Diffusion tensor imaging of vastus lateralis in patients with inflammatory myopathies.

OBJECTIVE: Inflammation in patients with myositis would increase diffusion of water molecules across sarcolemma that could be detected with the help of diffusion tensor imaging (DTI). We aimed to determine an association between DTI of vastus lateralis (VL) and histopathological findings in cases of myositis and to estimate diagnostic performance of different MRI variables in predicting histopathological outcomes. METHODS: This prospective cross-sectional observational study included 43 patients with myositis. MRI of bilateral thighs with DWI/DTI protocol was performed in all the patients. Thirty-three patients further underwent biopsy of right VL muscle. Imaging analysis included grading of "Muscle oedema" based on signal intensity (SI) and extent and "fatty infiltration" based on extent on conventional sequences and, acquiring DWI and DTI parameters. Gold standard method to determine inflammation in muscles was histopathological examination. Comparison of DTI/DWI variables with clinical and histopathological variables was done. RESULTS: The average DWI apparent diffusion coefficient (ADC) and DTI ADC values in the patients were 1.77 ± 0.35 and 2.06 ± 0.35 respectively. The average functional anisotropy (FA) was 0.39 ± 0.17 and, the 3 eigenvalues in the patients were 2.96 ± 0.63, 2.05 ± 0.32, and 1.20 ± 0.39 respectively. VL oedema SI weighted score was the best parameter for predicting effaced fascicular architecture and marked lymphocytic inflammation in endomysium on histopathology. VL fatty infiltration weighted score was the best parameter in predicting perifascicular atrophy. CONCLUSION: Addition of DWI or DTI didn't add significantly in determining active inflammation in cases of myositis.

Role of prazosin in patients with Guillain-Barré syndrome with sympathetic overactivity: A cohort study.

INTRODUCTION/AIMS: In Guillain-Barré syndrome (GBS), patients with dysautonomia demonstrate sympathetic overactivity (SO). This study assessed the role of prazosin (α1-blocker) in the management of SO. METHODS: This cohort study was conducted from January 2022 to September 2023. Thirty-two GBS patients with SO received prazosin (2.5-10 mg three times a day) (prazosin group). For comparison, we included historical controls that included 33 GBS patients having SO with similar baseline characteristics, including median age and disability, who did not receive prazosin, from a GBS registry of patients admitted during February 2018-December 2021. The primary endpoint was days to resolution of SO. Secondary endpoints were daily fluctuations in the systolic (SBP) and diastolic blood pressure (DBP), duration of hospital stay, in-hospital mortality, and disability at 3 months. RESULTS: The median ages of both the treatment and the control groups were 36 (IQR 25-49) years and 43 (66.2%) were males. The demographic and clinical parameters were comparable. Prazosin resulted in significantly earlier normalization of SO compared to the control group (median 15 vs. 20 days; p = .01). The mean fluctuations in the SBP and DBP at 15 days were significantly lower in the prazosin group. However, the duration of hospital stay and good recovery at 3 months were comparable. Three patients developed hypotension, while two patients died (ventilator-associated pneumonia) in the prazosin group. DISCUSSION: This study provides new evidence supporting the role of prazosin in SO, and needs randomized trials to confirm our findings.

Outcome of Guillain-Barré syndrome following intravenous immunoglobulin compared to natural course.

BACKGROUND AND PURPOSE: Intravenous immunoglobulin (IVIg) is recommended in Guillain-Barré syndrome (GBS), but its efficacy may vary in different subtypes. We report the outcomes of patients with GBS following IVIg treatment compared to the natural course (NC). We also compare the effect of IVIg treatment in different subtypes of GBS. METHODS: From a cohort of 528 GBS subjects, we have extracted 189 patients who received IVIg and compared their outcomes with 199 age- and peak disability-matched patients who did not receive IVIg, plasmapheresis, or corticosteroid. Disability was assessed using the 0-6 Guillain-Barré Syndrome Disability Scale (GBSDS). Clinical and neurophysiological subtypes were recorded. The primary outcome was functional disability at 6 months, which was categorized as complete (GBSDS ≤ 1), partial (GBSDS 2-3), or poor (GBSDS > 3). The secondary outcomes were in-hospital death, duration of hospitalization, and mechanical ventilation. RESULTS: In-hospital death (2.6% vs. 2%, p = 0.74) and 3-month poor recovery (20.7% vs. 18%) were similar in the IVIg and NC groups. At 6 months, however, a lesser proportion of patients in the IVIg group had poor recovery (2.2% vs. 8.3%, p = 0.026). The outcomes of IVIg and NC were compared in 72 acute motor axonal neuropathy (AMAN) and 256 acute inflammatory demyelinating polyradiculoneuropathy (AIDP) patients. IVIg therapy did not alter the outcome in AMAN but resulted in a lesser proportion of poor recovery at 6 months in AIDP (0.8% vs. 6.6%, p = 0.03). CONCLUSIONS: IVIg is beneficial in AIDP variants of GBS but not in the AMAN subtype. A customized treatment may be cost-effective until a randomized controlled trial is conducted in AMAN.

Renin, antidiuretic hormone (ADH), and ADH receptor levels in cerebral salt wasting associated with tuberculous meningitis.

BACKGROUND: To evaluate serum antidiuretic hormone (ADH), its receptors, and renin levels in cerebral salt wasting (CSW) in tuberculous meningitis (TBM). METHODS: Patients diagnosed with definite (n = 30) or probable TBM (n = 47) who developed hyponatremia (CSW, SIADH, or miscellaneous causes) were included. Sequential measurement of serum ADH, ADH-R, and renin activity by enzyme-linked immunosorbent assay was done and correlated with serum sodium level, urinary output, and fluid balance. RESULTS: Out of 79 TBM patients, CSW was observed in 36, SIADH in four, and miscellaneous hyponatremia in eight patients. CSW patients had a longer hospital stay (P < 0.001), lower GCS score (P < 0.007), higher MRC grade (P < 0.007), and a lower serum Na (P < 0.001) compared to non-CSW TBM patients. In severe CSW patients, serum ADH and ADH-R were correlated with hyponatremia and returned to baseline on correction; however, serum renin levels remained elevated. Serum ADH was related to hyponatremia but ADH-R and renin were not. ADH-R and renin levels did not significantly differ in CSW and SIADH. CONCLUSION: CSW is the commonest cause of hyponatremia in TBM and correlates with disease severity. ADH is related to hyponatremia, but ADH receptor and renin are not.

Neurological manifestations of coronavirus disease 2019: exploring past to understand present.

SARS-CoV-2 infection, resulting in Coronavirus disease 2019 (COVID-19), has significantly affected the entire world. It was labelled a pandemic by World Health Organization. Although it commonly produces respiratory symptoms, neurological features have been described. Neurological manifestations may vary from non-specific symptoms such as headache, dizziness, myalgia and/or fatigue, olfactory or taste dysfunction to specific syndromes including meningitis, stroke, acute transverse myelitis and Guillain-Barre syndrome. This review describes potential pathogenetic mechanisms and neurological manifestations of COVID-19 along with its management. Considering structural and pathogenetic similarity of SARS-CoV-2 with SARS-CoV and MERS viruses, we compared their neurological manifestations and mentioned few features expected in COVID-19 in future. Interestingly, many COVID-19 cases may present with pure neurological manifestations at onset with non-neurological features manifesting few days later and we propose the term "Neuro-COVID syndrome" for such cases. Awareness of neurological manifestations may facilitate its management and improve outcome in such patients.

Role of IV Immunoglobulin in Indian Children With Guillain-Barré Syndrome.

OBJECTIVES: To evaluate the outcome of Indian children with Guillain-Barré syndrome who received IV immunoglobulin compared with those who did not receive any specific therapy. DESIGN: Single center, prospective cross-sectional study. SETTING: Tertiary care neurology teaching hospital. PATIENTS: Children (≤ 18 yr old) with Guillain-Barré syndrome were included from a prospectively maintained Guillain-Barré syndrome registry from January 2008 to April 2017. Children were classified into acute inflammatory demyelinating polyradiculoneuropathy, acute motor axonal neuropathy, acute motor-sensory axonal neuropathy, and inexcitable motor nerves based on nerve conduction study. INTERVENTIONS: Out of 138 pediatric Guillain-Barré syndrome, 50 received IV immunoglobulin and another 50 age and peak disability matched controls (who did not receive IV immunoglobulin or plasmapheresis) were selected from the same registry for comparison. MEASUREMENTS AND MAIN RESULTS: Outcome at 3 and 6 months was defined on the basis of a 0-10 Clinical Grading Scale into complete (Clinical Grading Scale < 3), partial (Clinical Grading Scale 3-5), and poor (Clinical Grading Scale > 5) recovery. The primary outcome was proportion of patients with complete recovery at 3 and 6 months in IV immunoglobulin and non-IV immunoglobulin groups. Secondary outcomes included in-hospital deaths, duration of mechanical ventilation, and hospital stay. Subgroup analysis was done in acute motor axonal neuropathy and acute inflammatory demyelinating polyradiculoneuropathy groups. The baseline characteristics were similar except for shorter duration of illness and higher proportion of facial palsy in IV immunoglobulin group. Hospital deaths, duration of mechanical ventilation, hospital stay, and outcome at 3 and 6 months were not different between the two groups. Children with acute motor axonal neuropathy had better recovery at 6 months on IV immunoglobulin (58.3% vs 11.1%; p = 0.03), but not those with acute inflammatory demyelinating polyradiculoneuropathy (58.3% vs 72.2%; p = 0.22). In nonambulatory Guillain-Barré syndrome children, complete recovery at 6 months was similar in IV immunoglobulin and non-IV immunoglobulin group (57.4% vs 57.1%; p = 0.98). CONCLUSIONS: In Indian children with Guillain-Barré syndrome, the outcome at 6 months in IV immunoglobulin treated group was similar to non-IV immunoglobulin group. Children with acute motor axonal neuropathy responded better to IV immunoglobulin.

Prospective comparison of acute motor axonal neuropathy and acute inflammatory demyelinating polyradiculoneuropathy in 140 children with Guillain-Barré syndrome in India.

INTRODUCTION: There have been few reports on subtypes of Guillain-Barré syndrome (GBS) in children. We compared clinical and laboratory findings of acute inflammatory demyelinating polyradiculoneuropathy (AIDP) and acute motor axonal neuropathy (AMAN). METHODS: One hundred forty children with GBS were included. Based on nerve conduction study (NCS) findings, patients were subclassified as AIDP, AMAN, acute motor sensory axonal neuropathy (AMSAN), and equivocal. RESULTS: Clinically, 72.1% of patients had pure motor, 24.3% motor sensory, and 3.4% Miller Fisher syndrome. Based on NCS, 67.8% of patients had AIDP, 23.6% had AMAN, and 4.3% had AMSAN. By 3 months, 2.1% patients had died, 47.1% had complete recovery, and 24.3% had poor recovery (wheelchair-bound). Children with AMAN had more frequent lower limb weakness (P = 0.02) and a lower probability of complete recovery (P = 0.01) at 3 months than children with AIDP (56% vs. 30%). DISCUSSION: AIDP is the most common GBS subtype in children. It is characterized by better recovery at 3 months when compared with AMAN. Muscle Nerve 57: 761-765, 2018.

High dose intravenous immunoglobulin may be complicated by myocardial infarction.

Intravenous immunoglobulin [IVIg] is useful for treating several clinical conditions and is largely considered safe, without major adverse events. Here we report a case of acute ST elevation myocardial infarction associated with high dose IVIg administration in a previously healthy 69-year-old male patient of Guillain Barre syndrome. The case is being reported to emphasize the need for treating physicians to be aware of thrombotic complications associated with IVIg. The thrombotic complications associated with IVIg are reviewed in brief, and the measures to reduce them are discussed.

Effect of intravenous immunoglobulin and plasmapheresis on nerve conduction parameters compared to the natural course of Guillain-Barré syndrome.

BACKGROUND: Intravenous immunoglobulin (IVIg) and plasmapheresis (PLEX) are recommended in moderate to severe Guillain-Barré Syndrome (GBS), but there is paucity of studies evaluating its effect on nerve conduction studies (NCS). We report the effect of IVIg and PLEX on the NCS parameters and clinical outcomes compared to natural course (NC) of GBS patients. METHOD: Moderate to severe GBS patients were included based on clinical, cerebrospinal fluid, and NCS finding. Six motor and sensory nerves were evaluated at admission, one month and 3 months, and NCS subtyping was done. Axonal and demyelination burden in motor nerves and early reversible conduction block (ERCB) were noted. Patients receiving IVIg, PLEX or on NC were noted. Outcome was defined at 3 months into complete, partial and poor using a 0-6 GBS Disability Scale (GBSDS). RESULT: Seventy-two patients were included, whose median age was 36 years and 22(30.6 %) were females. 44 patients received IVIg, 9 PLEX and 19 were in NC, and they had comparable peak disability. AIDP was the dominant subtype at admission (58.3 %), which remained so at 3 months (50 %). The shift of subtypes was the highest from the equivocal group followed by AMAN and the least from AIDP. IVIg and PLEX group had more reduction in axonal burden and had ERCB compared to NC. 33(44 %) patients had complete recovery, and 40(55.5 %) patients had concordance in clinical and neurophysiological outcome. CONCLUSION: Transition of GBS subtype may occur at follow-up from all the subtypes, the highest from the equivocal and the lowest from the AIDP group. IVIg/PLEX treatment may help in reducing conduction block and axonal burden.

Comparison of local versus intramuscular steroid injection in patients with carpal tunnel syndrome: A prospective open label randomized clinical trial.

AIM: This study compared the efficacy and safety of local corticosteroid injection (LCI) vs intramuscular (IM) corticosteroid injection in mild to moderate carpal tunnel syndrome (CTS). METHODS: This is an open labeled, randomized controlled trial conducted during December 2021 to August 2023. Eighty-six patients with CTS were randomized in 1:1 ratio to receive either single 40 mg methylprednisolone (MP) injection at the wrist (LCI arm), or single 40 mg MP intramuscular injection (IM arm) in the deltoid. Primary outcome was absolute Symptom Severity Scale (SSS) at 3 months. Secondary outcomes were SSS score at 1-month, Functional Status Scale (FSS) score at 1 and 3 months, and recurrence at 3 months and injection site pain assessed on visual analog (VAS) scale. RESULTS: Median age was 45 (range 22 - 80) years, and 86 % were females. Baseline characteristics were comparable between groups. Mean SSS score at 3 months was similar in two arms (1.72 ± 0.71 vs 2.0 ± 0.93) with mean difference (MD) -0.03 (-0.31 to 0.25, p = 0.83) after adjusting for baseline SSS scores. LCI, however, resulted in significantly better SSS (1.48 ± 0.51 vs 1.88 ± 0.69, adjusted p = 0.04), and FSS scores (1.57 ± 0.44 vs 1.80 ± 0.66, adjusted p = 0.03) at 1 month compared to IM arm. Response rate (67.4 % vs 55.8 %; p = 0.30) and recurrence rate (17.1 % vs 22.6 %, p = 0.74) at 3 months were similar. Injection site pain was severe in LCI arm[median 5 (range 3 - 8) vs median 3 (range 2 - 6)]. CONCLUSIONS: In patients with mild to moderate CTS, LCI resulted in better improvement in the BCTQ scores at 1 month compared to IM steroid. However, 3-month's outcome was similar. Intramuscular steroid injection was better tolerated.

Clinical Reasoning: A 22-Year-Old Woman With Episodic Weakness and Jaundice.

We report a case of a 22-year-old woman who presented with recurrent episodes of quadriparesis, often accompanied by jaundice. Neurologic examination showed symmetrical proximal predominant quadriparesis with generalized hyporeflexia. The differential diagnoses included were of metabolic, inflammatory, genetic (including channelopathies), and autoimmune causes. Serum creatine phosphokinase levels and electrophysiologic studies helped narrow the differential. The final diagnosis was one that was responsive to vitamin supplementation. This report provides a systematic clinical approach to a case of episodic weakness with jaundice and respiratory failure.

Connecting the Dots: Exploring the Relationship between Optical Coherence Tomography and 99mTc-TRODAT-1 SPECT Parameters in Parkinson's Disease.

Background and Objective: While optical coherence tomography (OCT) is explored as a potential biomarker in Parkinson's disease (PD), technetium-99m-labeled tropane derivative (99mTc-TRODAT-1) single-photon emission computed tomography (SPECT) imaging has a proven role in diagnosing PD. Our objective was to compare the OCT parameters in PD patients and healthy controls (HCs) and correlate them with 99mTc-TRODAT-1 parameters in PD patients. Materials and Methods: This cross-sectional study included 30 PD patients and 30 age- and gender-matched HCs. Demographic data, PD details including Movement Disorders Society Unified Parkinson's Disease Rating Scale-III (MDS-UPDRS-III) and Hoehn-Yahr (HY) staging, and OCT parameters including macular and peripapillary retinal nerve fiber layer (RNFL) thickness in bilateral eyes were recorded. PD patients underwent 99mTc-TRODAT-1 SPECT imaging. The terms "ipsilateral" and "contralateral" were used with reference to more severely affected body side in PD patients and compared with corresponding sides in HCs. Results: PD patients showed significant ipsilateral superior parafoveal quadrant (mean ± standard deviation [SD] = 311.10 ± 15.90 vs. 297.57 ± 26.55, P = 0.02) and contralateral average perifoveal (mean ± SD = 278.75 ± 18.97 vs. 269.08 ± 16.91, P = 0.04) thinning compared to HCs. Peripapillary RNFL parameters were comparable between PD patients and HCs. MDS-UPDRS-III score and HY stage were inversely correlated to both ipsilateral (Spearman rho = -0.52, P = 0.003; Spearman rho = -0.47, P = 0.008) and contralateral (Spearman rho = -0.53, P = 0.002; Spearman rho = -0.58, P < 0.001) macular volumes, respectively. PD duration was inversely correlated with ipsilateral temporal parafoveal thickness (ρ = -0.41, P = 0.02). No correlation was observed between OCT and 99mTc-TRODAT-1 SPECT parameters in PD patients. Conclusion: Compared to HCs, a significant thinning was observed in the ipsilateral superior parafoveal quadrant and the contralateral average perifoveal region in PD patients. Macular volume and ipsilateral temporal parafoveal thickness were inversely correlated with disease severity and duration, respectively. OCT and 99mTc-TRODAT-1 SPECT parameters failed to correlate in PD patients.

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.

Dose-Effect Relationship of Motor Nerve Inexcitability on Outcome in Guillain-Barré Syndrome: A Prospective Cohort Study.

Objective: One or more inexcitable motor (IM) nerves are common during electrodiagnostic (EDx) study in Guillain-Barré syndrome (GBS). This study assessed the dose-effect relationship of IM nerves on outcome in patients with acute inflammatory demyelinating polyneuropathy (AIDP) and acute motor and/or sensory axonal neuropathy (AMAN and AMSAN). Materials and Methods: Eighty-eight GBS patients admitted during May 2018-June 2023 underwent detailed clinical evaluation and EDx study. Admission and follow-up disability were assessed on a 0-10 Clinical Grading Scale (CGS). Outcome was recovery at 6 months, defined as good (CGS <3) and poor (CGS ≥3). Binary multivariate logistic regression with backward elimination was used to calculate independent predictors of outcome. Results: Proportion of patients with complete recovery decreased significantly with increasing numbers of IM nerves (P < 0.01). Seventy-six patients were followed for 6 months. Among patients with IM nerves (n = 28), complete recovery was similar between AIDP and axonal GBS (70% vs. 50%, respectively; P = 0.40). However, in patients with recordable compound muscle action potentials (CMAPs) in all the motor nerves (n = 26), axonal GBS had significantly poor recovery compared to AIDP (75% vs. 9.1%; P = 0.01). Among patients receiving intravenous immunoglobulin (IVIg; n = 42), poor recovery was seen in 53.6% with IM nerves compared to 35.7% without (P = 0.28), while it was 37.5% versus 5.6% (P = 0.04), respectively, in those who did not receive IVIg (n = 34). However, only admission disability (odds ratio [OR] 0.88, 95% confidence interval [CI] 0.81-0.97; P = 0.007) was found to be an independent predictor of outcome. Conclusion: Although increasing numbers of IM nerves were associated with poor outcome on univariate analysis, they did not predict 6 months' outcome independently. Outcome did not differ between axonal GBS and AIDP among those with IM nerves. IVIg improved outcome in patients with IM nerves.

Migraine and Tension-type Headache in Parkinson's Disease and Progressive Supranuclear Palsy/Corticobasal Syndrome.

Objective: To compare the prevalence and characteristics of migraine and tension-type headache (TTH) among patients with Parkinson's disease (PD), progressive supranuclear palsy/corticobasal syndrome (PSP/CBS), and healthy controls (HCs). Methods: This cross-sectional study involved the collection of data from consecutive PD (n = 81) and PSP/CBS (n = 21) patients along with 104 HCs. Migraine and TTH were diagnosed using the International Classification of Headache Disorders 3rd edition criteria. Demographic data, PD or PSP/CBS details, and the presence and characteristics of migraine and TTH were collected. Montreal Cognitive Assessment Scale, Patient Health Questionnaire-9, and Pittsburgh Sleep-Quality Index were used to assess cognition, depression, and sleep quality, respectively. Results: A comparable proportion of PD and PSP/CBS patients reported lifetime headache (46.9% vs 23.8%; P = 0.06). TTH was more common, observed in 84.3%, 100%, and 93.5% of PD, PSP/CBS, and HCs with lifetime headache, respectively. A comparable proportion of participants in all three groups had bilateral (P = 0.10), dull-aching headache (P = 0.09), and occurring <5/month (P > 0.99). The mean severity score of headache among three groups was comparable (P = 0.39). Although the demographic and clinical characteristics of PSP/CBS patients with and without headache were comparable, PD patients with headache had a higher MDS-UPDRS-III score than those without. More than two-third PD and all PSP/CBS patients with lifetime headache reported headache improvement following parkinsonism onset. Conclusion: The prevalence and characteristics of migraine and TTH were comparable in PD, PSP/CBS, and HCs. Headache was associated with greater motor severity in PD. Following parkinsonism onset, headache improved in the majority of PD and PSP/CBS patients with lifetime headache.

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.