Phenomenological patterns and aetiological spectrum in patients visiting a tertiary care Movement disorders service in India: An observational study.

OBJECTIVE: Although previous studies have described phenomenological diagnoses, they lacked description of aetiological spectrum in patients visiting movement disorders (MD) service. Herein, we classify the MD phenomenology and describe aetiology wise distribution of each phenomenology in patients visiting a tertiary care movement disorders service. METHODS: Collected information included demographic profile (age of onset, age at presentation, gender, duration of illness before presentation), predominant MD phenomenology [such as parkinsonism, dystonia, ataxia, tremor, chorea, ballism, myoclonus, tics, stereotypy, restless legs syndrome (RLS) and others], diagnostic evaluations and detected aetiology. RESULTS: This observational study included 1140 MD patients over a span of 5 years. The median (IQR) age of onset was 49 (35-60) years and age at presentation was 54 (40-65) years, with median duration of illness being 36 (18-72) months. Nearly two-third of patients were males (M:F=731:409). Parkinsonism (n=494, 43.3 %) was the most common MD phenomenology observed, followed by dystonia (n=219, 19.2 %), ataxia (n=125, 11 %), tremor (n=118, 10.4 %), myoclonus (n=73, 6.4 %), chorea (n=40, 3.5 %), spasticity (n=22, 1.9 %), tics (n=8, 0.7 %), and RLS (n=8, 0.7 %). Thirty-three (2.9 %) patients were grouped under miscellaneous MDs. Overall, neurodegenerative disorders (57.4 %) were the most common cause of MDs. Parkinson's disease, genetic dystonia, essential tremor, genetic ataxias, hemifacial spasm, and Huntington's disease were the most common aetiologies for parkinsonism, dystonia, tremor, ataxia, myoclonus, and chorea, respectively. CONCLUSION: Parkinsonism was the most common phenomenology observed in MD patients, and was followed by dystonia, ataxia and tremor. Neurodegenerative disorders were the most common aetiology detected.

Clinical and Electrophysiological Characteristics of Very Early Guillain-Barré Syndrome.

PURPOSE: This study compared the clinical and electrodiagnostic (EDX) features and long-term outcomes of patients with very early Guillain-Barré syndrome (VEGBS, duration of illness ≤4 days) and those with early/late (>4 days)-presenting GBS. METHODS: One hundred patients with GBS were clinically evaluated and categorized into VEGBS and early/late GBS groups. Electrodiagnostic studies were performed on the bilateral median, ulnar, and fibular motor nerves and the bilateral median, ulnar, and sural sensory nerves. Admission and peak disability were assessed using the 0 to 6 Guillain-Barré Syndrome Disability Scale (GBSDS). The primary outcome was disability at 6 months, which was categorized as complete (GBSDS ≤1) or poor (GBSDS ≥2). The secondary outcomes were frequencies of abnormal electrodiagnostic findings, in-hospital progression, and mechanical ventilation (MV). RESULTS: Patients with VEGBS had higher peak disability (median 5 vs. 4; P = 0.02), frequent in-hospital disease progression (42.9% vs. 19.0%, P < 0.01), needed MV (50% vs. 22.4%; P < 0.01), and less frequent albuminocytologic dissociation (52.4% vs. 74.1%; P = 0.02) than those with early/late GBS. Thirteen patients were lost to follow-up at 6 months (nine patients with VEGBS and four patients with early/late GBS). The proportion of patients with complete recovery at 6 months was comparable (60.6% vs. 77.8%; P = ns). Reduced d-CMAP was the most common abnormality, noted in 64.7% and 71.6% of patients with VEGBS and early/late GBS, respectively (P = ns). Prolonged distal motor latency (≥130%) was more common in early/late GBS than in VEGBS (36.2% vs. 25.4%; P = 0.02), whereas absent F-waves were more frequent in VEGBS (37.7% vs. 28.7%; P = 0.03). CONCLUSIONS: Patients with VEGBS were more disabled at admission than those with early/late GBS. However, 6 month's outcomes were similar between the groups. F-wave abnormalities were frequent in VEGBS, and distal motor latency prolongation was common in early/late GBS.

Comparison of clinico-radiological profile, optical coherence tomography parameters, and outcome in MOGAD and Neuromyelitis optica spectrum disorder subtypes: A prospective observational study.

Objectives: The objectives of the study were to compare the clinico-radiological profile, optical coherence tomography (OCT) parameters and outcome in Myelin Oligodendrocyte Glycoprotein-IgG-associated disorders (MOGAD) and Neuromyelitis Optica Spectrum disorder subtypes. Materials and Methods: This prospective study involved collection of data regarding neurological assessment, neuroimaging, cerebrospinal fluid analysis, OCT parameters, treatment and outcome. Disease severity and disability were assessed using Expanded Disability Status Scale and modified Rankin scale. Patients were categorized into aquaporin-4 (AQP4+), MOGAD, and double negative (DN; both AQP4 and MOG negative). Results: Among 31 patients included, 42% were AQP4+, 32.2% were MOGAD, and 25.7% were DN. The median age at onset was comparable (AQP4+ vs. MOGAD vs. DN = 28 years vs. 24.4 years vs. 31.5years; P = 0.31). Females predominated in AQP4+ compared to MOGAD group (76.9% vs. 30%; P = 0.02). Majority of patients (73.5%) had a relapsing course with a median of two (range = 1-9) relapses. Ninety-nine demyelinating events occurred: Transverse myelitis (TM) in 60/99 (60.6%), optic neuritis (ON) in 43/99 (43.4%), area postrema (AP) syndrome in 20/99 (20.1%), and optico-spinal syndrome in 10/99 (10.1%). ON was common in MOGAD than AQP4+ patients (58.6% vs. 32.1%; P = 0.03). Spinal cord and brain lesions on magnetic resonance imaging (MRI) were seen in 90.3% and 54.8% patients, respectively. A significantly higher proportion of AQP4+ patients showed longitudinally extensive transverse myelitis as compared to MOGAD group (69.2 % vs. 20 %; P = 0.04), specifically involving dorsal cord (92.3% vs. 50%; P = 0.02). MRI brain lesions, especially involving AP, was frequent in DN than MOGAD (47.1% vs. 6.9%; P = 0.003) and AQP4+ (47.1% vs. 18.9%; P = 0.03) patients. AQP4+ group showed significant nasal RNFL thinning on OCT (P = 0.04). Although 6-month good functional outcome was better in MOGAD than DN and AQP4+ (80% vs. 71.4% vs. 41.7%) groups, they were comparable (P = 0.13). Conclusion: Nearly three-fourth of our patients showed a relapsing course, with TM being the most common clinical presentation. AQP4+ group showed female preponderance, frequent dorsal cord longitudinally extensive transverse myelitis, less frequent ON, and greater nasal RNFL thinning compared to MOGAD group. MRI brain lesions were more common in DN patients. All three groups exhibited good response to pulse corticosteroids and showed a comparable functional outcome at 6-month follow-up.

Tenecteplase and Alteplase for Thrombolysis of Acute Ischemic Stroke within 4.5 Hours: An Efficacy and Safety Study.

Objective: To compare the efficacy and safety of thrombolysis using Tenecteplase (TNK) versus alteplase in acute ischaemic stroke (AIS) patients within 4.5-hour window period. Methods: This retrospective study involved the collection of data from consecutive AIS patients who underwent thrombolysis in the Department of Neurology at a tertiary care university hospital, between May 2018 to January 2021. Data including clinical history, neurological assessment using modified Rankin score (mRS), National Institutes of Health Stroke Scale (NIHSS), brain neuroimaging, treatment, and outcome details were collected. The primary efficacy outcome was the proportion of patients with good functional recovery (mRS of 0-2) at 90 days of follow-up. Results: Total of 42 patients with AIS underwent thrombolysis, of which 19 received alteplase and 23 got TNK. The median (range) onset to door time [120 (20-210) versus 120 (30-210) minutes; P = 0.823] and median (range) onset to needle time [150 (60-255) versus 160 (50-240) minutes; P = 0.779] were comparable in both alteplase and TNK groups, respectively. The primary outcome of good functional recovery (mRS ≤2) at 3 months was observed in more than half the patients in each group and was comparable (P = 0.701). Post-thrombolysis complications including cerebral haemorrhage (symptomatic or asymptomatic) were comparable between the two groups (31.6% vs 30.4%; P = 0.936), except a significantly higher proportion of patients on TNK required mechanical ventilation (10.5% v/s 43.5%; P = 0.019). Conclusions: This study showed a comparable efficacy and safety profile of alteplase and TNK in thrombolysis of AIS throughout the 4.5 hours window period. Moreover, the ease of administration and better pharmacodynamic properties favors tenecteplase.

Comparison of patterns of infarction in TB and cryptococcal meningitis.

BACKGROUND: To compare the frequency and patterns of stroke, the specificity of tubercular zone (TBZ) infarction and its effect on outcomes in TB (TBM) and cryptococcal meningitis (CM). METHODS: This retrospective study was conducted at two tertiary centres in India from May 2018 to July 2020. Sixty-one patients with TBM and 22 with CM were included. The primary outcome was the proportions of TBM and CM patients with infarction. Secondary outcomes included the anatomical locations of infarction and in-hospital mortality. RESULTS: Infarction was noted in 52.5% of patients with TBM and in 54.5% of those with CM (p=0.87), with caudate head infarcts in 9.4% vs 41.7% (p=0.01), cerebellar in 9.4% vs 33.3% (p=0.05), thalamic in 25% vs 0% and lobar in 28.1% vs 0%, respectively. In TBM, the infarcts were located in the TBZ in 3 (9.4%), in the ischaemic zone in 23 (71.9%), while 6 (18.8%) patients showed infarcts in both, while in CM, the infarcts were in 0 (0%), 6 (50%) and 6 (50%) patients, respectively. Infarcts were not associated with in-hospital mortality, either in TBM or CM. CONCLUSION: Caudate head and cerebellar infarction was more common in CM, while thalamic and lobar infarcts were more frequent in TBM. TBZ infarcts were not specific to TBM.

Prediction models for mechanical ventilation and outcome in Guillain-Barré syndrome.

Various prediction models have been formulated to predict the need for mechanical ventilation (MV). In this study, we compare the sensitivity and specificity of Erasmus GBS Respiratory Insufficiency Score (EGRIS) and Sharshar score with single breath count (SBC) at 2 Hz to assess their usefulness in predicting MV and functional outcome. The primary outcome was prediction of MV and relative usefulness of all three models based on sensitivity and specificity. The secondary outcome was functional recovery at 6 months using the cut off points for MV and functional outcome at 6months was assessed using Hughes scale and categorized as good (<2) and poor (≥2). The median age was 30 years, and 64 (69.6%) were males. The sensitivity and specificity of EGRIS score was 78.1% and 67.2%, Sharshar score was 75.0% and 64.1%, and SBC at 2 Hz was 78.1% and 75.0%. The area under the receiver operating curves was maximum for SBC at 2 Hz (0.86) compared to other models, although insignificant. EGRIS score < 5 had better functional recovery compared to those with score ≥ 5 (66% vs 34%, p = 0.001). Similarly, in Sharshar score, 23/59 (39%) with score ≥ 4 had good recovery compared to 36 (61%) with score < 4 (p = 0.01). All the three models have comparable predictive values for MV. Outcome prediction is the best with EGRIS.

Non-COVID Neurological Emergencies: A Silent Killer Going Unnoticed during COVID-19 Pandemic.

Objective This study assesses the impact of coronavirus disease 2019 (COVID-19) on the pattern of neurological emergencies reaching a tertiary care center. Materials and Methods This is a retrospective and single center study involving 295 patients with neurological emergencies mainly including acute stroke, status epilepticus (SE), and tubercular meningitis visiting emergency department (ED) from January 1 to April 30, 2020 and divided into pre- and during lockdown, the latter starting from March 25 onward. The primary outcome was number of neurological emergencies visiting ED per week in both periods. Secondary outcomes included disease severity at admission, need for mechanical ventilation (MV), delay in hospitalization, in-hospital mortality, and reasons for poor compliance to ongoing treatment multivariate binary logistic regression was used to find independent predictors of in-hospital mortality which included variables with p <0.1 on univariate analysis. Structural break in the time series analysis was done by using Chow test. Results There was 53.8% reduction in number of neurological emergencies visiting ED during lockdown (22.1 visits vs. 10.2 visits per week, p = 0.001), significantly affecting rural population ( p = 0.004). Presenting patients had comparatively severe illness with increased requirement of MV ( p < 0.001) and significant delay in hospitalization during lockdown ( p < 0.001). Poor compliance to ongoing therapy increased from 34.4% in pre-lockdown to 64.7% patients during lockdown ( p < 0.001), mostly due to nonavailability of drugs ( p < 0.001). Overall, 35 deaths were recorded, with 20 (8.2%) in pre-lockdown and 15 (29.4%) during lockdown ( p = 0.001). Lockdown, nonavailability of local health care, delay in hospitalization, severity at admission, and need for MV emerged as independent predictors of poor outcome in stroke and delay in hospitalization in SE. Conclusion COVID-19 pandemic and associated lockdown resulted in marked decline in non-COVID neurological emergencies reporting to ED, with more severe presentations and significant delay from onset of symptoms to hospitalization.