Patterns of sensory nerve conduction abnormalities in Fisher syndrome: More predominant involvement of group Ia afferents than skin afferents

• Published in: Clinical neurophysiology Vol. 124; no. 7; pp. 1465 - 1469
• Main Authors: Sekiguchi, Yukari, Misawa, Sonoko, Shibuya, Kazumoto, Mitsuma, Satsuki, Hirano, Shigeki, Ohmori, Shigeki, Koga, Shunsuke, Iwai, Yuta, Beppu, Minako, Kuwabara, Satoshi
• Format: Journal Article
• Language: English
• Published: Netherlands Elsevier Ireland Ltd 01.07.2013
• Subjects: Adolescent; Adult; Age Factors; Aged; Aged, 80 and over; Electric Stimulation; Evoked Potentials, Somatosensory - physiology; Fisher syndrome; Group Ia afferent; H-Reflex - physiology; Humans; Middle Aged; Miller Fisher Syndrome - pathology; Miller Fisher Syndrome - physiopathology; Nerve Fibers, Myelinated - physiology; Neural Conduction - physiology; Neurology; Sensory nerve conduction; Skin - innervation; Skin afferent; Statistics, Nonparametric; Young Adult; Sensory nerve conduction; Fisher syndrome; Skin afferent; Group Ia afferent
• ISSN: 1388-2457; 1872-8952; 1872-8952
• DOI: 10.1016/j.clinph.2013.01.020

► This study elucidated electrophysiologic features of sensory nerve involvement in 47 patients with Fisher syndrome (FS). ► Major abnormalities included absent/reduced soleus H-reflexes with rarely reduced sensory nerve potentials, suggesting more predominant involvement of group Ia afferents than cutaneous afferents. ► Ataxia and areflexia in FS are presumably caused by immune attack by anti-GQ1b antibodies to group Ia neurons in the dorsal root ganglia in FS. To elucidate the features of sensory nerve involvement in Fisher syndrome (FS), this study extensively investigated sensory electrophysiology. In 47 consecutive FS patients, results of sensory nerve conduction studies in the median, ulnar and sural nerves, soleus H-reflexes, and median or tibial somatosensory-evoked potentials (SEP) were reviewed. Because of the large effects of age on amplitude of sensory nerve action potentials (SNAP), we strictly defined reduction of SNAP amplitudes by using a nomogram which age and amplitude obtained from 87normal subjects. In routine nerve conduction studies, SNAP amplitude was reduced only in 32% of the patients, and conduction velocity was decreased in 2%. In contrast, soleus H-reflexes were frequently absent or reduced (67%). SEPs were abnormal only in 17%. In FS, absent soleus H-reflexes are the most frequent electrophysiologic abnormalities, whereas SNAPs amplitudes are rarely affected. The pattern is characterized by predominant involvement of group Ia afferents with relatively preserved cutaneous afferents without evidence suggestive of demyelination. The major targets of immune attack by anti-GQ1b antibodies in FS appear to be group Ia neurons in the dorsal root ganglia, and this is presumably responsible for ataxia and areflexia in FS.