ABSTRACT
We report a 13 year-old female child with a idiopathic acute sensory neuronopathy mimicking a sensory form of Guillain-Barr syndrome, identified with electrodiagnosis and spine MRI. Motor conduction results were normal, but sensory nerve action potentials were not evoked in all four extremities. On MRI of the whole spine, the diffuse gadolinium-enhancement of dorsal roots in the spinal canal was detected without evidence of intramedullary lesion. The clinical symptoms and electrodiagnostic abnormalities had persisted for more than 18 months follow-up.
Subject(s)
Adolescent , Child , Female , Humans , Action Potentials , Electrodiagnosis , Extremities , Follow-Up Studies , Ganglia, Spinal , Magnetic Resonance Imaging , Spinal Canal , Spinal Nerve Roots , SpineABSTRACT
We have had an opportunity to study a patient with acute sensory neuronopathy. The patient was a 32-yearold housewife; the rapidly spreaded tingling sensation along both arms and legs developed, rendering her severely ataxic. There was no history of antecedent illness, familial neurological disease, or exposure to toxins and special drugs. On examinations, there was no abnormality in her mental and cranial nerve function. There was no motor weakness. She showed the profound loss of kinesthetic sense which was acutely progressive and associated with severe sensory ataxia and pseudoathetosis. All tendon reflexes were absent. However, cutaneous senses were preserved. There was no significant abnormal laboratory finding except elevated CSF protein content. On electrophysiologic findings, the decrease in the amplitude of action potentials with only mild slowing of conduction velocities of sensory nerves were found even though motor nerve conduction studies were normal. Median and tibial somatosensory evoked potentials could be elicited, although the median N19 scalp response and tibial N45 waveforms were prolonged in latency. Plasmapheresis were provided; clinical features improved. However, the electrophy-siological abnormalites remained. Thus we wish to report an additional case of woman suffering from the acute sensory neuronopathy, complementing the cases described by Stemm, Schaumburg and Asbury.