Ann Rehabil Med.  2014 Aug;38(4):568-574. 10.5535/arm.2014.38.4.568.

Medical Management for Intractable Pain Arising From Primary Sjogren Syndrome Involving Both Brain and Spinal Cord: A Case Report

• Lee KM
• Han KY
• Kwon OP

• KMID: 2165742
• DOI: http://doi.org/10.5535/arm.2014.38.4.568

Abstract

Primary Sjogren syndrome, which involves lesions in both the brain and spinal cord, is rarely reported. Related symptoms, such as intractable pain due to central nervous system involvement, are very rare. A 73-year-old woman diagnosed with primary Sjogren syndrome manifested with subacute encephalopathy and extensive transverse myelitis. She complained of severe whole body neuropathic pain. Magnetic resonance imaging demonstrated a non-enhancing ill-defined high intensity signal involving the posterior limb of the both internal capsule and right thalamus on a T2 fluid-attenuated inversion recovery image. Additionally, multifocal intramedullary ill-defined contrast-enhancing lesion with cord swelling from the C-spine to L-spine was also visible on the T2-weighted image. Her intractable pain remarkably improved after administration of concomitant oral doses of gabapentin, venlafaxine, and carbamazepine.

Keyword

Sjogren's syndrome; Extensive transverse myelitis; Neuropathic pain

MeSH Terms

Aged
Brain*
Carbamazepine
Central Nervous System
Extremities
Female
Humans
Internal Capsule
Magnetic Resonance Imaging
Myelitis, Transverse
Neuralgia
Pain, Intractable*
Sjogren's Syndrome*
Spinal Cord*
Thalamus
Venlafaxine Hydrochloride
Carbamazepine

Figure

• 

  Fig. 1 Magnetic resonance images of the brain and spinal cord. (A) T2 fluid-attenuated inversion recovery axial imaging demonstrated a non-enhancing ill-defined high intensity signal involving the posterior limb of the both internal capsule and right thalamus (arrow). (B-D) T2-weighted sagittal imaging demonstrated multifocal intramedullary ill-defined contrast-enhancing lesion with cord swelling from the C-spine to the L-spine (arrows).

• 

  Fig. 2 Magnetic resonance images of the brain. (A, B) T2 fluid-attenuated inversion recovery (FLAIR) axial imaging demonstrated a newly-appeared nodular high intensity signal involving the right middle cerebellar peduncle and left side of medulla (arrows). (C) T2 FLAIR axial imaging demonstrated a decreased size and less prominent patchy intensity signal involving the posterior limb of the right internal capsule (arrow).

Reference

1. Govoni M, Padovan M, Rizzo N, Trotta F. CNS involvement in primary Sjögren's syndrome: prevalence, clinical aspects, diagnostic assessment and therapeutic approach. CNS Drugs. 2001; 15:597–607. PMID: 11524032.

2. Choi HJ, Shin K, Kang EH, Im CH, Lee YJ, Lee EB, et al. Primary Sjögren's syndrome presenting as acute transverse myelitis. Korean J Med. 2005; 68:463–466.

3. Kim HA, Ma EJ, Lee J, Ha CK. The Primary Sjögren's syndrome involving central and peripheral nervous system. J Korean Neurol Assoc. 2007; 25:108–111.

4. Tennant F, Hermann L. Intractable or chronic pain: there is a difference. West J Med. 2000; 173:306. PMID: 11069861.

5. Dworkin RH, O'Connor AB, Backonja M, Farrar JT, Finnerup NB, Jensen TS, et al. Pharmacologic management of neuropathic pain: evidence-based recommendations. Pain. 2007; 132:237–251. PMID: 17920770.

6. Vitali C, Bombardieri S, Jonsson R, Moutsopoulos HM, Alexander EL, Carsons SE, et al. Classification criteria for Sjögren's syndrome: a revised version of the European criteria proposed by the American-European Consensus Group. Ann Rheum Dis. 2002; 61:554–558. PMID: 12006334.

7. Delalande S, de Seze J, Fauchais AL, Hachulla E, Stojkovic T, Ferriby D, et al. Neurologic manifestations in primary Sjögren syndrome: a study of 82 patients. Medicine (Baltimore). 2004; 83:280–291. PMID: 15342972.

8. Finnerup NB, Otto M, McQuay HJ, Jensen TS, Sindrup SH. Algorithm for neuropathic pain treatment: an evidence based proposal. Pain. 2005; 118:289–305. PMID: 16213659.

9. Hanna M, O'Brien C, Wilson MC. Prolonged-release oxycodone enhances the effects of existing gabapentin therapy in painful diabetic neuropathy patients. Eur J Pain. 2008; 12:804–813. PMID: 18262450.

10. Zin CS, Nissen LM, O'Callaghan JP, Duffull SB, Smith MT, Moore BJ. A randomized, controlled trial of oxycodone versus placebo in patients with postherpetic neuralgia and painful diabetic neuropathy treated with pregabalin. J Pain. 2010; 11:462–471. PMID: 19962354.