|Year : 2014 | Volume
| Issue : 3 | Page : 195-198
Extensive intracranial calcifications on computerized tomography in a young adult with idiopathic hypoparathyroidism
Manash Kumar Bora, Najah Ismail Kunju, BP Venkatesh, Kalia Perumal
Department of Radiology, AVMCH, Puducherry, India
|Date of Web Publication||17-Sep-2014|
Manash Kumar Bora
Department of Radiology, AVMCH, Puducherry - 607 402
Source of Support: None, Conflict of Interest: None
Pathological basal ganglia calcification can be due to various causes such as metabolic disorders, infections and genetic diseases. Hypoparathyroidism is one of the most common causes of pathological basal ganglia calcification. However, the presence of extensive intracranial calcifications involving other areas of the brain is uncommon. Patients with hypoparathyroidism can present with neurological symptoms related to hypocalcemia, extrapyramidal signs and seizures. We report the case of a young adult who presented with seizures, signs of cerebellar involvement and extensive intracranial calcifications. Investigations revealed that the patient had hypocalcemia, hyperphosphatemia and low parathyroid hormone levels. Since adequate treatment of hypoparathyroidism may lead to marked clinical improvement, this entity should be considered as a differential diagnosis in patients presenting with seizures and extensive intracranial calcifications.
Keywords: Computed tomography, idiopathic hypoparathyroidism, intracranial calcification, seizures
|How to cite this article:|
Bora MK, Kunju NI, Venkatesh B P, Perumal K. Extensive intracranial calcifications on computerized tomography in a young adult with idiopathic hypoparathyroidism. J NTR Univ Health Sci 2014;3:195-8
|How to cite this URL:|
Bora MK, Kunju NI, Venkatesh B P, Perumal K. Extensive intracranial calcifications on computerized tomography in a young adult with idiopathic hypoparathyroidism. J NTR Univ Health Sci [serial online] 2014 [cited 2020 Dec 5];3:195-8. Available from: https://www.jdrntruhs.org/text.asp?2014/3/3/195/140946
| Introduction|| |
Hypoparathyroidism is a disorder of parathormone deficiency. Patients usually presents with neurological symptoms related to hypocalcemia like muscular cramps, paresthesia, fatigue, anxiety etc., extrapyramidal signs and seizures. Association of bilateral basal ganglia calcifications in hypoparathyroidism is well-established. Pathological basal ganglia calcification can be due to various other causes such as metabolic disorders, infections and genetic diseases. Hypoparathyroidism is one of the most common cause of pathological basal ganglia calcification. However, the presence of extensive intracranial calcifications involving other areas of the brain is uncommon. We present a case of idiopathic hypoparathyroidism with seizures, signs of cerebellar involvement and bilateral basal ganglia calcifications and extensive intracranial calcifications of other areas of brain parenchyma.
| Case Report|| |
A 24-year-old male patient with history of seizures over the past 2-years was presented to the casualty department with episodes of seizures. His past medical history was unremarkable. He had no history of thyroid disorders any neck surgery or long-term intake of medications. On examination, his vitals were stable and higher mental functions were normal. His neurological examination showed right sided upper motor neuron facial palsy and right plantar extensor response. He also had scanning speech and dysdiadochokinesia. Chvostek and Trousseau signs were absent and fundoscopic examination was normal. The clinical findings were suggestive of right internal capsular and cerebellar involvement.
Ultrasonography abdomen was normal and showed normal sized kidneys with no parenchymal lesion.
Non-enhanced computed tomography (CT) scan brain, revealed multi focal, extensive, bilaterally symmetrical calcifications, involving bilateral cerebellar hemispheres [Figure 1], bilateral basal ganglia [Figure 2], bilateral internal capsules and subcortical white matter of frontal and parietal lobes [Figure 3]. Extensive bilaterally symmetrical calcifications were also noted in the periventricular white matter and centrum semiovale of both the parietal lobes [Figure 4].
Laboratory investigations revealed hypocalcemia (6.3 mg/dl), decreased parathyroid hormone (PTH) levels (2.5 pg/ml) and hyperphosphatemia (4.5 mg/dl). Serum albumin and magnesium levels were within normal limits. Reduced levels of serum calcium and elevated serum phosphate levels were suggestive of hypoparathyroidism.
|Figure 1: Non enhanced computed tomography brain at the level of fourth ventricle showing bilaterally symmetrical dense calcifi cation of cerebellar hemispheres including dentate nuclei|
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|Figure 2: Non enhanced computed tomography brain at the level of third ventricle showing bilaterally symmetrical dense calcification of caudate nucleus, lentiform nucleus and subcortical white matter|
of frontal lobe
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|Figure 3: Non enhanced computed tomography brain at the level of thalamus showing bilaterally symmetrical dense calcifi cation of thalamus, internal capsule and subcortical white matter of frontal and parietal lobes|
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|Figure 4: Non enhanced computed tomography brain at a higher level showing calcifi cation in the periventricular white matter and centrum semiovale|
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The clinical history along with biochemical reports and CT findings lead to the diagnosis of Idiopathic hypoparathyroidism with extensive intracranial calcifications.
The patient was started on calcium and vitamin D replacement along with supportive treatment with anti-epileptics.
| Discussion|| |
Hypoparathyroidism is a clinical disorder that manifests when the PTH produced by the parathyroid gland is insufficient to maintain the extracellular fluid calcium within normal range, or when adequate circulating concentrations of PTH are unable to function optimally in target tissues to maintain normal calcium levels. 
Hypoparathyroidism may result from agenesis of parathyroid glands like DiGeorge syndrome or destruction of the parathyroid glands following neck surgery or in autoimmune diseases, from reduced secretion of PTH like neonatal hypocalcemia or hypomagnesemia, or resistance to PTH which may occur as a primary disorder as in pseudohypoparathyroidism (PHP) or secondary to hypomagnesaemia.
It may occur as an inherited disorder that may either be part of a complex congenital defect like DiGeorge syndrome, or as a part of polyglandular autoimmune disorder, or as solitary endocrinopathy, which has been referred to as isolated or idiopathic hypoparathyroidism. Hypoparathyroidism may also complicate iron storage disease, especially secondary hemochromatosis in children and adolescents. 
The clinical features of hypoparathyroidism, when symptomatic, can include any of the following signs or symptoms like circumoral numbness, paresthesias, carpal and pedal muscle spasms, laryngeal spasm, tetany and/or seizures. 
Laboratory evaluation in diagnosing hypoparathyroidism should include serum calcium, phosphorous, serum albumin, creatinine, magnesium, intact PTH and 25-hydroxyvitamin D levels. Biochemical abnormalities in hypoparathyroidism include hyperphosphatemia in addition to decreased detectable levels of serum PTH and calcium. 
Measurement of serum total calcium is commonly used to assess calcium status. At physiological pH albumin binds approximately 45% of serum total calcium. Variation in serum albumin concentration therefore alters the concentration of serum total calcium, while the concentration of physiologically important ionized calcium remains constant. 
Magnesium deficiency or excess can impair PTH secretion and also result in hypoparathyroidism. 
Hypoparathyroidism is usually characterized by bilateral symmetrical calcification of basal ganglia which was first noted by Eaton et al. in 1939. 
Extensive calcification involving other areas of the brain is an unusual presentation as was noted in our case.
The mechanism of intracranial calcification in hypoparathyroidism, more often seen in PHP than in idiopathic hypoparathyroidism, has not been completely elucidated. It may be related more to the duration of hypocalcemia and hyperphosphatemia than PTH itself. Hypophosphatemia promotes ectopic calcification in brain tissue in hypoparathyroidism. 
Other important alternatives in the radiologic differential diagnosis for extensive bilaterally symmetrical intracranial calcification include Fahr disease or Fahr syndrome and PHP, which can be confirmed with measurements of serum calcium, phosphorus and PTH levels. ,
Fahr disease also known as bilateral striopallidodentate calcinosis is a rare neurodegenerative disease that is characterized by the bilaterally symmetric deposition of calcium and other minerals in the basal ganglia, thalamus, dentate nuclei and centrum semiovale with normal biochemical parameters.
PHP is an autosomal dominant disorder characterized by hypocalcemia and hypophosphatemia due to PTH resistance rather than PTH deficiency typically in association with distinctive skeletal and developmental defects. 
PHP, in which there is no abnormality of calcium metabolism in asymptomatic patients, is another possible diagnosis in patients with widespread cerebral calcification. 
| Conclusion|| |
Of the many causes of extensive intra cranial calcification as detected in unenhanced CT, hypoparathyroidism is one of the common causes of pathological basal ganglia calcification. Here, the calcifications are coarse or nodular, nearly symmetrical and usually localized to basal ganglia and adjacent structures. However, the presence of extensive intracranial calcifications involving other areas of the brain like cerebellar hemispheres, periventricular and sub cortical white matter is uncommon entity as noted in our case.
Since early diagnosis with adequate treatment may lead to marked clinical improvement of hypoparathyroidism this entity should be considered as a differential diagnosis in patients presenting with seizures and extensive intracranial calcifications in basal ganglia or other areas of brain parenchyma.
| References|| |
|1.||Gionanlis L, Vainas A, Bamihas G, Veneti P, Sobolos K. Brain calcinosis in a dialysis patient with hypoparathyroidism. NDT Plus 2008;1:36-40. |
|2.||Thakker RV. Parathyroid disorders and diseases altering calcium metabolism. In: Warrell DA, Cox TM, Firth JD, editors. The Oxford Textbook of Medicine. 5 th ed., Vol. 2. Oxford: Oxford University Press; 2010. p. 1864-8. |
|3.||Shoback D. Clinical practice. Hypoparathyroidism. N Engl J Med 2008;359:391-403. |
|4.||Payne RB, Little AJ, Williams RB, Milner JR. Interpretation of serum calcium in patients with abnormal serum proteins. Br Med J 1973;4:643-6. |
|5.||Rosen CJ, Brown S. Severe hypocalcemia after intravenous bisphosphonate therapy in occult vitamin D deficiency. N Engl J Med 2003;348:1503-4. |
|6.||Eaton LM, Camp JD, Love JG. Symmetric cerebral calcification, particularlyof the basal ganglia, demonstrable roentgenographically; calcificationof the finer cerebral blood vessels. Arch Neurol Psychiatry 1939;41:921-42. |
|7.||Fujita T. Mechanism of intracerebral calcification in hypoparathyroidism. Clin Calcium 2004;14:55-7. |
|8.||Avrahami E, Cohn DF, Feibel M, Tadmor R. MRI demonstration and CT correlation of the brain in patients with idiopathic intracerebral calcification. J Neurol 1994;241:381-4. |
|9.||Smith AB, Smirniotopoulos JG, Rushing EJ, Goldstein SJ. Bilateral thalamic lesions. AJR Am J Roentgenol 2009;192:W53-62. |
[Figure 1], [Figure 2], [Figure 3], [Figure 4]