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• Vol.62 No.9
• CLINICA NEUROL, 62: 736−739, 2022
• Vol.62 No.9 contents

Case Report

Isolated ACTH deficiency clinically presented as stiff-person syndrome, successfully treated by hormonal replacement：a case report

Tomone Taneda, M.D.¹⁾, Shunsuke Okawara, M.D.¹⁾, Masaki Namekawa, M.D.¹⁾, Yoshitaka Umeda, M.D.¹⁾, Mutsuo Oyake, M.D.¹⁾ and Nobuya Fujita, M.D.¹⁾

¹⁾ Department of Neurology, Nagaoka Red Cross Hospital

A 65-year-old man was admitted to our hospital with a 6-year history of painful muscle stiffness in his trunk and lower limbs, preventing him from walking. Stiff-person syndrome (SPS) was diagnosed because the patient had symptoms of painful muscle spasms elicited by tactile stimulation without joint contracture. Although SPS- related autoantibodies in the serum, including anti-glycine R, anti-amphiphysin, anti-glutamic acid decarboxylase (GAD), antidipeptidyl peptidase-like protein (DPPX) and anti-ϒ-aminobutyric acid-A (GABA_A) R, were negative, the ACTH and cortisol levels were low. On the basis of additional loading tests for anterior pituitary function and ACTH, isolated ACTH deficiency (IAD) was diagnosed. Hormonal replacement therapy with hydrocortisone at 15 mg/day ameliorated the condition quickly, and the patient became asymptomatic after three months. Flexion contractures have been reported as musculoskeletal symptoms of IAD, but are not usually evident in patients with SPS. The present case illustrates that the painful muscle spasms elicited by tactile stimulation without joint contracture characteristic of SPS can also be symptoms of IAD.
Movie Legend
Movie 1 Before treatment.
Painful muscle spasms induced by joint movement and tactile stimulation were seen in the lower limbs without any limitation of joint range of motion.
The patient was walking clumsily with his hip and knee joints in the flexion position.
Movie 2 After treatment.
Five days after treatment, the muscle spasms in the lower limbs disappeared. After 40 days, the patient was able to walk normally.
Movies are published with patient's permission.
Full Text of this Article in Japanese PDF (756K)

(CLINICA NEUROL, 62: 736−739, 2022)
key words: isolated ACTH deficiency, stiff-person syndrome, flexion contracture, hormonal replacement therapy

(Received: 14-Apr-22)