Biopsy-Proven Small-Fiber Neuropathy in Primary Sjögren's Syndrome: Neuropathic Pain Characteristics, Autoantibody Findings, and Histopathologic Features.

OBJECTIVE: Painful small-fiber neuropathies (SFNs) in primary Sjögren's syndrome (SS) may present as pure or mixed with concurrent large-fiber involvement. SFN can be diagnosed by punch skin biopsy results that identify decreased intra-epidermal nerve-fiber density (IENFD) of unmyelinated nerves. METHODS: We compared 23 consecutively evaluated patients with SS with pure and mixed SFN versus 98 patients without SFN. We distinguished between markers of dorsal root ganglia (DRG) degeneration (decreased IENFD in the proximal thigh versus the distal leg) versus axonal degeneration (decreased IENFD in the distal leg versus the proximal thigh). RESULTS: There were no differences in pain intensity, pain quality, and treatment characteristics in the comparison of 13 patients with pure SFN versus 10 patients with mixed SFN. Ten patients with SFN (approximately 45%) had neuropathic pain preceding sicca symptoms. Opioid analgesics were prescribed to approximately 45% of patients with SFN. When compared to 98 patients without SFN, the 23 patients with SFN had an increased frequency of male sex (30% versus 9%; P < 0.01), a decreased frequency of anti-Ro 52 (P = 0.01) and anti-Ro 60 antibodies (P = 0.01), rheumatoid factor positivity (P < 0.01), and polyclonal gammopathy (P < 0.01). Eleven patients had stocking-and-glove pain, and 12 patients had nonstocking-and-glove pain. Skin biopsy results disclosed patterns of axonal (16 patients) and DRG injury (7 patients). CONCLUSION: SS SFN had an increased frequency among male patients, a decreased frequency of multiple antibodies, frequent treatment with opioid analgesics, and the presence of nonstocking-and-glove pain. Distinguishing between DRG versus axonal injury is significant, especially given that mechanisms targeting the DRG may result in irreversible neuronal cell death. Altogether, these findings highlight clinical, autoantibody, and pathologic features that can help to define mechanisms and treatment strategies.

Catheter-Based Transepidural Approach to Cervical and Thoracic Posterior and Perineural Epidural Spaces: A Cadaveric Feasibility Study.

BACKGROUND AND OBJECTIVES: Approaching the cervical and high thoracic level epidural space through transepidural route from lumbar region represents a method to lower the occurrence of complications associated with direct approach. The authors performed a cadaveric pilot project to determine the feasibility of various catheter-based manipulation and cephalad advancement using the transepidural route. STUDY DESIGN AND METHODS: Two cadavers were used to determine the following: 1. Ability to place a guide sheath over a guidewire using a percutaneous approach within the posterior lumbar epidural space; 2. The highest vertebral level catheter can be advanced within the posterior epidural space; 3. Ability to cross midline within the posterior epidural space; and 4. Ability to catheterize the perineural epidural sheaths of the nerve roots exiting at cervical and thoracic vertebral levels. RESULTS: We were able to advance the catheters up to the level of cervical vertebral level of C2 within the posterior epidural space under fluoroscopic guidance from a sheath inserted via oblique parasagittal approach at the lumbar L4-L5 intervertebral space. We were able to cross midline within the posterior epidural space and catheterize multiple perineural epidural sheaths of the nerve roots exiting at cervical vertebral level of C2, C3, and C4 on ipsilateral or contralateral sides. We also catheterized multiple epidural sheaths that surround the nerve roots exiting at the thoracic vertebral level on ipsilateral or contralateral sides. CONCLUSIONS: We were able to advance a catheter or microcatheter up to the cervical vertebral level within the posterior epidural space and catheterize the perineural epidural sheath of the nerve root exiting at cervical and thoracic vertebral levels. Such observations support further exploration of percutaneous catheter based transepidural approach to cervical and thoracic dorsal epidural spaces for therapeutic interventions.

Neuroanatomical correlates of atrial fibrillation: a longitudinal MRI study.

BACKGROUND AND PURPOSE: To determine baseline volume and rate of volume change of whole brain, hippocampus, and entorhinal cortex in patients with atrial fibrillation. METHODS: We analyzed clinical and neuroimaging data collected as part of Alzheimer's Disease Neuroimaging Initiative in the United States and Canada. Patients with atrial fibrillation were identified based on baseline clinical/cognitive assessments, and age and gender-matched controls without atrial fibrillations were selected (1:1 ratio). All participants underwent 1.5 T structural magnetic resonance imaging (MRI) at specified intervals (6 or 12 months) for 2-3 years. RESULTS: A total of 33 persons with atrial fibrillation were included. There was no difference in whole brain and ventricular volumes at baseline MRI between cases and controls. There was significantly lower entorhinal cortex volume on right (p = 0.01) and left (p = 0.01) sides in patients with atrial fibrillation. There was significantly lower volume for middle temporal lobes on right (p = 0.04) and left (p = 0.001) sides. The rate of progression of atrophy in entorhinal cortex and middle temporal lobes was not different between patients with atrial fibrillation and controls. CONCLUSIONS: The association of atrial fibrillation with volume loss in entorhinal cortex and middle temporal lobes may provide new insights into pathophysiology of atrial fibrillation.