SUN-520 Brain Calcinosis Syndrome Manifesting as Cerebellar Ataxia in a Chronic Hypoparathyroid Patient

Abstract

BACKGROUND Chronic hypoparathyroidism in adults is usually secondary to previous thyroid surgery and is characterized by low calcuim and inappropriately low circulating PTH levels. The clinical presentation of hypoparathyroidism can vary with the serum calcium levels and chronicity of hypocalcemia. Hypoparathyroidism can cause intracranial calcifications but extensive intracranial calcifications are rare. Brain calcinosis syndrome is defined as bilateral calcium accumulation in the brain parenchyma, most often within the basal ganglia. Such intracranial calcifications occur in 0.3-1.5% of patients with hypoparathyroidism, often detected incidentally. The mechanism of intracranial calcification is not completely understood. Its occurrence with hypocalcemia suggests that increased calcium-phosphorus complex formation plays a role. THE CASE A 63-year old female was admitted due to dizziness. She had a fall prior confinement due to loss of consciousness lasting for less than 5 minutes, however, recovered afterwards. No head injury was detected at the referring hospital. She is hypertensive with good BP control on Amlodipine 2.5 mg and Losartan 50 mg taken once daily. She had total thyroidectomy for colloid adenoma 20 years ago, prescribed with Levothyroxine 100 mcg once daily but taken with poor compliance. She had episodes of cold intolerance, hoarseness, and weight gain. On examination, she was conscious and coherent. Blood pressure was 95/60 mm Hg, heart rate of 67 beats per minute. A midline neck scar from previous surgery was visible, no palpable neck mass or lymph nodes. Notable on the neurological examination were positive Rhomberg test and dysmetria evaluated by finger-to-nose test. Chvostek’s and Trosseau’s signs were negative. Motor, sensory and cranial nerve functions were intact. Plain cranial CT scan showed extensive cranial calcifications on both basal ganglia, corona radiata, brainstem and cerebellum. Blood tests revealed low levels of ionized calcium (0.91 mmol/L; N 1.12-1.32 mmol/L)), intact PTH (0.51 pg/ml; N 15-65 pg/ml), and FT4 (5.54 pmol/L; N 12-22 pmol/L). TSH was elevated (32.85 uIU/ml; N 0.27- 4.2 uIU/ml). Calcium with active vitamin D and levothyroxine were given. Physical rehabilitation initiated. Patient was eventually discharged improved, with decreased dizziness. CONCLUSION Basal ganglia calcifications present with diverse manifestations and include seizures, mental deterioration and disorders of the cerebellar or extrapyramidal function. Treatment usually involves calcium and vitamin D supplementation. Marked clinical improvement usually results from treatment of hypoparathyroidism. Measurement and monitoring of serum calcium, phosphorus and intact PTH is warranted. REFERENCE: Ramen C. Basak. A Case Report of Basal Ganglia Calcification - A Rare Finding of Hypoparathyroidism. Oman Med J. 2009 Jul; 24(3): 220-222.