Ataxic sensory neuropathy and dorsal root ganglionitis associated with Sjögren's syndrome

J. W. Griffin, D. R. Cornblath, E. Alexander, J. Campbell, Phillip Anson Low, S. Bird, E. L. Feldman

Research output: Contribution to journalArticle

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Abstract

Thirteen patients, 11 women and 2 men, developed sensory and autonomic neuronopathies in association with features of primary Sjögren's syndrome. In 11, Sjögren's syndrome had not been previously diagnosed at the time of neurological presentation. All had prominent loss of kinesthesia and proprioception. Pain and thermal sensibility were less severely affected. Most had evidence of autonomic insufficiency. In some this was severe, with Adie's pupils, fixed tachycardia, and orthostatic hypotension. The course ranged from an abrupt, devastating onset to indolent progression over years. Stabilization or functional improvement occurred in 6 patients, 2 of whom received no drug therapy. Sensory nerve conduction studies and examination of nerve biopsy specimens demonstrated a wide spectrum in the severity of loss of large myelinated fibers. The cutaneous nerves of 6 patients had perivascular mononuclear infiltrates without necrotizing arteritis. Examination of biopsy specimens of dorsal root ganglia in 3 patients revealed lymphocytic (T-cell) infiltration in the dorsal roots and ganglia, with focal clusters around neurons. In the more mildly affected ganglia, individual sensory neurons were undergoing degeneration. In the most advanced case, very few neurons remained. The possibility of Sjogren's syndrome should be considered in patients, especially women, who develop acute, subacute, or chronic sensory and autonomic neuropathies, with ataxia and kinesthetic loss.

Original languageEnglish (US)
Pages (from-to)304-315
Number of pages12
JournalAnnals of Neurology
Volume27
Issue number3
StatePublished - Mar 1990

Fingerprint

Spinal Nerve Roots
Spinal Ganglia
Tonic Pupil
Kinesthesis
Biopsy
Neurons
Proprioception
Polyarteritis Nodosa
Orthostatic Hypotension
Sjogren's Syndrome
Neural Conduction
Sensory Receptor Cells
Ataxia
Tachycardia
Ganglia
Hot Temperature
T-Lymphocytes
Drug Therapy
Pain
Skin

ASJC Scopus subject areas

  • Neuroscience(all)

Cite this

Griffin, J. W., Cornblath, D. R., Alexander, E., Campbell, J., Low, P. A., Bird, S., & Feldman, E. L. (1990). Ataxic sensory neuropathy and dorsal root ganglionitis associated with Sjögren's syndrome. Annals of Neurology, 27(3), 304-315.

Ataxic sensory neuropathy and dorsal root ganglionitis associated with Sjögren's syndrome. / Griffin, J. W.; Cornblath, D. R.; Alexander, E.; Campbell, J.; Low, Phillip Anson; Bird, S.; Feldman, E. L.

In: Annals of Neurology, Vol. 27, No. 3, 03.1990, p. 304-315.

Research output: Contribution to journalArticle

Griffin, JW, Cornblath, DR, Alexander, E, Campbell, J, Low, PA, Bird, S & Feldman, EL 1990, 'Ataxic sensory neuropathy and dorsal root ganglionitis associated with Sjögren's syndrome', Annals of Neurology, vol. 27, no. 3, pp. 304-315.
Griffin JW, Cornblath DR, Alexander E, Campbell J, Low PA, Bird S et al. Ataxic sensory neuropathy and dorsal root ganglionitis associated with Sjögren's syndrome. Annals of Neurology. 1990 Mar;27(3):304-315.
Griffin, J. W. ; Cornblath, D. R. ; Alexander, E. ; Campbell, J. ; Low, Phillip Anson ; Bird, S. ; Feldman, E. L. / Ataxic sensory neuropathy and dorsal root ganglionitis associated with Sjögren's syndrome. In: Annals of Neurology. 1990 ; Vol. 27, No. 3. pp. 304-315.
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