Clinical Presentation of Oral Manifestations and Intraoral Somatosensory Changes in Fahr’s Disease

Abstract

Fahr’s disease is a rare congenital disorder characterized by abnormal calcium deposition with subsequent atrophy involving the basal ganglia, cerebral and cerebellar cortical regions. Very little information is available regarding non-neurological manifestations of this disease, and almost no information is available on oral findings. Moreover, information is unavailable regarding intraoral somatosensory changes in Fahr’s disease. We report oral findings and intraoral somatosensory changes in patient with Fahr’s disease. A 62-year-old Japanese woman was referred to the Department of Oral and Maxillofacial Surgery at Tokyo Women’s Medical University Hospital with symptoms of bleeding gums. The patient had been diagnosed with Fahr’s disease by the Department of Psychiatry 1 year earlier. Intraoral examination showed poor oral and dental hygiene with gingival hyperplasia on the buccal aspects of the upper and lower incisors. Generalized attrition of the teeth was seen. Panoramic radiography showed horizontal bone resorption, but no evidence of congenital absence of any teeth. Gingival bleeding associated with poor periodontal condition was diagnosed. As an additional symptom, the patient reported intraoral burning sensation of the tongue. Qualitative sensory testing was performed for the tongue, upper and lower gingiva and lip mucosa, showing heat and cold hyperalgesia of the tongue. Mechanical allodynia located in the upper and lower lip mucosa was also reported. In accordance with these clinical findings, we diagnosed primary and/or secondary burning mouth syndrome related to Fahr’s disease. This case represents only the second instance for which intraoral findings of Fahr’s disease have been reported. Somatosensory changes were also found to be associated with the present case of Fahr’s disease. Intraoral somatosensory changes related to Fahr’s disease may be due to progressive lesions associated with this disease. Continuous follow-up and qualitative sensory testing to assess disease progression may be needed for clarification of these issues.