Acute Onset of Severe Psychosis and Paranoia in a Patient with Congenital Hypoparathyroidism and Basal Ganglia Calcification

We report a rare case of congenital hypoparathyroidism presenting with severe psychosis and paranoia, found to have basal ganglia calcification in the computer tomography (CT) scan. The symptoms resolved after electrolyte replacement without any antipsychotic medications.

Case Description :

A 63-year-old woman with a past medical history of congenital hypoparathyroidism presented with paranoia and severe psychosis symptoms. Further history revealed that several days before this presentation, the patient stopped taking vitamin D and calcium supplements because of gastrointestinal disturbance. Vital signs upon admission were normal. Upon physical examination, she was severely psychotic, had paranoid thoughts, and had brisk deep tendon reflexes. The serum calcium level was 5.6 mg/dL (reference range, 8.0 to 10.6 mg/dL) and the phosphorus level was 7.6 mg/dL (reference range, 2.3 to 5.0 mg/dL). Serum intact parathyroid hormone was undetectable. Infectious, other metabolic abnormalities, and recreational drug use were ruled-out as a potential reason for the acute mental status change.  Because of the new acute onset of psychosis, CT of the head was done and revealed symmetrical calcifications in bilateral basal ganglia and thalamus.

Fluid and electrolytes were replaced appropriately, and calcium and calcitriol were prescribed. The patient’s psychosis improved significantly after the electrolytes were corrected. The patient recovered and was discharged from the hospital on calcium supplementation and calcitriol. Follow-up appointments were scheduled with an endocrinologist and a psychiatrist.

Discussion :

Idiopathic basal ganglia calcification is a rare disorder, known as Fahr’s disease. The prevalence of Fahr's disease is 1: 1.000.000. The clear etiology and pathophysiology of this disease are not understood. Chronic congenital hypoparathyroidism with an electrolyte abnormality can be proposed as one of the underlying pathophysiologic mechanisms of basal ganglia calcification. Basal ganglia calcifications usually manifest with multiple nonspecific neuropsychiatric symptoms, such as cerebellar ataxia, seizure, insomnia, depression, psychosis, Parkinson-like symptoms, etc.
This case emphasizes the importance of brain imaging for any acute onset of neuropsychiatric symptoms in a patient with congenital hypoparathyroidism with disrupted phosphocalcic metabolism to rule out or confirm underlying possible brain calcification. Prompt diagnosis and early electrolyte replacement may help avoid prescribing antipsychotics.