Peripheral nervous system involvement in systemic sclerosis: the median nerve as target structure.
OBJECTIVE
To investigate the frequency and the main electrophysiological characteristics of the canalicolar passage nerve involvement in patients with systemic sclerosis (SSc).


METHODS
Thirty-two SSc patients were enrolled in the study, classified according to the type (diffuse or limited) and the duration (> / < 5 years) of the disease. Sensory-motor nerve conduction studies (NCS) of the upper and lower limbs, in particular at the critical canalicolar points, were conducted by recording the Compound Muscular Action Potential (CMAP) and the Sensory Action Potential (sNAP). The following parameters were evaluated: Motor Nerve Conduction Velocity (MNCV) and Sensory Nerve Conduction Velocity; distal and proximal latency of the CMAP and the onset and peak latency of the sNAP; peak-peak amplitude and negative-peak area of the CMAP and sNAP; and the Terminal Latency Index (TLI) (Terminal Distance/MCNV x Distal latency).


RESULTS
Four (12.5%) patients had a distal neuropathy of the upper limbs (one with monolateral and two with bilateral involvement of the median nerve and one bilateral involvement of the ulnar nerve). Fourteen (43.7%) patients showed a decrement of the median nerve TLI and seven (21.8%) of either the median or the ulnar nerve (Table I). Motor and sensitive conduction velocity and latency studies did not show a statistical difference between SSc patients and controls. The amplitude and area of the CMAP (distal and proximal), sNAP and of the median nerve TLI were significantly decreased in patients with respect to controls.


CONCLUSIONS
Distal mononeuropathy of the median nerve was the most frequent result in our patients. The involvement of the peripheral nervous system seems to be strictly topographical, following the modifications of the tissues and vascular tone (Raynaud's phenomenon) at the upper acral level. The neurophysiological alterations detected in our study at the wrist level may not be linked merely to a compressive event but also to microvascular involvement. Nerve involvement closely connected with the pathogenesis and distribution of SSc should be considered when peripheral nervous system involvement is the initial symptom of the disease.
Lori
S
S
Servizio di Neurofisiopatologia, USL 10, Florence, Italy.
Matucci-Cerinic
M
M
Casale
R
R
Generini
S
S
Lombardi
A
A
Pignone
A
A
Scaletti
C
C
Gangemi
P F
PF
Cagnoni
M
M
D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D004594:Electrophysiology / Q000379:methods; D019054:Evoked Potentials, Motor; D005260:Female; D006801:Humans; D008297:Male; D008475:Median Nerve / Q000503:physiopathology*; D008875:Middle Aged; D009431:Neural Conduction / Q000502:physiology*; D010523:Peripheral Nervous System Diseases / Q000209:etiology* / Q000503:physiopathology; D011597:Psychomotor Performance / Q000502:physiology*; D012595:Scleroderma, Systemic / Q000150:complications* / Q000503:physiopathology; D014459:Ulnar Nerve / Q000503:physiopathology