Abstracts / Journal of the Neurological Sciences 357 (2015) e255–e294 doi:10.1016/j.jns.2015.08.933 875 WFN15-1278 Movement Disorders Collecting pieces in a possible case of adrenoleukodystrophy A.C.A.F. Ferreira, M. Marco Orsini, C.A. Pereira, A. Kirmse, T.P.R. Benevides, T.N. Magalhães, M.A.A. Leite, A.P.C. Matta, J.G.R. Silva, A. Fernandes, L. kosow, J.M. Osvaldo, M.R.G. Nascimento Freitas. Neurology, Universidade Federal Fluminense, Rio de Janeiro, Brazil ED The aim of this study is to evaluate the efficacy of DBS STN patients with advanced Parkinson's disease on cognitive and affective disorders. Three-year prospective non-randomized trial was organized. The study involved 22 patients treated with STNDBS; 28 patients with PD receiving conservative treatment formed the control group (mean age 54,2 years, mean disease duration 9,6 years). Patients were evaluated under "OFF" and "ON"- medication conditions at 3, 6, 9, 12, 24 and 36 months from the start of the study. Mini-Mental State Examination – MMSE; Frontal Assessment Battery; Montreal Cognitive Assesement MoCА; Hamilton Rating Scale for Depression; The Hamilton Anxiety Rating Scale; Columbia Suicide Severity Rating Scale; The SAD PERSONS Scale; Obsessive– compulsive disorder Self-Test; Questionnaire for ImpulsiveCompulsive Disorders in Parkinson’s Disease – Anytime During Full and short, were used. Improvement of neurodynamic criteria in the first year with insignificant deterioration (for 4%) by three years of supervision was noted in all patients. Logical and visual memory become noticeably better within the first year, with borderline deterioration by the third year of observation. Improvement of mentation remained stable within three years. An insignificant decrease in verbal activity on the direct (7,2%) and controlled association (8,5%) was noted. Additionally, a reduction the severity of a depressive syndrome (for 46%), trait (for 22%) and state anxiety (for 26%) had place. No apparent transient situational personality disorders, such as hypomania, maniacal states, psychoses and aggressive behavior for all the time of supervision were observed. OO F N. Goubareva, N.V. Fedorova, E.V. Bril, A.A. Tomskiy, A.A. Gamaleya, V.A. Shabalov, A.A. Bondarenko, S.B. Buklina. Burdenko Neurosurgery Centre, Russian Academy of Medical Sciences, Moscow; Neurology Department of Russian Medical Academy of Post-Graduate Education, Centre for research of extrapyramidal disorders, Moscow, Moscow, Russia 47-year-old woman, with type 2 diabetes, started presenting painful spasms and rigidity of lower limbs 16 years ago. The symptoms intensified and ascended to upper limbs. She used to fall, developed fear of walking and became bedridden. She also showed startle reflexes. Moreover, it was detectable scanning speech dysmetria and movement decomposition. Anti-GAD was positive. ENMG evinced continuous firing of paravertebral and limb muscles. MRI displayed cerebellar atrophy. High doses of benzodiazepines and muscle relaxers attenuated the painful spasms. Conclusion: Stiff-Person Syndrome is a rare disorder described by muscle rigidity and spasms. Limb dystonia is an atypical manifestation and may be explained by decreased GABA-inhibition which could lead to excessive plasticity and functional impairment. Cerebellar ataxia in SPS has been documented in few cases; our second case endorses the possible association between GAD-Abs and cerebellar ataxia. PR 873 WFN15-0313 Movement Disorders Efficiency of deep brain stimulation of the subthalamic nucleus in patients with advanced Parkinson's disease on mood and cognition Background: Leukodystrophies compass a wide range of progressive degenerative diseases that affect white matter. Objective: Register a possible case of leukodystrophy showing intriguing particularities Patients and methods: Collect data from prompt-book and compare to the literature from Pubmed® database. Results: A 28-year-old man started at the age of 16 with spastic paresis of his left leg. At 23 years old he was paraparetic. Two years later he presented sphincter dysfunction. Brain MRI only displayed discrete sulci; CSF analysis was normal and negative for HTLV-1. One year later, he presented with urinary infection besides cognitive decline. The neurological deterioration developed fast. He kept spastic paraparesis with catatonia, including mutism. T2-weighted MRI showed diffuse atrophy with symmetrical hyperintesity in parietooccipital regions including posterior limb of internal capsule and U fibers but sparing brainstem and cerebellum. These data are suggestive of cerebral adrenoleukodystrophy despite adrenal functioning and normal plasma concentration of very-long-chain fatty acids (VLCFA). Adrenoleukodistrophy is an X-linked disorder that compromises VLCFA oxidation. Therefore, VLCFA gets accumulated in adrenal, spinal cord and brain. This case could represent an adrenomyeloneuropathy form (AMN) which evolves into cerebral form only in 20% after 10 years. However the patient didn’t pass through adrenal failure (present in 70% of the AMN cases). These particularities characterize an even lesser common presentation of a rare disease. Conclusion: Leukodystrophies involve numerous classifications with different phenotypes. Image patterns are a useful tool to complement diagnosis since molecular test is expensive and may cause false negative. CT OR RE doi:10.1016/j.jns.2015.08.932 874 WFN15-1564 Movement Disorders Two atypical cases of stiff-person syndrome UN C A.C.A.F. Ferreira, M.A.A. Leite, T.N. Magalhães, A. Kirmse, L. kosow, T.P.R. Benevides, F. Gaitan, M.A. Orsini, N. Osvaldo J.M., E.R. Davidovich. Neurology, Universidade Federal Fluminense, Rio de Janeiro, Brazil Background: Stiff-person syndrome (SPS) is a rare autoimune movement disorder, characterized by muscular rigidity, painful muscle spasms predominantly affecting paraspinal muscles. Objective: Report two cases of SPS with atypical manifestations. Patients and Methods: Collect data from medical records and compare to medical literature. Results: 60-year-old woman started presenting shakings in her right lower limb associated to cramps and instability three years ago. She also manifested dystonia-like postures of the limb extremity, but without pattern of repetition. Support was indispensable to walk. She developed panic disorder and severe startle reflex. Serology for anti-GAD was positive. Immunoglobulin therapy provided decrease in falls and dystonic movements; stiffness was sustained. e265 doi:10.1016/j.jns.2015.08.934