Objective: To educate readers about a unique presentation of hemidystonia-hemiatrophy syndrome which presented after an infection with COVID-19

Background: Hemidystonia-hemiatrophy syndrome is a rare disorder that can present years or even decades after a cerebral insult to the basal ganglia.

Method: Case study with images

Results: A 25-year-old right-handed female presented to clinic with a 2-year history of involuntary posturing of her right arm and leg. She reported that she had a normal birth and development and had no medical issues or difficulty using the right side of her body prior to 2 years ago. In November 2020, she contracted COVID-19. Over the next few weeks, she noticed that her right foot would turn inward, and her first toe would extend involuntarily. Over the next several months, the posturing in her leg worsened and spread to involve her arm with flexion of her fingers, wrist, and elbow that was worse with voluntary movement.  

Multiple neurologists evaluated her before she was seen in our clinic. MRI brain without contrast in 2021 and repeat MRI brain with and without contrast and MRA brain/carotids in 2022 were unremarkable per the radiology reports. At the time of our evaluation, her symptoms had been stable for at least one year. She failed trihexyphenidyl, baclofen, tizanidine, levodopa, and neurotoxin injections due to lack of efficacy and/or side effects. The examination was significant for right hemidystonia involving the arm and leg and slightly decreased size of her right hand compared to the left. Upon our review of the previously completed MRIs, she had atrophy of the left caudate and putamen. DaTscan showed essentially absent activity of the left corpus striatum.

Conclusion: We believe her unusual presentation of hemidystonia-hemiatrophy was most consistent with a peripartum insult to her left basal ganglia that she compensated for until she developed COVID-19 infection, which resulted in recrudescence of the prior insult.

MRI Brain depicting atrophy of the left striatum

DaTscan with absent activity in the left striatum

Photo of patients’ hands

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MDS Abstracts - https://www.mdsabstracts.org/abstract/recrudescence-of-a-compensated-hemidystonia-hemiatrophy-syndrome-after-covid-19-infection/