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Background: Parkinson's disease (PD), the second most prevalent neurological disorder in the elderly, manifests with distinctive movement disorders, including bradykinesia, resting tremor, and stiffness. With a progressive course, current treatment strategies primarily target symptomatic relief. Crocin is a chemical compound isolated from the dry stigma of Crocus sativus, and has demonstrated neuroprotective properties., Objectives: This study explores the impact of crocin on movement disorders and neuronal oxidative DNA damage in PD patients., Method: Conducted as a randomized, blinded, and controlled trial, this research focused on patients aged 30 to 80 with idiopathic PD. Using the second and third parts of the movement disorder society-unified PD rating scale (MDS-UPDRS), aspects of daily life activity and movement disorders were assessed before and after an 8-week intervention. Patients in the crocin groups received capsules containing 30 mg of crocin twice daily. Additionally, the 8-hydroxy-2-deoxydiguanosine (8-OHdG) to urinary creatinine ratio (8-OHdG/uCr) was measured to evaluate neuronal oxidative DNA damage., Results: Out of the initially evaluated 164 patients, 30 were randomly assigned to each group, with 53 subjects completing the study. Within-group analysis revealed a significant improvement in the second and third parts of MDS-UPDRS after 8 weeks of crocin intervention (P < 0.05). However, the 8-OHdG/uCr did not show significant changes. The well-tolerated daily dose of 60 mg of crocin demonstrated minimal side effects., Conclusion: This study establishes the efficacy of crocin in enhancing daily life activities and mitigating movement disorders, suggesting its potential as a supplementary intervention alongside conventional PD medications., Competing Interests: Declaration of competing interest The authors declare that they have no known competing financial interests or personal relationships that could have appeared to influence the work reported in this paper., (Copyright © 2024 Elsevier Ltd. All rights reserved.)

This study aimed to assess the prevalence of neurological symptoms and related imaging findings in patients with von Hippel-Lindau (VHL) at Rasool Akram Hospital from September 2018 to September 2021. This analytical observational study examined eligible patients over the period from September 2018 to September 2021. We collected demographic information (age, gender) along with imaging findings and results of neurological and eye examinations. Comparison between qualitative variables was also done using the Chi-square test or Fisher's exact test. Also, an independent t -test was used to compare quantitative variables between the two groups. SPSS version 22 software was used for statistical analysis of data. A significant level was considered less than or equal to 0.05. Of the 54 examined patients (48.1% were male and 51.9% were female) with an average age of 36.42 ± 13.37 years. A significant majority (87.0%) reported a positive family history of the disease. The most common type of disease was Type 1 observed in 94.4% of cases and Type 2A was the next most frequent (3.7%). The most common pattern of retinal pathological lesions seen in the examination was related to bilateral lesions (79.6%). The most common pathological finding was related to the presence of a mass in cerebellar magnetic resonance imaging (48.1%). Considering the findings of the present study, which highlight a significant frequency of bilateral retinal lesions as well as masses in the central nervous system and endocrine system, it is evident that patients require careful follow-up and various interventions after being diagnosed with the disease. This approach is essential to manage and potentially mitigate the complications associated with these conditions., Competing Interests: The authors declare no potential conflicts of interest with respect to research, authorship, and/or publication of this article., (Copyright: Habibi SAH., et al.)

Background: Olfaction has recently found clinical value in prediction, discrimination and prognosis of some neurodegenerative disorders. However, data originating from standard tests on olfactory dysfunction in Huntington's disease are limited to odour identification, which is only one domain of olfactory perceptual space., Method: Twenty-five patients and 25 age- and gender-matched controls were evaluated by the Sniffin' Sticks test in three domains of odour threshold, odour discrimination, odour identification and the sum score of them. Patients' motor function was assessed based on the Unified Huntington's Disease Rating Scale., Results: Compared with controls, patients' scores of all olfactory domains and their sum were significantly lower. Besides, our patients' odour threshold and odour discrimination impairments were more frequently impaired than odour identification impairment (86 per cent and 81 per cent vs 34 per cent, respectively)., Conclusion: Olfactory impairment is a common finding in patients with Huntington's disease; it is not limited to odour identification but is more pronounced in odour discrimination and odour threshold.

Background: The unique neurovascular structure of the retina has provided an opportunity to observe brain pathology in many neurological disorders. However, such studies on neurodegeneration with brain iron accumulation (NBIA) disorders are lacking., Objectives: To investigate NBIA's neurological and ophthalmological manifestations., Methods: This cross-sectional study was conducted on genetically confirmed NBIA patients and an age-gender-matched control group. The thickness of retinal layers, central choroidal thickness (CCT), and capillary plexus densities were measured by spectral domain-optical coherence tomography (SD-OCT) and OCT angiography, respectively. The patients also underwent funduscopy, electroretinography (ERG), visual evoked potential (VEP), and neurological examination (Pantothenate-Kinase Associated Neurodegeneration-Disease Rating Scale [PKAN-DRS]). The generalized estimating equation model was used to consider inter-eye correlations., Results: Seventy-four patients' and 80 controls' eyes were analyzed. Patients had significantly decreased visual acuity, reduced inner or outer sectors of almost all evaluated layers, increased CCT, and decreased vessel densities, with abnormal VEP and ERG in 32.4% and 45.9%, respectively. There were correlations between visual acuity and temporal peripapillary nerve fiber layer (positive) and between PKAN-DRS score and disease duration (negative), and scotopic b-wave amplitudes (positive). When considering only the PKAN eyes, ONL was among the significantly decreased retinal layers, with no differences in retinal vessel densities. Evidence of pachychoroid was only seen in patients with Kufor Rakeb syndrome., Conclusion: Observing pathologic structural and functional neurovascular changes in NBIA patients may provide an opportunity to elucidate the underlying mechanisms and differential retinal biomarkers in NBIA subtypes in further investigations. © 2023 International Parkinson and Movement Disorder Society., (© 2023 International Parkinson and Movement Disorder Society.)

Production of verb morphology, especially tense inflection, is usually impaired in individuals with agrammatism. There have been relatively few studies on treatment of verb tense inflection in agrammatic aphasia. In this study we adapted ACTION (a linguistically motivated treatment) to Persian language, to treat the production of regular and irregular verbs separately in sentence context. A single-subject multiple-baseline across behaviors design was used to establish the treatment effect. Using a non-probable convenience sampling, four Persian agrammatic patients with Broca's aphasia resulting from cerebrovascular accident (CVA) were recruited for this study. Two participants received treatment first for regular verbs (Phase 1, 4 weeks), and then for irregular verbs (Phase 2, 4 weeks). The other two participants received treatment in reverse order. In the final phase of treatment (Phase 3, 4 weeks), all 4 participants underwent a sentence construction treatment. All participants showed improvement in the production of trained tenses. Treatment also generalized to production of untrained regular verbs while generalization to irregular verbs was modest. Furthermore, improvement was found on narrative scores (e.g. MLU) after treatment. These findings suggest that Persian individuals with agrammatism could be trained to correctly apply temporal information to verb inflection in elicited speech.

Background: Transcranial Sonography is a non-invasive technique that has been used as a diagnostic tool for a variety of neurodegenerative disorders. However, the utility and potential application of this technique in NBIA disorders is scarce and inconclusive., Methods: In this cross-sectional retrospective case-control study, the echogenicity of Substantia Nigra (SN), Lentiform Nucleus (LN), and Diameter of the Third Ventricle (DTV) were assessed by TCS in genetically confirmed NBIA patients referring to the movement disorder clinic. The normal echogenicity area of SN was defined based on the 90th percentile of an age-and-gender-matched control group. NBIA patients underwent neurologic examination at each visit, but their brain magnetic resonance imaging and demographics were extracted from electronic records., Results: Thirty-five NBIA patients of four subtypes with a mean disease duration of 10.54 years and 35 controls were enrolled. The normally defined SN echogenicity in controls was 0.23 cm 2 . DTV and SN echogenicity areas were significantly higher in patients compared to the controls (P = 0.002 and < 0.001, respectively). Around 85% and 63% of the patients showed LN and SN hyperechogenicity at least on one side, respectively. Disease duration was positively correlated with DTV (r = 0.422, p = 0.015). Cases with Pantothenate Kinase Associated Neurodegeneration (n = 23) also had significantly higher DTV and SN echogenicity area compared to the controls., Conclusion: Despite most NBIA patients displayed increased DVT and higher SN and LN hyperechogenicity than healthy controls, the discriminatory role of TCS on different NBIA subtypes remains to be determined., Competing Interests: Declaration of Competing Interest None., (Copyright © 2023 Elsevier B.V. All rights reserved.)

Background: Neurodegeneration with Brain Iron Accumulation (NBIA) disorder is a group of ultra-orphan hereditary diseases with very limited data on its course., Objectives: To estimate the probability of preserving ambulatory ability and survival in NBIA., Methods: In this study, the electronic records of the demographic data and clinical assessments of NBIA patients from 2012 to 2023 were reviewed. The objectives of the study and factors impacting them were investigated by Kaplan-Meier and Cox regression methods., Results: One hundred and twenty-two genetically-confirmed NBIA patients consisting of nine subtypes were enrolled. Twenty-four and twenty-five cases were deceased and wheelchair-bound, with a mean disease duration of 11 ± 6.65 and 9.32 ± 5 years. The probability of preserving ambulation and survival was 42.9% in 9 years and 28.2% in 15 years for classical Pantothenate Kinase-Associated Neurodegeneration (PKAN, n = 18), 89.4% in 7 years and 84.7% in 9 years for atypical PKAN (n = 39), 23% in 18 years and 67.8% in 14 years for Mitochondrial Membrane Protein-Associated Neurodegeneration (MPAN, n = 23), 75% in 20 years and 36.5% in 33 years for Kufor Rakeb Syndrome (KRS, n = 17), respectively. The frequencies of rigidity, spasticity, and female gender were significantly higher in deceased cases compared to surviving patients. Spasticity was the only factor associated with death (P value = 0.03)., Conclusions: KRS had the best survival with the most extended ambulation period. The classical PKAN and MPAN cases had similar progression patterns to loss of ambulation ability, while MPAN patients had a slower progression to death. Spasticity was revealed to be the most determining factor for death., (© 2023 International Parkinson and Movement Disorder Society.)

Background: Parkinson's disease (PD) is one of the common neurodegenerative diseases, and there has been an increasing interest in the potential role of intestinal dysbiosis in its pathogenesis and related gastrointestinal complications such as constipation., Objectives: This study aims to evaluate the effects of multi-strain probiotics on constipation and motor function in PD patients., Methods: This study was a blinded, randomized controlled trial (RCT) that involved 27 PD patients who were diagnosed with constipation according to the ROME IV criteria for functional constipation. The primary outcome measured before and after the intervention in both the placebo and probiotic groups was the frequency of defecation. Secondary outcomes evaluated were laxative use, sense of complete evacuation, Bristol Stool Scale for consistency, and Unified Parkinson's Disease Rating Scale (UPDRS) scale. The study lasted for eight weeks. Both groups also were educated about lifestyle modification., Results: Of 30 included patients (15 in each group), 13 were women, and 17 were men. Three patients dropped out of the study. Between-group analysis showed that the frequency of bowel movements significantly increased in the probiotic group 4 [3 - 5] in comparison with 2 [2 - 3] in placebo (P = 0.02). Stool consistency also improved in the probiotic group (P = 0.04). However, there were no significant differences in other outcomes. The within-group analysis showed improvement in stool consistency in both probiotics and placebo groups (P = 0.01 and P = 0.007, respectively), while stool frequency and sense of complete evacuation significantly improved only in the probiotic group (P < 0.05)., Conclusions: This study demonstrated that multi-strain probiotics could improve frequency, consistency, and sense of complete evacuation in PD patients, while there was no significant effect on motor functions in 8 weeks. It is suggested that additional studies be conducted on longer-term effects., Competing Interests: The authors have no conflict of interest., (Copyright © 2023, Ghalandari et al.)

Background: Although speech and voice disorders are common in Parkinson's disease (PD), there is insufficient evidence to support the effectiveness of behavioural speech therapies in these patients., Aims: This study aimed to examine the effects of a new tele-rehabilitation program, a combining of conventional speech therapy and singing intervention, on voice deficits in patients with PD., Methods & Procedures: This study was a three-armed, assessor-masked, randomised controlled trial. Thirty-three people with PD were randomly assigned to the combination therapy, conventional speech therapy, or singing intervention group. This study followed the Consolidated Standards of Reporting Trials guidelines for non-pharmacological treatment. Each patient participated in 12 tele-rehabilitation sessions over 4 weeks. The combination therapy group received speech and singing interventions simultaneously (respiratory, speech, voice, and singing exercises). Voice intensity as a primary outcome and the voice handicap index (VHI), maximum frequency range, jitter and shimmer as secondary outcomes were evaluated 1 week before the first intervention session, 1 week after the last intervention session and 3 months after the last evaluation., Outcomes & Results: The results of repeated measures analysis of variance showed a significant main effect of time on all outcomes in all three groups after treatment (p < 0.001). There was a significant group effect for voice intensity (p < 0.001), VHI (p < 0.001), maximum frequency range (p = 0.014) and shimmer (p = 0.001). The combination therapy group demonstrated a significant outperformance in the VHI and shimmer than the speech therapy (p = 0.038) and singing intervention (p < 0.001) groups. The results of this study also indicated that combination therapy group compared to singing intervention group had a larger effect on voice intensity (p < 0.001), shimmer (p < 0.001) and maximum frequency range (p = 0.048)., Conclusions & Implication: The results demonstrated that combining speech therapy with a singing intervention delivered through tele-rehabilitation might be more effective in improving voice problems in patients with PD., What This Paper Adds: What is already known on the subject Parkinson's disease (PD) is a neurological disorder that frequently causes disturbances in speech and voice, which negatively affect patients' quality of life. Although speech difficulties occur in 90% of patients with PD, evidence-based treatment options for speech and language problems in these patients are limited. Therefore, further studies are required to develop and assess evidence-based treatment programs. What this study adds The findings of this study showed that a combination therapy program including conventional speech therapy approaches and individual singing intervention provided through tele-rehabilitation may have a greater effect on the improvement of voice problems in people with PD compared to speech therapy and singing intervention alone. What are the clinical implications of this work? Tele-rehabilitation combination therapy is an inexpensive and enjoyable behavioural treatment. The advantages of this method are that it is easy to access, appropriate for many stages of voice problems in PD, requires no prior singing training, encourages voice health and self-management and maximises treatment resources available to people with PD. We believe that the results of this study can provide a new clinical basis for treatment of voice disorders in people with PD., (© 2023 Royal College of Speech and Language Therapists.)

Background: Patients with extensive left hemisphere damage frequently have ideational apraxia (IA) and transcortical sensory aphasia (TSA). Difficulty with action coordination, phonological processing, and complex motor planning may not be indicative of higher-order motor programming or higher-order complex formation. We report on the effects of IA and TSA on the visual and motor skill of stroke patients., Purpose: The study aims to address the question of whether IA and TSA in bilingual individuals are the results of an error of motor function alone or due to a combined motor plus and cognitive dysfunction effect., Method: Twelve bilingual patients (seven males, and five females) were diagnosed with IA and TSA, and are divided into two groups of six patients. Then, 12 healthy bilingual controls were evaluated for comparing with both groups. Bilingual aphasia testing (BAT) and appropriate behavioral evaluation were used to assess motor skills, including coordination, visual-motor testing, and phonological processing., Results: Findings (pointing skills) show that the performance of the L1 and L2 languages are consistently significant ( p  < 0.001) in healthy individuals compared to the IA and TSA groups. Command skills for L1 and L2 languages were significantly higher in healthy individuals compared to IA and TSA controls ( p  < 0.001). Further, the orthographic skills of IA and TSA vs controls in both groups were significantly reduced ( p  < 0.01). Visual skills in the L1 language were significantly improved ( p  < 0.05) in IA and TSA patients compared to healthy controls after 2 months. Unlike orthographic skills which were improved in IA and TSA patients, languages in bilingual patients did not simultaneously improve., Conclusion: Dyspraxia is a condition that affects both motor and visual cognitive functions, and patients who have it often have less referred motor skills. The current dataset shows that accurate visual cognition requires both cognitive-linguistic and sensory-motor processes. Motor issues should be highlighted, and skills and functionality should be reinforced along with the significance of treatment between IA and TSA corresponding to age and education. This can be a good indicator for treating semantic disorders.

Purpose: Patients with idiopathic Parkinson's disease (PD) suffer from different non-motor symptoms, including pain. The present study aimed to measure the psychometric properties of the Brief Pain Inventory (BPI) in patients with PD during ON- and OFF-states., Methods: We recruited 460 patients with PD and 100 non-PD controls. The pain was assessed by the BPI, King's Parkinson's disease Pain Scale (KPPS), Neuropathic Pain Symptom Inventory (NPSI), Visual Analogue Scale-Pain (VAS-pain), and short-form McGill Pain Questionnaire-2 (SF-MPQ-2) in both medication states. Internal consistency and test-retest reliability was examined using Cronbach's alpha coefficient and intra-class correlation coefficient (ICC). Dimensionality and convergent validity of BPI were also investigated. Diagnostic accuracy and discriminative validity were determined by Receiver Operating Characteristics (ROC) curve analysis and Area Under the Curve (AUC)., Results: Cronbach's alpha was satisfactory ( α  = 0.91-0.97) in both states. The ICC values were 0.85-0.96 in ON- and OFF-state. Factor analysis revealed two factors. A high correlation was obtained between BPI subscales and other scales. AUC >0.91, sensitivity, and specificity> 0.77 were observed for discriminating different pain levels. Furthermore, appropriate diagnostic accuracy was found (AUC, sensitivity, and specificity >0.67) between non-PD control and PD patients., Conclusion: The BPI has acceptable psychometric features as well as diagnostic accuracy for patients with PD.Implications for rehabilitationPain as a non-motor symptom in PD can affect daily and social activities.The BPI is used to assess pain severity and interference in activities.For better treatment, pain should be assessed in off-state like to on-state.BPI has satisfactory reliability and validity in different medication states in PD.

Background: Up to know, limited and contradictory results have been published about the role of prognostic values of lipid profile and proprotein convertase subtilisin/kexin type 9 (PCSK9) in Parkinson's disease (PD). The aim of the present study is to investigate the role of lipid profile and PCSK9 in patients with PD and compare it with healthy individuals. Methods: In this case-control study, 31 individuals diagnosed with PD were compared with 31 healthy individuals. The lipid profile and PCSK9 of research participants were measured and the resulting data were analyzed using SPSS software. The P-values smaller than 0.05 were considered significant. Results: The mean age of participants in the PD and control group was 56.9 ± 8.8 and 53.7 ± 10.1 years, respectively (P > 0.050). 27 individuals (87.1%) in the PD group and 13 individuals (41.9%) in the control group were men. Low-density lipoprotein (LDL) level (84.2 ± 24.9 ml/dl vs. 105.5 ± 16.8, P < 0.001), high-density lipoprotein (HDL) level (45.5 ± 8.7 ml/dl vs. 51.1 ± 9.5 ml/dl, P < 0.001), and total cholesterol (155.3 ± 31.2 ml/dl vs. 192.8 ± 32.5 ml/dl, P < 0.001) were lower and triglyceride (TG) level was higher in the PD group (133.3 ± 79.3 ml/dl vs. 131.2 ± 58.6 ml/dl, P = 0.900) compared with the control group. PCSK9 level was higher in the PD group, but no significant difference was found (141.6 ± 70.0 vs. 129.7 ± 51.0 ng/ml, P = 0.500) compared to healthy subjects. Moreover, there was no relation between PCSK9 and severity of PD. Conclusion: Our findings showed that individuals with PD had lower levels of HDL, LDL, and total cholesterol compared with the control group. However, higher concentrations of PCSK9 were observed in patients with PD compared with healthy volunteers., Competing Interests: The authors declare no conflict of interest in this study., (Copyright © 2022 Iranian Neurological Association, and Tehran University of Medical Sciences Published by Tehran University of Medical Sciences.)

Background: Retinal biomarkers in neurodegenerative disorders have attracted much attention in recent years. Recent studies have reported visual dysfunction in Huntington's disease (HD). However, little is known about retinal structural changes in HD., Methods: A total of 50 subjects, including 25 motor-manifest HD patients and 25 gender- and age-matched controls, were enrolled. Unified Huntington's Disease Rating Score-Motor part was assessed in HD patients. Spectral-domain Optical Coherence Tomography (SD-OCT) was used to evaluate the macular thickness and peripapillary retinal nerve fiber layer (pRNFL). Superficial and deep capillary plexus densities were measured using OCT angiography (OCTA). To account for inter-eye correlation, generalized estimating equation (GEE) model was used., Results: HD patients had a significant reduction in macular thickness in both inner and outer superior sectors and the inferior outer sector. Inferior pRNFLs were significantly decreased in thickness. There was no significant difference in retinal capillary plexus density between the two groups. Age and disease duration were negatively correlated with macular thickness in HD patients. However, the severity of motor involvement was not correlated with SD-OCT or OCTA parameters., Conclusions: We observed attenuated pRNFL and macular retinal thickness in patients with HD, independent of macular capillary plexus parameters. It can support the hypothesis that the retina may be a potential biomarker for monitoring the neurodegenerative process in HD., (© 2022. Fondazione Società Italiana di Neurologia.)

Objective: Sleep problems are nonmotor symptoms in Parkinson's disease that should be carefully evaluated for better management and treatment. Parkinson's Disease Sleep Scale (PDSS-2) is one of the most reliable tools for measuring sleep difficulties in people with Parkinson's disease. This study investigated the psychometric properties of the Persian version of PDSS-2., Methods: Four hundred and fifty-six people with Parkinson's disease with a mean age ±standard deviation of 60.7 ± 11.3 years were engaged in this study. Acceptability was assessed by floor and ceiling effects. Dimensionality was measured by exploratory factor analysis. The convergent validity of PDSS-2 with the Hospital Anxiety and Depression Scale (HADS) was assessed. Internal consistency and test-retest reliability were assessed with Cronbach's alpha and intraclass correlation coefficient (ICC), respectively., Results: No noticeable ceiling and floor effect was detected. The dimensionality analysis showed three factors. A high correlation was obtained between PDSS-2 and HADS (anxiety subscale). Excellent internal consistency with α  = 0.94, and good test-retest reliability with ICC = 0.89 were obtained., Conclusion: This study showed that the Persian version of Parkinson's Disease Sleep Scale has acceptable validity and reliability for measuring sleep disturbances in people with Parkinson's disease., Competing Interests: The authors declare that they have no conflicts of interest., (Copyright © 2021 Mohammad Taghi Joghataei et al.)

Objective: Neuropathic pain is a type of pain reported in people with Parkinson's disease. There are various scales to evaluate the characteristics of this kind of pain. The purpose of this study was to investigate the psychometric properties of the Neuropathic Pain Symptom Inventory (NPSI), a specific scale that measures neuropathic pain in Iranian people with Parkinson's disease., Method: Four hundred forty-seven individuals with Parkinson's disease were recruited in the study. Acceptability, internal consistency (Cronbach's alpha), and test-retest reliability (intraclass correlation coefficient, ICC) of NPSI were calculated. Dimensionality was examined through exploratory factor analysis. For convergent validity, correlations of NPSI with Douleur Neuropathic 4, Brief Pain Inventory, King's Pain Parkinson disease Scale, and Visual Analog Scale-Pain were used. Discriminative validity and sensitivity to change between On- and Off- medication states were analyzed., Results: A marked floor effect was observed for this scale (64.2%). Cronbach's alpha and ICC were 0.90 and 0.87, respectively. Items of NPSI were placed in 4 factors. A moderate to the strong association (r s  = 0.55 to 0.85) between NPSI and other scales was obtained. The results of discriminative validity and sensitivity to change indicate the ability of NPSI to show differences between medication states., Conclusion: The results of this study suggest that NPSI has acceptable reliability, validity, and sensitivity to change, indicating that this scale is suitable for measuring neuropathic pain in Iranian people with Parkinson's disease., (© 2020. Fondazione Società Italiana di Neurologia.)

Objectives: Physicians have prescribed anticholinergic agents as monotherapy or adjuvant therapy in patients with Parkinson's disease for decades. However, these medications can cause many adverse effects including gait freezing and falling. Herein we assessed the effects of anticholinergic medications on motor function, freezing of gait and falling in a group of patients with PD., Patients and Methods: This prospective study evaluated the effect of gradual discontinuation of anticholinergics on motor function in 131 outpatients with Parkinson's disease. We assessed patients' motor function at baseline six and twelve months later using the UPDRS-III. We also evaluated freezing of gait and falling in patients using UPDRS-II part 14 and 13 respectively. The anticholinergics were tapered and gradually discontinued and additional levodopa doses were added as patients needed., Results: 131patients successfully discontinued their anticholinergic medications. Stopping anticholinergics significantly improved the motor symptoms in PD patients as reflected in the change between the mean (±SD) UPDRS-III score of 36.85(±11.5) at the baseline to 32.51(±11.4) and 31.43 (±11.3) after six and twelve months (P < 0.001). The mean (±SD) scores of freezing of gait (FOG)significantly changed from 1.34(±1) to 1.17(±1) and 0.6(±0.7) and for falling down from 0.62(±0.8) to 0.5 (±0.8) and 0.29(±0.5) respectively (p-value of <0.001)., Conclusion: Our finding demonstrated an improvement in motor function and FOG and falling incidences in PD patients, after discontinuation of anticholinergic drugs. As motor complications adversely affect the quality of life in PD patients, clinicians must be careful with the unnecessary use of anticholinergic drugs in their treatment strategies., (Copyright © 2021. Published by Elsevier B.V.)

Background: Pain is one of the most common non-motor symptoms in Parkinson's disease (PD). Using an appropriate and specific measuring tool would be helpful in managing the pain. King's Parkinson's disease Pain Scale (KPPS) is an instrument designed to specifically measure pain in people with PD., Purpose: This study aimed to examine the psychometric properties of the Persian version of KPPS (KPPS-P) and its cut-off points for pain severity levels., Methods: A total of 480 people with PD (with a mean (SD) age of 60.89 (10.98)) were recruited. The acceptability of KPPS-P was calculated. The structural validity and discriminant validity for different levels of pain was explored via the factor analysis, and Receiver Operating Characteristics (ROC) curves, respectively. Internal consistency, test-retest, and inter-rater reliability were estimated by Cronbach's alpha and Interclass Correlation coefficient (ICC). Convergent validity was established between KPPS-P and other scales including Visual Analog Scale-Pain, Douleur Neuropathic 4, Brief Pain Inventory, Short-form McGill Pain Questionnaire-2, and Parkinson's Disease-8., Results: A significant floor effect was observed. The exploratory factor analysis revealed 4 factors. Cronbach's alpha and ICC values were higher than 0.80. The correlation range between KPPS-P and other scales was 0.35-0.76. Cut-off points of 0, 17, and 68 were obtained to discriminate pain severity levels between no pain, mild, moderate, and severe pain, respectively, with sensitivity and specificity above 0.80., Conclusion: Our results indicate that the Persian version of KPPS not only has acceptable psychometric properties to assess pain in PD but also has the ability to distinguish between different levels of pain severity., (Copyright © 2021 Elsevier B.V. All rights reserved.)

Objectives: Primary generalized dystonia (PGD) due to heterozygous torsin 1A (TOR1A) gene mutation (DYT1) is a childhood onset dystonia with rapid deterioration of symptoms, leading to severe disability in adolescence. Globus pallidus interna deep brain stimulation (GPi-DBS) has been shown to provide significant improvement in these cases., Methods: This was a retrospective study of TOR1A mutation positive dystonia patients, conducted at a university hospital from 2006 to 2018. Burke-Fahn-Marsden Dystonia Rating Scale (BFM-DRS) was used to evaluate dystonia severity before and after surgery. Emergence of postsurgical parkinsonian symptoms was evaluated using the Unified Parkinson Disease Rating Scale (UPDRS) part III. Montreal Cognitive Assessment (MOCA) was applied to assess cognitive dysfunction. SPSS version 18 was used for data analysis., Results: Eleven patients entered for analysis with an average age of 22.36 (±3.35) years (range: 18-28). Seven patients (63.6 %) were female. Mean follow-up period was 8.72 (±0.87). Difference between baseline and most recent BFM scores was significant (disability: 10.5 ±4.52 versus 2.09 (±3.20), P: 0.001; severity: 48.45 (±17.88) versus 9.36 (±10.47), P<0.001). The mean MOCA and UPDRS III scores after 7-9 years of DBS were 27.18 (±2.99), and 6.09 (±4.15), respectively., Conclusion: Our experience confirms that GPi-DBS in pediatric patients with DYT1 dystonia is overall successful, with significant and long-lasting positive effects on motor and cognitive functions. There was no prominent side effect in long-term follow up., (Copyright © 2020 Elsevier B.V. All rights reserved.)

Introduction: Freezing of gait, a common PD motor symptom, could increase the risk of falling. This study aimed to investigate the clinimetric attributes of the Freezing of Gait Questionnaire (FOGQ) for people with Parkinson disease in the "off" state., Methods: A total of 115 patients with Parkinson disease (PD; mean age, 60.25 years) were included. Acceptability, internal consistency (by the Cronbach alpha, and test-retest by Intraclass Correlation [ICC]), and reliability of the Persian-translated version of the FOGQ were examined. Dimensionality was estimated by Exploratory Factor Analysis (EFA). Fall efficacy scale-international, unified Parkinson disease rating scale-II, Berg balance scale, functional reach test, and Parkinson disease questionnaire-39 were applied to determine the convergent validity. Diagnostic accuracy for obtaining optimal cutoff point, separating faller and non-faller groups, was analyzed by Receiver Operating Characteristics (ROC) curve analysis and Area Under the Curve (AUC). All tests were carried out in an "off" state., Results: The Cronbach alpha was high (α=0.92). The test-retest showed high reliability (ICC=0.89). The FOGQ was unidimensional according to the EFA and had acceptable convergent validity with moderate to high correlation with other clinical scales. The optimal cutoff point to discriminate fallers from non-fallers during the "off" state was 9/10, with an AUC of 0.92., Conclusion: Our results suggest that the FOGQ has appropriate reliability, validity, and discriminative ability for measuring FOG in patients with PD during the "off" state., Competing Interests: Conflict of interest The authors declared no conflict of interest., (Copyright© 2021 Iranian Neuroscience Society.)

Background: Independence in activities of daily living (ADL) is one of the most important aspects in planning treatment for people with Parkinson disease (PD). The Barthel Index (BI) and modified Rankin Scale (mRS) are commonly used in neurological diseases., Objective: This study was conducted to confirm the validity and reliability of the BI and mRS in PD during ON and OFF medication phases., Methods: We included 260 individuals with a diagnosis of idiopathic PD. The disability in ADL was measured by the BI, mRS, Parkinson's Disease Questionnaire-39 (PDQ-39), Unified Parkinson Disease Rating Scale-Activities of Daily Living (UPDRS-ADL), and Schwab and England ADL scale (SE). Test-retest, inter-rater reliability, and internal consistency were assessed by the intra-class correlation (ICC) and Cronbach α coefficients. Dimensionality was evaluated by factor analysis. Convergent validity was assessed by the SE, Berg Balance Scale (BBS), PDQ-39 and UPDRS-ADL., Results: For the 260 participants (187 [71.9%] males; mean [SD] age 60.3 [12.3] years), both the BI and mRS achieved an acceptable level of test-retest and inter-rater reliability (ICC=0.77 to 0.91) in ON and OFF medication phases. The Cronbach α for BI was 0.85 and 0.88, respectively. We found 1 and 2 factors for BI in ON and OFF phases, respectively. Investigation of convergent validity showed moderate to high correlation for the BI with the UPDRS-ADL, SE, PDQ-39 (ADL), BBS and mRS scores in ON and OFF phases (ρ=0.51-0.74) and mRS with SE, UPDRS-ADL, PDQ-39 (ADL) and BBS scores (ρ=0.48-0.82)., Conclusion: The BI and mRS showed acceptable validity and reliability to measure the degree of disability in patients with PD in daily activities in both ON and OFF medication phases., (Copyright © 2019 Elsevier Masson SAS. All rights reserved.)

Background: Nowadays, many neurological conditions, including Parkinson's disease (PD), are treated with deep brain stimulation (DBS). Life-threatening consequences can occur from DBS hardware failure or sudden implantable pulse generator (IPG) battery depletion. This issue may potentially worsen in concomitance with medical or infectious conditions, requiring stronger emergency management. Methods: We present here a 58 year-old PD patient with DBS, whose IPG replacement surgery was complicated by severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) infection and we report management of this patient along with recommendations for patients with similar situation. Results: As the newly-emerged coronavirus disease 2019 (COVID-19) is now announced to be pandemic, new protocols and specific measures for each individual group of patients with chronic diseases seem obligatory. Regarding our recent experience with a patient suffering from PD, on DBS treatment, who needed hospitalization, we felt useful to share our experience as a recommended protocol for similar patients in the time of current pandemic. Conclusion: Close monitoring of laboratory and clinical signs should be warranted in patients with PD awaiting IPG replacement in order to be prepared in these novel conditions that may precipitate an akinetic crisis/dystonic storm and to prevent life-threatening complications during the current pandemic., (Copyright © 2020 Iranian Neurological Association, and Tehran University of Medical Sciences Published by Tehran University of Medical Sciences.)

Purpose: Pain is one of the nonmotor symptoms of Parkinson's disease (PD) that, in order to be better managed, requires to be evaluated. Evaluations are done using pain assessment scales such as the Short-Form McGill Pain Questionnaire-2 (SF-MPQ-2). The goal of this study was to assess the psychometric properties of SF-MPQ-2 to measure pain in people with PD., Methods: Four hundred and twenty-eight PD patients with a mean (SD) age of 60.11 (11.44) years were included. Accessibility was measured through floor and ceiling effects. Dimensionality was estimated by exploratory factor analysis. The association between SF-MPQ-2 and other scales such as Neuropathic Pain Symptom Inventory, Douleur Neuropathic 4, Brief Pain Inventory, King's Pain Parkinson's Disease Scale, and Visual Analog Scale-Pain was considered to calculate convergent validity. Internal consistency and test-retest reliability were assessed by Cronbach's alpha and intraclass correlation coefficient (ICC), respectively., Results: A noticeable floor effect was found. Dimensionality results indicated four factors for this scale. A strong relationship was found between the SF-MPQ-2 total score and other scales ( r  = 0.55 to 0.85). In reliability analysis, Cronbach's alpha and ICC were 0.93 and 0.94 for SF-MPQ-2, respectively., Conclusion: The results of this study showed that SF-MPQ-2 has adequate validity and reliability to measure pain in people with Parkinson's disease., Competing Interests: The authors declare that there are no conflicts of interest regarding the publication of this paper., (Copyright © 2020 Maryam Mehdizadeh et al.)

Background: Tongue protrusion dystonia is an uncommon focal dystonia involving the lingual muscles. Causes of tongue protrusion dystonia include tardive dystonia, posthypoxic dystonia, neuroacanthocytosis, pantothenate kinase-associated neurodegeneration, and Lesch-Nyhan syndrome., Method: We summarize three children with pantothenate kinase-associated neurodegeneration and tongue protrusion dystonia. All three patients underwent careful neurological examination, brain magnetic resonance imaging, and genetic testing., Results: Tongue protrusion dystonia was a prominent and disabling symptom in all three patients. Brain magnetic resonance imaging revealed a typical eye of the tiger sign in all patients. Two patients had the same genetic mutation (c.1168 A>T mutation, p.I390F)., Conclusions: Tongue protrusion dystonia may be a clue to the underlying etiology of dystonia, including hereditary forms of dystonia. Among them, pantothenate kinase-associated neurodegeneration is an important cause, especially in children., (Copyright © 2019 Elsevier Inc. All rights reserved.)

Background There is growing evidence that exercise modalities have considerable effects on Parkinson's disease (PD). This trial aimed to provide a more detailed viewpoint of short-term and long-term treadmill training (TT) effects on some motor and non-motor features of PD. Methods In this prospective, randomized, single-blind clinical trial, 20 mild to moderate PD patients, admitted in Rasoul-e-Akram hospital in Tehran, Iran, were randomly allocated in case (11) and control (9) groups. Treadmill intervention was performed at moderate intensity with 60% of heart rate reserved (HRR) in two 30-min sessions/week for a duration of 10 weeks. Both the groups were evaluated for three times; at the baseline, 2 months later and then 2 months after the second evaluation. We assigned the Timed Up and Go test (TUG), 6-min walk test (6MW), and the SF-8 healthy questionnaire, for assessment of balance, functional capacity, and Quality of life (QoL), respectively. Results Balance and functional capacity were significantly improved in the case group after the intervention (TUG p-value: 0.003, 6MW p-value: 0.003). Moreover, the long-term analysis revealed significant results as well (TUG p-value: 0.001, 6MW p-value: 0.004). Mental condition's scores of SF-8 in cases were not statistically different in short-term follow-up (F/U). However, analysis illustrated p-value: 0.016 for long-term assessment. The intervention induced significant changes in physical condition's scores in both of the F/Us (PC p-value: 0.013). Conclusions This study provides evidence that a TT of mild to moderate intensity has significant and persistent benefits for the balance, functional capacity, and QoL in PD.

Background: Tremor is one of the most common symptoms of Parkinson's disease (PD), which is widely being used in the diagnosis procedure. Accurate estimation of PD tremor based on Unified PD Rating Scale (UPDRS) provides aid for physicians in prescription and home monitoring. This article presents a robust design of a classification system to estimate PD patient's hand tremors and the results of the proposed system as compared to the UPDRS., Methods: A smartphone accelerometer sensor is used for accurate and noninvasive data acquisition. We applied short-time Fourier transform to time series data of 52 PD patients. Features were extracted based on the severity of PD patients' hand tremor. The wrapper method was employed to determine the most discriminative subset of the extracted features. Four different classifiers were implemented for achieving best possible accuracy in the estimation of PD hand tremor based on UPDRS. Of the four tested classifiers, the Naive Bayesian approach proved to be the most accurate one., Results: The classification result for the assessment of PD tremor achieved close to 100% accuracy by selecting an optimum combination of extracted features of the acceleration signal acquired. For home health-care monitoring, the proposed algorithm was also implemented on a cost-effective embedded system equipped with a microcontroller, and the implemented classification algorithm achieved 93.8% average accuracy., Conclusions: The accuracy result of both implemented systems on MATLAB and microcontroller is acceptable in comparison with the previous works., Competing Interests: There are no conflicts of interest.

In this study, the role of known Parkinson's disease (PD) genes was examined in families with autosomal recessive (AR) parkinsonism to assist with the differential diagnosis of PD. Some families without mutations in known genes were also subject to whole genome sequencing with the objective to identify novel parkinsonism-related genes. Families were selected from 4000 clinical files of patients with PD or parkinsonism. AR inheritance pattern, consanguinity, and a minimum of two affected individuals per family were used as inclusion criteria. For disease gene/mutation identification, multiplex ligation-dependent probe amplification, quantitative PCR, linkage, and Sanger and whole genome sequencing assays were carried out. A total of 116 patients (50 families) were examined. Fifty-four patients (46.55%; 22 families) were found to carry pathogenic mutations in known genes while a novel gene, not previously associated with parkinsonism, was found mutated in a single family (2 patients). Pathogenic mutations, including missense, nonsense, frameshift, and exon rearrangements, were found in Parkin, PINK1, DJ-1, SYNJ1, and VAC14 genes. In conclusion, variable phenotypic expressivity was seen across all families.

Purpose: Parkinson disease (PD) is one of the common causes of imbalance, and the balance assessment is necessary for treatment and rehabilitation of these patients. The Berg Balance Scale (BBS) has been the main instrument used to evaluate balance impairment. The purpose of this study is to investigate reliability and validity of the Persian translation of BBS in Parkinson disease., Methods: One hundred PD patients (with mean age of 56.8 ± 15.13 years) were included. Interrater reliability was measured with the Kappa statistics and interclass correlation coefficients., Results: The mean values of the BBS scored by the two evaluators were 47/85 ± 11/09 and 48/03 ± 10/90, respectively. The mean of Kappa coefficient between two examiners was 0.76, which was between 0.38 and 0.93 for various items. The total score recorded by both examiners, interclass correlation coefficient, was 0.99, which is excellent. Cronbach's alpha for Iranian version of BBS was 0.92, which shows the excellent reliability of the questionnaire (0.62-0.9 for all items)., Conclusion: The Persian version of the BBS has excellent interrater reliability and internal consistency for the assessment of PD patients.

Background: Despite the negative effect of fear of falling during functioning and social participation of patients with Parkinson' disease, so far, only few studies have investigated its effect on the quality of life in these patients. We aimed to investigate the association between fear of falling and quality of life controlling for balance impairments based on hip and ankle strategy in drug On- and Off-phase of patients with idiopathic Parkinson' disease. Methods: In this non-experimental cross-sectional study, 139 patients with idiopathic Parkinson' disease (100 male, 39 female) by mean± SD age of 60.2±12.27 years, mean±SD time since diagnosis of 6.7±5.53 years and mean±SD Hoehn and Yahr stage of 2.8±1.49 were selected by a simple non-probability method. Balance function was measured by a functional reach test with hip and ankle strategy. The Persian version of the selfcompleted Fall Efficacy Scale-International and Parkinson's disease quality of life questionnaire was used to evaluate fear of falling and quality of life, respectively. Results: The results showed that the score of all dimensions of quality of life (i.e., mobility, activities of daily living, emotional wellbeing, stigma, social support, cognition, communication and bodily discomfort) were significantly affected by the intensity of fear of falling. Multiple regression analysis indicated a significant association between fear of falling and quality of life in a way that fear of falling explained 11% to 47% and 12% to 43% of variance in drug On-phase, as well as 8% to 45% and 9% to 48% of variance in the drug Off-phase in dimensions of quality of life after controlling for balance function based on hip and ankle strategy, respectively. In the drug On-phase, the strongest association (R=0.85, p<0.001) was found between fear of falling and mobility dimension of quality of life. In the drug Off-phase, the strongest relation was observed between fear of falling and mobility (R=0.82, p<0.001) as well as activities of daily living (R=0.78-0.79, p<0.001) dimensions. Conclusion: This study found that fear of falling affects the quality of life of patients with Parkinson' disease beyond its relationship with balance impairments based on the hip and ankle strategy in both drug On- and Off-phase.