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Journal List > Ann Clin Neurophysiol > v.21(1) > 1121234

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

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.

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References

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acn-21-61f1.tif
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.
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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
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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