Topic C: DISEASE ENTITIES (HUMAN) Interpretation: We have shown a specific sensory profile with an isolated small fiber loss in male Fabry patients which differs in the characteristics from painful neuropathies of other origin. As a result of these findings, it is possible to establish a simplified neurological testing procedure to detect M. Fabry in an early stage of the disease. 462 MOTOR CORTEX STIMULATION FOR PAIN CONTROL INDUCES METABOLIC CHANGES IN THE ENDOGENOUS OPIOID SYSTEM J. Maarrawi1,2 ° , P. Mertens1,2 , R. Peyron1,3 , N. Costes4 , M. Sindou2 , B. Laurent1,3 , L. Garcia-Larrea1 . 1 INSERM E342 – Central integration of pain in humans – Lyon & St Etienne; 2 Department of functional Neurosurgery – Hˆopital Neurologique de Lyon; 3 Department of Neurology – Hˆopital Bellevue – St Etienne; 4 CERMEP – Centre d’Etude et de Recherche M´edicales par Emission de Positons, Lyon, France Background and Aims: Motor cortex stimulation (MCS) for pain control induces focal cerebral blood flow changes, documented by Positron Emission Tomography (PET). Interestingly, the structures involved correspond to regions with high density of opioid receptors. The aim of this study was to investigate changes of opioid receptor availability induced by MCS. Methods: 15 patients (mean age: 52 years; M/F = 3/2) were included in this study. All of them suffered from strictly unilateral and refractory chronic neuropathic pain. Opioid receptors were studied using 11C-Diprenorphine PET-scans. Each patient underwent 2 preoperative PET-scans and 1 scan 6 months after chronic MCS. Results: No statistically significant difference was documented between the 2 preoperative PET-scans. Comparison of pre and post-operative PET-scans in the first 8 patients who finished the entire study revealed significant decrease of diprenorphine binding in the middle cingulate gyrus (MCG), periaqueductal gray (PAG), prefrontal cortex and cerebellum. Occupancy of receptors by endogenous opioids appears the most likely explanation for this effect. Binding changes in MCG and PAG were significantly correlated with pain relief. Conclusions: MCS may induce endogenous opioid release in cerebral structures implicated in the processing of acute and chronic pain. Correlation of this release with pain relief in at least 2 of these structures supports the hypothesis of a probable role of the endogenous opioid system in pain control induced by MCS. 463 CHARACTERISTICS OF SPREAD IN COMPLEX REGIONAL PAIN SYNDROME M.A. v. Rijn1 , J. Marinus1 ° , H. Putter2 , J.J. v. Hilten1 . 1 Department of Neurology, Leiden University Medical Center, Leiden; 2 Department of Medical Statistics, Leiden University Medical Center, Leiden, The Netherlands Background and Aims: Complex Regional Pain Syndrome (CRPS) is usually restricted to one extremity, but spreading to other extremities may occur. We collected many patients with multiple affected extremities, which presented a unique opportunity to evaluate the determinants and pattern of spreading. Methods: All CRPS patients who visited our outpatient clinic between 1998 and 2004 and met the IASP criteria for CRPS, were included. Results: A total of 288 patients were included. CRPS in multiple extremities (CRPS-M) occurred in 174 (60.4%) patients. CRPS-M patients were on average 5.5 years younger at onset and more often reported a spontaneous onset than patients with a single affected extremity (CRPS-S). Spreading to subsequent extremities most often occurred without an identifiable cause, except in patients where CRPS spread to the diagonal limb. The hazard of developing CRPS in a subsequent extremity increased with the number of affected extremities. The ‘natural’ pattern of spreading was studied in patients where CRPS started in one extremity and subsequently spread to a second extremity without an identifiable cause, which occurred in 72 of the 174 patients. Spreading to the symmetrical limb occurred in
S123 39 (54.2 %) patients, to the ipsilateral limb in 30 (41.7 %) patients, and to the diagonal limb in 3 (4.2 %) patients. Conclusions: M-CRPS more often develops spontaneously and is associated with younger age at onset. The hazard of spreading increases with the number of affected extremities. A symmetrical or ipsilateral pattern of spreading is frequently seen, but diagonal spreading is rare.
464 PROGRESSION OF DYSTONIA IN COMPLEX REGIONAL PAIN SYNDROME M.A. v. Rijn1 , J. Marinus1 ° , H. Putter2 , J.J. v. Hilten1 . 1 Neurology, Leiden University Medical Center, Leiden; 2 Medical Statistics, Leiden University Medical Center, Leiden, The Netherlands Background and Aims: Complex Regional Pain Syndrome (CRPS) is usually preceded by a trauma and may lead to dystonia in some patients. Information on the nature, chronology and clinical determinants of dystonia in CRPS patients may provide better insight in the pathophysiological mechanism of movement disorders (MDs) of CRPS. Methods: In a retrospective study we evaluated the clinical and temporal characteristics of MDs in patients with CRPS. A Cox’s proportional hazards model was used to evaluate factors influencing the onset of MDs. Results: Two-hundred-and eighty-eight patients suffered CRPS in one or more extremities. MDs occurred in 196 patients, with dystonia (92.9%) being the most prevalent. Sixty-three percent of these patients displayed MDs in multiple extremities. Patients with dystonia were on average eight years younger and more often had CRPS in multiple extremities. The interval between the onset of CRPS and dystonia in the first affected extremity varied from less than one week in 33% of the patients to more than one year in 25%. The hazard of developing dystonia in subsequent extremities increased with the number of extremities affected by dystonia. Conclusions: Dystonia in CRPS shows highly variable onset latency and is associated with younger age at onset and increased risk of development of dystonia in other extremities. The delayed onset and progression of dystonia in CRPS may indicate the involvement of a different underlying mechanism, possibly associated maladaptive neuroplasticity.
465 CHRONIC PAIN AFTER SPINAL CORD INJURY IN BOSNIA AND HERZEGOVINA K. Miladinovic1 ° , N. Vavra-Hadziahmetovic1 , A. Karkin-Tais2 , S. Hodzic3 . 1 Institute for Physical Medicine and Rehabilitation/University Clinical Center, Sarajevo; 2 Multidisciplinary HOPE 87 Pain Centre, Sarajevo; 3 Rehabilitation Center Fojnica, Bosnia and Herzegovina Background and Aims: The aim of this study was to assess the prevalence of chronic pain in a sample of patients with spinal cord injury (SCI), to classify the pain, to analyze its correlations with demographic and neurological factors, interference in daily activities and to investigate its treatment. Methods: An observational study based in a personal interview, Visual Analog Scale (VAS) and questionnaire for differentiation of neuropathic from non-neuropathic pain, e.g. the Leeds Assessment of Neuropathic Symptoms and Signs (LANSS) Pain Scale was conducted. Demographic, injury and pain related data were collected and statistically processed (percentage, Pearson correlation test). Results: One hundred patients with SCI, occurred in the period from 5–14 years, were included in the study. 50% reported chronic pain which fulfilled LANSS Pain Scale score for neuropathic pain in 91.1% of them. According to VAS higher intensity of pain was among man (average 7.4) than in women (6.6). We found correlation between VAS and LANSS Pain Scale (p = 0.002), correlation between VAS and age (p = 0.040) and between VAS and level of injury (Pearson rho = 0.329; p = 0.020). 60% patients declared pain interference in daily activities. Analysis of pain treatment showed three groups: no treatment (24.4%), treatment with physical procedures (16%) and pharmacological treatment (59.6%), mainly with nonsteroidals.