HP41: Idiopathic systemic capillary leak syndrome with severe peripheral nervous system involvement. Case report

Abstract

Idiopathic systemic capillary leak syndrome (ISCLS) a rare condition characterised by repeated relapses of leaking of plasma from blood vessels into body cavities and muscles. In one third of cases the disease is associated with variable severity peripheral nervous system damage. 26 years old male was admitted to hospital after repeated fainting with complains about progressive pain and numbness in lower limbs. In the hospital symptoms were rapidly progressing and patient developed asymmetrical painful tetraparesis (muscle strength in legs 0-1, muscle strength in arms 4). Systemic manifestations included hypotension, tachycardia, hemoconcentration, hypoalbuminemia, cardiac tamponade, crush syndrome. On 5th day from symptom onset treatment with intravenous immunoglobulin (IvIg) was started. After first IVIG treatment course systemic symptoms had resolved. Patient continued to receive IvIg monthly as a prophylactic treatment. After two courses of IvIg pain ceased, muscle strength improved (arm muscles 5, leg muscles 4). Patient died 20 months after symptom onset due to repeated systemic relapses. Electrodiagnostic study (EMG/NCS) was performed 3 times (on 3rd day from symptom onset, after first and second IvIg course). First EMG/NCS showed severe axonal motor and sensory neuropathy in legs, mild motor axonal damage in arms with massive spontaneous activity in all examined muscles. After repeated IvIg courses NCS in arms become normal, in legs tibial and peroneal MUAP remained absent, sural SNAP became normal; EMG showed slight evidence of reinnervation process without signs of active denervation. Conclusion. ISCLS can be considered in patients with rapidly progressive axonal polyradiculoneuritis with autonomic signs.