Disclosures: Matthew A. Erby, MD: Nothing to disclose

Case Description: A 59-year-old female patient with medical history of type II diabetes presented with recurrent seizures, fluctuating mental status, and hallucinations over the past 6 months requiring multiple hospitalizations. Workup revealed elevated thyroperoxidase and thyroglobulin antibodies. A steroid course was initiated for suspected Steroid Responsive Encephalopathy Associated with Autoimmune Thyroiditis (SREAT) and her level of arousal and motor strength improved, but she continued to have significant cognitive deficits.

Setting: Acute inpatient rehabilitation unit of a metropolitan hospital

Patient: 59-year-old female with SREAT syndrome.

Assessment/Results: Goal directed therapy with physical, occupational, and speech therapy in acute inpatient rehabilitation focused on improving cognition and functional independence. Cognitive evaluation and treatment was performed using the Boston Naming Test, the Cognitive-Linguistic Quick Test, and Scales of Cognitive Ability for Brain Injury. Patient improved from “standby assistance” to “modified independence” in speech-language and cognitive abilities during her ten-day inpatient stay, meeting all goals for delayed recall, thought processing, and organization. She remained without evidence of regression or relapse of the disease.

Discussion: SREAT syndrome typically presents with relapsing and remitting seizures, altered mental status, weakness, and myoclonus. SREAT syndrome is rare, with estimated prevalence of 2:100,000, and thus the role for cognitive rehabilitation is still relatively undefined in this population. Given the severity of the disease and high frequency of relapse, 16% of affected patients, it is critical to provide interventions that can improve recovery and reduce relapse rates. Cognitive therapy focused on maintaining cognitive strategies in a multidisciplinary acute rehabilitation setting resulted in a good outcome in this patient.

Conclusion: Intensive cognitive rehabilitation appears to play a role in executive function recovery in patients with SREAT syndrome. This successful outcome suggests a potentially vital role for cognitive therapy in addition to physical and occupational therapy in acute inpatient rehabilitation for restoration of functional independence in patients with SREAT syndrome.

Level of Evidence: Level V

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PM&R Meeting Abstracts - https://pmrjabstracts.org/abstract/the-role-of-acute-rehabilitation-in-a-patient-with-a-rare-neurocognitive-disease-a-case-report/