Meningoencephalitis in a Child Complicated by Myocarditis, Quadriparesis and Respiratory Failure

Abstract

An 8-year-old Hispanic male resident of Maryland developed fever to 38°C, diffuse abdominal pain and dizziness in August 2005. He was previously healthy, born and raised in the United States, but had traveled to Mexico a few years prior. He was treated for sinusitis by his physician, but was hospitalized 5 days later because of persistent fever, neck stiffness, nausea, vomiting and generalized weakness. On admission, vital signs showed a temperature of 38.5°C, heart rate of 126 beats/min, respiratory rate of 28 breaths/min, blood pressure of 102/45 mm Hg and oxygen saturation 95% in room air. Physical examination was notable for stiff neck, diffuse abdominal tenderness and a focal petechial rash over his left anterior chest. Neurologic examination was normal. A complete blood count revealed a white blood cell count of 4.2/ L (87% neutrophils, 2% band forms, 9% lymphocytes and 2% monocytes), hemoglobin concentration of 11 g/dL and platelet count of 161,000/ L. Cerebrospinal fluid (CSF) analysis showed 530 leukocytes/ L (88% granulocytes, 12% lymphocytes), red blood cells 20, with glucose 61 mg/dL and protein 148 mg/ dL. A Gram-stained smear of the CSF and bacterial cultures of the CSF and blood revealed negative results. Aspartate aminotransferase was 218 units/L (normal, 26), and alanine aminotransferase was 109 units/L (normal, 44). Serum electrolyte concentrations, urinalysis, urine toxicology screen and urine culture were normal. The patient was empirically treated with vancomycin and ceftriaxone for suspected bacterial meningitis. On the second hospital day, the child’s temperature increased to 39°C, and he continued to have abdominal pain and persistent nonbilious, nonbloody emesis. Neurologic examination remained normal, and mental status was appropriate. A chest radiograph and computed tomography of the brain were normal. Contrast-enhanced abdominal computed tomography examination revealed a mildly fatty liver with no focal lesions and a moderate quantity of retained stool. Early the next morning, the patient’s cardiorespiratory monitor alarm was activated, and he was found to be unresponsive with agonal breathing and weak gag and cough reflexes. He was hypothermic (34.5°C), heart rate was 145 beats/min and oxygen saturation was not detectable by pulse oximetry. The patient was intubated. Cardiology evaluation that morning demonstrated decreased electrocardiographic voltages throughout all leads and a prolonged QTc interval. Echocardiography showed a reduced shortening fraction of 23% (normal, 28–44%) and an ejection fraction of 40% (normal, 55–70%), consistent with myocarditis. Later in the afternoon, neurologic examination was remarkable for a comatose state and complete flaccid, areflexic quadriparesis. An electroencephalogram revealed diffuse slowing, consistent with meningoencephalitis. A repeat complete blood count showed a white blood cell count of 14,280/ L (44% neutrophils, 2% band forms, 35% lymphocytes, 11% monocytes, 1% basophils and 7% reactive lymphocytes), hemoglobin 10.5 g/dL and platelet count 210,000/ L. Aspartate aminotransferase and alanine aminotransferase were improved slightly to 117 and 76 units/L, respectively. Sodium had decreased to 126 mmol/L, and chloride was 93 mmol/L as a result of inappropriate antidiuretic hormone production and cerebral salt wasting. The other electrolytes were normal. Polymerase chain reaction tests on the CSF obtained from a repeat lumbar puncture on hospital day 4 for herpes simplex virus and enteroviruses yielded negative results. T2 magnetic resonance imaging on hospital day 6, obtained because of concern for cerebral damage resulting from hypoperfusion, demonstrated mild leptomeningeal enhancement and enhancing ventral nerve roots from thoracic level spine through the cauda equina. Antimicrobial therapy was empirically broadened on hospital day 4 when the patient developed respiratory failure and demonstrated an evolving flaccid paralysis. Acyclovir (for herpes simplex virus), doxycycline (for Rocky Mountain spotted fever), rifampin, isoniazid, pyrazinamide (for tuberculosis) and fluconazole (for cryptococcal meningitis) were added. Intravenous immunoglobulin (2 g/kg; Polygam SD; Baxter, Westlake Valley, CA) was administered on the fifth hospital day, after no neurologic improvement was noted because of concern for atypical Guillain-Barre syndrome. Increased alertness was observed over the next several days, during which time ptosis was noted that improved slightly over his hospital course. On the sixth hospital day, nerve conduction studies and an electromyogram suggested an early peripheral demyelinating polyneuropathy. By the eighth hospital day, the patient was able to open his eyes slightly to pain and had minimal ability to visually track from side to side. On hospital day 9, follow-up electrocardiogram revealed low, but improved, voltages and echocardiography showed improvement of the shortening fraction to 29% and ejection fraction to 52%. For denouement, see p. 855. Accepted for publication June 16, 2006. From the *Department of Infectious Disease, Children’s National Medical Center, the †Department of Pediatrics, Uniformed Services University of the Health Sciences, and the †Department of Neurology, Children’s National Medical Center, Bethesda, MD Copyright © 2006 by Lippincott Williams & Wilkins ISSN: 0891-3668/06/2509-0853 DOI: 10.1097/01.inf.0000234058.31683.70