Objective:

A case report of Burning Mouth Syndrome (BMS) as the initial presentation of Idiopathic Basal Ganglia Calcification (IBGC). The purpose is to enable a relatively new potential IBGC symptom, aiding diagnoses and broadening its clinical spectrum.  

Background:

IBGC is a rare condition that is associated with abnormal calcium deposition in basal ganglia, cerebral, and cerebellar cortical regions (1). IBGC is either familial or sporadic and presents with a wide range of clinical signs, including progressive mental deterioration of pyramidal, extra-pyramidal, and cerebellar signs. Limited information is available regarding the non-neurological manifestations of this disease, especially oral symptoms (2).  Characteristic intra-oral features include missing teeth, tooth mobility, gingival inflammation, and recession with associated periodontitis. However, only a few cases have been reported about intraoral somatosensory manifestations, including BMS (1).

Design/Methods:

Case report and literature review.

Results:

A 75-year-old patient with a past medical history of hypothyroidism, and restless leg syndrome presented to the Neurology clinic for a burning sensation of the tongue for six months. The patient reported gradual cognitive decline along with chronic penile pain. CT brain and MRI brain WO revealed areas of mineralization in bilateral periventricular regions, basal ganglia, and cerebellum. Serum parathyroid hormone, thyroid hormone, and ionized calcium were within normal limits. Somatosensory evoked potential testing for the trigeminal nerve was abnormal, showing bilateral conduction slowing. The patient has been advised Gabapentin (clonazepam was associated with side effects) and formal neuropsychological testing for cognitive changes.

Conclusions:

BMS has rarely been reported as a presenting feature of IBGC.  Calcium deposits in pain fibers are debated as causative, but the role of basal ganglia/thalamic nuclei calcifications and striatal dopamine systems failure remains unclear. This case report enhances IBGC understanding, stimulating further research to grasp the connection between these unique pain syndromes and basal ganglia calcification to develop advanced therapeutic options.