Movement Disorders Clinical Practice最新文献

Background: Reduced cerebrospinal fluid (CSF) clearance may play a vital role in the pathogenesis of normal pressure hydrocephalus (NPH), but the radiologic marker is yet to be elucidated.

Objectives: This open-label study presents two novel neuroimaging biomarkers based on enlarged perivascular spaces (ePVS) of the sub-insular territory: the Hedgehog and Hedgehog-Halo (H-H) sign, designed to predict gait symptom severity and tap response in NPH.

Methods: We retrospectively reviewed 203 patients with possible NPH with baseline magnetic resonance imaging and gait analyses before and after lumbar puncture (LP). The Hedgehog/H-H sign was scored using T2-weighted images. The clinical severity at baseline and post-tap gait improvement was compared in patients with and without Hedgehog/H-H sign. The association between Hedgehog/H-H sign and post-tap gait outcomes was assessed using multivariate regression. The diagnostic performance of Hedgehog/H-H sign was compared with conventional radiological markers.

Results: Patients with H-H showed higher global disability and more severe gait impairment than those without any signs. Following LP, patients with Hedgehog/H-H sign significantly improved in various gait parameters, unlike those with neither sign. Additionally, sub-insular ePVS was significantly associated with post-tap gait improvement after adjusting covariates. Finally, the Hedgehog/H-H sign showed a higher performance than conventional markers in predicting post-tap gait response.

Conclusions: The Hedgehog/H-H sign is a useful neuroimaging biomarker related to the severity and tap response in NPH. This biomarker can be readily applied in clinical practice before undergoing LP, independent of conventional radiological signs. Further research is warranted to determine applicability in predicting post-shunt gait response.

Background: Few studies have examined the effectiveness and duration of mindfulness-based therapies for tics in Tourette's syndrome. This study combined habit reversal therapy (HRT) with acceptance and commitment therapy (ACT).

Objectives: To evaluate the efficacy and response duration of HRT + ACT in reducing tic severity in adults with Tourette's Syndrome.

Methods: Tic severity was assessed at baseline, post-intervention, and at 6- and 12-month follow-ups using the Yale Global Tic Severity Scale (YGTSS) and video assessments. The intervention included eight weekly 1-h sessions.

Results: Mixed-effects regression showed significant reductions in tic severity post-treatment (b = -10.36, P = 0.002), maintained at 6 months (b = -8.19, P = 0.012) and 12 months (b = -8.82, P = 0.009). Video assessments confirmed these findings.

Conclusion: The HRT + ACT protocol effectively reduced tic severity, with benefits lasting 12 months. These results support further trials to compare HRT + ACT with HRT alone.

Background: Deep Brain Stimulation (DBS) has been demonstrated to improve quality of life in patients with refractory dystonia and Tourette's syndrome (TS). Because of the young age at onset of these disorders, and the marked benefit from DBS, pregnancy in patients who have received DBS is becoming a more frequent clinical occurrence, although clear management guidelines are lacking.

Cases: We report 14 new pregnancies in patients with dystonia or TS and DBS.

Literature review: Upon review of the literature, 23 pregnancies in patients with dystonia or TS were previously reported in seven articles.

Conclusion: Based on the available data from a total of 37 pregnancies, DBS does not seem associated with worse pregnancy outcome. However, careful planning and communication between neurologist, anesthesiologist and obstetrician are key. A registry on pregnancy outcome in patients with DBS should be generated to facilitate the development of guidelines.

Background: Patients with Parkinson's Disease (PD) frequently exhibit non-motor symptoms, particularly sleep disturbances. Sleep disorders in PD patients are intricately linked to the pathogenesis and progression of PD itself, exacerbating neurodegenerative processes and worsening patient quality of life.

Objectives: This review underscores the significance of sleep disorders in PD, highlighting their prevalence, impact on disease progression, and the bidirectional relationship between sleep disruption and neurodegeneration. It aims to enhance clinician awareness for better diagnosis and management of sleep-related comorbidities in PD.

Methods: A comprehensive literature search was conducted in PubMed and Scopus using key terms such as "sleep disorders", "Parkinson's disease", "REM sleep behavior disorder", "restless legs syndrome", "insomnia", "obstructive sleep apnea", "excessive daytime sleepiness", "circadian rhythm disorders", "sleep and neurodegeneration".

Results: Sleep disorders are prevalent in PD affecting up to 90% of patients. Conditions such as insomnia, REM sleep behavior disorder, restless legs syndrome, obstructive sleep apnea, excessive daytime sleepiness, and circadian rhythm disorders are commonly reported. These disorders are linked to multifactorial biological mechanisms and are associated with more severe disease phenotypes. Of note, several evidence shows that sleep abnormalities may contribute to neuroinflammation and neurodegeneration, further accelerating the disease course.

Conclusions: Sleep disturbances are critical non-motor symptoms in PD. Early diagnosis and tailored management of sleep disorders are essential for improving clinical outcomes and potentially offering neuroprotective benefits.