J Korean Neurosurg Soc.  2014 Jun;55(6):370-374. 10.3340/jkns.2014.55.6.370.

A Case of Cauda Equina Syndrome in Early-Onset Chronic Inflammatory Demyelinating Polyneuropathy Clinically Similar to Charcot-Marie-Tooth Disease Type 1

• Lee SE
• Park SW
• Ha SY
• Nam TK

• KMID: 2191120
• DOI: http://doi.org/10.3340/jkns.2014.55.6.370

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.

Keyword

Cauda equina syndrome; Chronic inflammatory demyelinating polyneuropathy; Charcot-Marie-Tooth disease; Laminectomy

MeSH Terms

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
Immunoglobulin G

Figure

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  Fig. 1 The patient's right foot shows deformities of pes cavus and hammertoes.

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  Fig. 2 Preoperative MRI of the lumbar spine, showing thickening of nerve roots of cauda equina. T1-weighted (A), T2-weighted (B), and post-contrast T1-weighted (C) sagittal image of MRI.

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  Fig. 3 Increased distal latency (arrow), temporal dispersion (arrow head) and conduction block (open arrow).

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  Fig. 4 The exposed nerve roots reveal diffuse enlargement with different sizes (A), decompressed spinal canal by laminectomy at L4-5 (B).

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  Fig. 5 The sural nerve biopsy shows large number of onion bulbs (arrows) (A), an electron micrograph shows typical configuration of an onion bulb, Schwann cell proliferation (open arrow) around a myelinated nerve fiber (B) (scale bar=20 µm).

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