SAT-LB50 Fahr’s Syndrome: A Rare Neurological Disorder Unmasked by a Psychiatric Illness

Fahr’s syndrome is a rare familial disorder characterized by abnormal accumulation of calcium deposits bilaterally at basal ganglia. It commonly affects middle-aged adults and presents with a range of neuropsychiatric symptoms. The exact prevalence of Fahr’s syndrome is uncertain; however, intracranial calcifications suggestive of this disorder are detected incidentally in approximately 0.3 % to 1.2 % of CT imaging of the brain with a prevalence of 1/1,000,000. It may be idiopathic or secondary to numerous causes dominated by phosphorous and calcium disorders, with the most common etiology being hypoparathyroidism. We report the case of a 27 years old female patient with a medical history of insulin-dependent Diabetes Mellitus type 1, Bipolar disorder, Autoimmune Polyglandular Syndrome Type 1, Thalassemia major, Primary Hypoparathyroidism and Bronchial Asthma who was admitted to the hospital after presenting an episode of dizziness, slurred speech and involuntary movements associated to hypoglycemia. The patient had a medical history of recurrent episodes of conscious self-induced hypoglycemia with double doses of insulin therapy and noncompliance with home medications. Upon evaluation, patient presents aggressive and defiant behavior. Physical and neurological examination was difficult to assess since she refused to be examined. Laboratories were remarkable for serum calcium of 6.2mg/dl, albumin of 3.5g/dl, with corrected calcium levels of 6.5mg/dl, suggestive of severe hypercalcemia. Head CT scan showed bilateral subcortical, basal ganglia clouded, thalamic, and cerebellar calcifications with preserved gray and white matter differentiation. Treatment was tailored to symptoms control and correction of underlying abnormalities. These case present the most critical features of the diagnostic criteria of Fahr’s syndrome. Pathologically, calcifications occur in the vascular walls and in the perivascular spaces of arterioles, capillaries, and veins. Clinical findings of Fahr’s syndrome vary from neurological disorder to those mimicking Bipolar disorder. In this case, there were no neurological symptoms, and this patient only presented with psychiatric manifestations suggestive of bipolar disorder. For any psychiatric condition, it is essential to rule out organic brain disorders before labeling a patient, especially one who is young and has multiple endocrinopathies which could be associated with this rare condition.