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A Rare Case of Guillain-Barre Syndrome Without Identifiable Trigger in Polycythemia Vera Patient
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A6877 - A Rare Case of Guillain-Barre Syndrome Without Identifiable Trigger in Polycythemia Vera Patient
Author Block: P. T. Taweesedt, A. Gupta, M. Bachan, Z. Khan; James J. Peter VA Medical center, Bronx, NY, United States.
IntroductionGuillain-Barré syndrome (GBS) is a rare condition in which the immune system attacks the peripheral nerves and is characterized by acute progressive symmetrical motor weakness and decreased or absent reflexes. It is often preceded by a trigger, commonly an infection or vaccination.
Case presentation
A 70 year-old male patient with Polycythemia Vera (PV) who presented with 4 days of rapidly progressive near-symmetrical weakness in his lower extremities. No history of recent infections or vaccination were identified prior to the onset of weakness. The initial presumptive diagnosis was a thrombotic event secondary to complication of uncontrolled PV since the patient had missed last several phlebotomy treatments for PV. He was admitted for inpatient phlebotomy. His neurological workup revealed absent deep tendon reflexes and weakness ascending to his upper extremities. A Lumbar puncture revealed elevated proteins and normal cell count in his cerebrospinal fluid consistent with GBS. He received 5 sessions of plasmapheresis over 10 days. During hospitalization his weakness plateaued with no worsening and no involvement of respiratory function/cranial nerves or autonomic nerves.
Discussion
GBS without any trigger is extremely rare. There was only one reported case of PV with GBS who developed cerebrovascular accident after receiving intravenous immunoglobulin G. Our patient did well with plasmapheresis and will be the second reported case of PV with GBS. Plasmapheresis is possible the treatment of choice for GBS in PV. Our patient is unique since he has GBS with PV. Is it possible that missed therapeutic phlebotomy in PV was the trigger?
Author Block: P. T. Taweesedt, A. Gupta, M. Bachan, Z. Khan; James J. Peter VA Medical center, Bronx, NY, United States.
IntroductionGuillain-Barré syndrome (GBS) is a rare condition in which the immune system attacks the peripheral nerves and is characterized by acute progressive symmetrical motor weakness and decreased or absent reflexes. It is often preceded by a trigger, commonly an infection or vaccination.
Case presentation
A 70 year-old male patient with Polycythemia Vera (PV) who presented with 4 days of rapidly progressive near-symmetrical weakness in his lower extremities. No history of recent infections or vaccination were identified prior to the onset of weakness. The initial presumptive diagnosis was a thrombotic event secondary to complication of uncontrolled PV since the patient had missed last several phlebotomy treatments for PV. He was admitted for inpatient phlebotomy. His neurological workup revealed absent deep tendon reflexes and weakness ascending to his upper extremities. A Lumbar puncture revealed elevated proteins and normal cell count in his cerebrospinal fluid consistent with GBS. He received 5 sessions of plasmapheresis over 10 days. During hospitalization his weakness plateaued with no worsening and no involvement of respiratory function/cranial nerves or autonomic nerves.
Discussion
GBS without any trigger is extremely rare. There was only one reported case of PV with GBS who developed cerebrovascular accident after receiving intravenous immunoglobulin G. Our patient did well with plasmapheresis and will be the second reported case of PV with GBS. Plasmapheresis is possible the treatment of choice for GBS in PV. Our patient is unique since he has GBS with PV. Is it possible that missed therapeutic phlebotomy in PV was the trigger?
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