Sex impacts treatment decisions in multiple sclerosis
ConclusionsReal-world treatment decisions are influenced by sex-related aspects. Awareness of these associations should prevent unwarranted differences in MS care. (Source: Journal of Neurology)
Source: Journal of Neurology - March 5, 2024 Category: Neurology Source Type: research

Jan Jarkowski (1880 –1929)
(Source: Journal of Neurology)
Source: Journal of Neurology - March 5, 2024 Category: Neurology Source Type: research

Evaluation of a self-administered iPad ®-based processing speed assessment for people with multiple sclerosis in a clinical routine setting
ConclusionsThe PST performed in a waiting room setting correlates strongly with established cognitive tests. It thus may be used to assess processing speed in a resource-efficient manner and complement cognitive assessment in clinical routine. Despite comparable validity of the PST and SDMT, we identified more pwMS with impaired processing speed using normative data of the SDMT compared to the PST and advise caution, that the common cut-off score of – 1.5 SD from the current PST is not appropriate in Europe. (Source: Journal of Neurology)
Source: Journal of Neurology - March 5, 2024 Category: Neurology Source Type: research

Visual and vestibular motion perception in persistent postural-perceptual dizziness (PPPD)
AbstractPersistent postural-perceptual dizziness (PPPD) is a chronic disorder of perceived unsteadiness. Symptoms can be exacerbated in visually complex stationary or moving environment. Visual dependence and increased motion sensitivity are predictors for PPPD but its pathophysiology remains unknown. We hypothesized an abnormal sensory –perceptual scaling mechanism in PPPD and tested visual- and vestibular perceptional thresholds in 32 patients and 28 age-matched healthy control subjects (HC). All participants showed normal vestibular function tests on quantitative testing. Visual motion coherence thresholds were assess...
Source: Journal of Neurology - March 5, 2024 Category: Neurology Source Type: research

Symptoms and signs did not predict outcome after surgery: a prospective study of 143 patients with idiopathic normal pressure hydrocephalus
This study confirms that the recorded clinical signs, symptoms, and impairments in the adopted clinical tests are characteristic findings in iNPH, based on that most of them improved after shunt surgery. However, our clinical data did not enable predictions of whether patients would respond to shunt surgery, indicating that the phenotype is unrelated to the reversibility of the iNPH state and should mainly support diagnosis. Absence of specific signs should not be used to exclude patients from treatment. (Source: Journal of Neurology)
Source: Journal of Neurology - March 5, 2024 Category: Neurology Source Type: research

Quantitative whole-body muscle MRI in idiopathic inflammatory myopathies including polymyositis with mitochondrial pathology: indications for a disease spectrum
AbstractObjectiveInflammatory myopathies (IIM) include dermatomyositis (DM), sporadic inclusion body myositis (sIBM), immune-mediated necrotizing myopathy (IMNM), and overlap myositis (OLM)/antisynthetase syndrome (ASyS). There is also a rare variant termed polymyositis with mitochondrial pathology (PM-Mito), which is considered a sIBM precursor. There is no information regarding muscle MRI for this rare entity. The aim of this study was to compare MRI findings in IIM, including PM-Mito.MethodsThis retrospective analysis included 41 patients (7 PM-Mito, 11 sIBM, 11 PM/ASyS/OLM, 12 IMNM) and 20 healthy controls. Pattern of ...
Source: Journal of Neurology - March 5, 2024 Category: Neurology Source Type: research

CHD8-related disorders redefined: an expanding spectrum of dystonic phenotypes
ConclusionsThis work validates our previous observation that dystonia is part of the phenotypic spectrum ofCHD8-related neurodevelopmental disorders with potential female preponderance, raising new challenges and opportunities in the diagnosis and management of this condition. It also highlights the importance of in-depth neurologic phenotyping of patients carrying variants associated with neurodevelopmental disorders, as the connection between neurodevelopmental and movement disorders is proving closer than previously appreciated. (Source: Journal of Neurology)
Source: Journal of Neurology - March 5, 2024 Category: Neurology Source Type: research

Supra- and infra-tentorial degeneration patterns in primary lateral sclerosis: a multimodal longitudinal neuroradiology study
ConclusionsPLS is not only associated with upper motor neuron dysfunction, but cerebellar cortical volume loss and deep cerebellar nuclear atrophy can also be readily detected. In addition to intra-cerebellar disease burden, cerebro-cerebellar connectivity alterations also take place. Our data add to the evolving evidence of widespread neurodegeneration in PLS beyond the primary motor regions. Cerebellar dysfunction in PLS is likely to exacerbate bulbar, gait and dexterity impairment and contribute to pseudobulbar affect. (Source: Journal of Neurology)
Source: Journal of Neurology - March 5, 2024 Category: Neurology Source Type: research

Outcome predictors of post-COVID conditions in the European Academy of Neurology COVID-19 registry
AbstractSeveral neurological manifestations are part of the post-COVID condition. We aimed to: (1) evaluate the 6-month outcome in the cohort of patients with neurological manifestations during the COVID-19 acute phase and surviving the infection, and find outcome predictors; (2) define the prevalence and type of neurological symptoms persistent at six months after the infection. Data source was an international registry of patients with COVID-19 infection and neurological symptoms, signs or diagnoses established by the European Academy of Neurology. Functional status at six-month follow-up was measured with the modified R...
Source: Journal of Neurology - March 4, 2024 Category: Neurology Source Type: research

Advancing understanding and treatment of spinal muscular atrophy with four SMN2 copies: a critical review
(Source: Journal of Neurology)
Source: Journal of Neurology - March 4, 2024 Category: Neurology Source Type: research

Effectiveness and tolerability of brivaracetam in patients with epilepsy stratified by comorbidities and etiology in the real world: 12-month subgroup data from the international EXPERIENCE pooled analysis
ConclusionsBRV as prescribed in the real world is effective and well tolerated among patients with CLD, psychiatric comorbidity, post-stroke epilepsy, BTRE, and TBIE. (Source: Journal of Neurology)
Source: Journal of Neurology - March 4, 2024 Category: Neurology Source Type: research

Tenecteplase versus alteplase for acute ischemic stroke: a systematic review and meta-analysis of randomized and non-randomized studies
ConclusionsModerate certainty evidence suggested that there was little to no difference between tenecteplase and alteplase in increasing the proportion of patients achieving excellent functional outcome at 3  months and the risk of symptomatic intracranial hemorrhage, while compared to alteplase, tenecteplase probably reduce all-cause mortality. Administration of 0.25 mg/kg tenecteplase after acute ischemic stroke is suggestive of increasing the proportion of patients that achieve excellent functional outcome at 3 months. (Source: Journal of Neurology)
Source: Journal of Neurology - March 4, 2024 Category: Neurology Source Type: research

Cerebellar compensation: a case of aphasia due to cerebellar hemorrhage
(Source: Journal of Neurology)
Source: Journal of Neurology - March 4, 2024 Category: Neurology Source Type: research

Extended interval dosing strategies in multiple sclerosis: insights from natalizumab and ocrelizumab trials
(Source: Journal of Neurology)
Source: Journal of Neurology - March 4, 2024 Category: Neurology Source Type: research

The efficacy and safety of FcRn inhibitors in patients with myasthenia gravis: a systematic review and meta-analysis
ConclusionFcRn inhibitors have good efficacy and safety in patients with MG. Among them, efgartigimod and nipocalimab were effective without causing an increased safety risk. Rozanolixizumab, despite its superior efficacy, caused an increased incidence of adverse events. Current evidence does not suggest that nipocalimab is effective in patients with MG. (Source: Journal of Neurology)
Source: Journal of Neurology - March 3, 2024 Category: Neurology Source Type: research