Suk-Won Ahn; Byung-Nam Yoon

doi:10.14253/acn.2019.21.1.61

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Ahn and Yoon: Motor dominant polyradiculopathy with Primary Sjögren's syndrome mimicking motor neuron disease

Case Report

Ann Clin Neurophysiol 2019;21(1):61-65.

Published online: 20 January 2019

DOI: https://doi.org/10.14253/acn.2019.21.1.61

Motor dominant polyradiculopathy with Primary Sjögren's syndrome mimicking motor neuron disease

Suk-Won Ahn¹, Byung-Nam Yoon^2,

¹Department of Neurology, Chung-Ang University Hospital, Chung-Ang University College of Medicine, Seoul, Korea

²Department of Neurology, Seoul Paik Hospital, Inje University College of Medicine, Seoul, Korea

Correspondence to Byung-Nam Yoon Department of Neurology, Seoul Paik Hos-pital, Inje University College of Medicine, 9 Mareunnae-ro, Jung-gu, Seoul 04551, Korea Tel: +82-2-2270-0946 Fax: +82-2-2270–0045 E-mail: nrybn1230@gmail.com

Received 19 September 2018 Revised 11 December 2018 Accepted 25 December 2018

This is an Open Access article distributed under the terms of the Creative Commons Attribution Non-Commercial License (http://creativecommons.org/licenses/by-nc/4.0) which permits unrestricted non-commercial use, distribution, and reproduction in any medium, provided the original work is properly cited.

Abstract

Sjögren's syndrome (SS)-associated polyradiculopathy is rarely reported. A 51-year-old woman presented with a history of gradual weakness in all four extremities for several months. Based on electrophysiological studies, spinal magnetic resonance imaging and cerebrospinal fluid examination, inflammatory polyradiculopathy was confirmed. During a search for the aetiol-ogy, the patient was ultimately diagnosed with SS. This study introduces SS-associated poly-radiculopathy that primarily presented with motor symptoms, thus mimicking motor neuron disease.

Keywords: Polyradiculopathy, Sjögren's syndrome, Motor neuron disease

References

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Fig. 1.

Cervical and thoracic spinal MRI. A T2 sagittal image (A) and T1 gadolinium-enhanced sagittal image (B) show unremarkable findings. T1 gadolini-um-enhanced axial images at the (C) C4 level and (D) C6 level show enhancement of the ventral nerve rootletsof the ventral nerve rootlets (white arrows). MRI, magnetic resonance imaging.

Table 1.

The results of nerve conduction study

Nerve	Stimulation	Latency (msec)	Amp.	Velocity (m/sec)	F-latency (msec)
Motor
Lt. median	Wrist	3.13 (<3.6)	11.3 (>5.0)		24.7
	Elbow		10.7	58.5 (>50.0)
	Axilla		10.4	60.1 (>56.0)
Rt. median	Wrist	3.17 (<3.6)	5.4 (>5.0)		26.6
	Elbow		5.3	53.7 (>50.0)
	Axilla		5.3	64.5 (>50.0)
Lt. ulnar	Wrist	2.46 (<2.5)	8.8 (>5.0)		21.4
	Elbow		8.8	62.7 (>50.6)
	Axilla		8.5	75.2 (>52.7)
Rt. ulnar	Wrist	2.29 (<2.5)	6.1 (>5.0)		20.8
	Elbow		5.6	54.9 (>50.6)
	Axilla		5.4	59.9 (>52.7)
Lt. Axillary	Erb	3.05 (<5.4)	8.9 (>4.6)
Rt. Axillary	Erb	4.01 (<5.4)	5.4 (>4.6)
Lt. MC	Erb	4.11 (<5.6)	5.4 (>4.0)
Rt. MC	Erb	4.23 (<5.6)	4.2 (>4.0)
Lt. peroneal	Ankle	3.25 (<4.8)	5.1 (>4.0)		42.5
	Popliteal fossa		5.1	46.4 (>41.9)
Rt. peroneal	Ankle	4.04 (<4.8)	5.3 (>4.0)		45.0
	Popliteal fossa		4.4	45.9 (>41.9)
Lt. tibial	Ankle	3.21 (<5.1)	12.9 (>5.0)		46.1
	Fibular neck		8.7	45.3 (>40.6)
Rt. tibial	Ankle	3.96 (<5.1)	10.9 (>5.0)		45.0
	Fibular neck		6.9	46.2 (>40.6)
Sensory
Lt. median	Digit 2	2.48	27.2 (>10.0)	44.4 (>41.3)
Rt. median	Digit 2	2.54	21.3 (>10.0)	43.3 (>41.3)
Lt. ulnar	Digit 5	1.98	10.4 (>10.0)	45.5 (>39.3)
Rt. ulnar	Digit 5	1.85	34.3 (>10.0)	48.6 (>39.3)
Lt. sural	Calf	2.71	9.9 (>6.0)	51.7 (>34.7)
Rt. sural	Calf	2.85	21.0 (>6.0)	49.1 (>34.7)
H reflex Lt		26.6
Rt		29.3

Latencies are in milliseconds, amplitudes of compound muscle action potentials in millivolts. amplitudes of sensory nerve action potentials in microvolts. velocities in m/sec.

Amp, amplitude; Lt, left; Rt, right; MC, musculocutaneous.

Table 2.

The findings of electromyography

Muscle	Spontaneous activity				Voluntary contraction
Muscle	IA	Fibrillation	PSWs	Amplitude	Duration	Recruitment	IP
Lt. ABP	↑	+1	+1	NL	NL	NL	NL
Rt. ABP	↑	0	0	↑	↑	↓	↓
Lt. FDI	↑	+1	+1	NL	NL	NL	NL
Rt. FDI	↑	+1	+1	NL	NL	NL	NL
Lt. FCR	↑	0	0	NL	NL	NL	NL
Rt. FCR	↑	+1	+1	↑	↑	↓	↓
Lt. biceps brachii	↑	+1	+2	↑	↑	↓	↓
Rt. biceps barchii	↑	+2	+2	↑	↑	↓	↓
Lt. triceps brahii	↑	+1	+2	↑	↑	↓	↓
Rt. triceps brachii	↑	+2	+2	↑	↑	↓	↓
Lt. vastus lateralis	↑	+1	+1	↑	↑	↓	↓
Rt. vastus lateralis	↑	0	+1	↑	↑	↓	↓
Lt. TA	↑	0	0	↑	↑	↓	↓
Rt. TA	↑	0	0	↑	↑	↓	↓
Lt. GCM	↑	0	0	↑	↑	↓	↓
Rt. GCM	↑	0	0	↑	↑	↓	↓
Lt. paraspinal C5-C8, T1, T6	↑	+1	+1
Rt. paraspinal C5-C8, T1, T6	↑	+1	+1
Lt. paraspinal L4–5	↑	+1	+1
Rt. paraspinal L4–5	↑	+1	+1
Lt. paraspinal S1	NL	0	0
Rt. paraspinal S1	NL	0	0
Lt. masseter	NL	0	0	NL	NL	NL	NL
Lt. tongue	NL	0	0	NL	NL	NL	NL

IA, insertional activity; PSW, positive sharp wave; IP, interference pattern; Lt, left; ABP, abductor pollicis brevis; NL, normal; Rt, right; FDI, first dorsal interosse-ous; FCR, flexor carpi radialis; TA, tibialis anterior; GCM, gastrocnemius medialis.

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