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   2021| Sep-Dec  | Volume 4 | Issue 3  
    Online since December 22, 2021

 
 
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REVIEW ARTICLES
Neuroprotective strategies in Parkinson’s disease: A long road ahead
Divyani Garg, Soaham Desai
Sep-Dec 2021, 4(3):99-110
DOI:10.4103/AOMD.AOMD_38_21  
Neuroprotection has been a fascinating area of research in Parkinson’s disease (PD). It offers the promise of disease modification, in turn, slowing the disease progression. A vast array of agents has been assessed for its neuroprotective properties. Although many of these agents have achieved varying degrees of efficacy in preclinical models of PD, definitive success has not been observed in clinical trials. The reasons underlying the lack of success lie within the intrinsic heterogeneity of PD. Instead of using a single agent for all patients in a “one-size-fits-all” approach, it is increasingly apparent that a specific study population with a well-defined predominant pathogenic mechanism should be selected for trials, assessing the role of each agent targeting a specific mechanism. Coenzyme Q10 may find use in an enriched cohort of PD patients with PARKIN mutations. The glucagon-like peptide 1 (GLP-1) analogue, exenatide, is currently being assessed in a phase III trial. Other GLP-1 agonists, such as liraglutide, lixisenatide, and semaglutide, are undergoing phase II trials. In addition, coffee has been shown to have a nonlinear relationship with PD risk. With increasing genetic and molecular understanding of PD, the dream of neuroprotection in PD may be realized in the near future. In this review, we summarize the current evidence on neuroprotection in PD.
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Infection-associated dystonia: A narrative review
Rahul Yadav, Vijay Shankar, Soaham Desai
Sep-Dec 2021, 4(3):111-120
DOI:10.4103/AOMD.AOMD_13_21  
Infectious diseases are common in tropical countries, and varied complications associated with such diseases are frequently encountered. Movement disorders are a complication of infectious diseases, and the spectrum of movement disorders differs between tropical countries and other countries. We screened three electronic databases for cases of dystonia presenting as a manifestation of infections diseases and selected cases and series describing chorea associated with infections. The studies were identified and data regarding the study design, sample size, neurological assessment, and diagnostic workup, including brain imaging and cerebrospinal fluid analysis were extracted. After a detailed review of 139 selected articles, 39 articles were referred to in the final manuscript of this narrative review. Dystonia is most commonly associated with Japanese encephalitis than other central nervous system infections. The hypothesized mechanisms of infection-related dystonia are vasculopathy, space-occupying lesions, autoimmune reactions, inflammation, or via anti-dopaminergic drug therapy. The infections presenting with dystonia include tuberculosis, Japanese encephalitis, streptococcal infections, varicella-zoster virus, subacute sclerosing panencephalitis, dengue, and neurocysticercosis. In this narrative review, we discuss the different types of central nervous system infections that present with dystonia.
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ORIGINAL ARTICLES
Pattern reversal and flash visual evoked potentials in essential tremor
ªencan Buturak, Halit Fidanci
Sep-Dec 2021, 4(3):131-135
DOI:10.4103/AOMD.AOMD_59_20  
BACKGROUND: There is evidence that nonmotor clinical findings may occur in essential tremor (ET). The aim of the study was to determine whether there is a subclinical impairment of visual pathways in ET by conducting visual evoked potential (VEP) studies on patients with ET. METHODS: Healthy individuals and patients with ET were included in the study. Individuals with visual impairment, eye disease, neurodegenerative diseases, or migraine were not included in the study. Pattern reversal VEP (PrVEP) and flash VEP (FVEP) were applied to all individuals. N75, P100, and N135 waves were obtained by PrVEP, whereas N1, P2, N2, P2, N3, and P3 waves were obtained by FVEP. The latencies and amplitudes of these waves were analyzed. RESULTS: Thirty-five healthy individuals (12 male, 23 female) and 29 patients with ET (16 male, 13 female) were included in the study. The mean age of the patients in the control group and the patients with ET was 33.9 ± 8.5 and 35.9 ± 16.8 years, respectively. Age and gender were not statistically significantly different between the two groups. The mean right/left P100 wave latencies of the control group and ET group were 90.3 ± 7.3/91.5 ± 6.1 ms and 99.4 ± 9.1/101.1 ± 7.8 ms, respectively (P = 0.009/P < 0.001). The mean right/left P2 wave latencies of the control group and ET group were 104.9 ± 15.1/104.8 ± 13.8 ms and 117.6 ± 15.4/118.3 ± 15.6 ms, respectively (P = 0.001/P = 0.001). CONCLUSION: This study showed that subclinical involvement of visual pathways may occur in ET.
  1,419 131 -
Burden of nonmotor symptoms in Parkinson’s disease patients from eastern India
Sulagna Sahu, Adreesh Mukherjee, Samar Biswas, Valentina Leta, Katarina Rukavina, Shyamal Kumar Das, Atanu Biswas
Sep-Dec 2021, 4(3):121-130
DOI:10.4103/AOMD.AOMD_5_21  
BACKGROUND: Parkinson’s disease (PD) is classically characterized by motor features. However, nonmotor symptoms (NMSs) represent an important aspect of the disease with a significant impact on quality of life (QoL). OBJECTIVES: The aim of this study was to evaluate NMS in patients with PD, to determine their various correlates, and to assess the impact on QoL. METHODS: This cross-sectional study included 150 consecutive patients with PD and 150 age- and sex-matched healthy controls. NMSs were assessed using the NMS Questionnaire (NMSQuest) and NMS Scale (NMSS). QoL was evaluated by the 8-item Parkinson’s Disease Questionnaire scored as summary index (PDQ-8-SI). RESULTS: Every patient experienced NMS and 90% had more than five NMSs. Patients with PD had a significantly higher prevalence of NMS compared to healthy controls. The most common NMSs in patients with PD were unexplained pain, anxiety, constipation, insomnia, and memory impairment. Miscellaneous was the most prevalent domain of NMSS, followed by sleep/fatigue, gastrointestinal tract, and mood/cognition. Attention/memory impairment and pain were greater in females. Cardiovascular/falls and perceptual/hallucination showed a positive correlation with duration of disease. Sexual dysfunction decreased with increasing age. In the young-onset group (YOPD), mood/cognition involvement was higher. PDQ-8-SI showed a significant correlation with total NMSS score and most of the individual domain scores. Contrary to other Indian studies, our patients reported restless legs more frequently, whereas urinary symptoms were less common. Our observations showed a greater prevalence of pain and constipation compared to the Western studies. CONCLUSIONS: All of our patients experienced NMS. The prevalence of various NMS in our study showed differences with previous reports. NMS had a significant impact on QoL.
  1,324 119 -
CASE REPORTS
Autosomal recessive spastic ataxia of Charlevoix Saguenay (ARSACS) in three brothers of a family from Kerala
Sasikumar Sheetal, Amith K Sasikumar, Lovin G Tomy
Sep-Dec 2021, 4(3):149-152
DOI:10.4103/AOMD.AOMD_60_20  
Autosomal recessive spastic ataxia of Charlevoix Saguenay (ARSACS) is an uncommon spastic-ataxic syndrome that is characterized by cerebellar ataxia, spasticity, and peripheral neuropathy. Though initially reported from Charlevoix-Saguenay-Lac-St-Jean region in Canada, this neurodegenerative disorder has been reported from other regions of the world. There have been very few reports on this condition from Kerala, India. We hereby report a family of three brothers from Kerala who were diagnosed with this condition.
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ORIGINAL ARTICLES
Spectrum of childhood dystonia evaluated at a tertiary care center from south India
Ganaraja V H, Netravathi M, Nitish Kamble, Vikram V Holla, Dwarakanath Srinivas, Ravi Yadav, Pramod Kumar Pal
Sep-Dec 2021, 4(3):136-142
DOI:10.4103/AOMD.AOMD_8_21  
INTRODUCTION: Dystonia is one of the most common hyperkinetic movement disorders observed in children with neurological disorders. The objective of this study was to evaluate the demographic, etiological, and radiological profile of childhood dystonia. METHODS: This study is a retrospective chart review of children with dystonia (onset ≤18 years) who were admitted to our hospital from 2013 to 2017. All the relevant demographic data including the ethnicity, socioeconomic and cultural background, examination findings, electrophysiological, and other investigations were retrieved from the medical records. RESULTS: A total of 814 children were admitted and evaluated in the pediatric ward, of which 85 (38 girls) children had dystonia. Mean age at onset was 6.21 ± 5.21 years. Mean duration of illness was 3.51 ± 4.23 years. Generalized dystonia was noticed in 83.5% followed by focal dystonia in 8.2%. Hemidystonia and segmental dystonia were less commonly seen with 4.7% and 3.5% of cases, respectively. Brain magnetic resonance imaging (MRI) was available in 82 patients; and diagnostic in 56.1% in the form of Wilson’s disease (14.1%) neurodegeneration with brain iron accumulation (14.1%), perinatal insult (8.2%), encephalitis (7.1%), mitochondrial cytopathy (3.5%), glutaric aciduria (2.4%), isovaleric acidemia (1.2%), metachromatic leukodystrophy (1.2%), acute disseminated encephalomyelitis (1.2%), and neuronal ceroid lipofuscinosis (1.2%). Two patients underwent surgical therapy in the form of deep brain stimulation and bilateral pallidotomy. CONCLUSION: Dystonia constitutes 10.4% of pediatric neurological admissions in our study cohort. Generalized dystonia is the commonest subtype. Brain MRI is useful in identifying etiology and it was diagnostic in nearly half of the patients (56.1%).
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Analysis of dysgraphia in advanced Parkinson’s disease patients following bilateral STN-DBS: a prospective study
Swapnil Kolpakwar, Rajesh Alugolu, Vijaya Saradhi Mudumba, Rukmini Kandadai, Rupam Borgohain
Sep-Dec 2021, 4(3):143-148
DOI:10.4103/AOMD.AOMD_4_21  
BACKGROUND: Parkinson’s disease (PD) is characterized by varying intensities of bradykinesia, rigidity, and tremor leading to disturbances in writing skills of the patient. We undertook this study to evaluate dysgraphia features in advanced PD cases and changes in these features after bilateral subthalamic nucleus deep brain stimulation (DBS). METHODS: All idiopathic PD cases who underwent DBS at our center were included in this study. Patients were assessed preoperatively for dysgraphia by analysis of handwriting in “off” phase by a single investigator on bedside testing. For quantification of micrographia, vertical length of first letter and width of the word written were calculated. An analysis of legibility of handwriting was also done for all patients using Fahn-Tolosa-Marin Tremor Rating Scale (FTMTRS). Patients were analyzed for dysgraphia at 6 months post-surgery in drug ‘off’ phase, and outcomes were correlated with baseline parameters. RESULTS: There were a total of 51 patients who were included in our study. Significant reduction was noted in postoperative Unified Parkinson’s Disease Rating Scale part III (UPDRS III) scores (P = 0.0001). Age more than 65 years was associated with less improvement in FTMTRS grades. Prevalence of micrographia reduced in the postoperative period, but the difference was not statistically significant. Median FTMTRS grade in preoperative and postoperative “off” phase was 3 and 2, respectively. Difference in pre- and postsurgery FTMTRS grades was found to be statistically significant (P = 0.00001). CONCLUSION: Subthalamic nucleus DBS results in substantial improvement in legibility of handwriting of patients, particularly in cases with age less than 65 years.
  868 88 -
CASE REPORTS
Huntington’s disease presenting as adult-onset tourettism: a case report
Mitesh Chandarana, Udit Saraf, Kalikavil Puthanveedu Divya, Syam Krishnan
Sep-Dec 2021, 4(3):153-156
DOI:10.4103/AOMD.AOMD_1_21  
Huntington’s disease (HD) is an autosomal dominant progressive neurodegenerative disease, caused by trinucleotide repeat expansion (CAG) in the Huntingtin gene (HTT) on chromosome 4. It is typically characterized by the combination of chorea with or without other extrapyramidal symptoms, oculomotor abnormalities, cognitive decline, and neuropsychiatric manifestations. However, HD consists of considerable phenotypic variability. Though chorea is the most common extrapyramidal manifestation, it is also associated with other movement disorders such as dystonia, myoclonus, tics, parkinsonism, and ataxia.
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Functional movement disorders during the COVID-19 pandemic
Sneha Dayanand Kamath, Nitish Kamble, Sindhu D M, Kasturi A Sakhardande, Chethan Basavarajappa, Pramod Kumar Pal
Sep-Dec 2021, 4(3):161-163
DOI:10.4103/AOMD.AOMD_26_21  
Functional movement disorders (FMDs) are a heterogenous group of movement abnormalities that greatly affect the quality of life of patients. They usually manifest as a result of underlying psychological or psychiatric illnesses without any known structural or neurochemical diseases. Various neurological disorders such as encephalitis, stroke, demyelination, seizures, and neuropathy have been reported by otherwise healthy individuals during the ongoing coronavirus disease 2019 (COVID-19) pandemic. Here, we describe the case of a 27-year-old woman who presented to our outpatient department with episodes of deviation of angle of mouth with variability and distractibility. Following thorough clinical evaluation and appropriate investigation, the underlying etiology was identified as FMD secondary to the restrictions imposed during the COVID-19 pandemic to contain the transmission of the virus. The lockdown, isolation, financial strain, and other pandemic-related issues are stressors that may contribute to psychogenic disorders in people.
  682 100 -
PIGG gene mutation associated with Uner Tan syndrome: A first case report
Gautam Wali, Gurusidheshwar M Wali, Carolyn M Sue
Sep-Dec 2021, 4(3):157-160
DOI:10.4103/AOMD.AOMD_28_21  
Uner Tan syndrome (UTS) is a rare neurogenetic disorder characterized by poor cognition, dysarthric speech, and habitual quadrupedal locomotion, and is associated with cerebellar hypoplasia. Mutations in the VLDLR, CA8, WDR81, ATP8A2, and TUBB2B genes are commonly associated with UTS. However, here, we report the case of a patient presenting with quadrupedal locomotion and other clinical features similar to UTS caused by a mutation in the PIGG gene. To the best of our knowledge, this is the first case in which a mutation in the PIGG gene is associated with UTS. We believe that our finding will help broaden the genetic spectrum of the syndrome.
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