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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Main Authors: Goh, Kian Guan, Yusof Khan, Abdul Hanif Khan, Nasruddin, Azraai
Format: Article
Published: Karger 2022
Online Access:http://psasir.upm.edu.my/id/eprint/103299/
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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
building UPM Institutional Repository
collection Online Access
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 upm-1032992023-06-26T07:19:22Z http://psasir.upm.edu.my/id/eprint/103299/ Stiff person-like syndrome: an unusual presentation of pituitary macroadenoma with panhypopituitarism Goh, Kian Guan Yusof Khan, Abdul Hanif Khan Nasruddin, Azraai 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. Karger 2022 Article PeerReviewed Goh, Kian Guan and Yusof Khan, Abdul Hanif Khan and Nasruddin, Azraai (2022) Stiff person-like syndrome: an unusual presentation of pituitary macroadenoma with panhypopituitarism. Case Reports in Neurology, 14 (1). 157 - 161. ISSN 1662-680X https://karger.com/crn/article/14/1/157/822774/Stiff-Person-Like-Syndrome-An-Unusual-Presentation 10.1159/000522253
spellingShingle 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
url http://psasir.upm.edu.my/id/eprint/103299/
http://psasir.upm.edu.my/id/eprint/103299/
http://psasir.upm.edu.my/id/eprint/103299/