Pradeep Sreekumara, Calwin David Singha, K Umakanthana
a. Department of Medicine, Coimbatore Medical College, Coimbatore

Corresponding Author: Dr. Pradeep Sreekumar, Junior Resident, Department of Medicine, Coimbatore Medical College, Coimbatore. Phone: 09994361627. Email: pradeepsreekumar@gmail.com

ABSTRACT

Idiopathic hypoparathyroidism rarely presents with choreoathetoid movements and extensive intracranial calcification, though neurological manifestations like seizures and tetany are more common. We report a case idiopathic hypoparathyroidism with both   choreoathetoid movements and extensive cerebral calcification.

Introduction

Hypoparathyroidism is an inherited or acquired deficiency of the parathyroid hormone (PTH) or its action. PTH secretion by the parathyroid glands (prime regulators of serum calcium concentration) maintains serum calcium within a strict range. Biochemical hallmarks of PTH insufficiency are hypocalcemia and hyperphosphatemia.

Basal ganglion calcification is a common manifestation of hypoparathyroidism, but calcification of the cerebral cortex is extremely rare.

We present a rare case of idiopathic hypoparathyroidism with extensive intracranial calcifications (including thalamus, cerebellum) and choreoathetoid movements.

Case History

Forty two year old male patient presented to the medical ward with history of involuntary movements involving both upper limbs and lower limbs on and off for the past 2 months. These movements are present for forty five minutes to one hour duration and disappears during sleep. He has a past history of seizures for which he is on phenytoin. On examination patient had chorea, positive chovstek sign and trousseau sign. There were no focal neurological deficits. Investigations revealed serum calcium of 8.5 mg/dl (9 to 11 mg/dl) and phosphate of 6.5 mg/dl (3.5 to 5.5). His parathyroid level by radioimmunoassay was 2.0 pg/ml (12 – 55pg/ml). Magnesium results and renal function tests were normal. CT scan showed bilateral basal ganglia, thalamus, cerebellar calcifications. He was treated with clonazepam and his involuntary movements subsided. He was treated with vitamin D and calcium supplementation.

Discussion

01-CT-Brian

Figure 1. CT Brain-Note the extensive intracranial calcifications involving both cerebellum, thalamus, basal ganglia.

Intracranial calcifications commonly basal ganglia calcifications are common. But extensive intracranial calcifications are very rare but reported1 in hypoparathyroidism. Other causes of intracranial calcifications include2  neoplasms  like craniopharyngioma, glioma, vascular like angiomas, aneurysms, infections like toxoplasmosis, herpes, rubella and  metabolic  include Fahr’s syndrome, idiopathic     basal ganglia calcification, pseudohypoparathyroidism, Tuberous sclerosis, Neurofibromatosis., Lissencephaly. All possible other causes of intracranial calcification was ruled out with appropriate investigations.

Hypoparathyroidism with basal ganglia calcification (in about 50%6of patients) and frontal lobe calcification has been reported.3 Caudate, lenticular, thalamic and frontal lobe calcifications have also been reported.5

Figure-2.-Basal-ganglia-and-Thalamic-calcifications

Figure 2. Basal ganglia and Thalamic calcifications-a closer view

Usual neurological manifestations include muscle spasms called tetany (can lead to spasms of the larynx, causing  breathing difficulties), convulsions (seizures). Choreo athetoid movements have been rarely reported in hypoparathyridism.4 Our patient had choreoathetoid movements which subsided with clonaze pam. He  also  developed  episodes  of hypocalcimea  with low s.calcium during attacks, positive trosseus sign, chvosteks sign which responded to IV calcium gluconate.

Severe hypocalcemia, a potentially life-threatening condition, should be treated as soon as possible with intravenous calcium (e.g. as calcium gluconate). Long-term treatment of hypoparathyroidism is with calcium and Vitamin D3 supplementation (D1 is ineffective in the absence of renal conversion). Teriparatide,  might become the treatment of choice for PTH supplementation .In the event of a life- threatening attack of low calcium levels or tetany (prolonged muscle contractions), calcium is administered  by  intravenous  (IV )  infusion.  Precautions are taken to prevent seizures or larynx spasms.

The  mechanism  of   calcification  in hypoparathyroidism has not been completely elucidated. Participation of PTH receptor 2 in the brain and superoxide production by mitochondria in hypoparathyroidism should be explored with reference  to  intracerebral  calcification  and neurodegerative diseases. Our patient was adviced to take lifelong supplementation of oral calcium and to continue clonazepam for control of choreo athetoid movements.

End Note

Author Information

  1. Dr. Pradeep Sreekumar, Junior Resident, Department of Medicine, Coimbatore Medical College, Coimbatore.
  2. Dr. Calwin David Singh, Assistant Professor, Department of Medicine, Coimbatore Medical College, Coimbatore.
  3. Dr. K Umakanthan, Professor, Department of Medicine, Coimbatore Medical College, Coimbatore

Conflict of Interest: None declared

References

  1. Fulop M, Zeifer B. Case report: extensive brain calcification in hypoparathyroidism. Am J Med Sci. 1991 Nov;302(5):292–5. [Pubmed]
  2. M    Mejboubi, T Zegerman, Journal  of  Neurology, Neurology & Psychiatry 2006
  3. Vakaet A, Rubens R, de Reuck J, vander Eecken H. Intracranial bilateral symmetrical calcification on CT-scanning. A case report and a review of the literature. Clin Neurol Neurosurg. 1985;87(2):103–11. [Pubmed]
  4. Girija AS. Idiopathic hypoparathyroidism with extensive intracranial calcification associated with kinesogenic choreoathetosis. J Assoc Physicians India. 2000 Sep;48(9):938–9. [Pubmed]
  5. J.B. Vogler III and J.H. Kim – Hypoparathyroidism, pseudohypoparathyroidism,  pseudopseudo hypoparathyroidism, chapter metabolic and endocrine disease of the skeleton Diagnostic Radiology 4th edition volume three.