Neurology

Livedo racemosa generalisata: an anthological vision through Vladímir Lébedev painting

Obituary for Prof. Dr. Dr. h.c. Dipl. Ing. Frank Lehmann-Horn

High-intensity training in patients with spinal and bulbar muscular atrophy Abstract Objective Long duration, moderate-intensity exercise is not well tolerated in patients with spinal and bulbar muscular atrophy (SBMA). This study investigated whether patients with SBMA can benefit from high-intensity training (HIT). Methods Ten patients with SBMA were randomized to 8 weeks of supervised HIT [n = 5; age = 50 (25–63) years] followed by 8 weeks of self-training or 8 weeks of no training followed by 8 weeks of non-supervised HIT [n = 5; age = 50 (26–54) years]. Training consisted of 2 × 5-min exercise periods with 1-min cyclic blocks of intermittent maximal intensity exercise on an ergometer bike. Maximal oxygen capacity (VO2max) and workload (Wmax) were measured before and after training by incremental exercise tests. Plasma creatine kinase levels, self-rated muscle pain, muscle fatigue, and activity level were monitored throughout the training period. Results Eight patients completed training. One patient dropped out after 5 weeks of training for private reasons. Another patient was excluded after 4 weeks due to lack of compliance. Eight weeks of training increased both VO2max(1.9 ± 2.3 ml min−1 kg−1; p = 0.04) and Wmax (15.6 ± 17.9 W; p = 0.03) in the 8 patients who completed training. There were no changes in plasma creatine kinase levels, self-reported muscle pain or muscle fatigue activity level after training. Conclusion This pilot study suggests that high-intensity training is safe and improves fitness in patients with SBMA. Unlike low- and moderate-intensity training, HIT is efficacious and favored over other training forms by the patients.

Anticholinergic drug usage and cognitive impairment: findings from three large European cohort studies

James Crichton-Browne (1840–1938)

"Can't seem to keep my mind to it": did Arthur Miller's Salesman have dementia with Lewy bodies? Abstract We present evidence that Willy Loman, the protagonist of Arthur Miller's play "Death of a Salesman", meets current diagnostic criteria for probable dementia with Lewy bodies. In particular, he presents with attentional deficits and executive dysfunction (with additional subtle visuoperceptual deficits) in addition to dramatic fluctuations in attention and vivid visual hallucinations. Dementia with Lewy bodies is the second most common neurodegenerative dementia. However, it is frequently misdiagnosed or underdiagnosed, even—we contest—in one of the most well-known characters in modern literature.

Anti-natalizumab antibodies during 8 years of natalizumab treatment: effect on natalizumab concentration and α4-integrin receptor saturation

Seizures and epilepsy in multiple sclerosis: epidemiology and prognosis in a large tertiary referral center Abstract Background Seizures and epilepsy may substantially add to the burden of disease in multiple sclerosis (MS), whereas the exact prevalence and prognosis of seizures and epilepsy in patients with MS remains largely unknown. Objectives We aimed to investigate the epidemiology and prognosis of seizures and epilepsy in MS. Methods We retrospectively analyzed a cohort of 4078 MS patients from a single tertiary referral clinic. Results After excluding 37 patients with unconfirmed MS and alternative seizure etiologies, we found seizures attributable to MS in 1.5% and epilepsy in 0.9% of patients. 40.4% of patients with a follow-up of at least twelve months experienced only a single seizure and 59.6% had recurring seizures. 39% of patients with recurrent seizures were considered drug-resistant, with 9.7% experiencing status epilepticus. Seizure recurrence after a first seizure depended significantly on the MS subtype and was seen more often if the first seizure occurred simultaneously with a MS relapse than in the absence of a relapse. Conclusion Our study shows a lower number of seizures and epilepsy in MS than previously reported. While a single seizure in MS usually has a good prognosis, relapse-associated seizures and established epilepsy in MS may not be as benign as previously assumed.

Occurrence of cerebral small vessel disease at diagnosis of MPO-ANCA-associated vasculitis Abstract Background Anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) often causes peripheral nervous system impairments. However, little is known about subclinical involvements of the central nervous system in AAV. We investigated the frequency and progression of cerebral small vessel disease (SVD) in patients with AAV. Methods This single-center, case–control study comprised 56 patients with myeloperoxidase (MPO)-ANCA-positive AAV. Cerebral SVD presenting periventricular and deep white matter hyperintensities was assessed using brain magnetic resonance imaging (MRI). Seventy-five patients with non-stroke-associated neurological diseases were employed as controls. Results At clinical diagnosis of MPO-ANCA-positive AAV, the frequency of periventricular hyperintensities in the AAV group was significantly higher than that in the control group (P = 0.014). Shinohara and Fazekas grades of periventricular hyperintensities in the AAV group were significantly higher than those in the control group (P = 0.019 and 0.020, respectively). In the AAV group, atherosclerosis-related factors, such as age and hypertension, were not associated with the Shinohara grades of periventricular hyperintensities, whereas serum CRP levels were significantly associated (odds ratio = 6.000, 95% confidence interval 1.648–21.840, P = 0.004). MRI changes were followed in 23 patients with AAV until 2 years after 6 months of diagnosis. Six of these patients worsened the grades of periventricular hyperintensities, while two of 27 in the control group worsened the grades (P = 0.013). Conclusion Inflammatory events are associated with the occurrence of cerebral SVD before clinical diagnosis of MPO-ANCA-positive AAV. The patients may be continuously exposed to the risk of cerebral SVD after immunosuppressive therapy.

In search of distinct MS-related fatigue subtypes: results from a multi-cohort analysis in 1.403 MS patients Abstract Fatigue is among the most disabling symptoms in patients with multiple sclerosis (PwMS). The common distinction between cognitive and motor fatigue is typically incorporated in self-rating instruments, such as the Chalder Fatigue Questionnaire (CFQ), the Fatigue Scale for Motor and Cognitive Functions (FSMC) or the Modified Fatigue Impact Scale (MFIS). The present study investigated the factor structure of the CFQ, the FSMC and the MFIS utilizing exploratory (EFA) and confirmatory factor analysis (CFA) as well as exploratory structural equation modeling (ESEM). Data of 1.403 PwMS were analyzed, utilizing four samples. The first sample (N = 605) was assessed online and split into two stratified halves to perform EFA, CFA, and ESEM on the CFQ and FSMC. The second sample (N = 293) was another online sample. It served to calculate CFA and ESEM on the CFQ and FSMC. The third sample was gathered in a clinical setting (N = 196) and analyzed by applying CFA and ESEM to the FSMC. The fourth sample (N = 309) was assessed in a clinical setting and allowed to run a CFA and ESEM on the MFIS. Proposed factor structures of all questionnaires were largely confirmed in EFA. However, none of the calculated CFAs and ESEMs could verify the proposed factor structures of the three measures, even with oblique rotation techniques. The findings might have implications for future research into the pathophysiological basis of MS-related fatigue and could affect the suitability of such measures as outcomes for treatment trials, presumably targeting specific sub-components of fatigue.

Alexandros Sfakianakis
Anapafseos 5 . Agios Nikolaos
Crete.Greece.72100
2841026182

6948891480

alsfakia@gmail.com