2. Introduction
Parathyroid hormone (PTH) regulates calcium and phosporus in the body
Hypoparathyroidism causing hypocalcemia and hyperphosphatemia
A rare metabolic disorder, with an estimated prevalence of 37 cases per
100.000
Etiology: most commond due to thyroid surgery. Only 1-5 % becomes
permanent
Can also causing Fahr Disease / Fahr Syndrome
Supit VD, Kurniawan D, Fatimah E. Fahr syndrome and neurological manifestations in hypoparathyroidism patients. Radiology Case Reports. 2024 Apr 1;19(4):1248-53.
Arruda AC, Guerra AC, Pessoa CH, Marquezine GF, Delfino VD. Hypoparathyroidism and Fahr’s syndrome: case series. Brazilian Journal of Nephrology. 2021 May 17;44:592-6.
3. Clinical Presentation
Paresthesia
Spasms (tetany)
Positive Chvostek sign
Parkinsonism and extrapyramidal symptoms
Cataracts
QT interval prolongation
Severe hypocalcemia (calcium < 5 mg/dL) can result in seizures and cardiac
arrhytmias
Abate EG, Clarke BL. Review of hypoparathyroidism. Frontiers in endocrinology. 2017 Jan 16;7:172.
Cating-Cabral MT, Clarke B. Epidemiology of hypoparathyroidism. InThe Parathyroids: Basic and Clinical Concepts: Third Edition 2014 Sep 5 (pp. 707-717). Elsevier Inc..
Supit VD, Kurniawan D, Fatimah E. Fahr syndrome and neurological manifestations in hypoparathyroidism patients. Radiology Case Reports. 2024 Apr 1;19(4):1248-53.
4. Pathophysiology
The reactive astrocytes and microglia accumulated around calcification
deposits suggest an ongoing mild inflammatory process
It takes around 8-10 years to cause basal ganglia calcification from
hypoparathyroidism alone
Intracranial calcification may worsen with advancing age and unregulated
calcium serum level
Mechanism of brain calcification in HP has been linked to long duration of
hyperphosphatemia and high calcium-phosphate product, resulting from
the disease itself and from long-term treatment with activated vitamin D
and calcium
Leyloh PA, Islamiyah WR. Fahr disease with seizure as clinical manifestation: a case series. Fahr Disease with Seizure as Clinical Manifestation: A Case Series. 2021 Sep 9;84(1):7-7.
Supit VD, Kurniawan D, Fatimah E. Fahr syndrome and neurological manifestations in hypoparathyroidism patients. Radiology Case Reports. 2024 Apr 1;19(4):1248-53.
Arruda AC, Guerra AC, Pessoa CH, Marquezine GF, Delfino VD. Hypoparathyroidism and Fahr’s syndrome: case series. Brazilian Journal of Nephrology. 2021 May 17;44:592-6.
6. Management
Maintain serum calcium levels within the low-normal range
The management target was to reduce symptoms, minimize the risk
of kidney stones and dysfunction, as well as prevent ectopic calcium
deposition in tissues
Calcium and vitamin D supplementation
AED: levetiracetam
7. Summary
Hypoparathyroidism most commonly caused by thyroid surgery
Causing disturbance of calcium and phosporus regulation in human body
May progress into Fahr disease brain abnormal calcifications
Neurological symptoms including seizures, parkinsonism, and cognitive
impairment
Treatment: calcium, vitamin D
Recommended AED option is levetiracetam