Pathophysiology and diagnosis of Guillain-Barré syndrome - challenges and needs.

Guillain-Barré syndrome (GBS) is an autoimmune polyneuropathy which presents with acute onset and rapid progression of flaccid, hyporeflexi quadriparesis. Both sensory and autonomic nerve involvement is seen. GBS has various subtypes that vary in their pathophysiology. The pathogenesis involves an immune response triggered by a preceding event which may be an infection, immunisation or surgical procedure. Clinical diagnosis has been largely the primary diagnosing criterion for GBS along with electrodiagnosis, which has several pitfalls and is supported by ancillary testing of cerebrospinal fluid (CSF) analysis and Nerve Conduction Studies. Measurement of anti-ganglioside antibodies is also an effective tool in its diagnosis. Further understanding of pathophysiology and better diagnostic methods are required for better management of GBS.

Guillain-barré syndrome (GBS) with antecedent chikungunya infection: a case report and literature review.

Guillain-Barré Syndrome (GBS) is an autoimmune neuropathy. Antecedent infections have been seen to be significant triggering factors for developing GBS. Among them, arboviral infections are rapidly gaining importance as significant triggers, especially in the areas where they are endemic. Chikungunya, an arboviral infection that usually causes a self-limiting acute febrile illness can lead to GBS as one its severe complications. Herein, we describe a case of a 21-year-old female who presented with weakness in all four limbs and paresthesia. Nerve conduction study and cerebrospinal fluid (CSF) analysis showed axonal, demyelinating motor and sensory neuropathy with albuminocytological dissociation indicating Acute Motor and Sensory Axonal Neuropathy (AMSAN) variant of GBS. Serum IgM antibodies against ganglioside GM1 were detected. Anti-Chikungunya IgM antibodies were found in both serum and CSF samples. The patient was initiated with Intravenous Immunoglobulin (IVIG) therapy. In view of hypoxia, she was intubated and was on mechanical ventilation. After 2 weeks of being comatose, the patient gradually improved and was discharged with no sequelae.A literature review on antecedent infections in GBS is presented alongside the case report to better understand the association of GBS with antecedent infections, especially the endemic arboviral infections like Chikungunya, Dengue and Zika. This will help in reinforcing the significance of having robust surveillance and public health control measures for infectious diseases.

Effectiveness of an Intensive, Functional, and Gamified Rehabilitation Program on Upper Limb Function in People With Stroke (EnteRtain): A Multicenter Randomized Clinical Trial.

BACKGROUND1: Despite a growing interest in gaming rehabilitation for upper limb (UL) recovery post-stroke, studies investigating the effects of game-based rehabilitation incorporating functional games are lacking. OBJECTIVE: To investigate the efficacy of an intensive, functional, gamified rehabilitation program compared to task-based training on UL motor function in acute/sub-acute stroke survivors. METHODS: This randomized, multicenter, single-blind, clinical trial comprises 120 participants with unilateral stroke who were randomized to receive either gamified training (n = 64) using the ArmAble™ [experimental group (EG)] or task-based training (n = 56) in conjunction with conventional therapy for 2 hours per day, 6 days per week for 2 weeks, followed by UL rehabilitation for another 4 weeks at home. Primary outcomes evaluated by a blinded assessor included the Fugl-Meyer Assessment-Upper Extremity (FM-UE), and Action Research Arm Test (ARAT). Data were analyzed using a linear mixed-effect regression model. RESULTS: The mean (standard deviation) age of the participants was 54.4 ± 11.7 years (78.1% men) in the EG and 57.7 ± 10.9 years (73.2% men) in the comparator group (CG). The median (interquartile range) time since stroke was 30.0 (54.0) days in the EG and 22.5 (45.0) days in the CG. Following the 2-week intervention, a statistically significant improvement was observed in the EG for the FM-UE [between-group mean differences (95% confidence interval): -3.9 (-6.5, -1.3); P = .003]; but not for the ARAT [-2.9 (-5.8, 0.0); P = .051]. Gains at 6 weeks were significantly greater in the EG for both FM-UE [-3.9 (-6.5, -1.3); P = .003]; and ARAT [-3.0 (-5.9, -0.0); P = .046]. CONCLUSION: Gamified rehabilitation using the ArmAble™ device has shown immediate and short-term improvement in UL function after acute/sub-acute stroke. CLINICAL TRIALS REGISTRY NUMBER: CTRI/2020/09/027651.

Serum Inflammatory Markers in Patients with Guillain Barre Syndrome.

Background: Guillain Barre syndrome (GBS) is an immune-mediated peripheral neuropathy characterized by the demyelination and axonal damage of the peripheral neurons. The pathogenesis of GBS involves the breakdown of the blood-brain barrier after which pro inflammatory cytokines attack the neurons in the peripheral nervous system. Aims: This study aims to evaluate five markers, namely matrix metalloproteinase (MMP)-2 and MMP-9, vascular endothelial growth factor (VEGF)-A, basic fibroblast growth factor (bFGF), and SFLT-1, which could have a role in the inflammatory response in patients with GBS and healthy controls. Settings and Design: In this prospective study, patients diagnosed with GBS at the department of neurology, Kasturba Medical College, Manipal, Karnataka were enrolled. Methods and Material: The markers selected for this study were analyzed using the ELISA method and expressed as given in the kit provided by the company. Ethical clearance was obtained from the Institutional Ethical Committee. Statistical Analysis: Results were evaluated using SPSS version 17.0 and expressed as mean ± SD. Error bars for each were drawn. Results: The levels of all five parameters showed a significant increase in patients as compared to controls. Conclusions: Disruption of the basement membrane of endoneurium by MMP-2 and MMP-9, recruitment and migration of macrophages and other cytokines by VEGF-A, bFGF, and soluble fms-like tyrosine kinase-1 (SFLT-1) are plausible, which leads to inflammation process and thus neuronal damage leading to the development of GBS.

Activity monitoring of stroke patients by physiotherapist and caregivers in a hospital setting: A pilot study.

Background: Activity monitoring is a necessary technique to ensure stroke survivors' activity levels in the hospital are within optimal levels as this is important for enhanced motor recovery. However, this could be time-consuming for healthcare professionals like physiotherapists. Activity monitoring by caregivers could be an alternate option. Therefore, our aim was to compare the activity monitoring of stroke survivors by caregivers and physiotherapists during early phase in a hospital setting. Methods: An observation study was carried out in the neuroscience ward in a tertiary care hospital among 17 stroke survivors. Physiotherapist and caregivers were instructed to use an activity log chart that was developed during previous research conducted by the same authors for observing the activities performed by the patients every 15 minutes from 8 AM to 5 PM across one day. Data collected were analysed using Stata 15. Kappa statistics were carried out to determine the agreement of the observations between the two raters. Results: A total of 10 male and seven female caregivers of stroke survivors with a mean age of 40.11 ± 9.2 years and a trained physiotherapist participated in the study. A total of 272 observations of caregivers were in agreement with that of the physiotherapist. Inter-rater Kappa statistics showed 60% agreement between the physiotherapist and the caregivers (p<0.05). Conclusions: There was moderate agreement between the physiotherapist and caregiver for activity monitoring of stroke survivors. This suggests behavioural mapping by caregivers may be a potential alternative solution in healthcare settings.

Variability in aphasia following subcortical hemorrhagic lesion.

BACKGROUND: Vascular lesion of the subcortical structures leads to aphasia. Cortical hypoperfusion has been proposed to be the etiological mechanism in aphasia following subcortical vascular lesion. Subcortical aphasia shows considerable variability in its clinical profile. Such variability has been attributed to the variable sites of cortical hypoperfusion following ischemic lesion of the subcortical structures. PURPOSE: This study investigated the variability in clinical aphasic profile following subcortical hemorrhagic lesion. METHODS: We retrospectively investigated the clinical aphasic profiles of twelve patients who reported to our hospital during a period of one year with subcortical hemorrhagic lesions. All patients underwent routine neurological examination, neuroimaging (CT/MRI) investigations and linguistic assessment. RESULTS: Eight patients exhibited lesion to the basal ganglia and four showed thalamic lesion. All of them showed considerable variability in their aphasic profile. CONCLUSION: Subcortical hemorrhagic lesion leads to variability in aphasia. Variability in aphasia may be considered as an important consequence in subcortical vascular lesion. Observations from this study were suggestive of better preservation of, and when affected, faster recovery of comprehension skills.

Global aphasia without hemiparesis: A case series.

BACKGROUND: Global aphasia without hemiparesis (GAWH) is a rare stroke syndrome characterized by the unusual dissociation of motor and language functions. Issues regarding its etio-pathogenesis, lesion sites, and recovery patterns are extensively being debated in contemporary neuroscience literature. MATERIALS AND METHODS: Four patients admitted in our hospital between 2005 and 2009 with GAWH caused by ischemic stroke were studied retrospectively with emphasis on number and site of lesions, etiology, and recovery patterns. RESULTS: The clinical findings from our subjects showed that GAWH could result from either single/multiple lesions including subcortical lesions. The recovery was rapid, although not complete. One case evolved into Wernicke's aphasia as seen in earlier studies. Two subjects revealed evolution to transcortical sensory aphasia and one to Broca's aphasia which is distinct from previous proposals. Two cases showed lack of clinico-anatomic correlation during recovery. CONCLUSIONS: GAWH could result from both embolic and large vessel strokes and single or multiple lesions. The recovery pattern may be variable and may show lack of clinico-anatomical correlation indicating anomalous cerebral functional reorganization, questioning the conventional teaching of language representation in the brain.