Neurology

Cross-cultural differences in dementia: Indian etiquette of honorifics Apoorva Pauranik Annals of Indian Academy of Neurology 2019 22(2):129-130

Can we treat secondary progressive multiple sclerosis now? Rohit Bhatia, Nishita Singh Annals of Indian Academy of Neurology 2019 22(2):131-136 Secondary progressive multiple sclerosis (SPMS) is characterized by progressive accumulation of disability without intermittent recovery. Treatment of these patients is challenging due to limited understanding of pathogenesis and fewer therapeutic options. This article summarizes difficulties in defining and conducting trials in SPMS, review major clinical trials on therapies approved and unapproved in SPMS and lastly, therapies in pipeline for use in SPMS.

Expert group meeting on aphasia: A report Apoorva Pauranik, Annamma George, Aparna Sahu, Ashima Nehra, Avanthi Paplikar, Chitralekha Bhat, Gopee Krishnan, Harsimar Kaur, Jitendra Saini, PA Suresh, Pawan Ojha, Pinky Singh, Pratap Sancheti, Prathibha Karanth, PS Mathuranath, Satyapal Goswami, Sonal Chitnis, N Sundar, Suvarna Alladi, Yasmeen Faroqi-Shah Annals of Indian Academy of Neurology 2019 22(2):137-146 A multidisciplinary team of experts took stock of the current state of affairs about many aspects of aphasia in India, including community burden, diagnostic assessment, therapy, rehabilitation, research, education, and advocacy. The broad spectrum of aphasiology was matched by the types of participants ranging from neurologists, speech-language pathologists, clinical psychologists, linguists, to experts in neuroimaging and computer sciences. Threadbare discussion in 16 sessions over 3 days leads to the identification of pressing problems and possible solutions. Many action plans have been envisaged and recommendations made. A few examples with high priority are community-based and hospital-based study incidence and prevalence of aphasia, development of test batteries for the assessment of many components of speech and communication in Indian languages which are validated on rigorous psychometric, and linguistic criteria, national registry for aphasia, educational modules about aphasia for different target groups, resources for advocacy and its training, a bank of research questions and outlines of research protocols for young professionals to pursue. The expert group will continue to oversee execution of some of the actionable plans in short and long term.

Aspirin and clopidogrel resistance in Indian patients with ischemic stroke and its associations with gene polymorphisms: A pilot study Samir Patel, Vandana Arya, Amrita Saraf, Manorama Bhargava, CS Agrawal Annals of Indian Academy of Neurology 2019 22(2):147-152 Introduction: Antiplatelet resistance is one of the urgent issues in current stroke care. One-third to one-half of the patients who experience a recurrent stroke is already on antiplatelet medications. We studied resistance to aspirin and clopidogrel in Indian stroke patients and its association with gene polymorphisms. Methods: Platelet function testing by light transmission aggregometry was performed on 65 patients with ischemic stroke who were stable on dual antiplatelet therapy (clopidogrel 75 mg OD and aspirin 75 mg OD) along with 65 age-matched controls. Aspirin resistance was considered as mean platelet aggregation ≥70% with 10 μM adenosine diphosphate (ADP) and ≥20% with 0.75 mM arachidonic acid. Clopidogrel resistance was defined as <10% decrease from the baseline in platelet aggregation in response to ADP 10 μM and semi-response as <30% decrease from the baseline. Polymorphisms CYP2C19 * 2 and GPIIb/IIIa (PLA1/A2) were genotyped by polymerase chain reaction-restriction fragment length polymorphism. Results: We found 64.6% (42/65) patients with inadequate response to clopidogrel (15.4% [10/65] resistant and 49.2% [32/65] semi-responders) and 4.6% (3/65) patients with inadequate response to aspirin (3.1% [2/65] resistant and 1.5% [1/65] semi-responder). The frequency of CYP2C19*2 mutant genotype was significantly higher in clopidogrel nonresponders compared to responders (P = 0.014). Clopidogrel nonresponsiveness was much higher in small vessel stroke. Conclusion: Unlike aspirin, a high proportion of nonresponders to clopidogrel was identified. In an interim analysis on 65 Indian patients, a significant association was found between CYP2C19*2 and clopidogrel nonresponsiveness.

Scrub typhus-associated opsoclonus: Clinical course and longitudinal outcomes in an Indian cohort Ravikar Ralph, AT Prabhakar, Sowmya Sathyendra, Ronald Carey, John Jude, George M Varghese Annals of Indian Academy of Neurology 2019 22(2):153-158 Context: Opsoclonus, a rare neurological manifestation in scrub typhus, causes significant distress and disability. There is a paucity of clinical data and outcomes in these patients. Aim: This study aims to describe the clinical and laboratory profile and longitudinal outcomes in a scrub typhus patient cohort with opsoclonus. Settings and Design: This retrospective study was conducted in a 2700-bed teaching hospital in South India, in scrub typhus patients with opsoclonus over a 5-year period. Patients and Methods: Clinical, laboratory, and radiological data and outcomes at discharge and 6- and 12-weeks postdischarge were documented. Results: Of 1650 scrub typhus patients, 18 had opsoclonus. 17 had opsoclonus at presentation, while one patient developed opsoclonus on the 5th admission day, 1-day postdefervescence. Opsoclonus was first noted after a median interval of 11 (7–18) days from fever onset. It was associated with myoclonus in 94% (17/18), cerebellar dysfunction in 67% (12/18), extrapyramidal syndrome (EPS) in 33% (6/18), and aseptic meningitis in 17% (3/18) patients. Mean cerebrospinal fluid (CSF) white blood cell (WBC) count was 9 ± 2.7 cells/cumm, with mean CSF protein 118.5 ± 53.9 mg% and mean CSF glucose 97 ± 13 mg% in 1l/15 patients. Brain magnetic resonance imaging was unremarkable in 75% (9/12). Case-fatality rate was 5.5% (1/18). Complete resolution of the index neurological syndrome occurred at 12-week postdischarge. Conclusions: Opsoclonus is a rare neurological manifestation in scrub typhus, usually occurring in association with myoclonus, cerebellar dysfunction, or EPS. It appears to occur during the resolving febrile phase, with neurological deficits completely resolving at 12 weeks.

Study of wrist ratio and wrist-to-palm index radio in individuals suffering from carpal tunnel syndrome Suchitra Sachin Palve, Sachin Bhaskar Palve Annals of Indian Academy of Neurology 2019 22(2):159-163 Background: Carpal tunnel syndrome (CTS) is the commonest median nerve entrapment neuropathy, with preponderance in females. Aims and Objective: The aim of the present study was to find out wrist ratio (WR) and wrist/palm ratio (WPR) in clinically diagnosed patients of CTS. Methodology: One hundred individuals (fifty patients of CTS and fifty as control group) aged between 30 and 50 years were recruited for the study. Early confirmation of clinically suspected patients of CTS was done by performing electrodiagnostic tests of median and ulnar nerves. Motor and sensory conduction velocities, distal motor and sensory latencies, and F-wave latencies were performed in the recruited volunteers. Results: The values for mean wrist ratio in control groups were 0.694, 0.703 respectively, and in patients with carpal tunnel syndrome, it was 0.704 and 0.719 respectively in moderate and severe type. The mean wrist to palm ratio (WPR) in control group was 0.371, while in patients with CTS, it was 0.374, 0.382, 0.387, and 0.401 based on progression of severity. Both were statistically significant for the last two groups (wrist to index finger [WIF] >4.4 m/s, moderate, and WIF nonrecordable, severe). Statistically significant (P < 0.001) decrease of motor conduction velocities for median nerve was seen in the CTS group as compared to control group. Statistically significant (P < 0.001) increase in distal motor and sensory latencies was observed for both median and ulnar nerves in CTS group with more increase in distal motor latency than sensory latency. Increase in F-wave latencies of both nerves was seen in CTS group. Conclusion: The study results confirm selective slowing of sensory and motor conduction within wrist-to-palm segment in patients of CTS. Both WR and WPR have a progressive correlation with the severity of CTS, but statistically significant changes were seen in the groups with moderate and severe CTS. Thus, WR/WPR can act as one of the essential parameters in the diagnosis of CTS with moderate-to-severe CTS.

A study of assessment of knowledge of childhood autism among medical students in Mumbai Samia D Ellias, Henal R Shah Annals of Indian Academy of Neurology 2019 22(2):164-169 Aims: This study is to study the knowledge and awareness on autism among medical students and the factors which impact their knowledge on autism. Settings and Design: For the study, a multicenter cross-sectional research has been carried out among medical students in seven medical colleges in Mumbai. Subjects and Methods: A total of 201 final-year medical students from seven medical colleges in Mumbai completed the knowledge on childhood autism among healthcare workers (KCAHW) survey and a semi-structured pro forma. Of the 201 students, 152 are from the public medical colleges and 37 from the private medical colleges. Statistical Analysis Used: Data collected have been represented in the form of frequency and percentage. SPSS 20.0 is used to analyze the mean distribution of KCAHW scores. A value of P < 0.05 is regarded as statistically significant. Results: The total mean score in the KCAHW questionnaire is 11.85 ± 3.15 out of 19. This is considered as poor knowledge. Our study reveals that students' knowledge on autism is limited. Knowledge gaps identified in certain autism spectrum disorder (ASD) symptoms are associated with the obsessive and compulsive repetitive pattern of behavior and that autism is associated with epilepsies. Conclusions: It is thus important that these children displaying ASD symptoms are diagnosed correctly and at the earliest. To enable this, doctors in all fields must be able to accurately identify the diagnostic features of autism both within and outside the four walls of the hospital.

Storage, degradation, and new connectivity of face-related semantic memory in Alzheimer's disease Sandip Pal, Prasenjit Sengupta, Malay Ghosal, Asutosh Pal, Rudraprasad Acharya, Debsadhan Biswas Annals of Indian Academy of Neurology 2019 22(2):170-174 Background: Excepting amnesia, impairment of other domains also hampers the activity of daily living in Alzheimer's disease (AD). Although prosopagnosia poses problem in interacting with other persons, it rarely causes problem during interaction with close relatives as known voice acts as cue for recognition. Objective: In a cohort of AD, we planned to study errors in recognition, naming, and assigning relationship of close relatives, to assess the type and frequency of errors and to explain with current knowledge and hypothesis. Materials and Methods: This cross-sectional study was conducted in Memory Clinic of Medical College Hospital, Kolkata, India, between July 2013 and June 2015. Patients were evaluated by history, general neurological examination, and neuropsychological tests. A structured questionnaire was used to assess recognition (use of honorifics) and naming defect of close relatives. Results: AD was diagnosed in 42 patients. Prosopagnosia was found in 14 and anomia in 6 patients. Four patients exhibited problem during conversation with close relatives. They assigned name and relation of one generation earlier to close relatives with proper recognitions. Discussion: We got predictive error of name and relation assignment of close relatives by one generation back with normal recognition. It can be explained by two memory traces in connection of face-visual and name (with/without relation) representation, earlier being hierarchically older and more resistant to wearing. Conclusions: We hypothesize that the name/relation store is orderly conserved. In AD, after degradation of part of name/relation store, a new wiring might be built up between these two traces.

Terminal latency index, residual latency, and median-ulnar F-wave latency difference in carpal tunnel syndrome Aslihan Uzunkulaoglu, Sevgi Ikbali Afsar, Betul Tepeli Annals of Indian Academy of Neurology 2019 22(2):175-179 Introduction: Carpal tunnel syndrome (CTS) is the most common entrapment neuropathy, but no electrodiagnostic test alone shows sufficient sensitivity for CTS. We aimed to investigate the value of median motor terminal latency index (mTLI), median motor residual latency (mRL), and median-ulnar F-wave latency difference (FdifMU) as additional tests to nerve conduction studies which are performed traditionally in electromyography laboratories. Methods: This is a retrospective study. The results of electrodiagnostic studies performed on patients with CTS were examined. We divided the enrolled hands of the patients diagnosed with CTS into two groups: affected hands with abnormal electroneuromyographic parameters indicating CTS diagnosis (CTS group) and hands with normal electroneuromyographic parameters (control group). Then, we analyzed the results of these completed electrodiagnostic studies. Results: A total of 320 hands of 160 patients were studied. FdifMU and mRL were found to be significantly higher in CTS group compared with the control group (P < 0.001). mTLIs were found to be significantly higher in control group compared with the CTS group (P < 0.001). Given that, the area under the curve is more than 70% for mTLI and mRL, but not for FdifMU. Conclusion: When combined with mMDL, both mTLI and mRL have excellent specificity for the diagnosis of mild and moderate CTS. However, the sensitivities for both parameters were lower. In suspected patients, FdifMU can be an additional tool for the diagnosis of CTS also, but alone it is not valuable.

Vasculitic neuropathy: A retrospective analysis of nerve biopsies and clinical features from a single tertiary care center Monalisa Hui, AK Meena, Liza Rajasekhar, Yareeda Sireesha, Jabeen Afshan, Rukmini Mridula, Rupam Borgohain, Megha S Uppin Annals of Indian Academy of Neurology 2019 22(2):180-186 Objective: Vasculitic neuropathy can be either restricted to the peripheral nerves or associated with systemic involvement of other organs. The objective of this study was to analyze the nerve biopsies reported as “vasculitic neuropathy” with clinical features. Materials and Methods: All cases diagnosed with vasculitic neuropathy were retrospectively analyzed and categorized as systemic vasculitis and nonsystemic vasculitic neuropathy based on the clinical features. The histological features were further evaluated and classified according to the Peripheral Nerve Society Guidelines. Results: Of the 126 cases, there were 65 nonsystemic vasculitis, 45 secondary systemic vasculitis, and 16 primary systemic vasculitis. Definite vasculitis was more common in the systemic vasculitis group. The epineurial vessels were predominantly involved with chronic axonal changes. Conclusion: The sensitivity of definite vasculitis on nerve biopsy was 54.76%. The sensitivity increases when the diagnostic criteria of definite and probable vasculitis were applied taking into account perivascular inflammation accompanied by vascular changes and axonopathy.