Altered Mental Status, Ataxia, and Ophthalmoplegia in an 8-year-old Girl

Leigh A. Stubbs; Sam N. Russo; Baruch R. Goldberg; Ankur A. Kamdar

doi:10.1542/pir.2019-0161

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Leigh A. Stubbs, MD, MPH*
Sam N. Russo, MD^†
Baruch R. Goldberg, MD^‡
Ankur A. Kamdar, MD^‡

^*Division of Community and General Pediatrics,
^†Division of Neurology, Department of Pediatrics,
^‡Division of Pulmonary, Allergy, Immunology, and Rheumatology, McGovern Medical School at The University of Texas Health Science Center at Houston, Houston, TX

Address correspondence to Leigh Anna Stubbs, MD, MPH, Feigin Tower, 1102 Bates, Ste. 330, Houston, TX 77030. E-mail: leigh.stubbs{at}bmc.edu

AUTHOR DISCLOSURE
Dr Stubbs’ current affiliation is Section of Pediatric Rheumatology, Department of Pediatrics, Baylor College of Medicine, Houston, TX; Dr Goldberg’s current affiliation is Section of Rheumatology, Department of Pediatrics, Emory School of Medicine, Atlanta, GA. The authors have no financial relationships relevant to this article to disclose. This commentary does not contain a discussion of an unapproved/investigative use of a commercial product/device.

Presentation

An 8-year-old African American, fully vaccinated girl with a past medical history of febrile seizures presents to the general pediatric ward with altered mental status (AMS), ataxia, and ophthalmoplegia. Over the course of 5 days before admission, she developed bilateral otalgia with upper respiratory symptoms, fever, headache, neck pain, and fatigue. Her mother denies that the patient has had any weight loss, hallucinations, seizures, significant family history, travel, insect bites, ingestions, undercooked or unpasteurized food, or known contacts with tuberculosis.

On physical examination, she is febrile (103°F [39.4°C]) and tachycardic (126 beats/min) without tachypnea or blood pressure abnormalities. She is ill-appearing, sleepy, and irritable, but she arouses with stimulation and follows simple commands. Her pupils are 3 mm, symmetrical, and equally reactive to light. She has a wide-based gait and ophthalmoplegia characterized by bilateral disconjugate gaze with esotropia. She exhibits difficulty with forward flexion of her neck but otherwise normal range of motion. The rest of her cranial nerve examination, deep tendon reflexes, tone, strength, and bulk are normal.

Her initial cerebrospinal fluid (CSF) analysis shows a white blood cell count of 470/μL (0.47 × 10⁹/L) with 50% lymphocytes and 50% atypical mononuclear cells, red blood cell count of 110/μL (0.11 × 10⁹/L), protein of 84 mg/dL (0.84 g/L), and glucose of 44 mg/dL (2.4 mmol/L) with concurrent serum glucose of 100 mg/dL (5.6 mmol/L). Her complete blood cell count is significant …