Abstract
Churg Strauss Syndrome (CSS) is a rare ANCA Associated Vasculitis (AAV) causes disseminated necrotizing vasculitis in patients with eosinophilia and Asthma. Initial presentation as Neuropathy is quite rare (15%).Usually Mono neuritis multiplex is the common presentation of Vasculitic neuropathy. Acute Neuropathy with GBS like initial presentation is extremely rare. There are only 6 case reports in literature. Here we present a 57 yr old male with history of asthma presenting with acute onset and progressive weakness of lower limbs followed by upper limbs with painful tingling and paresthesia over 3 weeks duration. On examination there was areflexic flaccid weakness of lower limbs and upper limbs both proximal and distal with distal sensory loss without any cranial nerve involvement. Investigations showed severe eosinophilia with proteinuria and urinary casts. Vasculitic workup showed high titre P-ANCA positivity. Nerve Conduction Studies showed severe axonal and demyelinating type of neuropathy effecting all four limbs. In view of vasculitis involving target organs patient was treated with pulse steroids and cyclophosphamide according to EUVAS Regimen. Patient showed good improvement in neurological deficits and proteinuria and is under regular follow up.
Keywords: Churg Strauss Syndrome GBS ANCA Associated Vasculitis Nephritis Asthma.
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Corresponding Author
Praveen Kumar Yadav
Consultant Neurologist and In Charge, Department of Neuromedicine,
The Mission Hospital, Durgapur
DOI: https://dx.doi.org/10.18535/jmscr/v5i11.168
