Successful therapeutic plasma exchange in a patient with Morvan syndrome

Rounak Dubey, Rajeev S Mallhi, Bhushan Asthana, Neerja Kushwaha, Arvind YadavAsian Journal of Transfusion Science 2019 13(1):73-75Morvan syndrome is a rare autoimmune disorder, characterized by hyperexcitability of both central and peripheral nervous systems, accompanied by autonomic dysfunction and hallucinations.[1] Therapeutic plasma exchange (TPE) has been found to be an effective mode of treatment for this disease, but there is limited literature supporting the same.[2] A 26-year-old male was admitted to our hospital and diagnosed with a case of Morvan syndrome, based on the clinical picture and laboratory findings. When standard drug therapy failed to show any improvement, a decision to carry out TPE was taken. The case presented with many peculiar challenges, mostly due to autonomic instability and hyperkinesia experienced by the patient while carrying out the procedure. All these challenges were diligently addressed and managed promptly. Clinical signs of improvement were evident from the 2nd TPE and by the time fifth TPE had finished, the patient was able to perform activities such as walking with support. His autonomic dysfunction and behavioral abnormalities had significantly subsided. This case report highlights the possible effectiveness of TPE in the management of a rare disease such as Morvan syndrome and appropriate application of basic principles and criteria for the use of TPE in cases where limited literature is available.
Source: Asian Journal of Transfusion Science - Category: Hematology Authors: Source Type: research