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SAT-357 First Case in Saudi Arabia Revealing Fahr Syndrome Secondary to Hypoparathyroidism: A Case Report

Introduction: Fahr Syndrome is a rare inherited or sporadic neurological disorder. It is characterized as in abnormal calcium deposition or in other words, calcification in the brain which almost always occurs bilaterally. Patients with Fahr syndrome usually presents with neurological symptoms such...

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Autores principales: Khan, Soha, Alhamdan, Ali Saleh, AlGhamdi, Ahmed, AlShehri, Abdulleh, Douba, Najah, AlAblani, Hamad, Husain, Ahmed Z
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Oxford University Press 2020
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Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7207827/
http://dx.doi.org/10.1210/jendso/bvaa046.1378
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author Khan, Soha
Alhamdan, Ali Saleh
AlGhamdi, Ahmed
AlShehri, Abdulleh
Douba, Najah
AlAblani, Hamad
Husain, Ahmed Z
author_facet Khan, Soha
Alhamdan, Ali Saleh
AlGhamdi, Ahmed
AlShehri, Abdulleh
Douba, Najah
AlAblani, Hamad
Husain, Ahmed Z
author_sort Khan, Soha
collection PubMed
description Introduction: Fahr Syndrome is a rare inherited or sporadic neurological disorder. It is characterized as in abnormal calcium deposition or in other words, calcification in the brain which almost always occurs bilaterally. Patients with Fahr syndrome usually presents with neurological symptoms such as seizures, tetany, speech impairment, dementia, deterioration of intelligence, involuntary movements. The etiology of Fahr syndrome is mostly associated with endocrine disorders especially hypoparathyroidism either primary or secondary or pseudo hyperparathyroidism; including adult onset neurodegenerative conditions, infectious disease like intrauterine and perinatal infections or inherited congenital causes are also considered. Clinical Case The authors report a 33-year-old Ethiopian female not known to any medical illness presented with abnormal involuntary left-hand movement, headache and dizziness. Further examination shows positive Chvostek’s and Trousseau’s signs. In addition, laboratory findings reveal decreased levels of serum calcium (1.227 mmol/L, normal range of 2.2-2.65 mmol/L), serum albumin (33.53 mg/dL, normal range of 35-52 g/L) and parathyroid hormone (0.3pmol/L, normal range of 1.1-8.43 pmol/L), decreased vitamin D serum level (14.52 ng/ml, normal range of 30-75 ng/ml). Interestingly, brain imaging shows bilateral symmetrical subcortical white matter, basal ganglia, cerebellar dentate nuclei calcifications. Thus, Fahr syndrome diagnosis was made. She was promptly treated with calcium and vitamin D replacement. Calcium gluconate was given intravenously with oral calcium carbonate and oral cholecalciferol. The patient recovered with this treatment leading to positive results without any recurring symptoms. INTERNAL Conclusion In conclusion, Fahr syndrome is a rare sequel of hypoparathyroidism, mostly presented with neurological symptoms due to hypocalcemia which after correction subdues the occurring manifestation such as in this case presented. Early diagnosis of Fahr syndrome due to hypoparathyroidism is crucial for prompt treatment and reversal of symptoms also to prevent complications. INTERNAL
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spelling pubmed-72078272020-05-13 SAT-357 First Case in Saudi Arabia Revealing Fahr Syndrome Secondary to Hypoparathyroidism: A Case Report Khan, Soha Alhamdan, Ali Saleh AlGhamdi, Ahmed AlShehri, Abdulleh Douba, Najah AlAblani, Hamad Husain, Ahmed Z J Endocr Soc Bone and Mineral Metabolism Introduction: Fahr Syndrome is a rare inherited or sporadic neurological disorder. It is characterized as in abnormal calcium deposition or in other words, calcification in the brain which almost always occurs bilaterally. Patients with Fahr syndrome usually presents with neurological symptoms such as seizures, tetany, speech impairment, dementia, deterioration of intelligence, involuntary movements. The etiology of Fahr syndrome is mostly associated with endocrine disorders especially hypoparathyroidism either primary or secondary or pseudo hyperparathyroidism; including adult onset neurodegenerative conditions, infectious disease like intrauterine and perinatal infections or inherited congenital causes are also considered. Clinical Case The authors report a 33-year-old Ethiopian female not known to any medical illness presented with abnormal involuntary left-hand movement, headache and dizziness. Further examination shows positive Chvostek’s and Trousseau’s signs. In addition, laboratory findings reveal decreased levels of serum calcium (1.227 mmol/L, normal range of 2.2-2.65 mmol/L), serum albumin (33.53 mg/dL, normal range of 35-52 g/L) and parathyroid hormone (0.3pmol/L, normal range of 1.1-8.43 pmol/L), decreased vitamin D serum level (14.52 ng/ml, normal range of 30-75 ng/ml). Interestingly, brain imaging shows bilateral symmetrical subcortical white matter, basal ganglia, cerebellar dentate nuclei calcifications. Thus, Fahr syndrome diagnosis was made. She was promptly treated with calcium and vitamin D replacement. Calcium gluconate was given intravenously with oral calcium carbonate and oral cholecalciferol. The patient recovered with this treatment leading to positive results without any recurring symptoms. INTERNAL Conclusion In conclusion, Fahr syndrome is a rare sequel of hypoparathyroidism, mostly presented with neurological symptoms due to hypocalcemia which after correction subdues the occurring manifestation such as in this case presented. Early diagnosis of Fahr syndrome due to hypoparathyroidism is crucial for prompt treatment and reversal of symptoms also to prevent complications. INTERNAL Oxford University Press 2020-05-08 /pmc/articles/PMC7207827/ http://dx.doi.org/10.1210/jendso/bvaa046.1378 Text en © Endocrine Society 2020. http://creativecommons.org/licenses/by-nc-nd/4.0/ This is an Open Access article distributed under the terms of the Creative Commons Attribution-NonCommercial-NoDerivs licence (http://creativecommons.org/licenses/by-nc-nd/4.0/), which permits non-commercial reproduction and distribution of the work, in any medium, provided the original work is not altered or transformed in any way, and that the work is properly cited. For commercial re-use, please contact journals.permissions@oup.com
spellingShingle Bone and Mineral Metabolism
Khan, Soha
Alhamdan, Ali Saleh
AlGhamdi, Ahmed
AlShehri, Abdulleh
Douba, Najah
AlAblani, Hamad
Husain, Ahmed Z
SAT-357 First Case in Saudi Arabia Revealing Fahr Syndrome Secondary to Hypoparathyroidism: A Case Report
title SAT-357 First Case in Saudi Arabia Revealing Fahr Syndrome Secondary to Hypoparathyroidism: A Case Report
title_full SAT-357 First Case in Saudi Arabia Revealing Fahr Syndrome Secondary to Hypoparathyroidism: A Case Report
title_fullStr SAT-357 First Case in Saudi Arabia Revealing Fahr Syndrome Secondary to Hypoparathyroidism: A Case Report
title_full_unstemmed SAT-357 First Case in Saudi Arabia Revealing Fahr Syndrome Secondary to Hypoparathyroidism: A Case Report
title_short SAT-357 First Case in Saudi Arabia Revealing Fahr Syndrome Secondary to Hypoparathyroidism: A Case Report
title_sort sat-357 first case in saudi arabia revealing fahr syndrome secondary to hypoparathyroidism: a case report
topic Bone and Mineral Metabolism
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7207827/
http://dx.doi.org/10.1210/jendso/bvaa046.1378
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