Endemic parkinsonism: clusters, biology and clinical features.

The term 'endemic parkinsonism' refers to diseases that manifest with a dominant parkinsonian syndrome, which can be typical or atypical, and are present only in a particular geographically defined location or population. Ten phenotypes of endemic parkinsonism are currently known: three in the Western Pacific region; two in the Asian-Oceanic region; one in the Caribbean islands of Guadeloupe and Martinique; and four in Europe. Some of these disease entities seem to be disappearing over time and therefore are probably triggered by unique environmental factors.

Cerebrospinal fluid and blood serum biomarkers in neurodegenerative proteinopathies: A prospective, open, cross-correlation study.

Neurodegenerative diseases are a broad heterogeneous group affecting the nervous system. They are characterized, from a pathophysiological perspective, by the selective involvement of a subpopulation of nerve cells with a consequent clinical picture of a disease. Clinical diagnoses of neurodegenerative diseases are quite challenging and often not completely accurate because of their marked heterogeneity and frequently overlapping clinical pictures.

Interhemispheric parietal cortex connectivity reflects improvement in post-stroke spasticity due to treatment with botulinum toxin-A.

In post-stroke spasticity (PSS), effective treatment with botulinum neurotoxin (BoNT) is associated with transient decrease in activation of the ipsilesional superior parietal lobule (SPL) and intraparietal sulcus (IPS). We hypothesized that this would be reflected in changes in resting-state functional connectivity (rsFC) of the SPL/IPS. Our aim was therefore to assess rsFC of the ipsilesional SPL/IPS in chronic stroke patients with hemiparesis both with and without PSS and to explore the relationship between SPL/IPS rsFC and PSS severity.

Imaging Modalities Used for Frameless and Fiducial-Less Deep Brain Stimulation: A Single Centre Exploratory Study among Parkinson's Disease Cases.

Deep brain stimulation (DBS) is a beneficial procedure for treating idiopathic Parkinson's disease (PD), essential tremor, and dystonia. The authors describe their set of imaging modalities used for a frameless and fiducial-less method of DBS. CT and MRI scans are obtained preoperatively, and STN parcellation is done based on diffusion tractography.

Lewy body disease or diseases with Lewy bodies?

The current nosological concept of α-synucleinopathies characterized by the presence of Lewy bodies (LBs) includes Parkinson's disease (PD), Parkinson's disease dementia (PDD), and dementia with Lewy bodies (DLB), for which the term "Lewy body disease" (LBD) has recently been proposed due to their considerable clinical and pathological overlap. However, even this term does not seem to describe the true nature of this group of diseases. The subsequent discoveries of α-synuclein (αSyn), SNCA gene, and the introduction of new immunohistochemical methods have started intensive research into the molecular-biological aspects of these diseases.

Temperature sensation in Parkinson's disease measured by quantitative sensory testing: a single-center, case-control study.

Background: The pathophysiology of abnormal temperature sensation in Parkinson's disease (PD) remains unclear. Abnormal thermal detection does not seem to depend on the dopaminergic deficit, suggesting that other systems play a role in these changes, probably both central and peripheral.

Methods: We measured thermal detection thresholds (TDT) using quantitative sensory testing (QST) in 28 patients with PD and compared them with 15 healthy controls.

Cortical somatosensory processing after botulinum toxin therapy in post-stroke spasticity.

In dystonic and spastic movement disorders, abnormalities of motor control and somatosensory processing as well as cortical modulations associated with clinical improvement after botulinum toxin A (BoNT-A) treatment have been reported, but electrophysiological evidence remains controversial. In the present observational study, we aimed to uncover central correlates of post-stroke spasticity (PSS) and BoNT-A-related changes in the sensorimotor cortex by investigating the cortical components of somatosensory evoked potentials (SEPs). Thirty-one chronic stroke patients with PSS of the upper limb were treated with BoNT-A application into the affected muscles and physiotherapy.

Deep brain stimulation electrode position impact on parkinsonian non-motor symptoms.

Background: In this study we evaluated the impact of location of deep brain stimulation electrode active contact in different parts of the subthalamic nucleus on improvement of non-motor symptoms in patients with Parkinson's disease.

Methods: The subthalamic nucleus was divided into two (dorsolateral/ventromedial) and three (dorsolateral, medial, ventromedial) parts. 37 deep brain stimulation electrodes were divided according to their active contact location.

Botulinum toxin injection changes resting state cerebellar connectivity in cervical dystonia.

In cervical dystonia, functional MRI (fMRI) evidence indicates changes in several resting state networks, which revert in part following the botulinum neurotoxin A (BoNT) therapy. Recently, the involvement of the cerebellum in dystonia has gained attention. The aim of our study was to compare connectivity between cerebellar subdivisions and the rest of the brain before and after BoNT treatment.

Contemporary clinical neurophysiology applications in dystonia.

The complex phenomenological understanding of dystonia has transcended from the clinics to genetics, imaging and neurophysiology. One way in which electrophysiology will impact into the clinics are cases wherein a dystonic clinical presentation may not be typical or a "forme fruste" of the disorder. Indeed, the physiological imprints of dystonia are present regardless of its clinical manifestation.

Premotor Parkinson's disease: Overview of clinical symptoms and current diagnostic methods.

Parkinson's disease (PD) is characterized by typical motor symptoms. However, recent studies show several non-motor features that may precede the development of the motor symptoms of PD. The best known premotor symptoms include hyposmia, REM sleep behavior disorder (RBD), constipation, and depression; other symptoms are excessive daytime somnolence, orthostatic hypotension and symptomatic hypotension, erectile or urinary dysfunction, musculoskeletal symptoms, pain, and global cognitive deficit.

O-Arm Navigated Frameless and Fiducial-Less Deep Brain Stimulation.

Object: Deep brain stimulation (DBS) is a very useful procedure for the treatment of idiopathic Parkinson's disease (PD), essential tremor, and dystonia. The authors evaluated the accuracy of the new method used in their center for the placing of DBS electrodes. Electrodes are placed using the intraoperative O-arm™ (Medtronic)-controlled frameless and fiducial-less system, Nexframe™ (Medtronic).

Deep Brain Stimulation Effects on Gait Pattern in Advanced Parkinson's Disease Patients.

Background: Gait disturbance accompanies many neurodegenerative diseases; it is characteristic for Parkinson's disease (PD). Treatment of advanced PD often includes deep brain stimulation (DBS) of the subthalamic nucleus. Regarding gait, previous studies have reported non-significant or conflicting results, possibly related to methodological limitations.

The Prevalence of Dystonic Tremor and Tremor Associated with Dystonia in Patients with Cervical Dystonia.

The link between dystonia and tremor has been known for decades, but the question of whether they are two separate illnesses or just different manifestations of one disease with the same pathophysiological background remains unanswered. We distinguish two types of tremor in dystonia: dystonic tremor (DT), which appears on the body part affected by dystonia, and tremor associated with dystonia (TAWD), which appears in locations where the dystonia does not occur. In this study, the frequency of occurrence of different forms of tremor was determined by clinical examination in a group of adult-onset isolated cervical dystonia (CD) patients treated with regular local injections of botulinum toxin A in our department.

Botulinum Toxin Modulates Posterior Parietal Cortex Activation in Post-stroke Spasticity of the Upper Limb.

Post-stroke spasticity (PSS) is effectively treated with intramuscular botulinum toxin type A (BoNT-A), although the clinical improvement is likely mediated by changes at the central nervous system level. Using functional magnetic resonance imaging (fMRI) of the brain, this study aims to confirm and locate BoNT-A-related changes during motor imagery with the impaired hand in severe PSS. Temporary alterations in primary and secondary sensorimotor representation of the impaired upper limb were expected.

Prevalence and evolution of spasticity in patients suffering from first-ever stroke with carotid origin: a prospective, longitudinal study.

Background And Purpose: The main aim of the study was to document the occurrence and evolution of post-stroke spasticity (PSS). The secondary goal was to identify predictors for increases and decreases in PSS rates during 12 months of subsequent follow-up.

Methods: In a longitudinal, multicenter, prospective cohort study, assessments were done at 7 days (V1), 6 months (V2), and 12 months (V3) after stroke onset.

New endemic familial parkinsonism in south Moravia, Czech Republic and its genetical background.

An increased prevalence of familial neurodegenerative parkinsonism or cognitive deterioration was recently found in a small region of southeastern Moravia.The aim of the study was to assess the genetic background of this familial disease.Variants in the ADH1C, EIF4G1, FBXO7, GBA + GBAP1, GIGYF2, HTRA2, LRRK2, MAPT, PRKN, DJ-1, PINK1, PLA2G6, SNCA, UCHL1, VPS35 genes were examined in 12 clinically positive probands of the pedigree in which familial atypical neurodegenerative parkinsonism was identified in previous epidemiological studies.

Changes in sensorimotor network activation after botulinum toxin type A injections in patients with cervical dystonia: a functional MRI study.

Botulinum toxin type A (BoNT) is considered an effective therapeutic option in cervical dystonia (CD). The pathophysiology of CD and other focal dystonias has not yet been fully explained. Results from neurophysiological and imaging studies suggest a significant involvement of the basal ganglia and thalamus, and functional abnormalities in premotor and primary sensorimotor cortical areas are considered a crucial factor in the development of focal dystonias.

Early manifestation of spasticity after first stroke in the territory of the internal carotid artery: A prospective multicenter study.

Aims: The main aim of this study was to provide an estimate of the incidence and prevalence of spasticity following stroke in the internal carotid artery territory for Regional Stroke Centers in the Czech Republic. A secondary goal was to identify predictors for the development of spasticity.

Methods: In a prospective cohort study, 256 consecutive patients with clinical signs of central paresis due to a first-ever stroke were examined in the acute stage.

Longitudinal brain activation changes related to electrophysiological findings in patients with cervical spondylotic myelopathy before and after spinal cord decompression: an fMRI study.

Background: Cervical spondylotic myelopathy (CSM) is the most common cause of spinal cord dysfunction, potentially leading to severe disability. Abnormal cervical spine magnetic resonance imaging (MRI) and motor evoked potentials (MEPs) are independent predictors of disease progression. Abnormal MRI is accompanied by various activation changes in functional brain MRI (fMRI), whereas preoperative and postoperative fMRI adaptations associated with abnormal preoperative MEP remain unknown.

Bilateral subthalamic deep brain stimulation initial impact on nonmotor and motor symptoms in Parkinson's disease: An open prospective single institution study.

Numerous studies document significant improvement in motor symptoms in patients with Parkinson's disease (PD) after deep brain stimulation of the subthalamic nucleus (STN-DBS). However, little is known about the initial effects of STN-DBS on nonmotor domains.Our objective was to elucidate the initial effects of STN-DBS on non-motor and motor symptoms in PD patients in a 4-month follow-up.

Changes in oxygen saturation and the retinal nerve fibre layer in patients with optic neuritis - a pilot study.

Purpose: Assessment of retinal oxygen saturation, thickness of a retinal nerve fibre layer (RNFL) and functional changes in the optic nerve during optic neuritis (ON) in patients with multiple sclerosis (MS).

Methods: Thirty-two patients with ON due to MS within 3 months of onset of symptoms were enrolled [22 females, 10 males, age 34 ± 9 years, median 32.5 years, 22 patients with the clinically isolated syndrome (CIS), 10 patients with relapsing-remitting from of MS (RRMS)].

Cerebrospinal fluid levels of chromogranin A and phosphorylated neurofilament heavy chain are elevated in amyotrophic lateral sclerosis.

Background: Various cerebrospinal fluid (CSF) biomarkers are being studied to improve the sensitivity and specificity of the diagnostic methods for amyotrophic lateral sclerosis (ALS).

Aims Of The Study: The aim of our study was to establish the CSF levels of chromogranin A (CgA) and phosphorylated neurofilament heavy chain (pNF-H) in patients with ALS in order to assess these proteins as possible biomarkers of ALS.

Methods: Cerebrospinal fluid levels of CgA and pNF-H were examined and mutually compared in 15 patients with sporadic ALS and 16 gender- and age-matched controls.