Susac Syndrome in an ICU Patient With History of Methamphetamine Use

Introduction: Susac syndrome is an extremely rare neurological condition characterized by a triad of symptoms that include branched retinal artery occlusion (BRAO), hearing loss, and encephalopathy. The underlying pathology is hypothesized to be an immune-mediated endothelial injury creating microinfarcts. This pathology is often seen in young patients with a history of drug abuse.


Description: The patient is a 32-year-old male with history of methamphetamine use admitted for acute encephalopathy. During hospital admission, he was upgraded to ICU level of care for worsening encephalopathy and oxygen requirement requiring intubation. His Glasgow Coma Scale was noted to be 3, and he was comatose with marked posturing in response to painful stimuli. Initial CT imaging showed hypoattenuation of the corpus callosum, and subsequent MRI noted punctate lesions in the central corpus callosum, pathognomonic for Susac syndrome.

The patient was determined to have Susac syndrome due to the combination of imaging findings, ongoing encephalopathy, and BRAO on fundoscopy. The patient was administered IV steroids, IVIG, and later Rituximab infusion. Rituximab was chosen over cyclophosphamide to minimize further immunosuppression in the setting of persistent respiratory infection. The patient also underwent tracheostomy placement for persistent ventilator dependence. The admission course was further complicated by septic shock and hydropneumothorax requiring chest tube placement. Two months after admission, the patient remains in a poor albeit improved neurologic state. He remains nonverbal but now spontaneously opens eyes and moves all extremities. Mental status continues to limit formal hearing assessment. Ultimate prognosis remains undetermined.


Discussion: The case highlights the challenges of diagnosis and management of Susac syndrome. Susac Syndrome in the ICU setting is rarely reported in medical literature, and recommendations regarding the management of Susac syndrome in the intensive care setting are even more scarce. Furthermore, the case underscores the challenges of initiating immunomodulators for Susac syndrome patients in the ICU setting. Early collaboration with a multidisciplinary team, especially with specialties such as neurology and ophthalmology, is essential for maximizing patient outcomes.