Acute sensory neuronopathy with preserved SEPs and long-latency reflexes.
A case of spontaneous acute sensory neuronopathy is described in a previously healthy 19-year-old male. He had marked sensory ataxia with relative sparing of cutaneous sensation. There was no recovery over 4 years. Sural nerve biopsy showed modest axonal loss. Sensory evoked potentials were mildly abnormal in the face of absent sensory nerve action potentials. The long-latency (R2) reflex was preserved whereas the short-latency (R1) reflex was absent. The findings indicate that loss of large-diameter (group Ia) ganglion cells are primarily responsible for this syndrome.
Knazan
M
M
Department of Medicine, University of British Columbia, Vancouver, Canada.
Bohlega
S
S
Berry
K
K
Eisen
A
A
D000208:Acute Disease; D000328:Adult; D005073:Evoked Potentials, Somatosensory; D005727:Ganglia, Spinal / Q000503:physiopathology; D006181:H-Reflex / Q000502:physiology; D006801:Humans; D008297:Male; D009475:Neurons, Afferent / Q000502:physiology*; D010523:Peripheral Nervous System Diseases / Q000503:physiopathology*; D012026:Reflex, Stretch / Q000502:physiology*; D013497:Sural Nerve / Q000503:physiopathology