Hypoparathyroidism and Fahr’s syndrome: case series

Abstract

Hypoparathyroidism (HP) is a rare metabolic disorder and causes hypocalcemia because parathyroid hormone secretion is inadequate to mobilize calcium from bone and reabsorb calcium from kidney and gut. Anterior neck surgery is the most common cause of acquired HP and autoimmune HP is the next most common form in adults. The duration, severity, and rate of development of hypocalcemia determine the clinical presentation. A variety of organs can be affected by calcification, more frequently kidneys, but also joints, eyes, skin, vasculature, and other organ systems and, although rarely seen, intracerebral calcifications. Case series: We report four cases of bilateral basal ganglia calcifications (BGC) also known as Fahr’s syndrome related to hypoparathyroidism. Discussion: Fahr’s syndrome is characterized by bilateral symmetrical calcification of areas of the brain that control movements including basal ganglia, thalamus, and others; it is a rare inherited or sporadic neurological disorder with a prevalence of less than 1/1.000.000. Main symptoms related to bilateral BGC include extra-pyramidal and cerebellar disorders, cognitive impairment, epileptic seizures, and psychiatric changes. BGC has been established as a possible outcome of HP. Its prevalence, demonstrated in the HP cohorts, varied significantly from 12 up to 74%. Currently, computed tomography (CT) is the most valuable method for diagnosis. The treatment include symptomatic support and identification of causes, but there is no specific treatment limiting the progression of calcification in the basal ganglia. Especially in HP, an early treatment can prevent calcification and neurophysiological disorders.

Fahr syndrome due to hypoparathyroidism presenting in a comatous patient: A Case Report

“…Presence of reduced PTH with reduced calcium and neuroimaging with gross calcifications suggests the diagnosis. 2 This case report emphasizes early treatment importance in suspected.…”

“…1 An important cause is primary hypoparathyroidism, for which diagnostic evaluation is imperative. 2 Clinical picture may involve acute or subacute presentations, in addition to asymptomatic cases. 3 Here we present a case of primary hypoparathyroidism causing Fahr's syndrome with an acute presentation with excellent therapeutic response.…”

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“…Onset of chronic renal failure later in her life may had augmented loss of serum calcium which was not counteracted by a balancing increase of PTH due to already existing hypoparathyroidism. Fahr's syndrome may arise from hypoparathyroidism [4][5][6] and may present with either dementia, extrapyramidal or psychiatric symptoms although at different age of onset [6][7][8]. Of note, the patient had never reported, nor she had at neurological examination, signs of increased neuromuscular excitability such as tetany or spasms, which are extremely frequent with an underlying severe hypocalcemia [3].…”

Seizures in activating calcium‐sensing receptor antibodies positive autoimmune hypoparathyroidism and preponderance of steroids therapy: A case report

“…The disorder may manifest acutely with seizures, bronchospasm, laryngospasm, or cardiac rhythm disturbances. 5 , 6 …”

“…As low PTH leads to low serum calcium levels, hypoparathyroidism often presents with paresthesia, hyperreflexia (carpal spasms known as Trousseau's sign and facial spasms known as Chvostek's sign), cramps, or tetany. The disorder may manifest acutely with seizures, bronchospasm, laryngospasm, or cardiac rhythm disturbances 5,6 …”