Prosthodontic rehabilitation in Fahr's syndrome: clinical challenges and oral manifestations

Fahr's syndrome, sometimes referred to as primary familial brain calcification, is an uncommon neurodegenerative disease that is typified by bilateral, symmetrical intracranial calcifications that primarily affect the cerebral cortex, thalamus, dentate nuclei, and basal ganglia. The condition is often associated with movement disorders, seizures, neuropsychiatric symptoms, and cognitive decline. However, it can sometimes go unnoticed and be discovered by accident when imaging for unrelated conditions. Early symptoms can be detected in non-neurological settings, even though neurologists deal with them most of the time. This highlights the importance of interdisciplinary vigilance in healthcare. Dental professionals, particularly prosthodontists, may occasionally experience systemic manifestations during routine diagnostic evaluations, emphasizing the need for a comprehensive clinical approach, including movement disorders and neuropsychiatric conditions.

A 39-year-old woman presented at the prosthodontics department of KGMU, complaining of difficulty chewing food due to missing posterior teeth. Her medical history included longstanding bilateral sensorineural hearing loss and epilepsy managed with antiepileptic medications for over 12 years. The results of a routine pre-prosthetic evaluation, which included panoramic radiography, raised the possibility of abnormal intracranial calcification. Additional evaluation using cranial computed tomography (CT) confirmed bilateral, symmetrical calcifications in the thalamus, subcortical white matter, and basal ganglia that resembled Fahr's syndrome. The patient was referred to the Department of Neurology for further assessment. In order to rule out secondary causes of calcification, other biochemical tests, such as serum calcium level, phosphate, parathyroid hormone, and vitamin D levels, were all within normal ranges. It was determined that idiopathic Fahr's syndrome was the cause. Since the condition is progressive, the patient was recommended to have frequent follow-ups even though there were no additional neurological symptoms. Potential neurological effects were closely monitored during the dental rehabilitation process.

In this case, Fahr's syndrome was accidentally discovered. This case underlines the importance of vigilance in dental practice, where incidental findings may lead to early diagnosis of systemic neurological conditions such as Fahr's syndrome.