Cognitive Reserve in Early Manifest Huntington Disease Patients: Leisure Time Is Associated with Lower Cognitive and Functional Impairment

We focused on Cognitive Reserve (CR) in patients with early Huntington Disease (HD) and investigated whether clinical outcomes might be influenced by lifetime intellectual enrichment over time. CR was evaluated by means of the Cognitive Reserve Index questionnaire (CRIq), an internationally validated scale which includes three sections: education, working activity, and leisure time. The clinical HD variables were quantified at three different time points (baseline-t0, 1 year follow up-t1 and 2 years follow up-t2) as per the Unified Huntington’s Disease Rating Scale (UHDRS), an internationally standardized and validated scale including motor, cognitive, functional and behavioral assays. Our sample consisted of 75 early manifest patients, withclinical stage scored according to the Total Functional Capacity (TFC) scale. Our correlational analysis highlighted a significant inverse association between CRIq leisure time (CRIq_LA) and longitudinal functional impairment (namely, the differential TFC score between t2 and t0 or ΔTFC) (p < 0.05), and the multidimensional progression of HD as measured by the composite UHDRS (cUHDRS, p < 0.01). CRIq_LA was significantly and positively associated with better cognitive performances at all time points (p < 0.05). Our results suggest that higher is the CRIq_LA, milder is the progression of HD in terms of functional, multidimensional and cognitive outcome.

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Longitudinal Change and Progression Indicators Using the Movement Disorder Society-Unified Parkinson’s Disease Rating Scale in Two Independent Cohorts with Early Parkinson’s Disease

Journal of Parkinson s Disease ◽

10.3233/jpd-212860 ◽

2021 ◽

pp. 1-16

Author(s):

Michael Bartl ◽

Mohammed Dakna ◽

Sebastian Schade ◽

Tamara Wicke ◽

Elisabeth Lang ◽

...

Keyword(s):

Parkinson’S Disease ◽

Parkinson's Disease ◽

Clinical Trials ◽

Rating Scale ◽

De Novo ◽

Longitudinal Change ◽

Single Center ◽

Square Roots ◽

Disease Rating

Background: The MDS-Unified Parkinson’s disease (PD) Rating Scale (MDS-UPDRS) is the most used scale in clinical trials. Little is known about the predictive potential of its single items. Objective: To systematically dissect MDS-UPDRS to predict PD progression. Methods: 574 de novo PD patients and 305 healthy controls were investigated at baseline (BL) in the single-center DeNoPa (6-year follow-up) and multi-center PPMI (8-year follow-up) cohorts. We calculated cumulative link mixed models of single MDS-UPDRS items for odds ratios (OR) for class change within the scale. Models were adjusted for age, sex, time, and levodopa equivalent daily dose. Annual change and progression of the square roots of the MDS-UDPRS subscores and Total Score were estimated by linear mixed modeling. Results: Baseline demographics revealed more common tremor dominant subtype in DeNoPa and postural instability and gait disorders-subtype and multiethnicity in PPMI. Subscore progression estimates were higher in PPMI but showed similar slopes and progression in both cohorts. Increased ORs for faster progression were found from BL subscores I and II (activities of daily living; ADL) most marked for subscore III (rigidity of neck/lower extremities, agility of the legs, gait, hands, and global spontaneity of movements). Tremor items showed low ORs/negative values. Conclusion: Higher scores at baseline for ADL, freezing, and rigidity were predictors of faster deterioration in both cohorts. Precision and predictability of the MDS-UPDRS were higher in the single-center setting, indicating the need for rigorous training and/or video documentation to improve its use in multi-center cohorts, for example, clinical trials.

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Cognitive reserve moderates the impact of subcortical gray matter atrophy on neuropsychological status in multiple sclerosis

Multiple Sclerosis Journal ◽

10.1177/1352458515579443 ◽

2015 ◽

Vol 22 (1) ◽

pp. 36-42 ◽

Cited By ~ 39

Author(s):

Claire M Modica ◽

Niels Bergsland ◽

Michael G Dwyer ◽

Deepa P Ramasamy ◽

Ellen Carl ◽

...

Keyword(s):

Multiple Sclerosis ◽

Cognitive Decline ◽

Cognitive Processing ◽

Gray Matter ◽

Cognitive Reserve ◽

Cognitive Outcome ◽

Brain Reserve ◽

The Impact ◽

Normal Controls

Background: Cognitive decline is characterized in multiple sclerosis (MS), but the rate and severity vary. The reserve hypothesis proposes that baseline neurological differences impact cognitive outcome in neurodegenerative disease. Objective: To elucidate how brain reserve and cognitive reserve influence subcortical gray matter (SCGM) atrophy and cognitive decline in MS over 3 years. Methods: Seventy-one MS patients and 23 normal controls underwent magnetic resonance imaging and cognitive assessment at baseline and 3-year follow-up. The influence of reserve on cognitive processing speed (CPS) and memory was examined. Results: SCGM volume and cognitive scores were lower in MS than normal controls ( P⩽0.001). Accounting for baseline, comparison of follow-up means yielded a difference between groups in SCGM volume ( P<0.001) but not cognition (NS). Cognitive reserve ( P=0.005), but not brain reserve, contributed to CPS, with only low cognitive reserve MS subjects showing decline in CPS ( P=0.029). SCGM change predicted CPS outcome in MS with low cognitive reserve ( P=0.002) but not high cognitive reserve. There were no effects in the domain of memory. Conclusions: SCGM atrophy occurs in normal controls, but significantly more so in MS. While CPS did not change in normal controls, low cognitive reserve was associated with CPS decline in MS. High cognitive reserve protect MS patients from cognitive decline related to SCGM atrophy.

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Long-term outcome of pallidal stimulation for Meige syndrome

Journal of Neurosurgery ◽

10.3171/2017.7.jns17323 ◽

2018 ◽

Vol 130 (1) ◽

pp. 84-89 ◽

Cited By ~ 8

Author(s):

Shiro Horisawa ◽

Taku Ochiai ◽

Shinichi Goto ◽

Takeshi Nakajima ◽

Nobuhiko Takeda ◽

...

Keyword(s):

Rating Scale ◽

Small Scale ◽

Initial Symptom ◽

Long Term Outcome ◽

Time Points ◽

Meige Syndrome ◽

Pallidal Stimulation ◽

The Mean

OBJECTIVEMeige syndrome is characterized by blepharospasm and varied subphenotypes of craniocervical dystonia. Current literature on pallidal surgery for Meige syndrome is limited to case reports and a few small-scale studies. The authors investigated the clinical outcomes of deep brain stimulation (DBS) of the globus pallidus internus (GPi) in patients with Meige syndrome.METHODSSixteen patients who underwent GPi DBS at the Tokyo Women’s Medical University Hospital between 2002 and 2015 were included in this study. Burke-Fahn-Marsden Dystonia Rating Scale (BFMDRS) movement subscale (BFMDRS-M) scores (range 0–120) obtained at the following 3 time points were included in this analysis: before surgery, 3 months after surgery, and at the most recent follow-up evaluation.RESULTSThe patients’ mean age (± SD) at symptom onset was 46.7 ± 10.1 years, and the mean disease duration at the time of the authors’ initial evaluation was 5.9 ± 4.1 years. In 12 patients, the initial symptom was blepharospasm, and the other 4 patients presented with cervical dystonia. The mean postoperative follow-up period was 66.6 ± 40.7 months (range 13–150 months). The mean total BFMDRS-M scores at the 3 time points were 16.3 ± 5.5, 5.5 ± 5.6 (66.3% improvement, p < 0.001), and 6.7 ± 7.3 (58.9% improvement, p < 0.001).CONCLUSIONSThe results indicate long-term efficacy for GPi DBS for the majority of patients with Meige syndrome.

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The unified huntington's disease rating scale for advanced patients: Validation and follow-up study

Movement Disorders ◽

10.1002/mds.25678 ◽

2013 ◽

Vol 28 (14) ◽

pp. 1995-2001 ◽

Cited By ~ 12

Author(s):

Katia Youssov ◽

Guillaume Dolbeau ◽

Patrick Maison ◽

Marie-Françoise Boissé ◽

Laurent Cleret de Langavant ◽

...

Keyword(s):

Huntington's Disease ◽

Huntington’S Disease ◽

Rating Scale ◽

Follow Up Study ◽

Disease Rating

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BILATERAL SUBTHALAMIC DEEP BRAIN STIMULATION IN A PATIENT WITH PARKINSON'S DISEASE WHO HAD PREVIOUSLY UNDERGONE THALAMOTOMY AND AUTOLOGOUS ADRENAL GRAFTING IN THE CAUDATE NUCLEUS

Neurosurgery ◽

10.1227/01.neu.0000245585.93284.15 ◽

2006 ◽

Vol 59 (5) ◽

pp. E1140-E1140 ◽

Cited By ~ 5

Author(s):

Francesco Vergani ◽

Andrea Landi ◽

Angelo Antonini ◽

Erik P. Sganzerla

Keyword(s):

Parkinson’S Disease ◽

Parkinson's Disease ◽

Deep Brain Stimulation ◽

Brain Stimulation ◽

Rating Scale ◽

Disease Rating ◽

Noticeable Reduction ◽

Surgical Treatments ◽

Deep Brain

Abstract OBJECTIVE Subthalamic (Stn) deep brain stimulation (DBS) is a valid surgical therapy for the treatment of severe Parkinson's disease. In recent years, StnDBS has been proposed for patients who previously received other surgical treatments, such as thalamotomy and pallidotomy. Nonetheless, there is no consensus about the indications of DBS in patients who previously underwent surgery. To the best of our knowledge this is the first reported case of a patient treated with DBS after previous thalamotomy and adrenal grafting. CLINICAL PRESENTATION A 62-year-old man with a long history (more than 30 yr) of Parkinson's disease received unilateral thalamotomy and autologous adrenal graft on two independent occasions. Thalamotomy led to a significant improvement, although limited to the control of contralateral tremor. The autologous adrenal graft was of no benefit. For the subsequent occurrence of L-dopa related dyskinesias and severe “off” periods, the patient was referred to our center for StnDBS. INTERVENTION The patient underwent bilateral StnDBS, obtaining a satisfactory improvement of rigidity and bradykinesia on both sides. The 1-year follow-up evaluation showed a 46% improvement in the Unified Parkinson's Disease Rating Scale motor section, along with a noticeable reduction in antiparkinsonian therapy (81%). CONCLUSION This case is consistent with previous reports from the literature, suggesting that StnDBS is feasible and safe, even in patients who previously received other surgical treatments for Parkinson's disease, such as thalamotomy or cell grafting.

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Anosognosia Is Associated With Greater Caregiver Burden and Poorer Executive Function in Huntington Disease

Journal of Geriatric Psychiatry and Neurology ◽

10.1177/0891988719856697 ◽

2019 ◽

Vol 33 (1) ◽

pp. 52-58

Author(s):

P. Wibawa ◽

R. Zombor ◽

M. Dragovic ◽

B. Hayhow ◽

J. Lee ◽

...

Keyword(s):

Executive Function ◽

Caregiver Burden ◽

Huntington Disease ◽

Rating Scale ◽

Demographic Data ◽

Caregiver Support ◽

Trail Making ◽

Disease Rating ◽

Executive Deficits ◽

State Examination

Background:Anosognosia, or unawareness of one’s deficits, is estimated to occur in 25% to 50% of Huntington disease (HD). The relationship between anosognosia and increased caregiver burden found in other dementias has not been determined in HD.Methods:Patient–caregiver dyads presenting to a statewide HD clinic were assessed using the Anosognosia Scale and grouped into “anosognosia” and “no anosognosia.” Caregiver burden, measured by Zarit Burden Interview (ZBI) and Caregiver Burden Inventory (CBI), demographic data, and Unified Huntington’s Disease Rating Scale, including Mini-Mental State Examination, Stroop, Trail Making, Verbal Fluency, and Symbol Digit Modalities Tests, were compared between groups.Results:Of the 38 patients recruited, 10 (26.3%) met criteria for anosognosia. Patients with anosognosia elicited higher caregiver burden ratings on both the ZBI (mean difference 16.4 [12.1], P < .001) and CBI (16.7 [15.0], P < .005) while also demonstrating poorer executive function. Except for CAG burden score, between-group characteristics did not differ significantly. Stroop Interference predicted both anosognosia and caregiver burden.Conclusions:In HD, anosognosia is associated with greater caregiver burden and executive deficits. Its occurrence should prompt further patient assessment and increased caregiver support.

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Interferential current and photobiomodulation in knee osteoarthritis: A randomized, placebo-controlled, double-blind clinical trial

Clinical Rehabilitation ◽

10.1177/02692155211012004 ◽

2021 ◽

pp. 026921552110120

Author(s):

Renata Alqualo-Costa ◽

Érika Patrícia Rampazo ◽

Gustavo Ribeiro Thome ◽

Mônica Rodrigues Perracini ◽

Richard Eloin Liebano

Keyword(s):

Clinical Trial ◽

Knee Osteoarthritis ◽

Pain Intensity ◽

Rating Scale ◽

Pain Modulation ◽

Double Blind ◽

Time Points ◽

Double Blind Clinical Trial ◽

Interferential Current

Objectives: To evaluate the effects of interferential current and photobiomodulation in patients with knee osteoarthritis. Design: A randomized, placebo-controlled, double-blind clinical trial. Setting: Physiotherapy Clinic of City University of São Paulo. Subjects: A total of 184 patients with knee osteoarthritis were recruited and, of these, 168 were included and randomized into four groups with 42 each: interferential current, photobiomodulation, interferential current plus photobiomodulation or placebo groups. One hundred and sixty-four patients completed the study. Intervention: Patients received 12 sessions (three times a week) of treatment: 30 minutes of interferential current (active or placebo) followed by photobiomodulation (active or placebo). Main measures: Primary outcome: pain intensity at rest and during movement (numeric rating scale) after 12 sessions. Secondary outcomes: functional capacity (Timed Up & Go and Sit and Lift tests and Lequesne and WOMAC questionnaires), pressure pain threshold, conditioned pain modulation, and muscle strength production (isokinetic evaluation). Patients were assessed at baseline, after 12 sessions, and three and six months after the end of the treatment. Results: Interferential current plus photobiomodulation reduced pain intensity at rest and during movement compared to placebo and interferential current at all time points ( P < 0.05). Photobiomodulation reduced pain intensity at rest compared to placebo at all time points ( P < 0.05) and compared to interferential current at six months follow-up ( P < 0.05). Photobiomodulation reduced pain intensity during movement compared to placebo at six months follow-up ( P < 0.05). Conclusion: Interferential current plus photobiomodulation or isolated photobiomodulation improve pain intensity in knee osteoarthritis.

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The severity progression of non-motor symptoms in Parkinson’s disease: a 6-year longitudinal study in Taiwanese patients

Scientific Reports ◽

10.1038/s41598-021-94255-9 ◽

2021 ◽

Vol 11 (1) ◽

Author(s):

Yi-Chieh Chen ◽

Rou-Shayn Chen ◽

Yi-Hsin Weng ◽

Ying-Zu Huang ◽

Chiung Chu Chen ◽

...

Keyword(s):

Parkinson’S Disease ◽

Parkinson's Disease ◽

Longitudinal Study ◽

Disease Duration ◽

Rating Scale ◽

Medical Center ◽

Motor Symptoms ◽

Disease Rating ◽

Non Motor Symptoms

AbstractNonmotor symptoms (NMSs) cause major burden in patients with Parkinson’s disease (PD). Previous NMSs progression studies mostly focused on the prevalence. We conducted a longitudinal study to identify the progression pattern by the severity. PD patients recruited from the outpatient clinics of a tertiary medical center were evaluated by the Unified Parkinson's Disease Rating Scale and Non-Motor Symptoms Scale (NMSS). A retrospective study with three-step analysis was performed. Step 1, the NMSs severity was compared among patients stratified by disease duration every 2 years up to 10 years. Step 2, patients with repeated tests in 2 years were categorized into 4 groups by the diseased duration of every 5 years. Step 3, the NMSS score changes in 6 years follow-up were determined, and the dosage of anti-PD drugs was compared to the NMSs severity changes. 676 patients completed the step 1 analysis, which showed a trend of NMSs worsening but not significant until the disease duration longer than 4–6 years. Furthermore, the severity did not change between repeated evaluations in 2 years in all patients. The progression became apparent after 6 years. Individual symptoms had different progression patterns and the increment of medications was independent to NMSs evolution. We demonstrated the NMSs severity progression in Taiwanese PD patients and the independence of the medications and NMSs progression.

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Impact of SAfinamide on Depressive Symptoms in Parkinson’s Disease Patients (SADness-PD Study): A Multicenter Retrospective Study

Brain Sciences ◽

10.3390/brainsci11020232 ◽

2021 ◽

Vol 11 (2) ◽

pp. 232

Author(s):

Esteban Peña ◽

Carmen Borrué ◽

Marina Mata ◽

Juan Carlos Martínez-Castrillo ◽

Araceli Alonso-Canovas ◽

...

Keyword(s):

Parkinson’S Disease ◽

Parkinson's Disease ◽

Serotonin Syndrome ◽

Rating Scale ◽

Motor Symptoms ◽

Motor Complications ◽

Daily Life Activities ◽

Patient Global Impression ◽

Disease Rating

Background: We aimed to assess the effects of safinamide on depression, motor symptoms, and the serotonin syndrome related to its co-administration with antidepressants in patients with Parkinson’s disease (PD). Methods: We retrospectively analyzed the data of patients at 1 and 3 months of follow-up compared to baseline. Results: n = 82 (safinamide 50 mg = 22, 100 mg = 60, with antidepressants = 44). First, we found improvement in depression (Hamilton Depression Rating Scale: −6 ± 5.10 at 1 month and −7.27 ± 5.10 at 3 months, p < 0.0001; Patient Global Impression of Improvement Scale: 60.3% and 69.5% of patients at 1 and 3 months reported some improvement). Second, safinamide improved the daily life activities and motor symptoms/motor complications (Unified Parkinson’s Disease Rating Scale (UPDRS-II): −2.51 ± 6.30 and −2.47 ± 6.11 at 1 and 3 months, p < 0.0001; III: −3.58 ± 8.68 and −4.03 ± 8.95 at 1 and 3 months, p < 0.0001; IV: −0.61 ± 2.61 and −0.8 ± 2.53 at 1 and 3 months, p < 0.0001). Third, 7.31% and 8.53% of patients developed non-severe adverse events related to safinamide at 1 and 3 months. Serotonin syndrome was not observed in the patients treated with antidepressants; some isolated serotonin syndrome symptoms were reported. Conclusions: Safinamide could be useful for treating depression in PD; it was effective for motor symptoms and motor complications and safe even when co-administered with antidepressants.

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