Background: Guillain-Barré syndrome (GBS) is a group of clinical syndromes involving acute polyneu­ropathy secondary to an immune-mediated process. The aim of the research is to study clinical profile and outcome in children aged 1-12 years presenting with GBS. Methods: It is a prospective plus retrospective observational study (5-year retrospective + 18 months prospective). All cases of GBS admitted from January 2020-july 2021 were enrolled for the study and retrospective cases were taken from case record from January 2015 onwards in a tertiary care centre. Results: Total 45 children were enrolled. The male: female ratio was 1.64:1. Mean age at presentation was 6.3 years, in a range of 1.5 -11.5 years of age. History of antecedent illness prior to the occurrence of GBS was present in 93.3% patients with upper respiratory tract infection being most common seen in 53.3% patients. Following variants of GBS were seen on NCV study performed in 34 patients, the most common was AIDP in 46.7% patients, followed by AMAN in 22.2% subjects and AMSAN in 6.7% patients. All children presented with lower limb weakness which progressed to involve upper limbs. Other associated features like bulbar cranial nerve involvement, respiratory muscle weakness, sensory symptoms and autonomic disturbance (arrythmia, hypotension, tachycardia, bradycardia) was observed in 30,12,7,16 patients respectively. Out of the total 45 patients, 12 patients required mechanical ventilation. Conclusions: Male predominance seen in GBS. AIDP the most common subtype of GBS. Respiratory distress and autonomic instability were associated with greater severity of Hughes disability score. Factors associated with poor outcome were sensory symptoms, autonomic instability, respiratory distress and bulbar cranial nerve involvement.

Background: Acquired demyelinating diseases (ADS) соnstitute а heterogeneous grоuр of central nervous system disorders of autoimmune origin that causes significant physical and cognitive disabilities. Early recognition and prompt management causes significant improvement in acute episodes of demyelinating disorders. Methods: 33 children diagnosed with demyelinating disorders at Lokmanya Tilal municipal medical college and hospital in Mumbai were enrolled. The study was conducted between January 2013 and November 2022.  Demographic data, clinical profile, CSF study, serum antibody, radiological findings were collected and results were analyzed. Statistical Data was analysed using statistical software GraphPad in Stat.V3.0. Data were presented in tables and figures whenever needed.  P value <0.05 considered as significant. Results: Of 33 patients, 21 (63%) were cases of acute disseminated encephalomyelitis (ADEM), 6 (18.1%) of transverse myelitis (TM), 1 case of ADEM + TM (3%), 3 (9%) of neuromyelitis optica, 1 (3%) of Optic neuritis and 1 (3%) of multiple sclerosis. ADEM patients presented with encephalopathy and multifocal neurological deficits, 40% were MOG positive. Two patients were of multiphasic ADEM. Patients of transverse myelitis had paraparesis or quadriparesis and sensory + bladder involvement. Patients with NMO presented with bilateral visual impairment with limb weakness and bladder involvement. Steroids were the primary treatment, 3 patients (9%) required intravenous immunoglobulin (IVIG) and 1 (3%) patient received plasma exchange therapy. Conclusions: ADEM is the most common ADS. Early diagnosis and management with steroids therapy improves outcome in most of the patients. Non response to steroids warrants second line treatment options like IV Immunoglobulin (IVIG) or Therapeutic plasma exchange.