Disclosures: Mirella Fernandez: No financial relationships or conflicts of interest

Case Diagnosis: Cerebellar ataxia

Case Description or Program Description: A 46-year-old male with Hashimato’s disease (HD) developed sudden onset dysarthria and ataxic gait. On neurologic exam, he had dysarthria, wide-based ataxic gait, dysmetria, absent postural responses, and grossly positive Romberg. Structural brain MRI revealed diffuse, severe cerebellar atrophy. He was found to have highly elevated thyroperoxidase antibody. Based on clinical, laboratory and imaging findings he was diagnosed with Hashimato’s encephalopathy and was treated with IV Solumedrol. However due to loss of Purkinje cells he developed permanent cerebellar ataxia.

Setting: Outpatient

Assessment/Results: Custom rehabilitation intervention was designed to improve motor control. Intervention was provided twice a week for 12 weeks. Sessions consisted of repetitive but increasingly challenging dynamic activities, each designed to challenge postural stability and multi-joint coordination. Pre and post-assessment of the following measures were obtained: disease severity was performed by using the International Cooperative Ataxia Rating Scale (ICARS); gait analysis (GAITRite®); posturography (Smart Equitest NeuroCom System)., and brain Magnetic Resonance Imaging (MRI) (anatomical brain segmentations used FreeSurfer v.6.3.0 analysis of T1-weighted MPRAGE images) In ICARS assessment his score improved 22 points(53.6%).Total Modified Dynamic Gait Index improved 4 points with increased velocity and step length, decreased base of support and toeing out. Limits of stability improved in reaction time, movement velocity and excursions. He also increased frontal and sagittal weight shifting speed on the Rhythmic Weight Shifting Test. Pre and post-intervention brain MRI’s obtained. Anatomical brain segmentations were done using FreeSurfer v.6.3.0 analysis of the T1-weighted MPRAGE images which showed 1.7% increase in total gray matter and 55% increase in cerebellar white matter.

Discussion (relevance): Our results highlight the importance of the custom rehabilitation intervention in a cerebellar ataxia patient who made relevant functional improvements that may be associated with specific patterns of structural brain plasticity.

Conclusions: This case report aims to draw attention to this rare but devastating neurological manifestation of HD and to highlight the potential functional recovery associated with remodeling of brain structures promoted by intense customized rehabilitation utilizing the methods to activate relearning of motor skills.

Level of Evidence: Level IV

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PM&R Meeting Abstracts - https://pmrjabstracts.org/abstract/structural-brain-plasticity-in-cerebellar-ataxia-induced-by-balance-training/