1626 - Memory in Motion: Rehabilitation of Wernicke's Encephalopathy in a Patient with Underlying McArdle’s Disease- A Case Report

A 29-year-old male with McArdle's disease and anxiety/depression presented to the emergency department for double vision, agitation, restlessness, and confusion after starting venlafaxine. He was admitted to the ICU for serotonin syndrome versus drug reaction and treated with IV benzodiazepines. Due to persistent encephalopathy, MRI brain was pursued and showed bilateral thalamic lesions suggestive of Wernicke’s encephalopathy and was treated with high dose IV thiamine. Of note, he did not have a history of alcohol use, therefore his only known risk factor was his underlying glycogen storage disease (GSD) and recent illness. Hospitalization was complicated by critical illness myopathy, resulting in severe weakness and muscle stiffness. He was admitted to inpatient rehab and underwent comprehensive rehabilitation, focusing on addressing his muscle stiffness, weakness, exercise intolerance, diplopia, and cognitive impairments.  

Discussions:

This report highlights rehabilitation considerations in a case of critical illness myopathy and Wernicke’s encephalopathy (WE) in the setting of underlying GSD. In GSD, enzyme deficiencies impair glycogen breakdown. This results in inadequate glucose levels which become exaggerated during periods of increased metabolic demand, leading to complications such as muscle atrophy, rhabdomyolysis, and myopathies. Rehabilitation should focus on early mobilization to prevent contractures, step-wise approach to restore muscle function, and optimization of nutrition and pain. Patients with WE pose unique challenges in rehab due to severe cognitive impairments, especially memory disturbances that ensue. This patient required a multidisciplinary team to address his impairments.

Conclusions:

GSD’s are rare and lead to debilitating complications while proposing difficulties in rehabilitation, especially when diagnosed with concurrent neurologic disorders such as WE. Physiatrists should be aware of the underlying complexities associated with GSD’s in order to address both the physical and cognitive challenges that these patients may face.