Session Title: Neurological Rehabilitation Case and Research Report
Session Time: 11:15am-12:45pm
Location: Research Hub - Kiosk 5
Disclosures: Emily R. Marquez, MD: Nothing to disclose
Case Description: The patient was admitted to an inpatient brain injury unit following recurrent falls that had resulted in a right subdural hematoma, left frontal contusion and intraparenchymal hemorrhage. The patient did not progress in therapies as expected. He was limited by profound orthostasis, refractory to supportive and pharmacologic interventions. In addition, he had other signs of autonomic dysfunction including urinary retention and his neurologic exam demonstrated anterocollis, dysarthria, and ataxia. This presentation was not consistent with traumatic encephalopathy, prompting investigation with MRI. It demonstrated pronounced volume loss at the pons and cerebellum, with cross-like regions of T2 hyper-intense signal in the pons (“hot cross bun sign”) suggestive of Multi System Atrophy (MSA,) a neurodegenerative disorder. Further history obtained from family confirmed that the patient had demonstrated signs of a progressive functional decline over the past few years. The remainder of the patient’s inpatient rehabilitation course focused on educating the patient and family on his new diagnosis, symptomatic management, and targeted therapies for identified impairments with notable improvement in functional status achieved by discharge.
Setting: Inpatient brain injury unit
Patient: 76-year-old male with Multi System Atrophy
Assessment/Results: The patient was discharged home with supportive family. He continued to require increasing levels of care due to the progressive nature of the disease.
Discussion: MSA has a reported annual incidence of 3 per 100,000 and is characterized by autonomic dysfunction. It is further sub-categorized into cerebellar and Parkinsonian variants. Diagnosis is made on the basis of clinical presentation and supported by imaging findings. Historically, neither variant responds to dopaminergic medications. Treatment for MSA is supportive with the goal of maintaining functional independence.
Conclusion: MSA is a rare neurodegenerative disease that presents with a unique constellation of symptoms and imaging findings. It is pivotal to consider among elderly patients presenting with atypical presentation of traumatic encephalopathy.
Level of Evidence: Level V
To cite this abstract in AMA style:Marquez ER, Driver SP. The Clue of the Hot Cross Bun: A Case Report [abstract]. PM R. 2019; 11(S2)(suppl 2). https://pmrjabstracts.org/abstract/the-clue-of-the-hot-cross-bun-a-case-report/. Accessed September 24, 2023.
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