Malignant Hemispheric Cerebral Infarction Associated with Idiopathic Systemic Capillary Leak Syndrome
In January 2012, a 40-year-old man was admitted to our hospital because of shock. Three days before admission, a flu-like illness that included nausea, general malaise, lower abdominal pain, and severe diarrhea was observed. At the previous hospital, 500 ml of 5% albumin and 1,500 ml/day of saline were administered. Three days later, the patient's consciousness had deteriorated, and right hemiparesis appeared. He was then referred to our hospital.\nPhysical examination revealed a temperature of 36.0°C, blood pressure of 95/72 mm Hg, pulse of 160 beats/min, and respiratory rate of 25 breaths/min. His SpO2 was 100% using a 10-liter/min O2 mask. The jugular vein was collapsed. The bilateral radial artery pulses were insufficiently palpable, and the extremities were cold. A whole-blood sample was coagulated easily and considered to be in a state of hyperviscosity. Laboratory data indicated polycythemia, hypoalbuminemia, severe leukocytosis, and elevated creatinine. Serum electrolytes were normal, but severe metabolic acidosis was found (table ). Influenza virus types A and B were shown to be positive using a rapid diagnostic kit. Pericardial effusion and a collapsed inferior vena cava were revealed in heart echocardiograph and chest computed tomography (CT) examinations (fig. ). Magnetic resonance (MR) angiography showed the occlusion of the division of the left M2 segment of the middle cerebral artery (MCA). Diffusion-weighted MR imaging revealed high signal intensity in the MCA territory (fig. ), suggesting acute cerebral infarction. Due to the midline shift of the head on CT after 20 h of hospitalization, external and internal decompression surgery was performed (fig. ). Postoperative intracranial pressure was 9 mm Hg, but gradually exceeded 25 mm Hg with cerebral edema progression. Excessive cerebral edema rapidly developed on the ischemic lesion in the leak phase, and internal decompression was added 12 h after the initial decompressive surgery. The main cause of the hypoproteinemia and hemoconcentration was thought to be intravascular volume depletion due to increased vascular permeability, with extravasation of albumin. It was consistent with the pathology of ISCLS. Mechanical ventilation and massive fluid resuscitation (10 liters/day) were implemented on the first day (fig. ).\nSwelling, redness, and heat sensation were noted in the lateral surfaces of the legs bilaterally. Pretibial compartment syndrome was diagnosed and bilateral calf fasciotomy was performed (fig. ). All samples were negative, including pan cultures of blood, sputum, and urine, and were analyzed for aerobic, anaerobic, atypical mycobacteria, and fungi. Toxic shock syndrome, sepsis, anaphylaxis, acute adrenal insufficiency, drug reactions, and polycythemia vera were considered as differential diagnoses, but there was no evidence suggesting its pathology.\nSevere right hemiparesis and Broca's aphasia persisted, and the patient was unable to walk without assistance at the 2-month follow-up. Two hundred milligrams of theophylline were administered twice daily as prophylaxis against ISCLS. Despite compliance with the prescribed medications, 7 attacks of ISCLS were documented during the first 12-month follow-up, with 2 severe episodes requiring fasciotomy (fig. ). Appropriate fluid resuscitation has resulted in no observed stroke recurrence. A monoclonal immunoglobulin (IgG kappa) was found in the clinical course using immunoelectrophoresis, though plasmacytosis was not found in bone marrow aspiration. The patient was also given an intravenous infusion of immunoglobulins, corticosteroids, and immunosuppressive therapy.