DIAGNOSTIC DILEMMAS AND CLINICAL REASONING A 33-Month-Old With Fever and Altered Mental Status

A 33-month-old girl presented with 3 days of fever and 1 day of multiple paroxysmal episodes of screaming with apparent unresponsiveness, flexed lower extremities, clenched hands, and upward eye deviation. These events lasted seconds to a minute at a time and occurred only during sleep. She slept peacefully between episodes and was easily awakened. She had a history of mild speech delay and mild intermittent asthma but was otherwise healthy. She was tired-appearing and fussy on examination with dry mucous membranes, but her examination was otherwise normal. A complete blood count with differential and serum levels of sodium, potassium, chloride, and calcium were normal, but her bicarbonate level was 12 mmol/L. Her fingerstick glucose level was 69 mg/dL. Urine dipstick was notable for large ketones, and a urine drug screen was normal. Cerebrospinal fluid examination yielded 2 white blood cells and 1040 red blood cells/mm with normal chemistries. A computed tomography (CT) scan of her head was unremarkable, and an abdominal ultrasound demonstrated no evidence of intussusception. Over the course of her hospitalization, these paroxysmal episodes persisted, and she subsequently developed mutism, right-sided weakness, and difficulty swallowing liquids. Here we present her case, diagnostic evaluation, and ultimate diagnosis. CASE HISTORY WITH SUBSPECIALTY INPUT Dr Andrew Lautz (Pediatrics, Chief Resident): A 33-month-old girl was admitted to the general pediatrics inpatient service for fever and altered mental status. During the 3 days preceding admission, she was febrile with a maximum temperature of 102°F. Her fever was accompanied by decreased activity, diminished oral intake, and limited urine output. On the night before admission, the patient’s mother noted multiple paroxysmal episodes of screaming with apparent unresponsiveness waking the child from sleep, each lasting seconds to a minute at a time. During these episodes the patient’s lower extremities were flexed to her abdomen, hands were clenched, and eyes were deviated upward. Afterward, she quickly returned to sleep. When awakened from sleep later, she seemed to be at her baseline mental status. Dr Goldberg, are there features of this history concerning for seizures? What else is in the differential diagnosis of these paroxysmal episodes? Dr Ethan Goldberg (Pediatric Neurology): These movements could be seizures. Seizures are extremely common in the pediatric age group, affecting up to 10% of children.1 As a neurologist, I am often asked about whether abnormal movements represent an epileptic seizure or a non-epileptic paroxysmal Divisions of General Pediatrics, Neurology, and Infectious Disease, The Children’s Hospital of Philadelphia, Philadelphia, Pennsylvania; Departments of Neurology and Pediatrics, The Perelman School of Medicine at the University of Pennsylvania, Philadelphia, Pennsylvania; and the Robert Wood Johnson Foundation Clinical Scholars Program, University of Pennsylvania, Philadelphia, Pennsylvania Dr Lautz contributed to the conception and design of this case presentation, drafted the initial manuscript, and revised the manuscript; Drs Jenssen, McGuire, and St. Geme III contributed to the conception and design of this case presentation and reviewed and revised the manuscript; and all authors approved the final manuscript as submitted. www.pediatrics.org/cgi/doi/10.1542/peds.2014-2405 DOI: 10.1542/peds.2014-2405 Accepted for publication Sep 8, 2014 Address correspondence to Andrew J. Lautz, MD, The Children’s Hospital of Philadelphia, 34th St and Civic Center Blvd, Room 7C26, Philadelphia, PA 19104. E-mail: [email protected] PEDIATRICS (ISSN Numbers: Print, 0031-4005; Online, 1098-4275). Copyright © 2015 by the American Academy of Pediatrics FINANCIAL DISCLOSURE: The authors have indicated they have no financial relationships relevant to this article to disclose. FUNDING: No external funding. POTENTIAL CONFLICT OF INTEREST: The authors have indicated they have no potential conflicts of interest to disclose. SPECIAL ARTICLE PEDIATRICS Volume 135, number 1, January 2015 by guest on April 5, 2017 Downloaded from movement of childhood. Fever can lower seizure threshold and can be an initial presenting trigger for epilepsy, but fever also lowers the threshold for many types of non-epileptic abnormal movements. In considering this patient, a history of eyes rolled back in the head is often present with a seizure, but this is not pathognomonic of seizures. Hands clenched into fists can be a seizure or a movement disorder but is often a response to pain or discomfort. Although this history could represent seizure activity, the child may simply be responding to pain.