다양한 신체증상을 주소로 내원한 부종양성 근강직증후군 1예 = A Case of Paraneoplastic Stiff-Person Syndrome Presenting Multiple Somatic Symptoms

다국어 초록 (Multilingual Abstract)

Stiff-person syndrome (SPS) is an unusual autoimmune neurological disease. We report a woman who developed stiff-person syndrome associated with thymoma. A 42-year-old woman visited a general hospital complaining of progressive rigidity in her neck and both lower legs. She also had other symptoms including whole body pruritus, dysphargia, dysarthria, diplopia, and a visual hallucination-like symptom. Emotional distress preceded her symptoms. After a extensive neurologic workup, she was transferred from the neurologic ward to the psychiatric ward under the impression of conversion
or somatoform disorder. During her psychiatric admission, we found a prolonged involuntary MUAP discharge on her electromyograph, and positive anti-GAD and anti-GQ1b antibodies. In addition, a chest CT scan revealed a thymic epithelial tumor. We report a case of stiff-person syndrome presenting multiple unexplained somatic symptoms, which was initially diagnosed as a conversion or somatoform disorder.

국문 초록 (Abstract)

근강직증후군은 드문 자가면역성 신경계 질환이다. 저자들은 흉선종과 동반한 근강직증후군 증례를 경험하여 보고하였다. 환자는 42세 여자 환자로 진행하는 경부와 양하지의 강직을 주소로 내원하였다. 환자는 전신 소양감, 연하곤란, 조음장애, 체간 강직, 복시 등의 증상을 보였으며, 또한 환각으로 의심되는 증상까지 있었다. 이런 증상은 감정적 스트레스 후에 발생하였기에 신체형 장애나 전환장애 같은 정신과적 질환의 가능성이 높다고 생각되어 신경과에서 정신과로 전과되었다. 그러나 정신과에서 시행한 근전도 검사상 지속 불수의적 근단위활동전위가 나타나고 항GAD 항체와 항GQ1 항체 양성을 보였기에 근강직증후군으로 진단하게 되었다.
흉부 방사선단층촬영상 흉선 상피종이 발견되었으며, 흉선절제술 후 임상증상이 호전되었다.

참고문헌 (Reference)

• 1 Levy LM, "The stiff-person syndrome: an autoimmune disorder affecting neurotransmission of gamma-aminobutyric acid" 131 : 522-530, 1999
• 2 Meinck HM, "The stiff-man syndrome: new pathophysiological aspects from abnormal exteroceptive reflexes and the response to clomipramine, clonidine, and tizanidine" 47 : 280-287, 1986
• 3 Dalakas MC, "The clinical spectrum of anti-GAD antibody-positive patients with stiff-person syndrome" 55 : 1531-1535, 2000
• 4 Mitsumoto H, "Sudden death and paroxysmal autonomic dysfunction in stiff-man syndrome" 238 : 91-96, 1991
• 5 Hagiwara H, "Stiff-person syndrome associated with invasive thymoma: a case report" 193 : 59-62, 2001
• 6 Murinson BB, "Stiff-person syndrome" 10 : 131-137, 2004
• 7 Black JL, "Stiff-man syndrome. Results of interviews and psychologic testing" 39 : 38-44, 1998
• 8 Lorish TR, "Stiff-man syndrome up dated" 64 : 629-636, 1989
• 9 Thomas S, "Stiff person syndrome with eye movement abnormality, myasthenia gravis, and thymoma" 76 : 141-142, 2005
• 10 Rakocevic G, "Stiff person syndrome with cerebellar disease and high-titer anti-GAD antibodies" 67 : 1068-1070, 2006
• 11 Essalmi L, "Stiff person syndrome associated with thymoma. Efficacy of thymectomy" 28 : 627-630, 2007
• 12 Tanaka H, "Stiff man syndrome with thymoma" 80 : 739-741, 2005
• 13 Meinck HM, "Stiff man syndrome and related conditions" 17 : 853-866, 2002
• 14 Espay AJ, "Rigidity and spasms from autoimmune encephalomyelopathies: stiff-person syndrome" 34 : 677-690, 2006
• 15 Barker RA, "Review of 23 patients affected by the stiff man syndrome: clinical subdivision into stiff trunk (man) syndrome, stiff limb syndrome, and progressive encephalomyelitis with rigidity" 65 : 633-640, 1998
• 16 Henningsen P, "Psychological factors in the diagnosis and pathogenesis of stiff-man syndrome" 47 : 38-42, 1996
• 17 Tinsley JA, "Psychiatric consultations in stiff-man syndrome" 58 : 444-449, 1997
• 18 McCabe DJ, "Paraneoplastic "stiff person syndrome" with metastatic adenocarcinoma and anti-Ri antibodies" 62 : 1402-1404, 2004
• 19 Folli F, "Autoantibodies to a 128-kd synaptic protein in three women with the stiff-man syndrome and breast cancer" 328 : 546-551, 1993
• 20 Solimena M, "Autoantibodies to GABA-ergic neurons and pancreatic beta cells in stiff-man syndrome" 322 : 1555-1560, 1990
• 21 Ameli R, "A neuropsychological assessment of phobias in patients with stiff person syndrome" 64 : 1961-1963, 2005
• 22 Gordon EE, "A critical survey of stiffman syndrome" 42 : 582-599, 1967