Mycobacterium bovis Infection of a Deep Brain Stimulation System Following Intravesical Bacillus Calmette-Guérin (BCG) Instillation.

Deep brain stimulation (DBS) is an advanced treatment in Parkinson's disease. We describe a 71-year-old patient in whom the DBS got infected with Mycobacterium bovis shortly after intravesical BCG instillations as an adjuvant treatment of bladder cancer. The DBS internal pulse generator and extension wires had to be replaced, and the patient was treated successfully with rifampicin, isoniazid, and ethambutol during three months.

Predicting Motor Outcome and Quality of Life After Subthalamic Deep Brain Stimulation for Parkinson's Disease: The Role of Standard Screening Measures and Wearable-Data.

Background: Standardized screening for subthalamic deep brain stimulation (STN DBS) in Parkinson's disease (PD) patients is crucial to determine eligibility, but its utility to predict postoperative outcomes in eligible patients is inconclusive. It is unknown whether wearable data can contribute to this aim.

Objective: To evaluate the utility of universal components incorporated in the DBS screening, complemented by a wearable sensor, to predict motor outcomes and Quality of life (QoL) one year after STN DBS surgery.

Functional Movement Disorders and Deep Brain Stimulation: A Multi-Center Study.

Background: Functional movement disorders (FMD) are a commonly under-recognized diagnosis in patients with underlying neurodegenerative diseases. FMD have been observed in patients undergoing deep brain stimulation (DBS) for Parkinson's disease (PD) and other movement disorders. The prevalence of coexisting FMD among movement disorder-related DBS patients is unknown, and it may occur more often than previously recognized.

Medical and Surgical Treatment for Medication-Induced Tremor: Case Report and Systematic Review.

Objective: To present a case of refractory medication-induced tremor successfully treated with deep brain stimulation (DBS) of the thalamic ventral intermediate nucleus (Vim) and to propose a medical and surgical treatment algorithm based on a systematical review of the literature.

Methods: Patient data were retrospectively collected. A systematic search was performed in PubMed, Embase, and Cochrane Library.

The actual use of directional steering and shorter pulse width in selected patients undergoing deep brain stimulation.

Introduction: Directional deep brain stimulation (DBS) and pulse with <60μs increase side-effects threshold, enlarging the therapeutic window. However, new systems allowing these advanced features are more expensive and often available only for a limited number of patients in some centers. It is unknown how many and which DBS patients actually need the advanced features because of an insufficient improvement with standard parameters.

Towards adaptive deep brain stimulation: clinical and technical notes on a novel commercial device for chronic brain sensing.

. Technical advances in deep brain stimulation (DBS) are crucial to improve therapeutic efficacy and battery life. We report the potentialities and pitfalls of one of the first commercially available devices capable of recording brain local field potentials (LFPs) from the implanted DBS leads, chronically and during stimulation.

Management of Parkinson's Disease During Pregnancy: Literature Review and Multidisciplinary Input.

Background: There are no standardized clinical guidelines for the management of Parkinson's disease (PD) during pregnancy. Increasing maternal age would suggest that the incidence of pregnancy in women diagnosed with PD is likely to increase.

Objective: To evaluate the evidence for the treatment of PD during pregnancy and to canvass opinion from patients and clinical teams as to the optimum clinical management in this setting.

Nomenclature of Genetically Determined Myoclonus Syndromes: Recommendations of the International Parkinson and Movement Disorder Society Task Force.

Genetically determined myoclonus disorders are a result of a large number of genes. They have wide clinical variation and no systematic nomenclature. With next-generation sequencing, genetic diagnostics require stringent criteria to associate genes and phenotype.

Skater's Cramp: A Possible Task-Specific Dystonia in Dutch Ice Skaters.

Background: Skater's cramp is an involuntary lower leg movement in skilled speed skaters. We aim to evaluate whether skater's cramp is compatible with task-specific dystonia.

Methods: A case-control study tested 5 speed skaters exhibiting symptoms of skater's cramp and 5 controls.

Botulinum neurotoxin treatment in jerky and tremulous functional movement disorders: a double-blind, randomised placebo-controlled trial with an open-label extension.

Objective: To study the effect of botulinum neurotoxin (BoNT) treatment in jerky and tremulous functional movement disorders (FMD).

Methods: Patients with invalidating, chronic (>1 year) symptoms were randomly assigned to two subsequent treatments with BoNT or placebo every 3 months with stratification according to symptom localisation. Improvement on the dichotomised Clinical Global Impression-Improvement scale (CGI-I) (improvement vs no change or worsening) at 4 months, assessed by investigators blinded to the allocated treatment was the primary outcome.

Movement disorders and nonmotor neuropsychological symptoms in children and adults with classical galactosemia.

Although movement disorders (MDs) are known complications, the exact frequency and severity remains uncertain in patients with classical galactosemia, especially in children. We determined the frequency, classification and severity of MDs in a cohort of pediatric and adult galactosemia patients, and assessed the association with nonmotor neuropsychological symptoms and daily functioning. Patients from seven centers in the United Kingdom and the Netherlands with a confirmed galactosemia diagnosis were invited to participate.

Progressive myoclonus ataxia: Time for a new definition?

Background: The clinical demarcation of the syndrome progressive myoclonus ataxia is unclear, leading to a lack of recognition and difficult differentiation from other neurological syndromes.

Objectives: The objective of this study was to apply a refined definition of progressive myoclonus ataxia and describe the clinical characteristics in patients with progressive myoclonus ataxia and with isolated cortical myoclonus.

Methods: A retro- and prospective analysis was performed in our tertiary referral center between 1994 and 2014.

The interrelation between clinical presentation and neurophysiology of posthypoxic myoclonus.

Objective: Posthypoxic myoclonus (PHM) in the first few days after resuscitation can be divided clinically into generalized and focal (uni- and multifocal) subtypes. The former is associated with a subcortical origin and poor prognosis in patients with postanoxic encephalopathy (PAE), and the latter with a cortical origin and better prognosis. However, use of PHM as prognosticator in PAE is hampered by the modest objectivity in its clinical assessment.

The efficacy of the modified Atkins diet in North Sea Progressive Myoclonus Epilepsy: an observational prospective open-label study.

Background: North Sea Progressive Myoclonus Epilepsy is a rare and severe disorder caused by mutations in the GOSR2 gene. It is clinically characterized by progressive myoclonus, seizures, early-onset ataxia and areflexia. As in other progressive myoclonus epilepsies, the efficacy of antiepileptic drugs is disappointingly limited in North Sea Progressive Myoclonus Epilepsy.

Unusual Course of Lafora Disease.

A 42-year-old male was admitted for refractory status epilepticus. At the age of 25, he had been diagnosed with juvenile myoclonic epilepsy. He had a stable clinical course for over a decade until a recent deterioration of behavior and epilepsy.

Distribution and Coexistence of Myoclonus and Dystonia as Clinical Predictors of SGCE Mutation Status: A Pilot Study.

Introduction: Myoclonus-dystonia (M-D) is a young onset movement disorder typically involving myoclonus and dystonia of the upper body. A proportion of the cases are caused by mutations to the autosomal dominantly inherited, maternally imprinted, epsilon-sarcoglycan gene (SGCE). Despite several sets of diagnostic criteria, identification of patients most likely to have an SGCE mutation remains difficult.

A novel diagnostic approach to patients with myoclonus.

Myoclonus is a hyperkinetic movement disorder characterized by brief, involuntary muscular jerks. Recognition of myoclonus and determination of the underlying aetiology remains challenging given that both acquired and genetically determined disorders have varied manifestations. The diagnostic work-up in myoclonus is often time-consuming and costly, and a definitive diagnosis is reached in only a minority of patients.

Myoclonus in childhood-onset neurogenetic disorders: The importance of early identification and treatment.

Background: In clinical practice, myoclonus in childhood-onset neurogenetic disorders frequently remains unrecognized, because it is often overshadowed by other neurological features. Since treatment can lead to significant functional improvement, accurate phenotyping is essential. To demonstrate the importance of early identification and treatment, we report on four patients with various childhood-onset neurogenetic disorders suffering from myoclonus.

[Recurrent rhabdomyolysis: screening for underlying disease].

Rhabdomyolysis is a rare potentially dangerous syndrome resulting from the dissolution of skeletal muscle fibres. An isolated attack of rhabdomyolysis can have various causes, such as trauma, hyperpyrexia, infections, electrolyte imbalances, seizures, severe exertion, and drugs or substance abuse or a combination of these. Recurrent episodes and/or a family history of rhabdomyolysis is more likely caused by an underlying genetic defect.