病情介绍-中英文版

姓名：蒋星禹病区神经内科病区病情摘要患儿蒋星禹，男，8岁，因“渐进性四肢无力2+月，尿便潴留1+月”为主诉入院。

患儿2+月前始（2013-01-14）无明显诱因出现右侧肢体无力，发病初期表现为右下肢跛行，右手持物不紧。

2013-01-19患者家属将其送至“重医附属儿童医院门诊”，行双下肢X片、神经传导速度测定、肌电图检查均正常。

2013-01-27患者家属将其送至“新桥医院”，行头颅MRI 平扫：脑干右分片状异常信号，呈长T1长T2flair。

患者当日于重医儿童医院住院治疗（2013-01-27至2013-02-12），入院查体：右侧上下肢肌力IV+，右侧巴彬斯基征可疑阳性。

余NS（－）。

入院后行脑脊液检查（2013-01-28）示：脑脊液微量蛋白：0.56（0.15-0.45g/L），葡萄糖：0.86（2.44 - 4.44mmol/L）；氯离子：124.2（102.7-132.1mmol/L）；血常规、尿常规、大便常规、甲胎蛋白、肝炎标志物、HIV、梅毒抗体阴性。

复查头颅MRI+增强（2012-01-29）发现：脊髓颈段和左侧小脑半球见斑片状异常信号。

颈段MRI（2013-01-31）发现：延髓至颈6椎体水平脊髓病变。

左侧小脑蚓部及小脑半球改变，建议增强检查助诊。

肺CT （2013-01-29）：未见明显异常。

视频脑电图（2013-01-31）：异常脑电图。

清醒背景6-7Hz θ活动增多。

心电图（2013-01-29）：窦性心律不齐。

心脏超声（2013-02-04）：心内结构未见异常，三尖瓣返流（轻度）。

P100（2013-02-08）：1.双眼全视野、左右单眼全视野P100波波幅明显降低。

2.左右单眼半视野各次P100波波幅降低或缺失。

SEP（2013-02-08）：双上肢SEP异常：皮层N20波引出，N20波潜伏期明显延长，伴时程增宽。

双下肢SEP无异常发现。

皮层P40波引出，P40波波幅无异常。

BAEP（2013-02-08）：1.左侧听神经远端及耳蜗可疑异常：1.左侧BAEP I 波潜伏期可疑延长；2.左侧上脑干异常：左侧III-V、I-V波间期延长，与右侧不对称；3.双侧BAEP听阈稍增高。

考虑：“1.急性播散性脑脊髓炎？2.颅内特殊病原感染？3.多发性硬化？4.神经系统肿瘤？”予地塞米松磷酸注射液10mg，2/日（3日后减量为醋酸泼尼松片，10mg，3/日）抗炎、丙种球蛋白支持治疗、20%甘露醇125ml，1/8小时（3日后减量为125ml，1/12小时）降颅压及对症处理、支持治疗。

2013-02-08患者出现尿便潴留。

出院时症状体征转归：右上肢数数，对指不能，右侧巴彬斯基征阳性。

余查体同入院。

2013-02-13患者转诊至北京儿童医院住院治疗（2013-02-13至2013-02-19），入院查体：右上肢肌力III-级，右下肢肌力III级，右侧上、中腹壁反射减弱，右侧下腹壁反射未引出。

双侧肱二、肱三头肌反射、膝腱、跟腱反射活跃。

右侧巴彬斯基征阳性。

右侧指鼻试验不稳。

余NS（—）。

行颈胸椎MRI平扫（2013-02-14）：延髓下段至颈5水平延髓级及脊髓增粗，髓内多发信号异常，左侧小脑半球下部分片状异常信号。

头颅MRI（2013-02-15）：结合颈段脊柱检查考虑颈髓占位性病变，并颅内室管膜下、蛛网膜下腔转移，累及脑组织可能性大。

腰骶椎MRI平扫（2013-02-16）：矢状面T8以下脊髓表面不光整，T8-10T2WI信号偏高，横轴位显示部分脊神经根增粗聚：结合颈部及头部MRI考虑颈髓占位，脑脊液播散转移可能性大。

脑脊液检查（2013-02-17）：常规+生化：脑脊液无色、透明。

氯124mmol/L、糖1.07mmol/L、蛋白144.45mg/L。

脑脊液墨汁染色、革兰染色、抗酸染色阴性。

脑脊液病理检查：涂片镜下见多量淋巴细胞，单核细胞及吞噬细胞。

骨髓检查（2013-02-07）：未见异常。

肝肾功、血常规、尿常规、大便常规、电解质、炎症系列均正常。

院外（天坛医院）会诊后考虑肿瘤可能性大，拟转入“中国人民解放军第二炮兵总医院”放疗中心治疗。

出院时症状体征转归：右上肢肌力II级，右下肢肌力III+级，余查体同入院。

2013-02-20转诊至“中国人民解放军第二炮兵总医院”住院治疗（2013-02-20至2013-03-04），入院查体：右上肢肌力II级，右下肢肌力III级，左上肢肌力IV级，左下肢肌力III级，右侧上、中腹壁反射减弱，右侧下腹壁反射未引出。

双侧肱二、肱三头肌反射、膝腱、跟腱反射活跃。

右侧巴彬斯基征阳性。

右侧指鼻试验不稳。

余NS（—）。

住院期间行PET-CT检查（2013-02-20）：1.左侧小脑、延髓及脊髓多发病变，其中延髓及脊柱病变FDG摄取增高，左小脑病变FDG摄取减低，与外院2013-01-29、2013-01-30、2013-02-13、2013-02-17MRI 所示病变一致，影像资料所示病变形态多变、范围广泛、部分病灶较前有变化，不符合典型恶性肿瘤影像学表现，考虑炎性改变可能，但需结合临床及相关实验室检查资料进一步除外。

2.右侧腹股沟处局部软组织较左侧略肿胀，FDG摄取增高，请结合临床。

3.盆腔少量积液。

治疗上予静注人免疫球蛋白治疗（10mg，1/日）、酸醋泼尼松龙片，30mg，1/日同前。

患者症状体征同入院。

2013-03-05转入我院神经外科，入院查体：右上肢II级，右下肢II+，左上肢肌力III级，左下肢III+级，膝、跟腱反射正常，双侧HBabinski征及Kernig征均阳性。

余NS（—）。

入院后行头颅MRI+颈胸部MRI（增强）（2013-03-08）：左侧小脑半球、延髓及上端颈髓多发异常信号影，并蛛网膜多发异常强化，考虑炎性病变可能。

颈段脊髓多发占位性病变，性质待定（感染性病变？新生物？）。

胸段脊髓未见明显异常。

腰段脊髓MRI平扫加增强未见确切异常；骶2骶管囊肿。

PET-CT（2013-03-11）示：1、左侧小脑、延髓及脊髓多发FDG 代谢增高灶，考虑炎性病变。

2、双侧口咽部、咽喉部FDG代谢增高，考虑炎性病变。

3、颈部、胸部、腹盆部及骨骼PET/CT显像未见明显异常。

血常规、血沉、肝炎十项、肿瘤标志物、凝血功能、血糖、尿常规、HIV、肝功、肾功、电解质、降钙素原检测、C反应蛋白、白介素6正常。

结核抗体阴性。

请全市会诊后考虑炎性病变可能性大。

予继续口服激素（逐渐减量）、诊断性抗结核治疗（异烟肼片，230mg，口服，1/日；利福喷丁胶囊，0.3g，口服，2/周；乙胺丁醇片，0.5g，口服，1/日；吡嗪酰胺片，0.125g，口服，3/日；）及针灸康复等治疗。

症状体征无明显改善。

2013-03-23转入我院神经内科，脑脊液检查（2013-03-25）：测初压100mmH2O。

留取15ml （常规+生化、三染色、免疫球蛋白+补体、脑脊髓液细胞学检查、结核分枝杆菌直接检测（MTD）、寡克隆带）测末压80mmH2O。

脑脊液常规+生化：脑脊液无色、透明，潘氏反应阳性+，蛋白质3.23g/L，白细胞0，糖0.70mmol/L（同期血糖4.9mmol/L），氯122.6mmlo/L，免疫球蛋白（脑脊髓）：I gG 0.57g/L，I gM 0.01 g/L，I gA 0.04 g/L，补体C3 0.06 g/L，I gE 5 IU/ml；免疫球蛋白（雪）：I gG 12.70g/L，I gM 1.20 g/L，I gA 1.46g/L，补体C3 1.24 g/L，I gE 111 IU/ml；脑脊液及血中未见寡克隆带。

腹部超声：肝、胆、胰、脾、双肾二维及彩色多普勒超声未见异常。

膀胱超声：膀胱大小6.4cm×6.4cm×8cm，膀胱充盈过度，壁粗糙，横切膀胱时，膀胱下部可见密集点状强回声堆积，范围 4.2cm×1.2cm，腔内另可见点状强回声漂浮，盆腔未见明显实质性占位。

[印象]膀胱充盈过度，壁粗糙，腔内沉积物，请结合临床。

淋巴结超声：双侧颈部、右侧腋窝淋巴可见。

目前诊断：延髓、颈髓炎性病变：考虑非特异性炎性病变可能性大，但患者小脑幕也有强化不支持。

患者三次脑脊液检查结果，蛋白持续升高，糖进行性下降，细胞数为0，氯化物正常，如为结核性，患者院外一直在口服激素治疗，无全身结核中毒症状，蛋白逐渐身高、糖低，但细胞数始终为0不支持。

按炎性病变给予糖皮质激素治疗（甲泼尼龙粉针，250mg，静滴，1/日）虽结核无依据，但激素使用期间慎重起见同时给予抗痨治疗。

治疗方案：按公斤体重予异烟肼片+利福平胶囊+乙胺丁醇片+吡嗪酰胺片抗痨、地塞米松抗炎及对症处理、支持治疗至今，患儿出现午后发热，体温37.8-38℃之间,4月6日以后又恢复正常。

4月9日脑脊液检查（2013-04-09）：测初压200mmH2O，脑脊液常规+生化：脑脊液无色、透明，潘氏反应阳性+，蛋白质3.65g/L，白细胞0，糖0.94mmol/L，氯111.5mmlo/L，。

HELP:Diagnosis and Treatment of an 8-year-old boyEight-year-old boy, who was admitted to our hospital on 14th Jan 2013 with chief complaint of “progressive weakness of limbs for more than 2 months, urinary retention for more than 1 month”.More than 2 months ago (14th Jan 2013), the patient developed weakness of the right limbs without obvious inducing factor. In the beginning, there was claudication on the right side and the patient could not hold an object firmly with the right hand. On 19th Jan 2013, the patient was sent to “Chongqing XX Hospital”, where bilateral lower limb X-ray, nerve conduction velocity determination and electromyogram showed normal results. On 27th Jan 2013, the patient was sent to another “Chongqing XXX Hospital”, where cranial MRI plain scan was performed, showing abnormal patchy signals in the right brain stem, and long T1 long T2 flair. On the same day, the patient was admitted to the former Hospital for inpatient treatment (from 27th Jan 2013 to 12th Feb 2013). Physical examination on admission: muscle strength of the right upper and lower limbs was IV+. Right Babinski’s sign was suspected positive. The others were normal. After admission, examination of cerebrospinal fluid (28th Jan 2013) showed: microalbumin in cerebrospinal fluid: 0.56 (0.15-0.45 g/L), glucose: 0.86 (2.44-4.44 mmol/L); chlorine ion: 124.2 (102.7-132.1 mmol/L); blood routine, urine routine, stool routine, alpha fetoprotein, hepatitis markers, HIV and syphilis antibody were negative. Re-examination of cranial MRI + enhancement (29th Jan 2012) showed: abnormal patchy signals in the cervical cord and left cerebellar hemisphere. Cervical MRI (31st Jan 2013) showed: myeleterosis at the levels from medulla oblongata to C6. Lesions in the left vermis cerebelli and cerebellar hemisphere were observed. Enhanced examination was recommended to facilitate diagnosis. Pulmonary CT (29th Jan 2013): no significant abnormality. Video EEG (31st Jan 2013): abnormal EEG. When awake, 6-7Hzθ activities increased. ECG (29th Jan 2013): sinus arrhythmia. Cardiac ultrasound (4th Feb 2013): cardiac structure showed no abnormality; tricuspid regurgitation (mild). P100 (8th Feb 2013): 1. P100 wave amplitude significantly decreased in binocular full field and left and right monocular full field. 2. P100 wave amplitude decreased or was absent in the left and right monocular half field. SEP (8th Feb 2013): bilateral upper limb SEP abnormality: cortical N20 wave was induced; latency of N20 wave was significantly prolonged, with broadening of time interval. Bilateral lower limb SEP showed no abnormality. Cortical P40 wave was induced. P40 wave amplitude showed no abnormality. BAEP(8th Feb 2013): 1. Distal end of the left auditory nerve and cochlea showed suspected abnormality: 1. Latency of the left BAEP I wave showed suspected prolongation; 2. Left upper brain stem showed abnormality: left III-V and I-V intervals were prolonged, asymmetric to the right side; 3. Bilateral BAEP auditory threshold was slightly increased. Diagnosis was considered to be “1. Acute disseminated encephalomyelitis? 2. Intracranial special pathogen infection? 3. Multiple sclerosis? 4. Nervous system tumor?” The patient received dexamethaso ne phosphate injection 10 mg, twice daily (changed to prednisone acetate tablets, 10 mg, 3 times daily after 3 days) as anti-inflammatory treatment, gamma globulin supportive therapy, 20% mannitol 125 ml, 1/8 hour (reduced to 125 ml, 1/12 hour after 3 days) to lower intracranial pressure, symptomatic treatment and supportive therapy. On 8th Feb 2013, the patient developed urinary and stool retention. On discharge on 12th Feb 2013 after 16 days treatment , outcome of symptoms and signs on of the boy: digital opposition was impaired; right Babinski’s sign was positive. The rest was the same as examination on admission.On 13th Feb 2013, the patient was transferred to “Beijing XXX Hospital”for inpatient treatment (from 13th Feb 2013 to 19th Feb 2013). Physical examination on admission: muscle strength of the right upper limb was grade III. Muscle strength of the right lower limb was grade III. Reflex on the right upper and middle abdominal wall was attenuated. Reflex on the right lower abdominal wall was not induced. Bilateral biceps reflex, triceps reflex, patellar tendon reflex and achilles tendon reflex were active. Right Babinski’s sign was positive. Right finger to nose test was unstable. The others were normal. Cervical and thoracic MRI plain scan was performed (14th Feb 2013): enlargement of the medulla and spinal cord from the lower part of medulla oblongata to C5; multiple abnormal signals in the spinal cord; abnormal patchy signals in the lower part of the left cerebellar hemisphere. Cranial MRI (15th Feb 2013): in combination with cervical spine examination, occupying lesion in the cervical cord was considered, accompanied by metastases in intracranial subependymal and subarachnoid spaces; involvement of brain tissue was probable. Lumbosacral MRI plain scan (16th Feb 2013): on sagittal plane, surface of the spinal cord below T8 was not smooth; T8-10T2WI signal was high; enlargement of some spinal nerve roots on transverse axis. In combination with cervical and cranial MRI, occupying lesion in the cervical cord was considered. Metastases spread by cerebrospinal fluid were probable. Cerebrospinal fluidexamination (17th Feb 2013): routine + biochemistry: cerebrospinal fluid was colorless and transparent. Chlorine 124 mmol/L, glucose 1.07 mmol/L, protein 144.45 mg/L. Cerebrospinal fluid ink staining, Gram staining and acid-fast staining were negative. Pathological examination of cerebrospinal fluid: under a microscopy, a large amount of lymphocytes, monocytes and phagocytes were observed on the smear. Bone marrow examination (7th Feb 2013): no abnormality. Liver and renal functions, blood routine, urine routine, stool routine, electrolytes and inflammation series were all normal. In another hospital (XXX Hospital), after consultation, the possibility of tumor was considered high. It was planned to transfer the patient to radiotherapy center in another “Beijing XXX Hospital” for treatment. On discharge on 19th Feb 2013 after 6 days treatment ,Outcome of symptoms and signs of the boy: muscle strength of the right upper limb was grade II. Muscle strength of the right lower limb was grade III+. The rest was the same as physical examination on admission.On 20th Feb 2013, the patient was transferred to “Beijing XXX Hospital” for inpatient treatment (from 20th Feb 2013 to 4th Mar 2013). Physical examination on admission: muscle strength of the right upper limb was grade II. Muscle strength of the right lower limb was grade III. Muscle strength of the left upper limb was grade IV. Muscle strength of the left lower limb was grade III. Reflex on the right upper and middle abdominal wall was attenuated. Reflex on the right lower abdominal wall was not induced. Bilateral biceps reflex, triceps reflex, patellar tendon reflex and achilles tendon reflex were active. Right B abinski’s sign was positive. Right finger to nose test was unstable. The others were normal. During hospitalization, PET-CT was performed (20th Feb 2013): 1. Multiple lesions in the left cerebellum, medulla oblongata and spinal cord, increased FDG uptake in lesions of medulla oblongata and spinal cord, decreased FDG uptake in lesions of the left cerebellum. Changes were consistent with results of MRI performed on 29th Jan 2013, 30th Jan 2013, 13th Feb 2013 and 17th Feb 2013 in other hospitals. 2. Local soft tissue in the right inguinal region showed slight swelling compared with the left side, with increased FDG uptake. Please combine with clinical observation. 3. A small amount of pelvic fluid. For treatment, the patient received intravenous injection of human immunoglobulin (10 mg, once daily), prednisolone acetate tablets, 30 mg, once daily as previous. On discharge on 4th Mar 2013 afer 12 days treatment ,t he patient’s symptoms and signs were the same as those on admission.On 5th Mar 2013, the patient was transferred to the neurosurgery department in our hospital. Physical examination on admission: muscle strength of the right upper limb was grade II, right lower limb grade II+, left upper limb grade III, and left lower limb grade III+. Patellar tendon r eflex and achilles tendon reflex were normal. Bilateral Babinski’s sign and Kernig’s sign were positive. The others were normal. After admission, cranial MRI + cervical and thoracic MRI (enhanced) were performed (8th Mar 2013): multiple abnormal signals in the left cerebellar hemisphere, medulla oblongata and upper end of cervical cord, multiple abnormal enhancements on arachnoid membrane; inflammatory changes were considered possible. There were multiple occupying lesions in the cervical cord, with nature to be determined . The thoracic cord showed no significant abnormality. Lumbar MRI plain scan plus enhancement did not show significant abnormality. There was a cyst in sacral canal at S2. PET-CT (11th Mar 2013) showed: 1. Multiple lesions with increased FDG metabolism in the left cerebellum, medulla oblongata and spinal cord;2. FDG metabolism was increased in bilateral pharynx oralis and laryngopharynx;3. PET-CT imaging did not show significant abnormality in the neck, thorax, abdomen, pelvis and bones. Blood routine, blood sedimentation, hepatitis 10 items, tumor markers, coagulation, blood glucose, urine routine, HIV, liver function, renal function, electrolytes, procalcitonin, C reactive protein and interleukin 6 were normal. Tuberculosis antibody was negative. After city-wide consultation, the patient continued to receive oral hormone (with gradual dose reduction), diagnostic anti-tuberculosis treatment (isoniazid tablets, 230 mg, oral, once daily; rifapentine capsules, 0.3 g, oral, twice weekly; ethambutol tablets, 0.5 g, oral, once daily; pyrazinamide tablets, 0.125 g, oral, 3 times daily) and acupuncture rehabilitation. The symptoms and signs were not significantly improved.On 23rd Mar 2013, the patient was transferred to the neurology department in our hospital. Examination of cerebrospinal fluid (25th Mar 2013): initial pressure 100 mmH2O. 15 ml was taken for various examinations (routine + biochemistry, 3 types of staining, immunoglobulin + complement, cytological examination of cerebrospinal fluid, direct detection of Mycobacterium tuberculosis (MTD) and oligoclonal band) and end pressure was 80 mmH2O. Cerebrospinal fluid routine + biochemistry: cerebrospinal fluid was colorless and transparent. Pandy test was positive,protein 3.23 g/L, white blood cell 0, glucose 0.70 mmol/L (concurrent blood glucose 4.9 mmol/L), chlorine 122.6 mmol/L; immunoglobulin (cerebrospinal fluid): IgG 0.57 g/L, IgM 0.01 g/L, IgA0.04 g/L, complement C3 0.06 g/L, IgE 5 IU/ml; immunoglobulin (blood): IgG 12.70 g/L, IgM1.20 g/L, IgA 1.46 g/L, complement C3 1.24 g/L, IgE 111 IU/ml; cerebrospinal fluid and blood did not show oligoclonal band. Abdominal ultrasound: 2-D and color Doppler ultrasound examination of liver, gall bladder, pancreas, spleen and both kidneys did not show abnormality. Urinary bladder ultrasound: size of the bladder was 6.4cm×6.4cm×8cm. The bladder was excessively filled. The wall was rough. On transverse section of the bladder, dense spots with strong echo were observed in the lower part, with area of 4.2cm×1.2cm. There were also floating spots with strong echo in the cavity. No significant parenchymal occupying lesion was observed in the pelvic cavity. There was deposition in the cavity. Treatment regimen: weight-based isoniazid tablets + rifampicin capsules + ethambutol tablets + pyrazinamide tablets for anti-tuberculosis, dexamethasone for anti-inflammation, symptomatic and supportive treatments till now.。