The acute sensory neuronopathy syndrome: A distinct clinical entity

Abstract

Four to twelve days following initial antibiotic treatment for a febrile illness, three adults suddenly experienced numbness and pain over the face and entire body. Each had received a penicillin or a semisynthetic derivative, and two patients also received other antibiotics. Signs appeared rapidly and included profound sensory ataxia, areflexia, and widespread sensory loss, primarily of large fiber modalities (proprioceptive sensibility). Slowed or absent sensory conduction was found. There was no weakness, and electrical study of muscle and motor nerve conduction was normal in all. The cerebrospinal fluid was acellular, and protein levels were elevated to 126 and 175 mg/dl in two cases and were normal in the other. Presently, all have a severe, static, residual sensory deficit. During follow-up of five years, no evidence of neoplastic disease or immunological disorder has appeared. Because of the rapid onset, widespread and pure sensory involvement, and poor recovery, the lesion is most likely confined to the dorsal root and gasserian ganglia (sensory neuronopathy). This pattern resembles that of the experimental lesions induced by doxorubicin and pyridoxine. It appears likely that either the previously administered antibiotics or the illness for which they were administered were of pathogenetic importance. We designate this previously unrecognized disorder the acute sensory neuronopathy syndrome and suggest that it represents a distinct, readily identifiable clinical entity.

Somatotopic Organization of Gentle Touch Processing in the Posterior Insular Cortex

“…The Ethical Review Board at the University of Gothenburg approved the study, and the experiments were performed in accordance with the Declaration of Helsinki. Informed consent was obtained from one subject (GL, age 56, right handed, female) with sensory neuronopathy syndrome (Sterman et al, 1980) and from six healthy subjects (aged 22-28 years, all right handed, three male).…”

“…Such stimulation of the skin invariably coactivates thick, myelinated fibers, preventing the selective study of CT afferents in healthy subjects. CT system physiology has, nevertheless, been successfully explored in a patient (GL) with neuronopathy syndrome (Sterman et al, 1980), who lacks large myelinated afferents but whose C fibers are intact (Olausson et al, 2002(Olausson et al, , 2008a. Despite asserting no touch sensation below the level of the nose in daily life, GL can, under focused concentration, detect the soft stroking of a brush and reports a vague pleasant sensation in response to the stimuli.…”

Machine Learning for Functional Brain Mapping

“…The C-tactile system is difficult to study in relation to brain processing, mainly since it cannot be activated selectively -any mechanical stimulation of the skin invariably co-activates thick, myelinated fibers in healthy subjects. C-tactile physiology has, nevertheless, been successfully explored in a patient (GL) with neuronopathy syndrome Sterman et al (1980), who lacks large myelinated Aβ afferents but whose C fibers are intact Olausson, Cole, Rylander, McGlone, Lamarre, Wallin, Krämer, Wessberg, Elam, Bushnell & Vallbo (2008); Olausson et al (2002). Due to the lack of Aβ fibers, GL has no sensation of touch.…”

“…Participants: Informed consent was obtained from six healthy subjects, as well as one subject (GL) with sensory neuronopathy syndrome Sterman et al (1980). At the age of 31, GL suffered permanent loss specifically of thick myelinated afferents (the so called Aβ fibers), leaving unmyelinated and small-diameter myelinated afferents intact Forget & Lamarre (1995).…”

Somatosensory Physiology Designated Lecture

“…The acute sensory neuronopathy syndrome (a crossreaction between an antibody raised to an infection and the large sensory myelinated neurones in the dorsal root ganglia), leaves the patient permanently without the perceptions of touch and movement/joint position sense below the neck (Sterman et al 1980). Those nerves concerned with pain, temperature and muscle fatigue and the motor nerves are clinically unaffected.…”