Atypical Hemolytic Uremic Syndrome Due to Neuroinfection : A Case Report and Pathophysiology

TMA (Thrombotic Microangiopathy), still a challenge in the medicine practice nowadays, one of the important factor is the high suspicion needed to consider the diagnosis in a patient with multiple signs and symptoms as manifestation of one disease. In this article, based on a clinical case, we explain and offer a simple algorithm that integrates diagnosis and treatment in a in a practical and feasible manner. A 36-year-old man, native of Venezuelan country, presented to the emergency department with complaint of malaise, bilateral ankle pain and fever for 1 week. He had medical comorbidities of mesangial proliferative glomerulonephritis and essential hypertension diagnosed 9 years ago, (being treated with nifedipine, mycophenolate and prednisone), his social history unremarkable, he denied drug abuse or smoking. On examination he was conscious and exhibited fluctuating level of alertness. He had a blood pressure of 156/106 mmHg, pulse of 86, respiratory rate of 18, and temperature of 99°F. On precordial exam he had normal heart sounds with no murmurs, on auscultation of lungs, he had bilateral air entry with no adventitious sounds. There was no jugular venous distention. The abdominal examination result was normal. Neurological exam initially was unremarkable. Laboratory workup showed a Complete Blood Count (CBC) with hemoglobin of 7.7g/dL, hematocrit of 22.8%, reticulocytosis of 3.2%, and a white cell count of 6.0 k/μL, platelet count was 29 k/μL. Peripheral smear was examined, which showed schistocytes (++) Drepanocytes (+) with screening test for sickle cell disease negative. Creatinine of 12.7 mg/dL (no baseline known), Blood Urea Nitrogen (BUN) 140 mg/dL, Arterial Blood Gases (ABG) with non-anion gap metabolic acidosis. A chest X-ray did not reveal any pathology, renal ultrasound findings showed changes compatible with long standing severe CKD. For the anterior data the patient was started on hemodialysis. Several hours later patient persisted febril without any infection signs. On the second day from admission, He had a sudden episode of shaking and deviation of the head and eyes to the left, with left-beating nystagmus. During this episode, the eye movements did not cross the midline, he was responsive to questions, and appeared fully alert, despite he denied clear memories of the event. His deteriorating mental status and inability to protect his airway required that he be placed on mechanical ventilation. Imaging studies that included a computed tomography scan of the brain showed no significant abnormalities to explain his altered mental status. He continued presenting seizure activity with decreasing interval of time, soon he was placed on complex therapy requiring benzodiazepines, valproate and the use of anesthetic therapy, after 24 hours we considered a super refractory status epilepticus condition. In order to discard neuroinfection a lumbar puncture was indicated, and it revealed lymphocytic pleocytosis, thereby the patient was placed on antimicrobial therapy (acyclovir) considering the possibility of herpetic meningitis. Nevertheless the most remarkable finding on this clinical case, was the simultaneous presence of schistocytes in peripheral blood, thrombocytopenia, and renal failure, leading to the suspicion of thrombotic microangiopathy. Once the ADAMTS13 level was measured (86.5%) and the absence of diarrhea history during present illness was confirmed and shiga toxin producing Escherichia coli test was negative, we concluded atypical hemolitic uremic syndrome (aHUS) as the most likely diagnosis, and the patient was as soon as possible initiated on eculizumab (after Neiserria meningitides vaccine), regardless of this effort patient died several days later (multiorgan failure), receiving just one dosis of Eculizumab. Citation: Londoño JL, Zapata MI, Maiguel JA (2019) Atypical Hemolytic Uremic Syndrome Due to Neuroinfection: A Case Report and Pathophysiology Emphasis Review. Int J Case Rep Ther Stud 1: 003.