1. 내분비 대사 내과 R1 김유진 / Prof. 우정택 근육 강직으로 내원한 50 세 여자 환자

2. Chief complaint Chief complaint Muscle weakness onset) 1 month ago Present illness 10’ 9/21 involuntary abdominal. Tension with LBP 으로 인근 정형외과에서 L-spine MRI 시행 후 HNP 발견되었으나 증상과 correlation 되지 않아 신경과 방문 권고 받 고 의정부 성모 병원 신경과 방문함. 환자 전신에 힘이 들어가고 간헐적인 경련이 일어나면서 몸을 움직이거나 일어나지 못 할 정도로 근 위약감 심해졌으며 체중감소 및 전신 소양감 증상도 동반되었음. 10’10/7~10/15 의정부 성모 병원 신경과 입원하여 evaluation 후 신경학적 검사상 증 상이 일관되지 않고 신경학적 기전과 일치 하지 않아 r/o conversion disorder 평가 위해 정신과로 전과되어 진료받음. Brain MR 상 특이소견 없었으며 medication 시행 하였으나 증상 호전되지 않음. 10’ 10/16~10/25 본원 한방 신경정신과로 전원 되어 치료받았으며 의정부 병원 방문 당시 발견된 hyperthyroidism 에 대한 치료 위해 내분비 내과로 전과됨. 12150546 지 O 순 (F/50) Adm : 2010-10-25

3. Past medical history DM / HTN / Tb / Hepatitis (-/-/-/-) Hyperthyroidism(+): 2010 년 10 월, 의정부 성모병원에서 진단 methimazol 10mg, propranolol 45mg OP Hx (-) Drug Hx(+) 의정부성모병원 신경정신과 입원 시 처방 :10/21 NR 진료의뢰 후 복용 중단 Duloxetine 60mg 1C qd Propranolol 10mg 1.5T bid Diazepam 2mg 1T bid Hydroxyzine 10mg 1T qd Mirtazapine 15mg 1T qd

4. Personal History Smoking (-) Alcohol (-) Occupation : 요식업 Family history(-) 육종암갑상선암

5. Review of Systems 1.General Fever (-) Chilling (-) Sweating(+) Weight loss(+): 5kg/2 주 Fatigue (+) 2.Skin Itching(+) : scratch wound Pigmentation (-) Rash(-) 3.Head & Neck Headache (-) Hoarseness(-) Sore throat (-) 4. E/ENT Discharge(-) Diplopia(+) Ptosis (-) Visual disturbance(+) Otalgia(-) Otorrhea(-) Tinnitus(-) Hearing disturbance(-) 5.Respiratory Cough (-) Sputum (-) Dyspnea (-) Hemoptysis (-) Pleuritic pain(-) 6.Cardiac Chest pain (-) DOE(-) Palpitation (-) Orthopnea(-) Nocturnal dyspnea(-)

6. Review of Systems 7.GI A/N/V/D/C (-/-/-/-/-) Abdominal pain (-) Hematochezia (-) Melena (-) 8.GU Flank pain (-) Dysuria (-) Frequency(-) Urgency (-) 9.Musculoskeletal Pain(+) Swelling(-) Back pain(+) Arthralgia(-) Muscle spasm(+) Stiffness(+) 10.Nervous Dizziness(-) Syncope(-) Seizure(-) Sensory loss(-) Motor weakness(-) Memory disturbance(-) Tremor(-) Numbness(-) Speech disturbance(+) - Lt. hand grip 시 Lt elbow joint extension 시 stiffness 보임. - 취침 시 flexion state  아침 기상 시 stiffness 심하고 활동 하면서 다소 증상이 호전됨. -Duration: 2~3 초 / 하루에 수 차례 - 주로 복부를 중심으로 통증을 동반 Lt. upper limb : proximal area

7. Physical Examination Ht 160 cm Wt 44.6 kgBMI :17.4 kg/m2 V/S 130/80mmHg – 96/min – 20/min – 36.0 °C General Alert consciousness Acute ill looking appearance Skin Rash(-) Warm and wet, Scratch wound Head & Neck Isocoric pupil c PLR (++/++) Cervical LN enlargement (-) No neck vein engorgement(-) Thyroid enlargement: slightly enlarged, hard PI (-) PTH (-/-) dry mouth(-)

8. Physical Examination Thorax Symmetrical chest expansion Clear breathing sounds s rale or wheezing Regular heart beat without murmur Abdomen hard & flat abdomen : board like Normo active bowel sound Tenderness (-) Rebound tenderness (-) No hepato splenomegaly Back & Extremity CVA Td (-/-) Pre tibial pitting edema(-/-)

9. Physical Examination Neurology Motor IV III Sensory 100 100 IV III 100 100 DTR: Normoreflex ++

10. Initial lab finding 1. CBC/DC 8810/ ㎕ (seg:69.5%) – 13.2g/ ㎗ – 38.7% - 368,000/ ㎕ 2. Chemistry Total bilirubin 0.61 mg/dL BUN/Cr 15/0.5 mg/dL Protein/Albumin 7.0/4.2 g/dL Na/K/Cl 141/3.9/104mEq/L AST/ALT 21/29 IU/L Ca/P/Mg 9.2/3.8/2.0 mg/dL ALP/rGT 92/14 IU/L CRP <0.3 mg/dL CK/LD 21/417IU/L 3. UA RBC 0~1/HPF WBC 2~4/HPF Ketone (-) Protein (-) Nitrite (-) Glucose (-)

11. Chest x-ray

12. EKG

13. Problem lists #1. Ant. mediastinal mass #2. Muscular symptom (spasm, pain, stiffness, weakness) #3. Known hyperthyroidism

14. Initial assessment & plan #1. Ant. mediastinal mass #2. Muscular symptom (spasm, pain, stiffness, weakness) r/o Thymoma with Neurologic Sx - Myasthenia Gravis - Neuromyotonia (Isaac synd.) - Lambert Eaton myasthenic syndrome - Stiff person syndrome Diagnosis plan) Chest CT(or MRI) Tensillon test, Jolly test, NCS, EMG Serum Ach-R Ab, anti MuSK AB, anti-VGKC Ab, anti-VGCC Ab etc. Treatment plan) Symptomatic control (Anticonvulsant etc.) Bridge therapy (Plasmapheresis ) Surgery Post-op Mx (Acetylcholineseterase inhibitor & immunosuppressive drug etc)

16. Harrison’s principle of internal medicine, 17 th edition

17. Initial assessment & plan #3. Known hyperthyroidism Diagnosis plan) TFT, Anti-thyroid auto Ab Treatment plan) methimazol & propranolol 60mg 유지

18. Clinical Course

19. 10/25 11/2 11/1211/611/411/210/3110/2910/2711/1011/8 Chest CT

20. -CT 10/27 Chest-CT

21. 10.2711.311.11 T3 (80~200ng/dL) 149126 Free T4 (0.77~1.94ng/dL) 2.793.01 TSH (0.3~4.0uU/mL) 0.060.14 Anti-TPO (0~8U/ml) 661234 TG-Ab (0~60U/ml) 54.734.5 TBII (0~10%) 39.127.9

22. 10/25 11/2 11/1211/611/411/210/3110/2910/2711/1011/8 Chest CT

23. 10/25 11/2 11/1211/611/411/210/3110/2910/2711/1011/8 Chest CT Tensilon Test Jolly Test NCS EMG Edrophonium IV negative

24. Nerve conduction Study(NCS)  Motor & sensory: WNL

25. Jolly’s test Low rate stimulation: Decremental response(-) High rate stimulation Incremetal response(-)

26. EMG(electromyography)  Abnormal sign(-)

27. TS transfer & Radical Thymectomy 0 AchR Ab: 8.077 nmol/L 0 10/25 11/2 11/1211/611/411/210/3110/2910/2711/1011/8 Chest CT Tensilon Test Jolly Test NCS EMG AchR Ab Edrophonium IV negative Plasmaph eresis #1 Plasmaph eresis #2 Plasmaph eresis #3 200mgCarbamazepem 600mg Etravil 10mg Muscle weakness, stiffness, pain  no interval change Thymoma B1 PRE OP

28. 2010/11/10 Radical thymectomy via sternotomy OP finding > 1. 5 x 5cm 크기의 thymic mass 가 관찰됨. 2. Left pleura 로 invasion 양상 보임. 3. Left lung 과 phrenic nerve 로의 invasion 양상 보이지 않음 4. Frozen bx - thymoma, r/o type B-I Diagnosis > Thymus, thymectomy: Thymoma (WHO classification B1)

30. 11/12 11/2 12/1812/612/211/2811/2411/2011/1612/1412/10 POST OP

31. 11/12 11/2 12/1812/612/211/2811/2411/2011/1612/1412/10 Carbamazepem 600mg Etravil 10mg 800mg1200mg 20mg 40mg Prednisone 80mgTapering to 0mg Pyridostigmine 90mg->stop (12/11) Diazepam 20mg M. weakness, stiffness, pain Generalzied twitching 수십회  no interval chagne 7.855 AchR Ab: 7.488 nmol/L 8.224 Anti - GAD Ab U/mL 10 배 희석 : >300 U 100 배 희석 : 630 Anti - GAD Ab >300 U/mL Paraneoplastic AutoAb : Neagtive M. weakness, stiffness, pain Generalzied twitching  거의 호전된 양상 POST OP 900->600mg->stop

32. 12/26 퇴원 11/2 4/144/3 adm 3/28 외래 #5 3/21 외래 #4 2/17 외래 #3 2/01 외래 #2 1/06 외래 #1 4/124/5 Methimazol 20mg Tramadol 50mg PO 10mg Tramadol 50mg IV Baclofen 15mg-->30mg Anti - GAD Ab Orbit CT M. weakness, stiffness, pain Generalzied twitching  거의 호전된 양상 으로 퇴원  일상생활 가능, 외래 통원 Discharge & OPD f/u & Admission Diazepam 20mg 20mg Tramadol 50mg 30mg 10mg 15mg 50mg 30mg 10mg no medication for 15days  symptom aggravation methimazol : TFT f/u 3/28 : 수면과다, 가라앉는 기분 ->diazepam 감량 Brain CT 내원 1 주일전부터 N/V 내원 3 일 전부터 poor oral intake Muscle stiffness 악화 Diplopia, nystagmus,dizziness 1. Stiff person syndrome 2. Graves’ Ophthalmopathy

33. 입원 전 11/2 HD14 외래 3-4 외래 1-2 퇴원수술 후 수술 전 입원 HD2 외래 5- 입원 Carbamazepem Etravil Prednisone Pyridostigmine 0~6 Lower trunk 1 Upper trunk 1 Leg 1 Arms 1 Face 1 Abdomen 1 Clinical course Baclofen Lower trunk 1 Upper trunk 1 Leg Arms Face Abdomen 1 Lower trunk 1 Upper trunk 1 Leg 1 Arms 1 Face Abdomen 1 1 1 : Assessment of severity : Class 0 — No symptoms or signs Class I — Only signs, no symptoms : lid retraction, lid lag Class II — Soft tissue involvement Class III — Proptosis : Exophthalmos, Lt eye Class IV — Extraocular muscle involvement : Diplopia, nystagmus Class V — Corneal involvement Class VI — Sight loss (optic nerve involvement) : Activity score : 0 Spontaneous retrobulbar pain Pain on eye movement Eyelid erythema Conjunctival injection Chemosis Swelling of the carbuncle Eyelid edema Diazepam

34. Final diagnosis 1. Stiff person syndrome 2. Thymoma 3. Graves’ disease