Case submitted by Caroline Oehlerich PA-C
Children’s National Hospital
History
6-year-old female with no significant past medical history who presents with 2 days of lower back pain. Pain is described as aching and non-radiating. Pain awakens her from sleep. No improvement despite Motrin. She was triaged by her PMD and advised to see care for further evaluation of possible UTI.
Mother notes urinary two days of urgency and frequency. No fever, dysuria, or hematuria. No prior history of UTIs. No abdominal pain. No diarrhea. She had two episodes of emesis following attempts at drinking water in the last 24 hours. She has voided at least three times in the last 24 hours. Her last bowel movement was 2 to 3 days prior and described as soft. No history of constipation.
Mother notes that her gait appears unsteady which she attributes to lower back pain. On further questioning, mother reports that she is frequently tripping over her own feet which is abnormal for her. At her baseline, she is playful and able to run, jump, skip and climb up stairs without difficulty. No headache. No neck pain/stiffness. No numbness or tingling. No change in speech. No change in mental status. No weight loss, night sweats, or chills. No known trauma to the back. No recent head trauma. Mother notes that patient’s classmates were sick with flu-like symptoms 3 weeks ago; the patient had fatigue at that time but no fever or URI symptoms.
Past medical, family, surgical, and social history reviewed and non-contributory. No chronic medications. No known allergies. Immunizations up to date.
Physical with vital signs
- T 36.7, HR 90, BP 120/82, SpO2 97% on RA
- General: Alert. appropriate for age. Cooperative. smiling. interacting.
- Skin: Warm. dry. intact. No rash on limited skin exam.
- Head: Normocephalic. atraumatic.
- Neck: Supple. trachea midline. no tenderness. no lymphadenopathy. Full range of motion, no stiffness.
- Eye: Pupils are equal, round and reactive to light. extraocular movements are intact. normal conjunctiva. no discharge. no jaundice. vision grossly normal.
- Ears, nose, mouth and throat: Tympanic membranes clear. Oral mucosa moist. No pharyngeal erythema or exudate.
- Cardiovascular: Regular rate and rhythm. No murmur. No gallop. Normal peripheral perfusion. Extremity pulses equal.
- Respiratory: Lungs are clear to auscultation. respirations are non-labored. breath sounds are equal. Symmetrical chest wall expansion.
- Gastrointestinal: Soft. Non distended. Normal bowel sounds. No organomegaly. Slightly tender to deep palpation in the right lower quadrant, no rebound tenderness or guarding, questionable palpable stool burden.
- Genitourinary: Normal genitalia for age. no tenderness. no discharge. no lesions.
- Back: Normal alignment. no step-offs. No tenderness to palpation over the spinous processes. Range of motion limited secondary to pain.
- Musculoskeletal: Normal ROM. no swelling. no deformity. moves all extremities.
Neurological
Alert. CN II-XII intact. No nystagmus. Normal speech observed. developmentally normal. Gait is abnormal; she appears hunched over at the waist and ataxic. Strength 5/5 in her upper and lower extremities bilaterally. Sensation is grossly intact to light touch throughout; did not assess for a sensory level. She is hyperreflexic (3+) at the patellar tendons. She has several beats of clonus at both ankles, right greater than left, and associated with thigh adduction (R more so than L). Reflexes of the upper extremities appear normal. No saddle anesthesia.
Psychiatric
Cooperative. appropriate mood & affect.
Urgent Care work up (labs, images, meds etc)
Patient transferred to Children’s ED for emergent neuroimaging given abnormal neurologic exam. CT brain without contrast showed regional hypoattenuation centered at splenium of corpus callosum, also involving immediately adjacent posterior thalami, concerning for toxic, metabolic, infectious, inflammatory, or post-infectious demyelinating process. CT lumbar spine with contrast was unremarkable except for bladder distension. CBC notable for leukocytosis (13), otherwise unremarkable. ESR, CRP, and CMP were within normal limits.
Brief one liner about disposition (admitted, transferred, discharged home with referral)
Patient admitted to the Neurology service for further evaluation, including MRI brain/spine, MRV of the head, and lumbar puncture. Studies were consistent with MOGAD, ADEM-ON phenotype. She had near-complete resolution of symptoms after a 5-day course of pulse-dose steroids.
Ultimate Diagnosis
Myelin oligodendrocyte glycoprotein antibody disease (MOGAD), acute disseminated encephalomyelitis-optic neuritis (ADEM-ON) phenotype.
At least 2-3 learning pearls about the diagnosis
- Evaluation of back pain in pediatric patients should include a comprehensive neurologic exam, including assessment of deep-tendon reflexes and gait.
- Initial symptoms of ADEM may occur 2-3 weeks after infection or vaccination. In addition to encephalopathy, the most common neurologic features of ADEM include pyramidal tract signs, acute hemiparesis, cerebellar ataxia, cranial neuropathies including optic neuritis, and spinal cord dysfunction.
- The differential diagnosis for ADEM is broad, and diagnosis is based on clinical and radiological findings and exclusion of mimickers, such as viral or bacterial meningoencephalitis/encephalitis, neoplasms, and other immune-mediated or inflammatory processes.
*Source: https://www.annchildneurol.org/journal/view.php?doi=10.26815/acn.2022.00220
