A 22-year-old African American man presented to the ED, complaining chiefly of weakness on the left side of his body for the past month, which does not improve with rest. He also complained of numbness on the left side of his body, including the left side of his chest. He does not have any muscle pain. He mentioned that he has had balance problems for the last 2-3 months. He also complained of headache in the temporal area, nausea, and vertigo.

The man also complained of visual difficulty, including double vision and blurring of vision in the left eye. He denied having any dysphagia or dysarthria – he had no slurring of speech or bowel/bladder dysfunction. He did not have vomiting, rash, fever, chills, rigors, cough, shortness of breath, chest pain, palpitation, or abdominal pain.

The man had been seen in the emergency department 3 months prior, complaining of severe nausea and vertigo. At that time, a CT scan showed decreased attenuation along the right periventricular area. The radiologist recommended correlation with MRI to exclude acute infarction. However, at that time, the patient did not have any weakness and was treated symptomatically for the diagnosis of acute labyrinthitis. He is otherwise healthy, with no history of sexually transmitted diseases, known drug allergies, or family history of neurologic disease.

On physical exam, his vital signs were within normal limits. An eye exam revealed a normal reactive pupil and incomplete left homonymous hemianopia. Cranial nerves III, IV, V, VI, VII, IX, X, and XII were intact.

Rinne and Weber tests showed abnormal lateralization to the left with decreased bone and air conduction in the left ear. On the left upper and lower extremities, the patient had a 20% reduction in power and strength and decreased sensation to touch, pinprick, and temperature. His deep tendon reflexes were 2+ in both upper extremities and 3+ in both lower extremities, and his plantar reflexes were flexor bilaterally. He had dysmetria and past-pointing on his left side. The Romberg sign was positive, with a tendency to fall toward left. He also had an ataxic gait with limping of his left leg. Initial laboratory data showed mild hypokalemia and elevated creatinine phosphokinase. His blood chemistry was otherwise normal.

A CT scan of the head showed patchy areas of decreased attenuation involving the left frontal lobe and the periventricular region, including on the right side posteriorly and on the left side in the region of the left frontal lobe and the centrum semiovale. There was no evidence of acute infarction. The radiologist recommended that the possibility of vasculitis should be excluded with a follow-up MRI. Neurology was consulted to rule out vasculitic cause of the symptoms. The patient’s history and physical examination suggested findings consistent with upper motor neuron disease, and possible demyelination, according to attending neurologist Dr. Victor Jaramillo and neurology resident Dr. Ninad Parekh of Conemaugh Memorial Hospital, Johnstown, Pennsylvania. They also advised performing MRI to better assess the brain lesions.

Follow-up MRI findings met four of the McDonald MR imaging criteria for the diagnosis of multiple sclerosis. However, the multiple large lesions found on the brain MRI did not correlate with the very limited neurologic deficits that were detected on physical exam. For further assessment of spinal involvement, cervical and thoracic MRIs were obtained as well. The cervical MRI also showed multiple areas of increased signal, while the thoracic MRI appeared normal.

The recommended diagnostic criteria for multiple sclerosis, published as guidelines from the international panel on the diagnosis of multiple sclerosis – more commonly known as “McDonald criteria” – were formulated in 2001. These criteria were further modified in 2005 to simplify and increase rapidity of diagnosis of MS while at the same time maintaining adequate sensitivity and specificity.

According to the modified McDonald criteria, “the revised MRI criteria for dissemination in time are detection of gadolinium enhancement at least 3 months after the onset of the first clinical event or detection of a new T2 lesion appearing at any time, compared with a reference scan done at least 30 days after the onset of the initial clinical event” (Ann. Neurol. 2005;58:840-6). “The revised MRI criteria for dissemination in space are three of the following: (1) one or more gadolinium-enhancing lesions or nine T2 hyperintense lesions; (2) one or more infratentorial lesions; (3) one or more juxtacortical lesions; or (4) three or more periventricular lesions.”

The patient was started on a high dose of intravenous steroids (1,000 mg/day methylprednisolone) for 5 days, followed by tapering does of oral prednisone. His neurologic symptoms were completely resolved at the follow-up visit 15 days later. He had no neurologic symptoms at a subsequent visit at 2 months. Three months later he was seen in the ED for cervical strain and urticaria, but at that time he was not having any neurologic symptoms.

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一名22岁非洲裔美国人因左侧躯体无力数月到急诊室就诊，该症状休息后也无法得到缓解，另外患者还合并包括左胸在内的左侧躯体麻木。患者没有肌肉疼痛病史。在过去2~3个月内，患者还出现了平衡障碍，并经常出现颞叶部位疼痛、恶心、眩晕等不适。

 

追问病史，发现患者还合并有视力障碍，包括复视、左眼视物模糊等，但没有吞咽困难和构音障碍，即患者未曾出现说话含混不清或胃肠道症状。患者亦无呕吐、皮疹、发热、寒颤、咳嗽、呼吸急促、胸痛、心悸及腹痛等不适。

 

患者3个月前曾因严重恶心、眩晕到急诊就诊。当时头CT扫描先是侧脑室旁有低密度灶，故放射科医师建议完善头MRI检查以除外急性脑梗死。但患者当时无任何无力症状，故最后诊断为急性迷路炎并给予对症治疗。患者既往基本健康，否认有性病、药物过敏等病史，否认家族遗传性神经系统疾病病史。

 

体检显示，患者视力正常、瞳孔对光反射正常，但存在不完全的左侧偏盲。第Ⅲ、Ⅳ、Ⅴ、Ⅵ、Ⅶ、Ⅸ、Ⅹ及Ⅻ对脑神经均正常。

 

Rinne试验及Weber试验显示偏左，提示左耳气导和骨导降低。左侧上下肢肌力减退20%，左侧痛温觉、触觉降低。患者上肢腱反射正常，下肢腱反射偏活跃，跖反射两侧对称。患者左侧肢体辨距不良、指鼻不准；Romberg征阳性，向左侧歪斜。行走时患者为共济失调步态，左侧跛行。入院化验检查显示患者存在轻度低钾血症和肌酸激酶增高，其他血生化检查大致正常。

 

头CT显示左额叶、双侧侧脑室旁及左侧半卵圆中心区域斑片状低密度灶，没有急性脑梗死迹象。放射科医师认为有血管炎的可能，故建议神经内科完善头MRI检查以除外血管炎。经过美国宾夕法尼亚州约翰斯敦市Conemaugh纪念医院神经内科Victor Jaramillo教授及Ninad Parekh住院医师会诊后，考虑到该患者病史及体检支持上运动神经元病变、脱髓鞘病变可能性大。同时两位医师也建议做头MRI检查以更好地明确病灶情况。

 

进一步行头MRI检查显示，该患者头颅内病灶符合McDonald影像学改变的诊断标准。不过该患者MRI中多发性脑内大片病灶与给患者查体发现的非常有限的神经系统局灶性缺损症状不相符，故有必要进一步检查脊髓情况，包括颈髓和胸髓病变情况。颈部MRI检查显示亦发现有增强信号，而胸髓MRI检查正常。

 

多发性硬化的推荐诊断标准是由国际多发性硬化研究组制订并发表的诊断指南，即所谓的“McDonald”诊断标准，于2001年发表。随后为了简化诊断步骤、提高诊断速度，于2005年做了修改，但修改后的诊断标准并没有因此降低诊断敏感性和特异性。

 

根据修改后的McDonald诊断标准，“修改后对时间的多发性MRI标准定义为出现第一次临床症状或任何时间出现新的T2病灶至少3个月后发现有钆增强病灶，而既往规定出现第一次临床症状后至少30天后做比较扫描”（Ann Neurol. 2005,58:840-6）。“修改后对空间的多发性MRI标准定义为符合下面各项中的3项：(1)一个或多个钆增强病灶或9个T2高信号；(2)一个或多个幕下病灶；(3)一个或多个近皮层病灶；(4)3个或更多侧脑室周围病灶。”

 

给予该患者大剂量激素冲击治疗（1,000mg/d甲基强的松龙）5天，随后逐渐减量并给予强的松口服治疗。在随后的随访中，患者15天后神经系统症状完全改善，2个月随访时未查到有神经系统定位体征。三个月后患者再次来急诊室就诊，不过这次是因为颈部紧张和荨麻疹来诊，而神经系统查体亦未见定位体征。

 

图：McDonald诊断标准对多发性硬化病灶空间的多发性定义为一个或多个钆增强病灶。

 

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