Case Report
A Rare Association of Fahr’s Syndrome and Hashimoto’s Encephalopathy
Manoj Prakash Jeyaseelan*, Shobhana N, Sadeesh Kumar V, Selvakumar CJ and Guru S
Department of Neurology, Coimbatore govt medical college and Hospital, Tamil Nadu, India.
*Corresponding author: Manoj Prakash Jeyaseelan, Department of Neurology, Coimbatore govt medical college and Hospital, Tamil Nadu, India. E-mail Id: jmprakas@gmail.com
Article Information:Submission: 29/09/2025; Accepted: 16/10/2025; Published: 18/10/2025
Copyright: © 2025 Manoj Prakash Jeyaseelan, et al. This is an open access article distributed under the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium,
provided the original work is properly cited.
Abstract
Background: Steroid Responsive Encephalopathy Associated with Autoimmune Thyroiditis (SREAT), also known as Hashimoto encephalopathy, is a rare but treatable cause of rapidly progressive dementia. Coexistence with Fahr’s syndrome, an idiopathic bilateral basal ganglia calcification, is
exceptionally uncommon and poses a diagnostic challenge.
Case Presentation: We report a 70-year-old female who presented with a 6-month history of insidious onset behavioral changes, psychosis, cognitive decline, and movement abnormalities including bilateral rest tremors. Laboratory investigations revealed elevated anti-thyroid peroxidase (anti-TPO) antibodies, thyroiditis on ultrasound, elevated parathormone, and vitamin D deficiency. Neuroimaging showed coarse bilateral calcifications in the basal ganglia, dentate nuclei, and occipital gyri consistent with Fahr’s syndrome. Serum autoimmune encephalitis markers were negative. The patient responded dramatically to intravenous pulse steroids and rituximab, with marked clinical improvement and cognitive recovery.
Conclusion: This case highlights the importance of considering SREAT in rapidly progressive dementia even when imaging shows typical Fahr’s syndrome calcifications. Early recognition and immunosuppressive treatment can lead to significant clinical improvement in this potentially reversible disorder, underscoring the need for vigilance in atypical presentations.
Case Presentation: We report a 70-year-old female who presented with a 6-month history of insidious onset behavioral changes, psychosis, cognitive decline, and movement abnormalities including bilateral rest tremors. Laboratory investigations revealed elevated anti-thyroid peroxidase (anti-TPO) antibodies, thyroiditis on ultrasound, elevated parathormone, and vitamin D deficiency. Neuroimaging showed coarse bilateral calcifications in the basal ganglia, dentate nuclei, and occipital gyri consistent with Fahr’s syndrome. Serum autoimmune encephalitis markers were negative. The patient responded dramatically to intravenous pulse steroids and rituximab, with marked clinical improvement and cognitive recovery.
Conclusion: This case highlights the importance of considering SREAT in rapidly progressive dementia even when imaging shows typical Fahr’s syndrome calcifications. Early recognition and immunosuppressive treatment can lead to significant clinical improvement in this potentially reversible disorder, underscoring the need for vigilance in atypical presentations.
Keywords:SREAT; Rapidly progressive dementia; Fahrs syndrome