Efficacy and safety of deferiprone for the treatment of superficial siderosis: results from a long-term observational study.

Background: Superficial siderosis (SS) of the central nervous system is a rare and heterogeneous condition due to deposition of hemosiderin on the surface of the brain and spinal cord. The usually progressive clinical course is characterized by a combination of hearing loss, cerebellar ataxia, and myelopathy. There is no known treatment for SS, but the iron chelator deferiprone (DFP) has been proposed as a potentially useful treatment.

Association between Objectively Measured Physical Activity and Gait Patterns in People with Parkinson's Disease: Results from a 3-Month Monitoring.

Background: Although physical activity (PA) is known to be beneficial in improving motor symptoms of people with Parkinson's disease (pwPD), little is known about the relationship between gait patterns and features of PA performed during daily life.

Objective: To verify the existence of possible relationships between spatiotemporal and kinematic parameters of gait and amount/intensity of PA, both instrumentally assessed.

Methods: Eighteen individuals affected by PD (10F and 8M, age 68.

Quantitative assessment of gait parameters in people with Parkinson's disease in laboratory and clinical setting: Are the measures interchangeable?

This study aimed to investigate possible differences in spatio-temporal gait parameters of people with Parkinson's Disease (pwPD) when they are tested either in laboratory using 3D Gait Analysis or in a clinical setting using wearable accelerometers. The main spatio-temporal gait parameters (speed, cadence, stride length, stance, swing and double support duration) of 31 pwPD were acquired: i) using a wearable accelerometer in a clinical setting while wearing shoes (ISS); ii) same as condition 1, but barefoot (ISB); iii) using an optoelectronic system (OES) undressed and barefoot. While no significant differences were found for cadence, stance, swing and double support duration, the experimental setting affected speed and stride length that decreased (by 17% and 12% respectively, P<0.

Quantitative assessment of upper limb functional impairments in people with Parkinson's disease.

Background: In clinical routine, upper limb motor disorders of people with Parkinson's disease are commonly assessed using scale- or timed tests, but such tools are not fully suitable for providing detailed information about their type and magnitude. To partly overcome these limitations, the present study aims to quantitatively investigate upper limb functional impairments through quantitative analysis of the "hand-to-mouth" task.

Methods: Twenty-five individuals with Parkinson's disease and 20 age-matched healthy individuals underwent a kinematic analysis of the hand-to-mouth task from which spatio-temporal and kinematic measures, including summary measures (Arm Variable Score and Arm Profile Score), were calculated and correlated with clinical scores (Hoehn & Yahr, H&Y and the Unified Parkinson Disease Rating Scale, UPDRS).

Spread of dystonia in patients with idiopathic adult-onset laryngeal dystonia.

Background And Purpose: Adult-onset laryngeal dystonia (LD) can be isolated or can be associated with dystonia in other body parts. Combined forms can be segmental at the onset or can result from dystonia spread to or from the larynx. The aim of this study was to identify the main clinical and demographic features of adult-onset idiopathic LD in an Italian population with special focus on dystonia spread.

The Use of Footstep Sounds as Rhythmic Auditory Stimulation for Gait Rehabilitation in Parkinson's Disease: A Randomized Controlled Trial.

Background: The use of rhythmic auditory stimulation (RAS) has been proven useful in the management of gait disturbances associated with Parkinson's disease (PD). Typically, the RAS consists of metronome or music-based sounds (artificial RAS), while ecological footstep sounds (ecological RAS) have never been used for rehabilitation programs.

Objective: The aim of this study was to compare the effects of a rehabilitation program integrated either with ecological or with artificial RAS.

Correction to: The Italian Dystonia Registry: rationale, design and preliminary findings.

In the original article, Gina Ferrazzano was affiliated to Department of Neurology and Psychiatry, Neuromed Institute IRCCS, Sapienza University of Rome, Pozzilli, Italy.The corrected affiliation should be: Neuromed Institute IRCCS, Pozzilli, IS, Italy.

The Italian Dystonia Registry: rationale, design and preliminary findings.

The Italian Dystonia Registry is a multicenter data collection system that will prospectively assess the phenomenology and natural history of adult-onset dystonia and will serve as a basis for future etiological, pathophysiological and therapeutic studies. In the first 6 months of activity, 20 movement disorders Italian centres have adhered to the registry and 664 patients have been recruited. Baseline historical information from this cohort provides the first general overview of adult-onset dystonia in Italy.

Differential induction of dyskinesia and neuroinflammation by pulsatile versus continuous l-DOPA delivery in the 6-OHDA model of Parkinson's disease.

Neuroinflammation is associated with l-DOPA treatment in Parkinson's disease (PD), suggesting a role in l-DOPA-induced dyskinesia (LID), however it is unclear whether increased inflammation is specifically related to the dyskinetic outcome of l-DOPA treatment. Diversely from oral l-DOPA, continuous intrajejunal l-DOPA infusion is associated with very low dyskinetic outcome in PD patients. We reproduced these regimens of administration in 6-OHDA-lesioned hemiparkinsonian rats, where dyskinetic responses and striatal neuroinflammation induced by chronic pulsatile (DOPAp) or continuous (DOPAc) l-DOPA were compared.

Effects of Physical Rehabilitation Integrated with Rhythmic Auditory Stimulation on Spatio-Temporal and Kinematic Parameters of Gait in Parkinson's Disease.

Movement rehabilitation by means of physical therapy represents an essential tool in the management of gait disturbances induced by Parkinson's disease (PD). In this context, the use of rhythmic auditory stimulation (RAS) has been proven useful in improving several spatio-temporal parameters, but concerning its effect on gait patterns, scarce information is available from a kinematic viewpoint. In this study, we used three-dimensional gait analysis based on optoelectronic stereophotogrammetry to investigate the effects of 5 weeks of supervised rehabilitation, which included gait training integrated with RAS on 26 individuals affected by PD (age 70.

Parkinsonism and dementia are negative prognostic factors for the outcome of subdural hematoma.

To determine, among a population with subdural hematoma (SH), whether patients affected by neurodegenerative disorders (parkinsonism and dementia) have a worse clinical outcome. We reviewed the data of patients diagnosed with fall-related SH discharged from the Departments of Neurology/Stroke unit, Neurosurgery, Intensive Care Unit at Brotzu General Hospital (Cagliari, Italy) between January 2010 and December 2013. Patients with severe traumatisms, evidence of spontaneous intracerebral bleeding or aged less than 50 were excluded.

The peripheral nerve involvement in Parkinson Disease: A multifaceted phenomenon.

In the last decade the possible relationship between Parkinson's disease (PD) and peripheral neuropathy (PN) has received increasing attention. Given that PN is quite common in Parkinson's disease, much controversy has arisen on whether it is part of the neurogenerative process itself - actually one of the possible causes - or a complication of levodopa administration. In this article we will discuss the different hypotheses, as well as our perspective on these open issues.

Levodopa-carbidopa intrajejunal gel in advanced Parkinson disease with "on" freezing of gait.

Freezing of gait is a common and disabling disorder in advanced Parkinson's disease (PD). The relationship with dopaminergic medication is complex and often non-linear, thus freezing may occur even when the core parkinsonian features (tremor, rigidity and bradykinesia) appear optimally controlled. We evaluated the effect of Levodopa-carbidopa intrajejunal gel in a group of seven non-demented PD patients with prominent episodes of freezing refractory to adjustments of oral therapy.

Efficacy and safety of deferiprone for the treatment of pantothenate kinase-associated neurodegeneration (PKAN) and neurodegeneration with brain iron accumulation (NBIA): results from a four years follow-up.

Objective: To evaluate the long-term effect of Deferiprone (DFP) in reducing brain iron overload and improving neurological manifestations in patients with NBIA.

Methods: 6 NBIA patients (5 with genetically confirmed PKAN), received DFP solution at 15 mg/kg po bid. They were assessed by UPDRS/III and UDRS scales and blinded video rating, performed at baseline and every six months.

Restless legs syndrome in multiple sclerosis: a case-control study.

The aim of our study was to explore restless legs syndrome (RLS) frequency in multiple sclerosis (MS)-patients and establish whether RLS could be a symptom of MS. Over a period of 1 year, we consecutively enrolled 202 MS-patients and 212 healthy controls, matched for sex and age, in a case-control study. All of them filled in a structured questionnaire according to IRLSSG criteria.

LRRK2 mutations and Parkinson's disease in Sardinia--A Mediterranean genetic isolate.

The Leucine-Rich Repeat Kinase 2 (LRRK2) Gly2019Ser mutation is frequent among Parkinson's disease (PD) patients from the Arab, Jewish, and Iberian populations, while another mutation, Arg1441Gly, is common in the Basque population. We studied the prevalence of these mutations in Sardinia, a Mediterranean genetic isolate with peculiar structure and similarities with the Basque population. Among 98 Sardinian PD probands we detected one heterozygous Gly2019Ser carrier.

Prevalence of primary blepharospasm in Sardinia, Italy: a service-based survey.

We performed a service-based epidemiological study of primary blepharospasm in the island of Sardinia (Italy). Due to its favorable geographical location, we are confident we will provide reliable data from patients seeking botulinum toxin treatment. A total of 53 patients were assessed.