Clinician’s Corner Case 2: A nine-year-old girl with prolonged fever and headache A previously healthy nine year-old girl presented to the authors’ institution (BC Children’s Hospital, Vancouver, British Columbia) with a four-week history of fevers, vomiting, headache and abdominal pain. She was first seen in a community hospital following a 24 h history of fever followed by headache, emesis and a generalized erythematous, macular rash. Petechiae were not noted. A diagnosis of viral gastroenteritis was made. The rash subsided quickly but the remainder of her symptoms persisted. Ten days later, she presented again to the same hospital with daily fevers, daily episodes of emesis and debilitating headaches. Her investigations included a normal complete blood cell count, and her urine culture was positive for Escherichia coli. She was discharged on cephalexin for five days; however, her symptoms failed to resolve. She was subsequently referred and admitted to the authors’ tertiary care centre. Review of her history revealed daily fevers accompanied by severe nausea and emesis, along with severe headaches without photophobia. There was no history of recent travel or ill contacts. She denied night sweats, neck stiffness or joint pain. Her records indicated a 5 kg weight loss over four weeks. Physical examination revealed a stable patient with normal vital signs. General examination was unremarkable and no neck stiffness or neurological deficit was detected. Laboratory investigations on admission showed leukocytosis (white blood cell count 11.2×109/L) and thrombocytosis (platelet count 423×109/L [normal range 150×109/L to 400×109/L]). Erythrocyte sedimentation rate was elevated (76 mm/h). Urine and blood cultures were negative. Abdominal ultrasound revealed a large echogenic right kidney, suggesting possible pyelonephritis. A computed tomography scan of the head and a chest radiograph were normal. A further investigation was performed to reveal the diagnosis. Correspondence (Case 2): Dr Keyvan Hadad, Division of General Pediatrics, BC Children’s Hospital, Room 2N-11 – 4480 Oak Street, Vancouver, British Columbia V6H 3N1. Telephone 604-602-0931, e-mail [email protected] Case 1 accepted for publication November 8, 2013. Case 2 accepted November 12, 2013 Paediatr Child Health Vol 19 No 4 April 2014 ©2014 Pulsus Group Inc. All rights reserved 177 Clinician’s Corner case 2 Diagnosis: Chronic Meningococcal Meningitis Given the ultrasound results, a provisional diagnosis of partially treated urinary tract infection was made. To complete the diagnostic workup, a lumbar puncture was performed at 12 h of admission and before initiation of antibiotic therapy. The cerebrospinal fluid (CSF) was cloudy, with an opening pressure of 38 cmH2O. Analysis of the CSF revealed a glucose level of <1.1 mmol/L, a protein level of 2.33 mg/L, white blood cell count of 1740×106/L and red blood cell count of 2×106/L. Polymerase chain reaction performed on the CSF was positive for Neisseria meningitidis serogroup B. The patient was started on intravenous cefotaxime and became afebrile within 12 h of therapy. To facilitate comprehensive evaluation of exposure and possible chemoprophylaxis of close contacts, the case was reported to the BC Centre for Disease Control (Vancouver, British Columbia). All symptoms resolved within 24 h to 48 h. She was discharged after completion of a seven-day course of intravenous cefotaxime. Of note, the patient was subsequently shown to have no complement deficiency, nor was she asplenic. At discharge, she was found to have moderately severe left-sided sensorineural hearing loss; this remained unchanged six weeks later. Infection with Neisseria meningitidis remains an important cause of morbidity and mortality. Prompt recognition of symptoms and initiation of therapy are essential to prevent possibly serious morbidity and mortality. The infection may be limited to blood or to the meninges, or may involve a combination of both. The most common findings of an acute meningeal infection are neck stiffness, fever and altered mental status. Here, however, we present a case of chronic meningococcal meningitis. Chronic and/or recurrent infections with N meningitidis are exceedingly rare, and are mostly confined to patients with deficiencies of terminal complement components (C5 to C9), C3 or properdin, or with anatomical or functional asplenia. Chronic meningococcemia presents as a triad of spiking fever, vasculitic rash and largejoint arthralgia. The diagnosis is challenging because bacterial cultures are frequently negative, at least in the initial stages of the illness. Meningeal involvement in chronic meningococcemia can occur as a late complication. 178 Our patient was both young and fully immunocompetent, in contrast to reports of this condition in the elderly (1) and in a patient with a complement deficiency (2-5). Additionally, our patient had no clinically detectable neurological findings; this differed from previous case reports (1,2,6) and increased the diagnostic challenge. Our case did, however, resemble other published reports on chronic meningitis due to N meningitidis in that the blood cultures were negative and the response to appropriate antibiotic treatment was rapid. The outcomes of published cases appear to be highly variable, ranging from none to mild gait ataxia or sensorineural hearing loss, as demonstrated in our patient. Clinical Pearls • Chronic meningococcal meningitis can exist in the absence of acute or chronic meningococcemia. • Patients with chronic meningococcal meningitis may not always demonstrate classic meningeal signs or neurological deficits. A lumbar puncture is, thus, essential for the diagnosis. • Chronic meningococcal meningitis should be considered in the differential diagnosis of fever or headache associated with emesis. Tahara Bhate BSc Division of General Pediatrics Tobias R Kollmann MD PhD Divison of Infectious Diseases Keyvan Hadad MD MHSc FRCPC Division of General Pediatrics Department of Pediatrics, University of British Columbia Vancouver, British Columbia Paediatr Child Health Vol 19 No 4 April 2014
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