Journal of Neurology

INTRODUCTION AND OBJECTIVE: Several prediction models for falls/near falls in Parkinson's disease (PD) have been proposed. However, longitudinal predictors of frequency of falls/near falls are poorly investigated. Therefore, we aimed to identify short- and long-term predictors of the number of falls/near falls in PD. METHODS: A prospective cohort of 58 persons with PD was assessed at baseline (mean age and PD duration, 65 and 3.2 years, respectively) and 3.5 years later...

OBJECTIVE: To determine if hyposmia in isolated REM sleep behavior disorder (IRBD) predicts short-term conversion to any α-synucleinopathy and declines with time. METHODS: Olfaction was tested using the University of Pennsylvania Smell Identification Test (UPSIT-40) in 140 consecutive patients with polysomnography-confirmed IRBD and in 77 matched controls. Patients were followed-up during 5.6 ± 3.9 (range 0.2-13) years. Twenty-one patients underwent serial UPSIT-40 evaluations at 1-3 and 4-6 years after baseline...

OBJECTIVE: This study aimed at developing a quantitative approach to assess abnormalities on MRI of the brachial plexus and the cervical roots in patients with chronic inflammatory demyelinating polyneuropathy (CIDP) and multifocal motor neuropathy (MMN) and to evaluate interrater reliability and its diagnostic value. METHODS: We performed a cross-sectional study in 50 patients with CIDP, 31 with MMN and 42 disease controls. We systematically measured cervical nerve root sizes on MRI bilaterally (C5, C6, C7) in the coronal [diameter (mm)] and sagittal planes [area (mm2 )], next to the ganglion (G0 ) and 1 cm distal from the ganglion (G1 )...

The coronavirus disease 2019 (COVID-19) crisis confronted us, like many researchers worldwide, with an unforeseen challenge during the final stages of a randomized controlled trial involving ataxia patients. Institutional guidelines suddenly no longer allowed regular follow-up visits to take place, impeding the clinical evaluation of long-term outcomes. Here, we discuss the various scenarios that we considered in response to these imposed restrictions and share our experience of home video recording by dedicated, extensively instructed family members...

OBJECTIVE: To investigate oculomotor impairment in subtypes of progressive supranuclear palsy (PSP) and its associations with clinical features and regional brain volumes in PSP. METHODS: We compared the video-oculography (VOG) findings of 123 PSP patients, consisting of 66 PSP-Richardson syndrome (PSP-RS), 28 PSP-parkinsonism (PSP-P), and 29 PSP-progressive gait freezing (PSP-PGF), along with 80 Parkinson's disease (PD) patients. We also investigated the associations of the VOG results with clinical features (disease duration, PSP rating scales [PSPRS] scores for dysphagia and postural stability) in the subtypes of PSP patients and with regional volumes in the brainstem, including the midbrain, pons, medulla, and the superior cerebellar peduncle (SCP), among the patients who had MRI images at the time of VOG (30 PSP)...

From March to May 2020, the Italian health care system, as many others, was almost entirely devoted to the fight against the COVID-19 pandemic. In this context, a number of questions arose, from the increased stroke risk due to COVID-19 infection to the quality of stroke patient care. The overwhelming need of COVID-19 patient management made mandatory a complete re-organization of the stroke pathways: many health professionals were reallocated and a number of stroke units was turned into COVID-19 wards. As a result, acute stroke care suffered from a shortage of services and delays in time-dependent treatments and diagnostic work-up...

We describe a rare case of post-infective Acute Motor Axonal Neuropathy (AMAN) variant of Guillain-Barrè Syndrome (GBS) associated with myelitis and anti-GD1b positivity after SARS-CoV-2 infection. The patient referred to the hospital reporting a history of ten days lasting moderate fever, myalgia and anosmia, with the onset of progressive quadriparesis and ascending paraesthesias in the four limbs since five days from defervescence. A chest computed tomography demonstrated interstitial pneumonia with "ground glass opacities", suggesting Coronavirus disease (COVID-19)...

No abstract text is available yet for this article.

BACKGROUND: Botulinum neurotoxin type A (BoNT-A) is an effective pharmacological treatment for the management of cervical dystonia (CD) that requires repeated administration at variable intervals. We explored patient perceptions of the impact of CD and the waning of BoNT-A therapeutic effects. METHODS: An internet-based survey was conducted through Carenity, a global online patient community, from May to September 2019. Eligible respondents were adults with CD who had ≥ 2 previous BoNT-A injections...

BACKGROUND: Nusinersen recently became available as the first treatment for Spinal Muscular Atrophy (SMA) and data on its effectiveness and safety in adult SMA patients are still scarce. METHODS: We evaluated the effectiveness and safety of nusinersen treatment during 14 months in 16 adult patients with SMA types 3 and 4 in a prospective study, and retrospectively detailed the natural history of 48 adult SMA patients types 2, 3 and 4. RESULTS: Hand grip strength (p = 0...

BACKGROUND: Development of "Wearing Off" (WO) of motor and non-motor function in Parkinson's disease (PD) adversely affects quality of life. This suggest that identifying and treating WO is important. However, identification of WO depends on people with PD (PwP) recognising and reporting WO and there is a perception that WO may be significantly underestimated. OBJECTIVE: We investigate the feasibility of identifying "Wearing Off" using objective measurement and assess the clinical benefit in rectifying it...

INTRODUCTION: Treatment with disease-modifying therapies (DMT) in patients with clinically isolated syndrome (CIS) represents standard care in multiple sclerosis (MS) patients nowadays. Since a proportion of patients may show no evidence of disease activity (NEDA) after some time of treatment, the question might arise about the risks of stopping DMT. METHODS: We present a cohort of 49 patients who started DMT immediately after CIS and had no evidence of disease activity (NEDA-3) for at least five years before discontinuation of therapy...

Down syndrome (DS) is one of the most well-recognized genetic disorders. Persons with DS are known to have a variety of co-morbid medical problems, affecting nearly all organ systems. Improved healthcare interventions and research have allowed for increased life span of persons with DS, although disorders of the neurologic system remain underexplored. The purpose of this systematic review is to provide clinically pertinent information on the neurological phenotypes of frequently occurring or clinically relevant conditions...

No abstract text is available yet for this article.

OBJECTIVE: To uncover possible impairments of walking and dynamic postural stability in patients with primary orthostatic tremor (OT). METHODS: Spatiotemporal gait characteristics were quantified in 18 patients with primary OT (mean age 70.5 ± 5.9 years, 10 females) and 18 age-matched healthy controls. One-third of patients reported disease-related fall events. Walking performance was assessed on a pressure-sensitive carpet under seven conditions: walking at preferred, slow, and maximal speed, with head reclination or eyes closed, and while performing a cognitive or motor dual-task paradigm...

Loss of peripheral vestibular function provokes severe impairments of gaze and posture stabilization in humans and animals. However, relatively little is known about the extent of the instantaneous deficits. This is mostly due to the fact that in humans a spontaneous loss often goes unnoticed initially and targeted lesions in animals are performed under deep anesthesia, which prevents immediate evaluation of behavioral deficits. Here, we use isolated preparations of Xenopus laevis tadpoles with functionally intact vestibulo-ocular (VOR) and optokinetic reflexes (OKR) to evaluate the acute consequences of unilateral VIIIth nerve sections...

Peripheral neuropathies have various causes, both infectious and non-infectious. When we think of "epidemics", we often refer to an infectious or even post-infectious origin. Nevertheless, the history of mankind is marked by episodes of epidemics of peripheral neuropathies of non-infectious nature, either of nutritional or toxic origin: we present here the main causes of such epidemics.

Disorders of consciousness (DOC) are an important but still underexplored entity in neurology. Novel electroencephalography (EEG) measures are currently being employed for improving diagnostic classification, estimating prognosis and supporting medicolegal decision-making in DOC patients. However, complex recording protocols, a confusing variety of EEG measures, and complicated analysis algorithms create roadblocks against broad application. We conducted a systematic review based on English-language studies in PubMed, Medline and Web of Science databases...

INTRODUCTION: Internal globus pallidus (GPi) deep brain stimulation (DBS) is a safe and effective alternative treatment in Parkinson's disease (PD) for patients with cognitive impairment. However, no study has yet investigated metabolic changes within a large series of patients undergoing GPi stimulation. OBJECTIVE: We assessed motor, cognitive and psychiatric changes, as well as modifications in brain glucose metabolism measured with FDG-PET, before and after bilateral GPi-DBS...

The history of mankind is marked by numerous epidemics, some of which involved diseases of the peripheral nervous system, either infectious or otherwise. We describe here the three main infectious causes of epidemics that affect the peripheral nervous system: leprosy, poliomyelitis and diphtheria. We then discuss the main epidemics of immune-mediated origin.