(822) Acute Disseminated Encephalomyelitis From Invasive Group A Streptococcal Infection

Introduction: Group A streptococcal (GAS) infections are known as some of the most frequent bacterial infections during childhood, often causing local infections. However, the infection can progress to invasive disease, such as scarlet fever, streptococcal toxic shock syndrome (STSS), necrotizing fasciitis (NF), and rarely acute disseminated encephalomyelitis (ADEM). With a recent surge in GAS infections worldwide, the risk of increased incidence of invasive GAS infections also rises. ADEM is an inflammatory demyelinating disease of the central nervous system that is both difficult to recognize, and therefore treat. As invasive GAS infections increase, ADEM poses a unique challenge for providers for rapid diagnosis and treatment to improve outcomes.

Description: An 8-year-old previously healthy male was admitted with depressed mental status, fever, GAS pharyngitis, and mild hyponatremia. He developed new-onset generalized tonic-clonic seizures leading to acute hypoxemic hypercarbic respiratory failure requiring tracheal intubation. Seizures were managed with levetiracetam and hyponatremia correction. Sepsis workup revealed GAS bacteremia, with normal spinal fluid analysis. Head CT was normal, and MRI of the brain and spine showed diffusion restriction in the cerebellum, brainstem, and in the upper cervical spinal cord with demyelinating lesions in the cervical cord consistent with ADEM. He was treated with ceftriaxone for GAS sepsis causing bacteremia and with high-dose steroids for ADEM. Upon tracheal extubation, he demonstrated significant myopathy and swallowing dysfunction requiring intensive inpatient rehabilitation.

Discussion: With a general increase in invasive GAS infections worldwide, it is imperative that the clinician have a low suspicion for infection complications such as ADEM. Although this is an incredibly rare presentation of invasive GAS, if left untreated it could lead to permanent neurologic deficits.