Extrapyramidal symptoms due to calcinosis cerebri in a patient with unknown primary hypoparathyroidism

Ramis, Joana Nicolau; Ibáñez, A. Espino; Irigoin, Rosmeri Rivera; Artigas, Carla Francés; Comas, Lluís Masmiquel

doi:10.1016/j.endoen.2011.03.003

Cited by 6 publications

(8 citation statements)

References 9 publications

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“…These symptoms have been explained by the symmetric, bilateral calcification within the nucleus pallidus, dentate nucleus, putamen and hemispheric white matter [2,3]. It is reported that in basal ganglia calcification, thalamocorticostriatal damage may trigger schizophrenialike syndrome with an incidence ranging from 0.3% to 1.2%, reported on routine radiological examinations [4].…”

Section: Discussionmentioning

confidence: 99%

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Bilateral Basal Ganglia Calcification Secondary to FAHRs Syndrome: A Rare Entity

Nagaraj¹,

Nair²

2016

J Neurol Disord

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A 33 year-old lady was admitted to the intensive care unit (ICU) with the history of a generalized tonic clonic seizure (GTCS) following a road traffic accident (RTA) 8 days ago. Regarding her past medical history, the patient had undergone a thyroidectomy two years ago for a benign thyroid condition. Her physical examination was unremarkable and vitals were within normal limits. On neurological assessment, she had spastic quadriparesis with stiffness of the shoulder girdle muscles. Chvostek and Trousseau signs were elicitable.On admission, blood investigations showed hypocalcemia (total calcium of 4.6 mg/dL (normal level 8.4-10.5). White blood cell count, and hemoglobin level were 21,600/mm 3 , and 9.6 g/dL, respectively. The levels of urea nitrogen and creatinine were normal (14 mg/dL and 0.9 mg/dL, respectively). The parathormone (PTH) level was 2.3 pg/mL (normal level 15-65 pg/mL) with an albumin of 3.3 g/dL. Vitamin D was found to be 24.4 ng/dL. Computed tomography scan (CT scan) of the brain showed intracranial symmetrical calcifications of the basal nuclei with no compressive effect (Figure 1). The clinical examination and laboratory values and subsequent radiological investigations drove us to a diagnosis of Fahr's syndrome secondary to endocrinological dysfunction due to post-thyroidectomy HPT.She was managed conservatively in the ICU with intravenous calcium gluconate, and ampicilin/sulbactam for respiratory infection and started on supportive oxygen therapy via 60% venturi. She was sedated to reduce the muscle stiffness and spasms.On the third day, post admission, we initiated oral calcium and calcitriol. On the sixth day, we took her out of sedation and the patient showed active movements but with loss of muscle strength. The total calcium reached a level of 8.9 mg/dL by the thirteenth day of initiation of therapy. On the sixteenth day, she was discharged, stable, but with Abstract Background and importance: Fahr's syndrome is also known as idiopathic calcification of the basal ganglia. It is described as a rare, degenerative, neuropsychiatric disorder characterized by seizures, extrapyramidal, and neuropsychiatric symptoms as a result of symmetric and bilateral calcifications within the basal ganglia. Involvement of the nucleus pallidus, the putamen, the dentate nucleus of the cerebellum, and the hemispheric white matter at the base of the skull, are common radiological hallmarks of this syndrome. The calcification probably occurs due to lipid deposition and demyelinization.

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Section: Discussionmentioning

confidence: 99%

“…Among these conditions, the commonest cause for FAHRs syndrome, by far, seems to be endocrine diseases [3].…”

Section: Discussionmentioning

confidence: 99%

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Bilateral Basal Ganglia Calcification Secondary to FAHRs Syndrome: A Rare Entity

Nagaraj¹,

Nair²

2016

J Neurol Disord

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“…Especially in HPT, an early treatment can prevent calcification and neurophysiological disorders [20,55,56].…”

Section: Discussionmentioning

confidence: 99%

“…Bilateral striopallidodentate calcinosis is mostly associated with a disorder of calcium and phosphate metabolism, especially hypoparathyroidism (HPT) [2,20,55,60]; however, different aetiology must be considered, including infectious, metabolic, and genetic diseases [65].…”

Section: Discussionmentioning

confidence: 99%

Fahr’s syndrome and clinical correlation: a case series and literature review

Pistacchi¹,

Gioulis²,

Sanson³

et al. 2016

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Hypoparathyroidism

Sanctis¹,

Soliman

Fiscina

2012

Current Opinion in Endocrinology, Diabetes &Amp; Obesity

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PTH promotes bone resorption, decreases urinary calcium excretion, enhances the conversion of 25-hydroxyvitamin D to 1, 25-dihydroxyvitamin D and increases intestinal calcium absorption and phosphate renal excretion. Understanding the molecular cause of the disease in patients and their families has the potential for proper tailoring of genetic counselling, family screening and treatment. Signs and symptoms may be associated not only with the severity, chronicity and therapeutic endpoints in HPT but also with the different causes of the disease. Hypocalcaemia may be an asymptomatic laboratory finding or a life-threatening metabolic disturbance. Although the therapy of acute hypocalcaemia is usually readily accomplished, chronic hypocalcaemia remains a very difficult treatment problem. Replacement therapy with PTH could be a therapeutic option for refractory HPT.

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Extrapyramidal symptoms due to calcinosis cerebri in a patient with unknown primary hypoparathyroidism

Cited by 6 publications

References 9 publications

Bilateral Basal Ganglia Calcification Secondary to FAHRs Syndrome: A Rare Entity

Bilateral Basal Ganglia Calcification Secondary to FAHRs Syndrome: A Rare Entity

Fahr’s syndrome and clinical correlation: a case series and literature review

Hypoparathyroidism

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