Myelopathy is a rare central nervous system (CNS) complication associated with systemic lupus erythematosus (SLE). Acute transverse myelitis (ATM) is the most frequent form of SLE-related myelopathy. Magnetic resonance imaging (MRI) typically shows increased signal intensity in T2-weighted images and cord swelling. In the present paper, we describe six cases of SLE-related myelopathy with multiple increased signals in the T2-weighted images involving continuous levels of the cervical and thoracic spinal cord, a distinctive feature recently named 'longitudinal myelitis'. The clinical and laboratory findings are similar to those presented by ATM patients, including paraparesis, sensory level and sphincter disturbances. Four patients had positive antiphospholipid antibodies (aPL) suggesting that this could be a characteristic of longitudinal myelitis. Treatment in all cases included high doses of corticosteroids and immunosuppressive agents (intravenous (i.v.) cyclophosphamide). Anticoagulation therapy was given to one patient and two others received low doses of aspirin. The outcome was mainly unfavorable with slow improvement in only one case, no improvement in two and relapse of the myelopathy in the remaining three. In conclusion, longitudinal myelitis is an unusual form of SLE-related myelopathy, it might be associated with aPL and it has a poor prognosis.

Neurophysiological tests of anorectal function can provide useful information regarding the integrity of neuronal innervation, as well as neuromuscular function. This information can give insights regarding the pathophysiological mechanisms that lead to several disorders of anorectal function, particularly fecal incontinence, pelvic floor disorders and dyssynergic defecation. Currently, several tests are available for the neurophysiological evaluation of anorectal function. These tests are mostly performed on patients referred to tertiary care centers, either following negative evaluations or when there is lack of response to conventional therapy. Judicious use of these tests can reveal significant and new understanding of the underlying mechanism(s) that could pave the way for better management of these disorders. In addition, these techniques are complementary to other modalities of investigation, such as pelvic floor imaging. The most commonly performed neurophysiological tests, along with their indications and clinical utility are discussed. Several novel techniques are evolving that may reveal new information on brain-gut interactions.

Background/Aim Brain-gut dysfunction has been implicated in gastrointestinal disorders but a comprehensive test of brain-gut axis is lacking. We developed and tested a novel method for assessing both afferent anorectal-brain function using cortical evoked potentials (CEP), and efferent brain-anorectal function using motor evoked potentials (MEP). Methods CEP was assessed following electrical stimulations of anus and rectum with bipolar electrodes in 26 healthy subjects. Anorectal MEPs were recorded following transcranial magnetic stimulation (TMS) over paramedian motor cortices bilaterally. Anal and rectal latencies/amplitudes for CEP and MEP responses and thresholds for first sensation and pain (mA) were analyzed and compared. Reproducibility and interobserver agreement of responses were examined. Results Reproducible polyphasic rectal and anal CEPs were recorded in all subjects, without gender differences, and with negative correlation between BMI and CEP amplitude (r −0.66, p=0.001). TMS evoked triphasic rectal and anal MEPs, without gender differences. Reproducibility for CEP and MEP was excellent (CV <10%). The inter-rater CV for anal and rectal MEPs was excellent (ICC 97–99), although there was inter-subject variation. Conclusions Combined CEP and MEP studies offer a simple, inexpensive and valid method of examining bidirectional brain-anorectal axes. This comprehensive method could provide mechanistic insights into lower gut disorders.

Desire to defecate is associated with a unique anal contractile response, the sensorimotor response (SMR). However, the precise muscle(s) involved is not known. We aimed to examine the role of external and internal anal sphincter and the puborectalis muscle in the genesis of SMR. Anorectal 3-D pressure topography was performed in 10 healthy subjects during graded rectal balloon distention using a novel high-definition manometry system consisting of a probe with 256 pressure sensors arranged circumferentially. The anal pressure changes before, during, and after the onset of SMR were measured at every millimeter along the length of anal canal and in 3-D by dividing the anal canal into 4 ϫ 2.1-mm grids. Pressures were assessed in the longitudinal and anterior-posterior axis. Anal ultrasound was performed to assess puborectalis morphology. 3-D topography demonstrated that rectal distention produced an SMR coinciding with desire to defecate and predominantly induced by contraction of puborectalis. Anal ultrasound showed that the puborectalis was located at mean distance of 3.5 cm from anal verge, which corresponded with peak pressure difference between the anterior and posterior vectors observed at 3.4 cm with 3-D topography (r ϭ 0.77). The highest absolute and percentage increases in pressure during SMR were seen in the superior-posterior portion of anal canal, reaffirming the role of puborectalis. The SMR anal pressure profile showed a peak pressure at 1.6 cm from anal verge in the anterior and posterior vectors and distinct increase in pressure only posteriorly at 3.2 cm corresponding to puborectalis. We concluded that SMR is primarily induced by the activation and contraction of the puborectalis muscle in response to a sensation of a desire to defecate. anal manometry; puborectalis IN HUMANS, BALLOON DISTENTION of the rectum normally induces the recto-anal inhibitory reflex (RAIR) (6, 11) as well as the recto-anal contractile reflex (RACR) (9, 10, 21). RAIR is an enteric neuronal reflex that is mediated by nitric oxide, vasoactive intestinal peptide, and adenosine triphosphate and causes relaxation of internal anal sphincter (IAS) (18). The rate and method of rectal distention (intermittent vs. ramp) affect the properties of the RAIR, and this reflex response is classically absent in Hirschsprung's disease and other conditions such as after circular rectal myotomy and after lower anterior resection (4,12,22).In addition to the aforementioned reflexes, recently, we have described the sensorimotor response (SMR) (5) that is also seen during balloon distention of the rectum. The SMR is a transient anal contraction that is usually seen overlying the initial relaxation phase of the RAIR (5). In healthy subjects, it coincides with the onset of a sensation for defecation (5). A recent study showed that the SMR was abnormal in patients with rectal hyposensitivity, suggesting a pathophysiological role for this response in anorectal disorders (19). However, the precise origin of the anal contractile response, in particular...