Chronic immune sensory polyradiculopathy: A possibly treatable sensory ataxia

M. Sinnreich, Christopher Jon Klein, J. R. Daube, J. Engelstad, R. J. Spinner, P. James B Dyck

Research output: Contribution to journalArticle

126 Citations (Scopus)

Abstract

Background: Chronic inflammatory neuropathies can present with a sensory ataxia due to involvement of dorsal root ganglia (DRG) or sensory nerves. Selective inflammatory involvement of sensory nerve roots proximal to the DRG has been postulated. Methods: The authors identified 15 patients with a sensory syndrome and normal nerve conduction studies. Sensory nerve root involvement was suggested by either somatosensory evoked potential (SSEP) or imaging abnormalities. CNS disease was excluded. Results: All patients had gait ataxia, large fiber sensory loss, and paresthesias, and nine had frequent falls. The disease course was chronic and progressive (median duration 5 years, range 3 months to 18 years). Sural sensory nerve action potential amplitudes were preserved and SSEP abnormalities were consistent with sensory nerve root involvement. Five patients had enlargement of lumbar nerve roots on MRI with enhancement in three. The CSF protein was elevated in 13 of 14 patients tested. Three patients had lumbar sensory rootlet biopsies that showed thickened rootlets, decreased density of large myelinated fibers, segmental demyelination, onion-bulb formation, and endoneurial inflammation. Six patients who required aids to walk were treated with immune modulating therapy and all had marked improvement with four returning to normal ambulation. Conclusion: Based on the described clinical features, normal nerve conduction studies, characteristic somatosensory evoked potential (SSEP) abnormality, enlarged nerve roots, elevated CSF protein, and inflammatory hypertrophic changes of sensory nerve rootlet tissue, we suggest the term chronic immune sensory polyradiculopathy (CISP) for this syndrome. This condition preferentially affects large myelinated fibers of the posterior roots, may respond favorably to treatment, and may be a restricted form of chronic inflammatory demyelinating polyradiculoneuropathy.

Original languageEnglish (US)
Pages (from-to)1662-1669
Number of pages8
JournalNeurology
Volume63
Issue number9
StatePublished - Nov 9 2004

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Polyradiculopathy
Ataxia
Somatosensory Evoked Potentials
Neural Conduction
Spinal Ganglia
Gait Ataxia
Chronic Inflammatory Demyelinating Polyradiculoneuropathy
Nerve Tissue
Sural Nerve
Onions
Paresthesia
Central Nervous System Diseases
Demyelinating Diseases
Action Potentials
Walking
Proteins
Inflammation
Biopsy
Therapeutics

ASJC Scopus subject areas

  • Neuroscience(all)

Cite this

Sinnreich, M., Klein, C. J., Daube, J. R., Engelstad, J., Spinner, R. J., & Dyck, P. J. B. (2004). Chronic immune sensory polyradiculopathy: A possibly treatable sensory ataxia. Neurology, 63(9), 1662-1669.

Chronic immune sensory polyradiculopathy : A possibly treatable sensory ataxia. / Sinnreich, M.; Klein, Christopher Jon; Daube, J. R.; Engelstad, J.; Spinner, R. J.; Dyck, P. James B.

In: Neurology, Vol. 63, No. 9, 09.11.2004, p. 1662-1669.

Research output: Contribution to journalArticle

Sinnreich, M, Klein, CJ, Daube, JR, Engelstad, J, Spinner, RJ & Dyck, PJB 2004, 'Chronic immune sensory polyradiculopathy: A possibly treatable sensory ataxia', Neurology, vol. 63, no. 9, pp. 1662-1669.
Sinnreich M, Klein CJ, Daube JR, Engelstad J, Spinner RJ, Dyck PJB. Chronic immune sensory polyradiculopathy: A possibly treatable sensory ataxia. Neurology. 2004 Nov 9;63(9):1662-1669.
Sinnreich, M. ; Klein, Christopher Jon ; Daube, J. R. ; Engelstad, J. ; Spinner, R. J. ; Dyck, P. James B. / Chronic immune sensory polyradiculopathy : A possibly treatable sensory ataxia. In: Neurology. 2004 ; Vol. 63, No. 9. pp. 1662-1669.
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