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   Table of Contents - Current issue
Sep-Dec 2020
Volume 3 | Issue 3
Page Nos. 125-192

Online since Saturday, November 7, 2020

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Neurosyphilis and movement disorder: Old box, new “candy” p. 125
Anumeha Mishra, Sanjay Pandey
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Newer therapies for advanced Parkinson’s disease: Choosing among “many rights” p. 127
Roopa Rajan, Kanwaljeet Garg
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Neurosyphilis-associated movement disorder: A literature review p. 129
Jamir Pitton Rissardo, Ana Letícia Fornari Caprara
Syphilis is a well-known “great simulator/mimicker” of other diseases. Over the last decades, the clinical features of neurosyphilis have changed with an increasing percentage of atypical manifestations. In this context, movement disorders caused by neurosyphilis are rare and challenging to diagnose. This literature review aimed to evaluate the clinical epidemiological profile, pathological mechanisms, and historical features of neurosyphilis-associated movement disorders. Relevant reports in six databases were identified and assessed by two reviewers without language restriction. A total of 84 reports containing 168 cases who developed a movement disorder related to neurosyphilis were reported. The mean and the median reported ages were 40.50 (standard deviation [SD], 20.30) and 43 years (2.5–72.5 years). The predominant sex was male (79.16%). Argyll Robertson pupils were found in 54.90% of the individuals. The movement disorders reported were tremor, chorea, parkinsonism, ataxia, myoclonus, dystonia, athetosis, and ballism. In the literature, we have a large number of reports about movement disorder associated with neurosyphilis. But, in the majority of them, the individuals had the syphilitic diagnosis based on unspecific methods, electrodiagnostic studies were not performed, or penicillin therapy was unavailable. Also, we believe that any patient presenting with a movement disorder should have a thorough neurological examination of pupillary reflex, and if any abnormality is present, syphilitic laboratorial tests should be done.
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Subcutaneous apomorphine in advanced Parkinson’s disease and its use in Indian population p. 145
Vinod Metta, Rupam Borgohain, Prashanth L Kukkle, Rukmini Mridula, Pankaj Agarwal, Asha Kishore, Vinay Goyal, Ray Chaudhuri
Although subcutaneously administered apomorphine is widely used as an effective adjunct therapy for Parkinson’s disease (PD), it was approved for use in India only in 2019. This review summarizes the history, pharmacology, clinical spectrum, indications, efficacy, and side effects of subcutaneous apomorphine use in patients with PD along with clinical recommendations for its use in Indian context. Intermittent subcutaneous apomorphine injection or continuous subcutaneous apomorphine infusion is effective adjunctive treatments for patients with advanced PD with levodopa-related refractory motor complications and some specific nonmotor symptoms (NMS) as growing evidence shows apomorphine also improves aspects of NMS of PD. Common side effects of subcutaneous apomorphine are skin nodules, nausea, and somnolence with incidence being higher with infusion than that with injection. Impulse control disorders and neuropsychiatric complications common to most dopamine agonists can also occur. As per National Institute for Health and Care Excellence (NICE), United Kingdom, apomorphine, as intermittent injection or continuous subcutaneous infusion, is one of the best medical therapies and may be considered before using deep brain stimulation (DBS) or levodopa/carbidopa intestinal gel (LCIG). Head-to-head open-label comparative multicenter data suggest that apomorphine is at least as effective as DBS or LCIG in relation to nonmotor and motor benefit. More studies are needed to reduce the paucity of apomorphine data in Indian population. We also discuss criteria to select one device therapy over another and newer apomorphine delivery strategies in the pipeline.
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Nonmotor symptoms and sleep disturbances in patients with blepharospasm p. 156
Somdattaa Ray, Pramod K Pal, Ravi Yadav
Blepharospasm is a disabling focal dystonia, which is characterized by involuntary bilateral synchronous, excessive blinking and closure of the eyes. This leads to functional impairment in vision and consequences such as inability to carry out daily activities such as driving a vehicle and walking. In addition, these patients can develop many nonmotor symptoms. There is a significant prevalence of anxiety, depression, and obsessive compulsion disorders in patients with blepharospasm. Sleep problems such as poor quality of sleep, delayed sleep latency, and reduced sleep duration are also seen in blepharospasm. This review aims to summarize the literature on the impact of nonmotor symptoms in patients with blepharospasm.
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Unawareness of hyposmia in patients with idiopathic Parkinson’s disease p. 163
Utkarsh Patel, Charulata Savant Sankhla
BACKGROUND: Loss of olfaction is a hallmark of neurodegenerative disorders such as idiopathic Parkinson’s disease (PD) and it may precede the clinical syndrome of PD by many years. Olfactory bulb is probably the earliest site of pathology in PD. This study examines the frequency of unawareness of hyposmia in study cohorts with and without PD and its correlation with cognitive impairment. The objective of this study was to assess olfactory function using the Indian Smell Identification Test (INSIT), which evaluates the unawareness of hyposmia in patients with idiopathic PD. MATERIALS AND METHODS: Olfaction was assessed in 30 PD patients and 30 healthy controls by using INSIT. During this test, the subjects were asked to identify smell from a set of choices and were scored out of 10. The cutoff used for hyposmia was an INSIT score of ≤4. Unawareness was defined as reporting a normal sense of smell in the setting of a low INSIT. Cognitive status was assessed by using Mini Mental State Examination (MMSE) to study the effect of cognitive status on unawareness of hyposmia in PD cases. RESULTS: The mean age for the PD and control groups was 64.3± 10.6 years and 63.9 ± 10.2 years, respectively. Most of the participants were males: 53% and 56% in the PD and control groups, respectively. Out of the 30 patients in each group, 18 patients were unaware of hyposmia in the PD group as compared with 4 patients in the control group. The mean smell identification score using INSIT was 4.13 ± 2.42 in the PD group and 6.86 ± 2.3 in the control group, which was found to be significant with a p value of <0.0001. In the PD group, the mean MMSE score in subjects who were unaware of hyposmia was 27.89, whereas the mean MMSE score in subjects who were aware of smell was 29.25. This was found to be not statistically significant with a p value of 0.1087. CONCLUSION: Unawareness of hyposmia in patients with PD is high as compared with that in the elderly without PD. Under-reporting of hyposmia is seen in patients with PD. There is no increase in the unawareness of hyposmia in patients with PD who exhibit dementia. All these lead to a premise whereby population screening using INSIT could be used for the early detection of PD in those who already harbor the earliest pathology of neurodegeneration.
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Does quality of sleep differ in familial and sporadic Parkinson’s disease? p. 167
Rohan R Mahale, Ravi Yadav, Pramod K Pal
Background: Sleep disturbances are commonly seen in Parkinson’s disease and are often under-recognized and under treated in clinical practice. Objectives: To determine the quality of sleep in patients with familial Parkinson’s disease (PD) and to note any difference from those with sporadic Parkinson’s disease. Methods: One hundred and fifty six patients with Parkinson’s disease (familial PD- 20, sporadic PD-136) were clinically examined and quality of sleep was determined using Pittsburgh sleep quality index (PSQI), Parkinson’s disease Sleep Scale (PDSS) and Epworth Sleep Scale (ESS). Other scales included Unified Parkinson’s Disease Rating Scale -part III (UPDRS-III), Hoehn & Yahr Stage, Mini Mental Status Examination, Hamilton anxiety rating scale and Hamilton depression rating scale. Results: Global PSQI score, percentage of patients with global PSQI score >5, total PDSS score, and percentages of patients with total PDSS scores < 82 was similar in both groups. The total ESS score was higher in sporadic PD as compared to familial PD (p=0.04) and the frequency of patients with total ESS score >10 was greater in sporadic PD (p=0.02). The frequency of insomnia, occurrence of nightmares, involuntary limb movements during sleep and sleep behaviour disrupting sleep continuity was similar in both groups. Conclusions: There was no difference in the sleep quality between familial PD and sporadic PD patients.
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Syringomyelia with Chiari 1 malformation presenting as focal hand dystonia p. 173
Dinesh Khandelwal, Vaibhav Mathur, Arvind Vyas, Mridula Singh
Syringomyelia is described as a fluid-filled cavity within the spinal cord devoid of an ependymal lining. It is best visualized on magnetic resonance imaging (MRI), with the cavity being low intensity on T1-weighted and high intensity on T2-weighted images. The association of syringomyelia with dystonia has been infrequently reported in the medical literature. We herein describe a case of syringomyelia with Chiari 1 malformation and hydrocephalus having writer’s cramp and pseudoathetosis, which as per our review, is a yet undescribed manifestation in previously published literature. Also, we emphasize on the usefulness of spinal MRI in a case of focal dystonia/writer’s cramp if cause is not apparent after initial evaluation and more so if associated with proprioceptive sensory impairment and pseudoathetosis.
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Can yoga lead to maladaptive plasticity resulting in disorder of motor control? p. 178
Nitish Kamble, Ketan Jhunjhunwala, Ravi Yadav, Amitabh Bhattacharya, Pramod Kumar Pal
The practice of yoga is based on the traditional Indian philosophy. Children during their development show adaptive neuroplasticity which is due to long-term potentiation that causes changes in the synaptic transmission. Excessive plasticity in the developing brain can lead to maladaptive neuronal circuits which can cause hyperkinetic movement disorders. It is not clear at what age yoga should be started and whether certain yogas or yogas done improperly can lead to maladaptive plasticity. We report here an unusual case of tic disorder which was precipitated by yoga.
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Hyperparathyroidism: A rare cause of worsening of parkinsonism p. 181
Bhushan R Mishal, Kartik J Kachhadiya, Shaival H Chandalia, Mehul S Bhansali, Pettarusp M Wadia
Parkinsonism secondary to hypoparathyroidism is well described. The association of hyperparathyroidism with parkinsonism is rare. We describe here a case of progressive supranuclear palsy (PSP) associated with hyperparathyroidism secondary to a parathyroid adenoma who improved significantly with surgical excision of the parathyroid adenoma.
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Dystonia and dysphagia in spinocerebellar ataxia 1 portends a severe phenotype p. 185
Sahil Mehta, Sucharita Ray, Sarath Aleti, Vivek Lal
A variety of manifestations are known to occur in spinocerebellar ataxias (SCAs) other than ataxia. Dystonia may be the presenting or dominant manifestation of certain types of SCAs, commonly in SCA 3, SCA 17, and SCA 2. In this paper, we present a case of genetically proven SCA 1 with dystonia as a dominant manifestation. She additionally manifested dysphagia early in the course of her illness which led to her death due to choking within 3 years of onset. Through this case we attempt to highlight the occurrence of dystonia as well as dysphagia in SCA 1 which usually lends a more aggressive course and poorer prognosis to the condition compared to other SCAs.
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Immune-mediated movement disorders, neuronal antibodies, and paraneoplastic syndromes p. 188
Jamir Pitton Rissardo, Ana Letícia Fornari Caprara
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Book review on “Parkinson’s disease in India: from clinic to the bench” p. 191
Roopa Rajan
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