Objective: To report a case of progressive supranuclear palsy cerebellar type (PSP-C) with choreiform movements.

Background: PSP-C is a rare subtype of PSP characterized by predominantly cerebellar ataxia (1). PSP diagnosis remains challenging prior to autopsy due to clinical heterogeneity and lack of specific biomarkers, making atypical presentations like PSP-C particularly difficult to identify. However, measures of midbrain atrophy, including a low midbrain-to-pons ratio, may be sensitive and specific signs to differentiate PSP from other causes of parkinsonism (2).

Method: A case report

Results: A 73 year old woman with hypertension and hyperlipidemia presented with progressive incoordination and slurred speech. Exam was notable for positive applause sign, procerus sign, vertical saccade slowing, head thrusts to initiate saccades, slight to mild symmetric bradykinesia, slight rigidity bilaterally, truncal more than appendicular cerebellar ataxia, dysdiadochokinesia, and left greater than right limb and truncal choreiform movements. Serology workup including ANA, ESR, Folic Acid, Heavy metal screen, MMA, VB12, B1, Vitamin D, Vitamin E, Uric acid, Syphilis screen and movement disorder autoimmune panel were unremarkable. MRI brain showed morning glory sign and midbrain to pons ratio 0.44 without significant cerebellar atrophy. The absence of phosphorylated alpha-synuclein pathology on skin biopsy and lack of autonomic features distinguished MSA from other causes of cerebellar ataxia. While the MDS-PSP clinical criteria does not apply to PSP-C, the patient did have oculomotor dysfunction (O2), postural instability (P1) and akinesis (A2) according to MDS-PSP clinical criteria (3).

Conclusion: Progressive supranuclear palsy should be considered for patients presenting with cerebellar ataxia and midbrain atrophy. While one case report includes a patient with PSP-C with hyperkinetic movement disorder, chorea is not a common feature in PSP.

References: [1] Koga S, Josephs KA, Ogaki K, et al. Cerebellar ataxia in progressive supranuclear palsy: An autopsy study of PSP-C. Movement Disorders. 2016;31(5):653-662. doi:https://doi.org/10.1002/mds.26499
[2] Oba H, Yagishita A, Terada H, et al. New and reliable MRI diagnosis for progressive supranuclear palsy. Neurology. 2005;64(12):2050-2055. doi:https://doi.org/10.1212/01.WNL.0000165960.04422.D0
[3] Höglinger GU, Respondek G, Stamelou M, et al. Clinical diagnosis of progressive supranuclear palsy: The movement disorder society criteria. Movement Disorders. 2017;32(6):853-864. doi:https://doi.org/10.1002/mds.26987

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MDS Abstracts - https://www.mdsabstracts.org/abstract/a-case-report-of-progressive-supranuclear-palsy-with-cerebellar-ataxia-and-chorea/