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American Journal of Emergency Medicine (2012) 30, 2085.e5–2085.e7

Case Report

Neurobrucellosis developing unilateral oculomotor nerveparalysis☆,☆☆

Abstract

Brucellosis is a zoonotic infectious disease that iscommon around the world. Its clinical course demonstratesgreat diversity as it can affect all organs and systems.However, the central nervous system is rarely affected in thepediatric population. Neurobrucellosis is most frequentlyobserved with meningitis and has numerous complications,including meningocephalitis, myelitis, cranial nerve paralyses,radiculopathy, and neuropathy. Neurobrucellosis affectsthe second, third, sixth, seventh, and eighth cranial nerves.Involvement of the oculomotor nerves is a very rarecomplication in neurobrucellosis although several adultcases have been reported. In this article, we present the caseof a 9-year-old girl who developed unilateral nerve paralysisas a secondary complication of neurobrucellosis andrecovered without sequel after treatment. This case isnotable because it is a very rare, the first within thepediatric population.

Our article emphasizes that neurobrucellosis should beconsidered among the distinguishing diagnoses in every casethat is admitted for nerve paralysis in regions where Brucellainfection is endemic.

Brucella is a zoonotic infectious disease that is commonin Turkey and around the world. It infects humansrandomly through meat, milk, diary products, and thesecretions of infected animals. As the active microorgan-ism affects all organs and systems, its clinical coursedemonstrates great diversity. Involvement of the centralnervous system is very rare in brucellosis, and itsfrequency is low among the pediatric age population. Ithas various complications, including neurobrucellosismeningoencephalitis, myelitis, cranial nerve paralyses,radiculoneuropathy, brain abscess, demyelinating disease,hydrocephalia, and meningovascular syndromes [1]. Ocu-

☆ Contributors: Işıkay S proposed the study and wrote the first draft.Yılmaz K and Ölmez A helped in writing. All authors contributed to thedesign and interpretation of the study. Işıkay S is the guarantor.

☆☆ Disclosures: The authors have reported no conflicts of interest andfinancial support.

0735-6757/$ – see front matter © 2012 Elsevier Inc. All rights reserved.

lomotor nerve paralysis is a very rare complication ofneurobrucellosis [2].

In this article, we present a third nerve paralysis thatdeveloped as a secondary complication of neurobrucellosisbecause it is very rare and is the first case in the pediatricage population.

A 9-year-old girl was admitted to hospital withcomplaints of nausea, headache, and neck pain. She alsohad diplopia for 3 days and right upper eyelid ptosis sincethat morning. Her history demonstrated that she hadexperienced weariness, trichiniasis, night sweats, weightloss, and occasional high temperatures for almost a month.There were no characteristics in her history, nor that of herfamily, other than a diagnosis of Brucella at a health carecenter where she had been admitted for hip pain 6 monthsearlier, followed by a 1-month Brucella treatment. Thephysical examination showed a body weight of 23 kg (10percentile), a height of 130 cm (50-75 percentile), bloodpressure at 120/75 mm Hg, a pulse rate of 74 per minute,rhythmic, a respiration rate of 20 per minute, and a bodytemperature of 36.6°C. Her general condition was good,and she was conscious, oriented, and cooperating. HerNeck stiffness were positive, and Kerning and Brudzenskiindicators were negative. Her right upper eyelid was ptoticand deviated outwards and downwards; her ipsilateralpupil was dilated and showed no reflex to direct light. Thesame eye had limitations in looking inwards and upwards.Ophthalmology of both eyes and other system examina-tions were considered normal. Laboratory examinationsfound the patient's hemoglobin level at 12 g/dL, a whiteblood cell count of 7650/mm3, thrombocyte count of266 000/mm3, mean corpuscular volume at 77 fL, meancorpuscular hemoglobin at 24 pg, and red cell distributionwidth (RDW) at 17. Her peripheral blood smear contained65% polymorphonuclear leukocyte, 34% lymphocyte, and1% monocyte, and erythrocytes had a hypochromemicrocytic morphology. Her erythrocyte sedimentationrate was 18 mm/h, and her C-reactive protein was 3.22mg/dL (vs normal value of 0-0.8 mg/dL). Blood glucosewas found to be 95 mg/dL; urea, 2.4 mg/dL; serumcreatinine, 0.3 mg/dL; serum sodium, 135 mEq/L; potas-sium, 3.9 mEq/L; Cl, 103 mEq/L; calcium, 9 mg/dL;alanine aminotransferase, 38 U/L; aspartate aminotransfer-ase, 27 U/L; total protein, 7.5 g/dL; and albumin, 3.9g/dL. Serum iron was 44 μg/dL, with serum iron binding

2085.e6 Case Report

capacity at 215 μg/dL, and ferritin was 8 ng/mL. Herposteroanterior lung graph was normal. The patient, whosecomputed brain tomography was considered normal, wasgiven lumbar puncture. Her cerebrospinal fluid (CSF)glucose was 30 mg/dL (synchronous blood sugar was98 mg/dL), protein was 56 mg/dL, and microscopic eva-luation identified 120/mm3 mononuclear cells withlymphocyte character. Cerebrospinal fluid gram and acid-resistant bacteria staining did not identify any microor-ganisms. The patient was diagnosed with meningitis andwas given ceftriaxone (100 mg/kg per day). A tuberculinskin test was negative. Brain, orbital magnetic resonanceimaging and magnetic resonance imaging angiographywere considered normal. A serum and CSF Rose Bengaltest was positive. A serum Brucella Wright agglutinationtest was 1/320, and her CSF Wright agglutination waspositive at 1/1280 titration. Based on these clinical andlaboratory findings, the patient was diagnosed withneurobrucellosis. Rifampicin and cotrimoxazole treatmentswere added to the ceftriaxone treatment. The patient, whohad third nerve paralysis, was given an intravenous pulsesteroid for 5 days, then treatment was continued orally.The patient's complaints were relieved on the fifth day oftreatment. Ceftriaxone treatment was completed in 10days. Blood and CSF cultures were all negative. Thirdnerve paralysis of the left eye was completely relieved onthe seventh day. The patient was discharged, after startingoral iron treatment for anemia. Her prednisolone treat-ment was decreased and discontinued at the end of thesecond month. Rifampicin and cotrimoxazole treatmentswere discontinued at the end of the sixth month whenserum Brucella Wright agglutination was identifiedpositive at 1/80 titration. The patient did not have anycomplaints at her 1-year follow-up and was then removedfrom follow-up.

Brucellosis is endemic around the world, particularly inMediterranean countries and in Turkey. In Turkish society,the prevalence of Brucella seropositivity varies from 2.6%to 14.4% [1]. The active microorganism may affect anyorgan or system of the human body. Neurobrucellosis is arare complication of brucellosis and occurs at a very lowfrequency in the pediatric age population [2]. It is known that0.8% of pediatric Brucella cases affect the central nervoussystem [3]. Neurobrucellosis, which can affect both thecentral and the peripheral nervous system, has many differentclinical forms. Its eye complications include papillitis,papiloedema, retrobulbar neuritis, and ophthalmoplegia[1,2]. Neurobrucellosis most frequently leads to eighthnerve paralysis and can also involve second, sixth, andseventh cranial nerves [4]. In the literature, there are reportsof several cases that involve concomitant optical, abducent,and eighth nerve paralysis [5]; optical and abducentnerve paralysis [6]; facial and vestibulocochlear nerve para-lysis [7]; and isolated optical, abducent [8], and vestibuloco-chlear nerve paralysis [9]. Oculomotor nerve paralysis isvery uncommon and has rarely been reported in adult series

[2]. Miyares et al [10] reported a neurobrucellosis case inan adult who presented with oculomotor nerve paralysis inthe right eye, abducent nerve paralysis in the left eye, andirreversible papillitis. Our searches on Pubmed and Googlesearch engines did not retrieve reports of any pediatriccases. We conclude that this is the first pediatric neuro-brucellosis case with isolated third nerve paralysis to bepresented in literature.

The primary reasons for third nerve paralysis duringchildhood are congenital, posttraumatic, or postinfectious.Brain stem vascular diseases, multiple sclerosis [11], tumor,aneurysm, temporal lobe herniation, infections, cavernoussinus thrombosis, diabetes mellitus, arteriovenous malforma-tions, Tolosa-Hunt syndrome, migraine, myastenia gravis,and carotid-cavernous fistula are the other reasons [12].Herpes simplex type 1, human herpes type 6, varicella zostervirus, Brucella, and tuberculosis infections are infectiouscauses of third nerve paralysis [12,13]. Our case had anegative tuberculin skin test, and all of the radiologic imageswere normal.

Neurobrucellosis is diagnosed with production of theorganism in the CSF culture, demonstration of antibodiesgenerated against Brucella in the CSF, and the presenceof findings indicating meningeal involvement. Positiveidentification of at least one of these findings is sufficientfor diagnosis. The CSF evaluation of patients indicatespleocytosis primarily with lymphocyte and an increase inCSF proteins. The CSF glucose level may be normal orslightly decreased. Isolation of the organism in CSF cultureis the criterion standard; however, this test may not bepossible at all times. Production has been reported forless than a quarter of all cases. Typical meningitis findingsare identified in less than half of the cases [1,2]. Treat-ment of neurobrucellosis is a subject of debate, and thereis no consensus on the duration of treatment. Variousstudies report different treatment durations and suggest anaverage treatment time of 3 to 9 months, with double ortriple treatment combinations. Rifampicin, doxycycline,cotrimoxazole, and aminoglycoside are the preferredtreatments [1-10]. Our case was given a combined treat-ment with rifampicin and cotrimoxazole for 6 months.There had been no relapses or sequels by the time of her1-year follow-up. Shakir et al [2] suggested the useof steroids, although their effectiveness has not beencompletely proven in cases of neurobrucellosis with mye-litis, cranial nerve involvement, and vascular and spinalcord involvement. We applied the pulse steroid treat-ment to our patient for 5 days as an inpatient and thencontinued with it for 2 months, finally discontinuing it bygradual decrease.

Neurobrucellosis may appear with different clinicalmanifestations, and the diagnosis may be difficult. Althoughrare in the pediatric age population, this infectious diseasemay lead to atypical clinical presentations, such asoculomotor nerve paralysis, and should be considered asone of the distinguishing diagnoses.

2085.e7Case Report

Sedat IşıkayKutluhan Yılmaz

Department of Pediatric NeurologyGaziantep University Faculty of Medicine

Gaziantep, TurkeyE-mail addresses: dr.sedatisikay@mynet.com

dr.sedatisikay@hotmail.com

Akgün ÖlmezDepartment of Pediatrics

Denizli State HospitalDenizli, Turkey

http://dx.doi.org/10.1016/j.ajem.2011.12.004

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