 7.5 vs. 32 ± 12.3 months, P = 0.036). After 12 months of follow‐up, cell reactivity was maintained in 33 patients (78%). Conclusion: A high percentage of patients exhibit a significant and maintained reactivity to myelin peptides over time. Therefore, this mix may be useful as a source of antigen in the development of protocols aimed at inducing specific tolerance in MS.     

The impact of silent vascular brain burden in cognitive impairment in Parkinson’s disease

Background and purpose: White matter hyperintensities (WMHs) detected by magnetic resonance imaging (MRI) of the brain are associated with dementia and cognitive impairment in the general population and in Alzheimer’s disease. Their effect in cognitive decline and dementia associated with Parkinson’s disease (PD) is still unclear. Methods: We studied the relationship between WMHs and cognitive state in 111 patients with PD classified as cognitively normal (n = 39), with a mild cognitive impairment (MCI) (n = 46) or dementia (n = 26), in a cross‐sectional and follow‐up study. Cognitive state was evaluated with a comprehensive neuropsychological battery, and WMHs were identified in FLAIR and T2‐weighted MRI. The burden of WMHs was rated using the Scheltens scale. Results: No differences in WMHs were found between the three groups in the cross‐sectional study. A negative correlation was observed between semantic fluency and the subscore for WMHs in the frontal lobe. Of the 36 non‐demented patients re‐evaluated after a mean follow‐up of 30 months, three patients converted into MCI and 5 into dementia. Progression of periventricular WMHs was associated with an increased conversion to dementia. A marginal association between the increase in total WMHs burden and worsening in the Mini Mental State Examination was encountered. Conclusions: White matter hyperintensities do not influence the cognitive status of patients with PD. Frontal WMHs have a negative impact on semantic fluency. Brain vascular burden may have an effect on cognitive impairment in patients with PD as WMHs increase overtime might increase the risk of conversion to dementia. This finding needs further confirmation in larger prospective studies.     

Orodispersible sublingual piribedil to abort OFF episodes: A single dose placebo‐controlled,randomized, double‐blind,cross‐over study

S90049, a novel sublingual formulation of the non‐ergoline D₂‐D₃ agonist piribedil, has a pharmacokinetic profile promising to provide rapid relief on motor signs in Parkinson's disease (PD). We assessed the efficacy and safety of S90049 in aborting OFF episodes responding to subcutaneous apomorphine in PD patients with motor fluctuations. This was a single‐dose double‐blind double‐placebo 3 × 3 cross‐over study. Optimal tested doses were determined during a previous open‐label titration phase (S90049 median dose: 60 mg, apomorphine: 5 mg). Primary endpoint was the maximal change versus baseline in UPDRS motor score (ΔUPDRS III) assessed after drug administration following an overnight withdrawal of antiparkinsonian medications. Thirty patients (age: 60 ± 8 years, PD duration: 12 ± 6 years, UPDRS III OFF: 37 ± 15) participated. S90049 wassuperior to placebo on ΔUPDRS III (?13 ± 12 versus ?7 ± 9 respectively; estimated difference ?5.2, 95% Confidence Interval (CI)[?10.4;0.05], P = 0.05). This was also true for secondary outcomes: number of patients switching from OFF to ON (17 on S90049 vs. 8 on placebo, P = 0.03), time to turn ON (P = 0.013) and duration of the ON phase (P = 0.03). In the 17 patients who switched ON on S90049, ΔUPDRS III was similar on S90049 (?21.2 ± 10.1) and apomorphine (?23.6 ± 14.1) (estimated difference: 4.0 95% CI [?2.9;10.9]). S90049 was well tolerated: no serious or unexpected adverse event occurred. A single dose of up to 60 mg of S90049 given sublingually was superior to placebo in improving UPDRS III and aborting a practical OFF in patients with advanced PD. Testing greater doses might improve response rate. © 2009 Movement Disorder Society     

Coenzyme Q10 and vitamin E deficiency in Friedreich’s ataxia: predictor of efficacy of vitamin E and coenzyme Q10 therapy

Background and purpose: A pilot study of high dose coenzyme Q₁₀ (CoQ₁₀)/vitamin E therapy in Friedreich’s ataxia (FRDA) patients resulted in significant clinical improvements in most patients. This study investigated the potential for this treatment to modify clinical progression in FRDA in a randomized double blind trial. Methods: Fifty FRDA patients were randomly divided into high or low dose CoQ₁₀/ vitamin E groups. The change in International Co‐operative Ataxia Ratings Scale (ICARS) was assessed over 2 years as the primary end‐point. A post hoc analysis was made using cross‐sectional data. Results: At baseline serum CoQ₁₀ and vitamin E levels were significantly decreased in the FRDA patients (P < 0.001). During the trial CoQ₁₀ and vitamin E levels significantly increased in both groups (P < 0.01). The primary and secondary end‐points were not significantly different between the therapy groups. When compared to cross‐sectional data 49% of all patients demonstrated improved ICARS scores. This responder group had significantly lower baseline serum CoQ₁₀ levels. Conclusions: A high proportion of FRDA patients have a decreased serum CoQ₁₀ level which was the best predictor of a positive clinical response to CoQ₁₀/vitamin E therapy. Low and high dose CoQ₁₀/vitamin E therapies were equally effective in improving ICARS scores.     

Physical exercise in myasthenia gravis is safe and improves neuromuscular parameters and physical performance‐based measures: A pilot study

Introduction: Due to the shortage of exercise‐related research in Myasthenia Gravis (MG), there are no consensus guidelines on physical exercise for MG patients. Methods: In this prospective pilot study, 10 MG patients with mild disease performed supervised aerobic and resistance training twice weekly for 12 weeks. The Myasthenia Gravis Composite (MGC) score, compound motor action potential (CMAP), repetitive nerve stimulation, muscle force, physical performance‐based measures, serum levels of interleukin‐6, muscle enzymes, and immuno‐microRNAs miR‐150‐5p and miR‐21‐5p were assessed before and after the training period. Results: Physical exercise was well tolerated, and the MGC score was unchanged. Muscle resistance weights and CMAP amplitudes increased for biceps brachii and rectus femoris muscles, and physical performance‐based measures improved. Muscle enzymes remained normal, whereas disease‐specific microRNAs miR‐150‐5p and miR‐21‐5p were reduced after the training period. Conclusions: We propose that general recommendations regarding physical exercise can be applied safely to well‐regulated MG patients. Muscle Nerve 56 : 207–214, 2017     

Four‐week trunk‐specific rehabilitation treatment improves lateral trunk flexion in Parkinson's disease

People with Parkinson's disease (PD) often have a posture characterized by lateral trunk flexion poorly responsive to antiparkinsonian drugs. To examine the effects of a rehabilitation programme (daily individual 90‐minute‐sessions, 5‐days‐a‐week for 4‐consecutive weeks) on lateral trunk flexion and mobility, 22 PD patients with mild to severe lateral trunk flexion, and 22 PD patients without trunk flexion were studied. Patients were evaluated using the Unified Parkinson's Disease Rating Scale motor subscale (UPDRS‐III) score, and the kinematic behavior of the trunk was recorded by means of an optoelectronic system to determine: a) trunk flexion, inclination and rotation values in the erect standing posture; b) ranges of trunk flexion and inclination during trunk movements. After the treatment, significant decreases in trunk flexion [24°(4) vs. 14°(3), P < 0.001] and inclination in the static condition [23°(5) vs. 12°(4), P < 0.001)] were observed, both of which were maintained at the 6‐month follow up. During the trunk flexion task, a significantly increased range of trunk flexion [64°(15) vs. 83°(15), P < 0.001] was observed; similarly, during the lateral bending task, the range of trunk inclination was found to be significantly increased, both toward the side of the trunk deviation [29°(8) vs. 42°(13), P < 0.01] and toward the contralateral side [14°(6) vs 29°(11), P < 0.01]. No further significant changes were observed at the 6‐month follow‐up. Trunk flexion and inclination values in the upright standing posture correlated slightly with the UPDRS‐III score. Our findings show that significant improvements in axial posture and trunk mobility can be obtained through the 4‐week rehabilitation programme described, with a parallel improvement in clinical status. © 2010 Movement Disorder Society     

Oral corticosteroid therapy and present disease status in myasthenia gravis

Introduction: The aim of this study was to elucidate the effectiveness of oral prednisolone (PSL) according to dosing regimen in 472 patients with myasthenia gravis (MG). Methods: We compared the clinical characteristics and PSL treatment between 226 patients who achieved minimal manifestations (MM) or better and 246 patients who remained improved (I) or worsened, according to the MG Foundation of America postintervention status. Results: Achievement of MM or better at peak PSL dose (odds ratio 12.25, P < 0.0001) and combined use of plasma exchange/plasmapheresis (PE/PP) and/or intravenous immunoglobulin (IVIg) (odds ratio 1.92, P = 0.04) were associated positively, and total PSL dose during the past year (odds ratio 0.17, P = 0.03) was associated negatively with present MM or better status. Conclusions: Higher PSL dose and longer PSL treatment do not ensure better outcome. In the absence of a good response, the PSL dose should be decreased by combining with modalities such as PE/PP or IVIg. Muscle Nerve 51 :692–696, 2015