Outpatient Motor Retraining for Functional Movement Disorder: Predictors of a Favorable Short-Term Response.

Background: Treating functional movement disorder (FMD) with motor retraining is effective but resource intensive. Objectives: Identify patient, disease, and program variables associated with favorable treatment outcomes. Methods: Retrospective review of the 1 week intensive outpatient FMD program at Mayo Clinic in Minnesota from February 2019 to August 2021. Outcomes included patient-reported measures (Canadian Occupational Performance Measure-Performance and Satisfaction subscales [COPM-P and COPM-S, range 0-10] and Global Rating of Change [GROC, -7 to +7]) and a retrospective investigator-rated scale (0-3, worse/not improved to significantly improved/resolved). Linear regression models identified variables predicting favorable outcomes. Results: Participants (n = 201, 74% female, mean age = 46) had median FMD duration of 24 months. The commonest FMD subtypes were gait disorder (65%), tremor (41%) and weakness (17%); 53% had ≥2 subtypes. Most patients (88%) completed a therapeutic screening process before program entry. Patient-reported outcomes at the end of the week improved substantially (COPM-P average change 3.8 ± 1.9; GROC post-program average 5.5 ± 1.7). Available investigator-rated outcomes from short-term follow-up were also positive (102/122 [84%] moderately to significantly improved/resolved). Factors predicting greater improvement in COPM-P were completing therapeutic screening, higher number of non-motor symptoms, shorter FMD duration, earlier program entry, lower baseline COPM scores, and (among screened patients) higher GROC between therapeutic screening and program start. Conclusion: Patients with diverse FMD subtypes improved substantially over a 1 week period. Utilization of therapeutic screening and greater improvement between therapeutic screening and program start were novel predictors of favorable outcomes. Non-motor symptoms did not preclude positive responses, although patients with predominant non-motor burden were excluded.

Clinical Reasoning: A 43-Year-Old Man With Subacute Onset of Vision Disturbances, Jaw Spasms, and Balance and Sleep Difficulties.

A brainstem syndrome is recognizable in patients presenting with a combination of visual disturbances, incoordination, gait problems, speech and swallowing difficulties, and new-onset sleep symptomatology. Brainstem disorders of subacute onset (onset and progression with accumulation of disabling deficits in 6-12 weeks) are generally of autoimmune, infectious, inflammatory, or infiltrative neoplastic cause. An autoimmune or infectious brainstem disorder may be referred to as brainstem encephalitis or rhombencephalitis. We describe a patient with paraneoplastic autoimmune rhombencephalitis, in whom diagnostic clues included the following: diverse visual and sleep symptoms, trismus, and choking in the history; see-saw nystagmus, opsoclonus, dysarthria, jaw dystonia, and episodic laryngospasm on examination; subtle but longitudinal and nonenhancing T2 MRI abnormalities in the brainstem and upper cervical cord; and oligoclonal bands in the CSF. His movement disorder-specific neural IgG profile revealed ANNA-2 (anti-Ri) and KLHL-11-IgG. Both are biomarkers of paraneoplastic brainstem encephalitis. KLCHL-11-IgG has been reported to accompany germ cell tumors, which was found in a solitary metastasis to the left inguinal lymph node in our patient, along with an atrophic left testis. Multidisciplinary treatment (autoimmune neurology, sleep medicine, ophthalmology, and physiatry) led to significant clinical improvements. This case provides a framework for the evaluation of patients with subacute-onset brainstem syndromes and the investigation and management of those with paraneoplastic and other autoimmune diseases.

Safety and efficacy of (+)-epicatechin in subjects with Friedreich's ataxia: A phase II, open-label, prospective study.

BACKGROUND: (+)-Epicatechin (EPI) induces mitochondrial biogenesis and antioxidant metabolism in muscle fibers and neurons. We aimed to evaluate safety and efficacy of (+)-EPI in pediatric subjects with Friedreich's ataxia (FRDA). METHODS: This was a phase II, open-label, baseline-controlled single-center trial including 10 participants ages 10 to 22 with confirmed FA diagnosis. (+)-EPI was administered orally at 75 mg/d for 24 weeks, with escalation to 150 mg/d at 12 weeks for subjects not showing improvement of neuromuscular, neurological or cardiac endpoints. Neurological endpoints were change from baseline in Friedreich's Ataxia Rating Scale (FARS) and 8-m timed walk. Cardiac endpoints were changes from baseline in left ventricular (LV) structure and function by cardiac magnetic resonance imaging (MRI) and echocardiogram, changes in cardiac electrophysiology, and changes in biomarkers for heart failure and hypertrophy. RESULTS: Mean FARS/modified (m)FARS scores showed nonstatistically significant improvement by both group and individual analysis. FARS/mFARS scores improved in 5/9 subjects (56%), 8-m walk in 3/9 (33%), 9-peg hole test in 6/10 (60%). LV mass index by cardiac MRI was significantly reduced at 12 weeks (P = .045), and was improved in 7/10 (70%) subjects at 24 weeks. Mean LV ejection fraction was increased at 24 weeks (P = .008) compared to baseline. Mean maximal septal thickness by echocardiography was increased at 24 weeks (P = .031). There were no serious adverse events. CONCLUSION: (+)-EPI was well tolerated over 24 weeks at up to 150 mg/d. Improvement was observed in cardiac structure and function in subset of subjects with FRDA without statistically significant improvement in primary neurological outcomes. SYNOPSIS: A (+)-epicatechin showed improvement of cardiac function, nonsignificant reduction of FARS/mFARS scores, and sustained significant upregulation of muscle-regeneration biomarker follistatin.

Musculoskeletal education in physical medicine and rehabilitation residency programs.

OBJECTIVE: To characterize current musculoskeletal (MSK) education experiences in physical medicine and rehabilitation residency programs and to identify perceived barriers to providing more extensive MSK education experiences. In addition, to establish utilization patterns for the PASSOR Physical Examination Core Competencies List. DESIGN: Between March and November 2003, all 81 physical medicine and rehabilitation residency program directors were asked to complete an MSK education survey developed by the authors. RESULTS: A total of 69 of 81 program directors (86%) responded after multiple contacts. The most frequently utilized MSK education formats were MSK lecture series, MSK departmental conferences, and physical examination workshops. Potential barriers to expanded MSK education included money, time, and staff number. Given unlimited resources, most residency programs would greatly increase utilization of visiting lecturers, CD-ROMs/DVDs, objective structured clinical examinations, and physical examination videos. Of the 30 program directors who recalled receiving the Core Competencies List, only 40% (12 of 30) have formally integrated the list into their residency training. Barriers to implementation included logistical challenges and lack of direction regarding implementation. CONCLUSIONS: Residency program directors indicate a strong interest in expanding resident MSK education through the use of CD-ROMs/DVDs, physical examination videos, objective structured clinical examinations, and visiting lecturer programs. CD-ROMs/DVDs and videos represent particularly attractive educational formats for supplementing resident MSK education due to the advantages of central production, nominal costs, widespread distribution, multimedia capabilities, and accessibility. These educational formats should be considered for targeted educational initiatives to enhance resident MSK education, regardless of residency program size or resources.