Brain Advance Access originally published online on June 8, 2009
Brain 2009 132(7):1723-1733; doi:10.1093/brain/awp136
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The pattern and diagnostic criteria of sensory neuronopathy: a case–control study
1 Centre de Référence Maladies Neuromusculaires Rares, Rhône-Alpes, France 2 Service de Neurologie. CHU de Saint-Etienne. France 3 Université de Saint-Etienne, France 4 Centre de Référence Maladies Rares "Syndromes neurologiques paranéoplasiques" 5 Inserm U842 Lyon-Saint-Etienne 6 Hôpital Neurologique, Hospices Civils de Lyon, France 7 Hôpital de la Croix-Rousse, Hospices Civils de Lyon, France 8 Université Lyon 1, Lyon, France
Correspondence to: Jean-Christophe Antoine, Service de Neurologie, CHU de Saint-Etienne, 42055 Saint-Etienne cedex 02, France E-mail: j.christophe.antoine{at}chu-st-etienne.fr
Acquired sensory neuronopathies encompass a group of paraneoplastic, dysimmune, toxic or idiopathic disorders characterized by degeneration of peripheral sensory neurons in dorsal root ganglia. As dorsal root ganglia cannot easily be explored, the clinical diagnosis of these disorders may be difficult. The question as to whether there exists a common clinical pattern of sensory neuronopathies, allowing the establishment of validated and easy-to-use diagnostic criteria, has not yet been addressed. In this study, logistic regression was used to construct diagnostic criteria on a retrospective study population of 78 patients with sensory neuronopathies and 56 with other sensory neuropathies. For this, sensory neuronopathy was provisionally considered as unambiguous in 44 patients with paraneoplastic disorder or cisplatin treatment and likely in 34 with a dysimmune or idiopathic setting who may theoretically have another form of neuropathy. To test the homogeneity of the sensory neuronopathy population, likely candidates were compared with unambiguous cases and then the whole population was compared with the other sensory neuropathies population. Criteria accuracy was checked on 37 prospective patients referred for diagnosis of sensory neuropathy. In the study population, sensory neuronopathy showed a common clinical and electrophysiological pattern that was independent of the underlying cause, including unusual forms with only patchy sensory loss, mild electrical motor nerve abnormalities and predominant small fibre or isolated lower limb involvement. Logistic regression allowed the construction of a set of criteria that gave fair results with the following combination: ataxia in the lower or upper limbs + asymmetrical distribution + sensory loss not restricted to the lower limbs + at least one sensory action potential absent or three sensory action potentials <30% of the lower limit of normal in the upper limbs + less than two nerves with abnormal motor nerve conduction study in the lower limbs.
Key Words: sensory neuronopathy; sensory ganglionopathy; sensory neuropathy; paraneoplastic neurological syndrome; cisplatin
Abbreviations: CSF, cerebrospinal fluid; ENMG, electroneuromyographic study; ROC, receiver operator characteristic; SAP, sensory action potentials; SNN, sensory neuronopathies
Received October 4, 2008. Revised December 29, 2008. Accepted April 21, 2009.
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