Eosinophilic granulomatous polyangiitis (EGPA) like other antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis has multisystemic involvement. It commonly manifests with prodromal pulmonary involvement as asthma, chronic sinusitis followed by systemic vasculitic complications associated with blood and tissue eosinophilia. Central nervous system manifestations at presentation are uncommon compared with peripheral nervous system involvement. Vasculitic neuropathy in EGPA commonly presents as mononeuritis multiplex but rarely as polyradiculopathy. Late onset EGPA often presents with systemic involvement, and early diagnosis is a key to prevent further complications. The neuropathy in late onset EGPA is often refractory to immunosuppression and corticosteroids treatment. We report a case of EGPA with late onset asthma presenting with acute infarct and demyelinating polyradiculoneuropathy that progressed with bulbar paralysis and profound dysautonomia. This illustrates simultaneous involvement of central and peripheral nervous system with EGPA. Autonomic dysfunction can occur in patients of EGPA with multisystem involvement, which may predict severe complications.
Background:Cerebral Venous Thrombosis (CVT) is a multifactorial condition with wide clinical presentation. They have favourable prognosis but 15% die or become dependent. Hence, there is need for user friendly risk scoring system which helps in clinical decision making and predicting prognosis. Methods:The present study was a prospective observational study of CVT patients from April, 2018 to February, 2019 at Department of Neurology, King George Hospital, Andhra Medical College, Visakhapatnam, and followed up for three months. Patients with confirmed diagnosis of CVT based on history and radiological findings were studied. All the patients were assessed for CVT Grading Scale, CVST Scoring and CVT Risk Score. Results:Sixty-six CVT patients were enrolled with mean age of 32.4years. CVT Risk score can predict poor outcome if cut off score is above 3 with sensitivity of 92 % and specificity of 20 %. Prediction of prognosis and brain lesions by CVT risk score analysed by Fischer exact test is highly significant (p= 0.00). CVT Risk Score and CVT Grade Scale had high sensitivity in predicting prognosis at one and three months. CVT grading scale with mild scores had good outcome and those with severe grading had poor outcome. Conclusion:Prognostic Scores in Indian population was a novel study in CVT patients. Despite low specificity, it can be used to avoid unnecessary or dangerous intervention in low-risk patients and identifies high-risk patients with poor prognosis, who require intensive interventions, such as local IV thrombolysis and decompressive surgery.
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