A 50-year-old previously healthy woman presented with a 4-day history of low back pain, nausea, vomiting, diarrhea, jaundice, and fever. She developed hypoxemic respiratory insufficiency and a chest radiograph showed diffuse alveolar and interstitial infiltrates. She was promptly intubated and became hemodynamically unstable and developed dialytic-renal insufficiency. At this time, laboratory workup revealed a hemoglobin level of 6.1 mg/dL (to convert to grams per liter, multiply by 10), leukocyte count of 0.2603/μL (to convert to ×109/L, multiply by 0.001), C-reactive protein level of 59 000 mg/L (to convert to nanomoles per liter, multiply by 9.524), platelet count of 28×103/μL (to convert to ×109/L, multiply by 1), serum urea level of 80 mg/dL (to convert to millimoles per liter, multiply by 0.357), serum creatinine level of 2.24 mg/dL (to convert to micromoles per liter, multiply by 88.4), serum potassium level of 0.72 mEq/L (to convert to millimoles per liter, multiply by 1), and abnormal coagulation profile (international normalized ratio of 1.8; D-dimer level of 125 200 mg/mL [to convert to nanomoles per liter, multiply by 5.476). A presumptive diagnosis of hemolytic-uremic syndrome led to a day of plasmapheresis, but then no schizocytes were found in the peripheral blood and results of the Coombs test were negative. Empirical antibiotic and antiviral treatment were started with ceftriaxone sodium, clarithromycin, and oseltamivir phosphate. Her blood and urine culture findings were negative. After 4 days of intensive care unit treatment, the patient progressively recovered and became hemodynamically stable. She no longer needed daily hemodialysis and she was being ventilated in spontaneous mode without sedative drugs. However, no awakening occurred. Neurological examination revealed a comatose patient with decorticated posturing after noxious stimuli, with normal brainstem functions and pupillary responses, without focal signs. Cranial computed tomography showed widespread cortical and deep brain hemorrhages (Figure, A and B). Brain magnetic resonance imaging and magnetic resonance angiography (Figure, C-G) revealed several foci of hemorrhages, internal jugular vein thrombosis, and thrombosis of multiple sinuses. Given the presence of cerebral venous thrombosis, a thorough search for autoimmune, prothrombotic, and infectious conditions was done. Serum human immunodeficiency virus; human T-lymphotropic virus; hepatitis A, B, and C; Venereal Disease Research Laboratory test; dengue and yellow fever; Rickettsial spotted fever; and leishmaniasis serologies were negative. An IgM immunoassay (enzyme-linked immunosorbent assay) for leptospirosis (Leptospira interrogans serovar icterohemorrhagiae) came out positive and was followed by microscopic agglutination test (MAT) titers of 1/400 at the first sample collected 5 days after the onset of symptoms. Another IgM enzyme-linked immunosorbent assay for leptospirosis performed 30 days after the onset of symptoms was still positive, with MAT titers of 1/100 for the serovar icterohemorrhagiae, lower than the initial values probably because a day of plasmapheresis was inadvertently performed before a hemolytic-uremic syndrome was ruled out. The final diagnosis was Weil syndrome complicated with cerebral venous thrombosis. The patient received low-molecular-weight heparin to treat the underlying prothrombotic state as well as ceftriaxone for 14 days. She was discharged from the intensive care unit without active interaction and without following commands, but her eyes had spontaneous opening. A cranial computed tomography scan was repeated 12 days after anticoagulation and showed a reduction of parenchymal edema and all hemorrhagic lesions. She was discharged from the hospital after 45 days with gastrostomy, tracheostomy, and an anticoagulation scheme. She started to follow simple commands but remained with anarthria and spastic tetraparesis.
Vale TC, Santos GCDT, Saturnino SF, Neto ASA, Amâncio FF, Oliveira MAA, Fernandes BFS, Gomez RS, Andrade MV, Teixeira AL. Weil SyndromeA Rare Cause of Cerebral Venous Thrombosis. JAMA Neurol. 2014;71(2):238-239. doi:10.1001/jamaneurol.2013.2175