Chin R L, Sander H W, Brannagan T H, Green P H R, Hays A P, Alaedini A, Latov N
Peripheral Neuropathy Center, Department of Neurology and Neuroscience, Weill Medical College of Cornell University, New York, NY 10022, USA.
Neurology. 2003 May 27;60(10):1581-5. doi: 10.1212/01.wnl.0000063307.84039.c7.
Celiac disease (CD) is a chronic inflammatory enteropathy resulting from sensitivity to ingested gluten. Neurologic complications are estimated to occur in 10% of affected patients, with ataxia and peripheral neuropathy being the most common problems. The incidence and clinical presentation of patients with CD-associated peripheral neuropathy have not previously been investigated.
To determine the incidence of CD in patients with neuropathy and to characterize the clinical presentation.
The records of 20 patients with neuropathy and biopsy-confirmed CD were reviewed.
Six of the 20 patients had neuropathic symptoms alone without gastrointestinal involvement, and neuropathic symptoms preceded other CD symptoms in another 3 patients. All patients had burning, tingling, and numbness in their hands and feet, with distal sensory loss, and nine had diffuse paresthesias involving the face, trunk, or lumbosacral region. Only two had weakness. Results of electrophysiologic studies were normal or mildly abnormal in 18 (90%) of the patients. Sural nerve biopsies, obtained from three patients, revealed mild to severe axonopathy. Using the agglutination assay, 13 (65%) of the patients were positive for ganglioside antibodies. Excluding patients who were referred with the diagnosis of celiac neuropathy, CD was seen in approximately 2.5% of all neuropathy patients and in 8% of patients with neuropathy and normal electrophysiologic studies seen at our center.
CD is commonly associated with sensory neuropathy and should be considered even in the absence of gastrointestinal symptoms.
乳糜泻(CD)是一种由对摄入麸质敏感引起的慢性炎症性肠病。据估计,10%的受累患者会出现神经并发症,共济失调和周围神经病变是最常见的问题。此前尚未对CD相关性周围神经病变患者的发病率和临床表现进行过研究。
确定神经病变患者中CD的发病率,并描述其临床表现。
回顾了20例经活检确诊为CD的神经病变患者的病历。
20例患者中有6例仅出现神经病变症状,无胃肠道受累,另有3例患者神经病变症状先于其他CD症状出现。所有患者均有手足部灼痛、刺痛和麻木感,伴有远端感觉丧失,9例患者有累及面部、躯干或腰骶部的弥漫性感觉异常。只有2例患者有肌无力。18例(90%)患者的电生理检查结果正常或轻度异常。对3例患者进行的腓肠神经活检显示有轻度至重度轴索性神经病。采用凝集试验,13例(65%)患者的神经节苷脂抗体呈阳性。排除那些以乳糜泻性神经病变诊断转诊的患者,在所有神经病变患者中,CD的发生率约为2.5%,在本中心接受电生理检查正常的神经病变患者中,CD的发生率为8%。
CD常与感觉神经病变相关,即使在没有胃肠道症状的情况下也应考虑该病。