Parkinsonism & related disorders
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Parkinsonism Relat. Disord. · Jan 2018
Transcranial sonographic findings may predict prognosis of gastroprokinetic drug-induced parkinsonism.
Drug-induced parkinsonism (DIP) is one important cause of parkinsonism and a major cause of misleading diagnosis of Parkinson's disease (PD). DIP is caused by dopamine receptor blocking agents. Its symptoms will improve after withdrawal of offending drugs. However, parkinsonism does not regress in several individuals. It may persist or exacerbate despite drug withdrawal. Transcranial sonography (TCS) of the substantia nigra (SN) has been widely used to diagnose PD and differentiate parkinsonism types. The objective of this study was to investigate the value of early TCS findings for predicting clinical outcome of patients with newly diagnosed gastroprokinetic drug-induced parkinsonism after withdrawal of dopamine receptor blocking agents. ⋯ SN echogenicity on TCS could be a useful tool to differentiate PD from DIP in clinical situations. Pure DIP and unmasked PD exhibited different SN echogenicity patterns. Early SN echogenicity findings on TCS could be used a biomarker to predict clinical prognosis of DIP.
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The dystonias are a group of disorders defined by over-contraction of muscles leading to abnormal movements and postures. In recent years, enormous advances have been made in elucidating the neurobiological mechanisms responsible for many types of dystonia. ⋯ Although the dystonias share certain clinical aspects such as over-contraction of muscles leading to abnormal movements and postures, they actually comprise a very clinically and etiologically heterogeneous group of disorders. Understanding their neurobiological basis is important for devising rational therapies appropriately targeted for specific subgroups of patients.
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Parkinsonism Relat. Disord. · Jan 2018
Comparative StudySquare biphasic pulse deep brain stimulation for essential tremor: The BiP tremor study.
Conventional deep brain stimulation (DBS) utilizes regular, high frequency pulses to treat medication-refractory symptoms in essential tremor (ET). Modifications of DBS pulse shape to achieve improved effectiveness is a promising approach. ⋯ BiP was safe, tolerable and effective on the tremor symptoms when tested up to 3 h. This study demonstrated the feasibility of applying a novel DBS waveform in the clinic setting. Larger prospective studies with longer clinical follow-up will be required.
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Parkinsonism Relat. Disord. · Oct 2017
Seven tesla MRI of the substantia nigra in patients with rapid eye movement sleep behavior disorder.
Susceptibility-weighted imaging of the substantia nigra (SN) both at 7 and 3 Tesla (T) has shown high accuracy in distinguishing patients with Parkinson's disease (PD) and healthy subjects (HS). Patients with rapid eye movement (REM) behavior disorder (RBD) can develop synucleinopathies, and such risk is higher with dopamine transporter single photon emission tomography (123I-FP-CIT SPECT) evidence of nigro-striatal dysfunction. We aimed at evaluating SN 7T magnetic resonance imaging (7T-MRI) in patients with RBD and determining the agreement between MRI and 123I-FP-CIT SPECT. ⋯ Gradient-recalled-echo multiecho susceptibility-weighted imaging of the SN at 7T is abnormal in 60% of patients with RBD. The 7T-MRI and 123I-FP-CIT SPECT results showed good agreement. 7T-MRI of the SN could represent a safe marker for neurodegenerative disease in patients with RBD, however longitudinal study is warranted.
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Perry syndrome (PS) is a rare hereditary neurodegenerative disease characterized by autosomal dominant parkinsonism, psychiatric symptoms, weight loss, central hypoventilation, and distinct TDP-43 pathology. The mutated causative gene for PS is DCTN1, which encodes the dynactin subunit p150Glued. Dynactin is a motor protein involved in axonal transport; the p150Glued subunit has a critical role in the overall function. ⋯ We recently had the opportunity to evaluate this family and to test the gene status of an affected family member. The Polish family is newly identified and is the first PS family in Poland. Although still rare, DCTN1-related neurodegeneration needs to be considered in a differential diagnosis of parkinsonian disorders, frontotemporal dementia, and motor-neuron diseases, especially if there is family history.