421 - Surviving Acute Hemorrhagic Leukoencephalitis and Regaining Function

Case Diagnosis: A Unique Presentation of Acute Hemorrhagic Leukoencephalitis

Case Description: A 39-year-old female with Sturge-Weber syndrome presented with two weeks of paresthesia and three days of unilateral weakness and facial droop. Her condition progressed with decreased sensation to all extremities, gait instability, confusion and impaired alertness. Five weeks after presentation, the patient was unresponsive to verbal and tactile stimuli and was withdrawing to pain only. Brain MRI showed hyperintense regions and brain biopsies showed active demyelination with microhemorrhages. The patient was admitted to acute rehabilitation and regained significant function through multidisciplinary therapy and rehab-focused medical management.

Discussions: AHLE is a rare demyelinating disease that usually results in death due to rapid increase in ICP. The majority of patients who survive are pediatric, and the disease is ⅔ more likely in men. Additionally, most cases involve rapid neurological deterioration. In adults that survive the ICP crisis, the rehabilitation process is not well-documented. Presented here is a unique case of AHLE in which a female adult patient survived an ICP crisis, underwent craniectomy, and progressed through acute rehabilitation. Challenges in rehab included drowsiness, agitation, headaches, and depression. Amantadine and citalopram displayed immense clinical benefit in alertness, attention, therapy participation, and speech.

Conclusions: AHLE is an understudied disease with complications that drastically affect a patient’s life. When creating a differential for neurological decline, it is important to consider atypical presentations of AHLE. Although most literature points to upper respiratory infection as a trigger for AHLE, this is not always the case. There is a paucity of large-scale research on the rehabilitation of AHLE patients due to the high mortality rate. Here we underline the need for more research on patients who survive AHLE and the progress they can make in rehabilitation, paving a path of recovery, with morbidity, not mortality, that we can potentially expect in surviving patients.