P2A287: Acute Disseminated Encephalomyelitis Presentation with Penile Pain and Acute Urinary Retention: A Case Report

Introduction: Acute disseminated encephalomyelitis (ADEM) is rare in childhood. It is a demyelinating disease of the central nervous system that appears to result from a cell-mediated delayed hypersensitivity reaction and primarily managed with high-dose corticosteroids. The classic presentation of this disease is meningoencephalitis. We present a unique pediatric case where penile pain and acute urinary retention were the initial presenting symptoms.

Case Description: 4-year-old male with no significant past medical history presented with penile pain and acute urinary retention of one-day duration following a five-day history of cough, congestion, and rhinorrhea. On examination, he exhibited irritability with palpation of the head and shaft of the penis. 320 mL of retained urine was detected on subsequent bladder scan. Straight catheter placement resulted in return of normal urine without resistance, followed by immediate relief of pain. Pertinent preliminary labs included: procalcitonin of 0.51 ng/mL, leukocytosis of 23.3 10*9/L, blood and urine cultures with no growth, and urinalysis with trace ketones and small blood. Shortly after catheterization, bilateral lower extremity pain with refusal to bear weight, irritability, listlessness, and continued urinary retention were noted. Magnetic Resonance Imaging (MRI) of the brain and spine with and without contrast demonstrated scattered supratentorial and infratentorial signal abnormalities and hyper-intense signal abnormalities throughout the spinal cord (Figures 1 and 2, respectively). Pertinent subsequent labs included: positive serum myelin oligodendrocyte glycoprotein immunoglobulin and negative cerebral spinal fluid (CSF) studies. Treatment with a five-day course of intravenous (IV) methylprednisolone and two doses of IV immunoglobulin (IVIG) were initiated at the recommendation of pediatric neurology. Retinal exam performed by ophthalmology was unremarkable. Neurologic symptoms, including penile pain secondary to urinary retention, significantly improved after treatment. Following completion of the corticosteroid and IVIG course, the patient was discharged to an inpatient rehabilitation facility with return to his neurologic baseline.

Discussion: ADEM typically presents with symptoms of meningoencephalitis. Penile pain and urinary retention are unusual presenting signs. While supportive techniques, such as straight catheterization, were initially performed for symptomatic relief, MRI and CSF studies were ordered immediately following neurologic deterioration on physical exam, confirming the diagnosis of ADEM. Rapid recognition of these acute changes is imperative for initiation of life-saving intervention.

Conclusion: This case highlights that atypical symptoms, such as penile pain and acute urinary retention, can constitute the initial presentation of ADEM. We also emphasize the importance of immediate further work-up for these patients, especially if the symptoms are persistent or accompanied by signs of early neurologic deterioration.