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Stiff Person-Like Syndrome: An Unusual Presentation of Pituitary Macroadenoma with Panhypopituitarism

Pituitary adenoma can manifest as pituitary hypofunction, which can cause symptoms of panhypopituitarism. Commonly, symptoms of hormonal deficiencies such as lethargy, weight change, cold intolerance, and sexual dysfunction are reported. Optic chiasmal compression leads to visual field changes and t...

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Autores principales: Goh, Kian Guan, Yusof Khan, Abdul Hanif Khan, Nasruddin, Azraai
Formato: Online Artículo Texto
Lenguaje:English
Publicado: S. Karger AG 2022
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9035960/
https://www.ncbi.nlm.nih.gov/pubmed/35530378
http://dx.doi.org/10.1159/000522253
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author Goh, Kian Guan
Yusof Khan, Abdul Hanif Khan
Nasruddin, Azraai
author_facet Goh, Kian Guan
Yusof Khan, Abdul Hanif Khan
Nasruddin, Azraai
author_sort Goh, Kian Guan
collection PubMed
description Pituitary adenoma can manifest as pituitary hypofunction, which can cause symptoms of panhypopituitarism. Commonly, symptoms of hormonal deficiencies such as lethargy, weight change, cold intolerance, and sexual dysfunction are reported. Optic chiasmal compression leads to visual field changes and the discovery of the pituitary lesion. However, limb stiffness is a rare presentation of hypopituitarism, especially hypocortisolism. We report a 68-year-old man who presented with progressive lower limb stiffness associated with truncal instability mimicking a stiff person syndrome (SPS). Hypoglycaemia and hyponatraemia prompted the discovery of pituitary macroadenoma with panhypopituitarism. Investigation showed pituitary macroadenoma on magnetic resonance imaging with hypocortisolism, hypothyroidism, and hypogonadotropic hypogonadism. After initiating hydrocortisone replacement, the patient had complete resolution of lower limb stiffness with no permanent neurological sequelae. It is postulated that hypocortisolism and hyponatraemia disrupt the metabolic function of muscle leading to stiffness. As a result, lower limb rigidity, flexion deformities, and pain are more common. Differentiating adrenal insufficiency associated with rigidity and SPS is important as the response to treatment for both conditions differs. Prompt treatment leads to fast resolution and prevents contractures in adrenal insufficiency-associated rigidity. Thus, recognizing limb rigidity as the first presentation of hypopituitarism is important to avoid long-term complications.
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spelling pubmed-90359602022-05-06 Stiff Person-Like Syndrome: An Unusual Presentation of Pituitary Macroadenoma with Panhypopituitarism Goh, Kian Guan Yusof Khan, Abdul Hanif Khan Nasruddin, Azraai Case Rep Neurol Single Case − General Neurology Pituitary adenoma can manifest as pituitary hypofunction, which can cause symptoms of panhypopituitarism. Commonly, symptoms of hormonal deficiencies such as lethargy, weight change, cold intolerance, and sexual dysfunction are reported. Optic chiasmal compression leads to visual field changes and the discovery of the pituitary lesion. However, limb stiffness is a rare presentation of hypopituitarism, especially hypocortisolism. We report a 68-year-old man who presented with progressive lower limb stiffness associated with truncal instability mimicking a stiff person syndrome (SPS). Hypoglycaemia and hyponatraemia prompted the discovery of pituitary macroadenoma with panhypopituitarism. Investigation showed pituitary macroadenoma on magnetic resonance imaging with hypocortisolism, hypothyroidism, and hypogonadotropic hypogonadism. After initiating hydrocortisone replacement, the patient had complete resolution of lower limb stiffness with no permanent neurological sequelae. It is postulated that hypocortisolism and hyponatraemia disrupt the metabolic function of muscle leading to stiffness. As a result, lower limb rigidity, flexion deformities, and pain are more common. Differentiating adrenal insufficiency associated with rigidity and SPS is important as the response to treatment for both conditions differs. Prompt treatment leads to fast resolution and prevents contractures in adrenal insufficiency-associated rigidity. Thus, recognizing limb rigidity as the first presentation of hypopituitarism is important to avoid long-term complications. S. Karger AG 2022-03-18 /pmc/articles/PMC9035960/ /pubmed/35530378 http://dx.doi.org/10.1159/000522253 Text en Copyright © 2022 by S. Karger AG, Basel https://creativecommons.org/licenses/by-nc/4.0/This article is licensed under the Creative Commons Attribution-NonCommercial-4.0 International License (CC BY-NC) (http://www.karger.com/Services/OpenAccessLicense). Usage and distribution for commercial purposes requires written permission.
spellingShingle Single Case − General Neurology
Goh, Kian Guan
Yusof Khan, Abdul Hanif Khan
Nasruddin, Azraai
Stiff Person-Like Syndrome: An Unusual Presentation of Pituitary Macroadenoma with Panhypopituitarism
title Stiff Person-Like Syndrome: An Unusual Presentation of Pituitary Macroadenoma with Panhypopituitarism
title_full Stiff Person-Like Syndrome: An Unusual Presentation of Pituitary Macroadenoma with Panhypopituitarism
title_fullStr Stiff Person-Like Syndrome: An Unusual Presentation of Pituitary Macroadenoma with Panhypopituitarism
title_full_unstemmed Stiff Person-Like Syndrome: An Unusual Presentation of Pituitary Macroadenoma with Panhypopituitarism
title_short Stiff Person-Like Syndrome: An Unusual Presentation of Pituitary Macroadenoma with Panhypopituitarism
title_sort stiff person-like syndrome: an unusual presentation of pituitary macroadenoma with panhypopituitarism
topic Single Case − General Neurology
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9035960/
https://www.ncbi.nlm.nih.gov/pubmed/35530378
http://dx.doi.org/10.1159/000522253
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