0334 Neurologic manifestations of systemic lupus erythematosus: A focus on peripheral neuropathy

S184

Poster Abstracts

Monday, November 7, 2005

32% of patients, peroneal nerve amplitude or conduction velodty was impaired. In this study, the average of motor nerve distal latendes in patients was significantly higher than control group. (p - 0.008). Likewise, the mean sensory nerve conduction velocity in patients was significantly lower than control group (p - 0.006). Conclusion: In tiffs survey, the prevalence of peripheral neuropathy was higher in diabetic a patient which is an indicator of early damage of nerves among diabetic patients or it is consequence of delayed diagnosis of these patients. 0331 Desert Hedgelmg-Patdled2 Expression in Peripheral Nerves during Wallerian Degeneration and Regeneration

Bajestan, SN a, Umehara, F a, Itoh, K a, Sharghi-NamSni, S~, Jessen, K R ~, Mirsky, R ~, Osame, M ~. 1Department of Neurology

arid Geriatrics, Kagoshima University Graduate School of Medical arid Dental Sciences, Kagoshima, Japan; 2Department of Anatomy" arid Developmental Biology, University College London, London, UK Background: Desert hedgehog (Dhh) signaling pathway, which is mediated by Patched (Ptc) and Smoothened (Smo) receptors, is involved in the development of peripheral nerves. Recently, the vertebrate Ptc fanffly has been extended by the identification of Ptc2. Method: Using RT-PCR and immunolffstochemistry in sciatic nerves of adult mice and purifed cultures of mouse Schwann cells and fibroblasts, the expression of Ptc receptors were examined. After sciatic nerve crush injury, wild type CWT) and dhh-null nerves were analyzed morphologically at different time points and expression of dhh and related receptors was also measured by RT-PCR in WT mice. Results: Both dhh and ptc2 m R N A were expressed in adult mouse sciatic nerves butptel m R N A was undetectable. Pte2 m R N A was also found in purified cultures of mouse Schwann cells, and at much lower levels, in purified fibroblasts. Ptc2 protein was expressed in unmyelinated nerve fibers. After crush injury, degeneration of myelinated fibers was more severe dhh-null mice than in WT mice. Furthermore, in regenerated nerves of dhh-null mice, minifascicular fomlation was more extensive in than in dhh-null intact nerves. Dhh and pte2 m R N A expression showed a marked decline during the degenerating phase after injury in WT mice followed by a slow increase in the regenerating phase. The ptcl m R N A was undetectable in both injured and control nerves and the smo m R N A levels did not change after injury. Conclusion: These results suggest that the Dhh-Ptc2 signaling pathway may be involved in the maintenance of adult nerves and degeneration and regeneration process after injury. 0332 Tomacula Iormation precedes seginental deinyelination in an avian riboflavin deficiency Inodel Ca[, Z ~'2, Finnie, J~, Manavis, j1, Ghabriel, M N 3, Thompson, PD ~, Blumbergs, PC 1.2'4. 1Hanson Institute Centre for Neurological Diseases,

Institute of Medical arid Veterinary Science, Adelaide, Australia; 2Department of Neurology" arid University Department of Medicine, Royal Adelaide Hospital; XDepartrnent of Anatomy and," 4Department of Pathology, The University of Adelaide, Australia Background: Focal myelin swellings or tomacula have been found in many hmnan hereditary and non-hereditary neuropathies. Tile pathogenesis of tomacala formation is not well understood and the redundant myelin folds comprising tomacula are generally considered a remyelination phenomenon. This study aimed to investigate the relationship between tomacula formation and demyelination in an avian riboflavin deficiency model of primary segmental demyelination. Method: Newborn broiler meat chickens were maintained either on a routine diet containing 5.0mg/kg riboflavin (control group) or a riboflavin deficient diet containing 1.Smg/kg riboflavin. Riboflavin

concentrations in the liver were measured at postnatal day 11. Peripheral nerves were morphologically examined at days 6, 11, 16 and 21 using LM, EM and teased nerve fibre techniques. Results: The riboflavin concentration in the liver of riboflavin-deficient chickens was significantly lower than that of control birds. Ribofiavindeficient chickens showed a demyelinating neuropathy with paranodal tomacula formation starting on day 11. Paranodal tomacula consisted of redundant myelin infoldings or outfoldings, increased in size and frequency with age and at day 16 showed prominent degenerative changes. The frequency of paranodal tomacula decreased at day 21 in contrast to an increased frequency of myelinated fibres showing demyelination. Conclusion: Paranodal tomacula formation is an early change that precedes segmental demyelination in an avian riboflavin deficiency model of demyelination.

0333 Paranodal palhology in Tangier disease wilh Renfitting-Relapsing Multifocal Neuropathy CM, Z 1;2, Blumbergs, P C ~:~.3, Cash, K a, Rice, JP~, Manavis, ja, Swift, f , Ghabriel, M N 4, Tiwmpson, PD a. 1Hanson Institute Centre

for Neurological Diseases, Institute of Medical arid Veterinary Science, Adelaide, Australia; 2Department of Neurology" arid University Department of Medicine, Royal Adelaide Hospital; 3Department of Pathology and," 4Department of Anatomy, The University of Adelaide, Australia Pathological studies of a sural nerve biopsy in a man with Tangier disease presenting as a renfitting-relapsing multifocal neuropathy showed abnormalities in the paranodal regions, including lipid deposition (65°,5), redundant myelin foldings with various degrees of myelin splitting and vesiculation (43%) fanning small tomacula and abnormal myelin terminal loops (14%). The internodal regions were normal in the majority of myelinated fibres. Abnormal lipid storage was also present in tile Schwann cells of the majority of umnyelinated fibres (67"/0). The evidence suggests that the noncompacted myelin region of tile paranode is a preferential site for lipid storage in the myelinated Schwann cell and that the space occupying effects of the cholesterol esters leads to paranodal malfunction and tomacula formation as the pathological basis for the multifocal relapsingremitting clinical course.

0334 Neurologie Manifestations of Systemic Lupus Erythematosus: A Focus On Peripheral Neuropathy Cheng, WK ~, Kwan, M C ~, Chung, TH a, Ko, K F ~. 1Kwong Wah

Hospital, Hang Kong Objective: To determine the prevalence of neurological manifestations and peripheral neuropathy in a group of patients with SLE, define the characters and investigate any possible association with clinical and laboratory parameters of the disease. Patients and methods: 152 SLE out-patients in Kwong Wah Hospital were reviewed retrospectively for neurological manifestations. A subset o f 76 patients was studied prospectively and consecutively by clinical exanffnation, laboratory test and neurophysiological studies. Results: There were 139 women and 13 men. The mean age was 40.7 years and a mean follow-up of 88 months. 54 (35.5%) patients had suffered from one or more neurological manifestations. Most frequent findings were tension headache (33.3%) and cerebrovascular accident (124°,5). Other prevalent findings included mood disorder, seizure, anxiety and migraine attack (14.8%). Significant association was found with livedo reticularis, low C3 & CA level, presence of anticardiolipid antibodies and use of cyclophosphamide. Neurophysiological study showed peripheral neuropathy to be present in 34.2%. Half of them (55.3°,5) had positive neuropathy symptom score but more than 70% were found to be normal on neurological examination. The most frequently affected nerves were, in descending orders, sural