A Case of Cauda Equina Syndrome in Early-Onset Chronic Inflammatory Demyelinating Polyneuropathy Clinically Similar to Charcot-Marie-Tooth Disease Type 1.
10.3340/jkns.2014.55.6.370
- Author:
Seung Eun LEE
1
;
Seung Won PARK
;
Sam Yeol HA
;
Taek Kyun NAM
Author Information
1. Department of Neurosurgery, Chung-Ang University Hospital, Chung-Ang University College of Medicine, Seoul, Korea. nspsw@cau.ac.kr
- Publication Type:Case Report
- Keywords:
Cauda equina syndrome;
Chronic inflammatory demyelinating polyneuropathy;
Charcot-Marie-Tooth disease;
Laminectomy
- MeSH:
Adult;
Biopsy;
Cauda Equina;
Charcot-Marie-Tooth Disease*;
Congenital Abnormalities;
Constipation;
Foot Deformities;
Humans;
Immunoglobulin G;
Laminectomy;
Low Back Pain;
Magnetic Resonance Imaging;
Nerve Fibers, Myelinated;
Neural Conduction;
Polyneuropathies*;
Polyradiculoneuropathy, Chronic Inflammatory Demyelinating;
Polyradiculopathy*;
Recurrence;
Schwann Cells;
Spinal Nerve Roots;
Spine;
Sural Nerve;
Urinary Incontinence
- From:Journal of Korean Neurosurgical Society
2014;55(6):370-374
- CountryRepublic of Korea
- Language:English
-
Abstract:
To present a case of cauda equina syndrome (CES) caused by chronic inflammatory demyelinating polyneuropathy (CIDP) which seemed clinically similar to Charcot-Marie-Tooth disease type1 (CMT1). CIDP is an immune-mediated polyneuropathy, either progressive or relapsing-remitting. It is a non-hereditary disorder characterized by symmetrical motor and sensory deficits. Rarely, spinal nerve roots can be involved, leading to CES by hypertrophic cauda equina. A 34-year-old man presented with low back pain, radicular pain, bilateral lower-extremity weakness, urinary incontinence, and constipation. He had had musculoskeletal deformities, such as hammertoes and pes cavus, since age 10. Lumbar spine magnetic resonance imaging showed diffuse thickening of the cauda equina. Electrophysiological testing showed increased distal latency, conduction blocks, temporal dispersion, and severe nerve conduction velocity slowing (3 m/s). We were not able to find genetic mutations at the PMP 22, MPZ, PRX, and EGR2 genes. The pathologic findings of the sural nerve biopsy revealed thinly myelinated nerve fibers with Schwann cells proliferation. We performed a decompressive laminectomy, intravenous IgG (IV-IgG) and oral steroid. At 1 week after surgery, most of his symptoms showed marked improvements except foot deformities. There was no relapse or aggravation of disease for 3 years. We diagnosed the case as an early-onset CIDP with cauda equine syndrome, whose initial clinical findings were similar to those of CMT1, and successfully managed with decompressive laminectomy, IV-IgG and oral steroid.
