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慢性炎症性脱髓鞘性多发性神经根神经病患者因神经根明显肿大导致的脑积水。

Hydrocephalus due to marked enlargement of spinal roots in a patient with chronic inflammatory demyelinating polyradiculoneuropathy.

机构信息

Department of Neurology, Dokkyo Medical University, Tochigi, Japan.

出版信息

Eur J Neurol. 2020 Nov;27(11):2385-2388. doi: 10.1111/ene.14492.

DOI:10.1111/ene.14492
PMID:32853462
Abstract

BACKGROUND

Hydrocephalus or papilledema has rarely been reported in patients with chronic inflammatory demyelinating polyradiculoneuropathy (CIDP).

METHODS

We report a 65-year-old woman with a 12-year history of CIDP presenting with progressive dementia, hallucination and deterioration of gait.

RESULTS

Neurological examination revealed cognitive impairment, symmetric proximal and distal weakness with areflexia and muscle atrophy in the distal four limbs. The cerebrospinal fluid examination showed marked elevation of protein concentration. Magnetic resonance imaging revealed hydrocephalus and marked enlarged cervical and lumbar roots and plexus. The cervical cord and cauda equina were compressed by the swollen roots. A ventriculoperitoneal shunt resulted in reduction of the ventricles size along with improvement of her cognitive impairment.

CONCLUSION

In our patient with CIDP, hydrocephalus was likely caused by hypertrophic nerve roots. Our findings suggest that CIDP patients with pronounced hypertrophic nerve roots require careful observation.

摘要

背景

慢性炎症性脱髓鞘性多发性神经病(CIDP)患者很少出现脑积水或视乳头水肿。

方法

我们报告了一例 65 岁女性,患有 CIDP12 年,表现为进行性痴呆、幻觉和步态恶化。

结果

神经系统检查显示认知障碍、四肢远近端对称无力、反射消失和肌肉萎缩。脑脊液检查显示蛋白浓度显著升高。磁共振成像显示脑积水和明显增大的颈、腰神经根和丛。肿胀的神经根压迫颈髓和马尾。脑室-腹腔分流术使脑室缩小,认知障碍改善。

结论

在我们的 CIDP 患者中,脑积水可能是由肥大的神经根引起的。我们的发现表明,有明显肥大神经根的 CIDP 患者需要密切观察。

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