Late-onset Parry-Romberg Syndrome with atypical neurological manifestations: A case report

Sema Akkus; Suban Amatya; Kriti Shrestha; Shitiz Sriwastava; Demetrios A Karides

doi:10.1016/j.radcr.2023.10.032

Late-onset Parry-Romberg Syndrome with atypical neurological manifestations: A case report

Radiol Case Rep. 2023 Nov 17;19(1):459-463. doi: 10.1016/j.radcr.2023.10.032. eCollection 2024 Jan.

Authors

Sema Akkus¹, Suban Amatya², Kriti Shrestha², Shitiz Sriwastava³, Demetrios A Karides⁴

Affiliations

¹ Department of Neurology, Icahn School of Medicine at Mount Sinai, New York, NY, USA.
² Department of Medicine, Patan Academy of Health Sciences, Lalitpur, Nepal.
³ Division of Multiple Sclerosis and Neuroimmunology, Department of Neurology, McGovern Medical School (UT Health), University of Texas Health Science Center at Houston, Houston, TX, USA.
⁴ Department of Neurology, Mount Sinai Health System, New York, NY, USA.

Abstract

Parry-Romberg Syndrome (PRS) is a rare neurocutaneous disorder characterized by gradual facial hemiatrophy. We present a case study of a 64-year-old woman with late-onset PRS and linear scleroderma. The patient exhibited atypical PRS symptoms including leg numbness, hyper-reflexia, trigeminal neuralgia, and severe headaches. Diagnostic evaluations revealed chronic left-sided cerebral infarction, microhemorrhages, and nerve involvement. Treatment options for PRS are limited and aim to manage symptoms. This case highlights the diagnostic challenges of late-onset PRS, emphasizing interdisciplinary approach. Further research and improved therapies are essential for better patient outcomes.

Keywords: Atypical manifestations; Brain MRI; Late-onset; Neuroimaging; Parry-Romberg syndrome; Progressive facial hemiatrophy.

Publication types

Case Reports