relapsing polychondritis and lymphocytic meningitis with varied neurological symptoms

1
context. This phenomenon is called attentional boost, which is impaired in individuals with hippocampal atrophy. In this study, we examined the effect of l-DOPA in attentional boost. We report data from 20 patients with Parkinson's disease (PD) before and after l-DOPA therapy. Participants received a letter detection task, while also viewing a series of briey presented natural and urban scenes in the background of the letters. Before l-DOPA treatment (unmedicated state), the performance of PD patients was similar to that of the controls: they exhibited higher levels of scene recognition performance when scenes were presented with target letters relative to baseline (scenes alone) and distractors. After l-DOPA treatment, target-associated scene recognition was further enhanced, but PD patients also improved recognition when scenes were presented with distractor letters outperforming con- trols. l-DOPA did not affect baseline scene recognition. Changes in subthreshold impulsive traits and psychosis-like experiences (follow-up minus baseline) correlated with paradoxically enhanced scene recogni- tion at distractor letters. These results suggest that l-DOPA provides an attentional boost at both behaviorally relevant and irrelevant points of time when distractors are presented. A behaviorally irrelevant boost may account for subthreshold psychiatric symptoms detectable in PD. doi:10.1016/j.jns.2013.07.628 Abstract WCN 2013 No: 889 Topic: 2 Movement Disorders Relapsing polychondritis and lymphocytic meningitis with varied neurological symptoms R.D.-P. Ducci a , F.M. Branco Germiniani a , L.E. Augustin Czecko a , E. Paiva b , H.A.G. Teive a . a Neurology, Hospital de Clínicas Universidade Federal do Paraná, Curitiba, Brazil; b Rheumatology, Hospital de Clínicas Universidade Federal do Paraná, Curitiba, Brazil Background: Relapsing polychondritis is a rheumatological disease characterized by bilateral auricular chondritis, vestibular compromise and varied neurological symptoms. Objective: To report the case of a patient with relapsing polychondritis and several neurological manifestations. Patient and method: We report the case of a patient with relapsing polychondritis with several neurological manifestations. Results: A 69 year-old male was admitted with a 20-day history of ataxia, paraparesis, tinnitus, vertigo and confusion. Two months before he started with bilateral auricular chondritis and arthritis of metacarpophalangeal joints and ankles. He had been previously seen at another hospital, where he had been treated for herpetic encephalitis, with improvement of confusion. On examination he had downward nistagmus, rigidity of upper limbs, paraparesis, absent reexes, tactile hypoesthesia, dismetric movements, gross postural and action tremor, bradikynesia and truncal ataxia. He also had swelling and a purplish erythema of both ear lobes and arthritis in the metacarpophalangeal joints of the right hand. Brain and cervical MRI disclosed a mild thickening of the dura. A new lumbar puncture conrmed the presence of elevated leukocytes and laboratory exams disclosed augmented inammatory activity. A diagnosis of relapsing polychondritis was made based on the association of chondritis, arthritis and vestibular ataxia with predominant neurological symptoms. Following a course of Prednisone 1 mg/kg qid there was major improvement of chondritis, arthritis, ataxia and paraparesis, but the tremor remained unchanged. Conclusion: Relapsing polychondritis is a multisystemic disease that can manifest not only with bilateral auricular chondritis and vestibular compromise, but also with varied neurological symptoms. doi:10.1016/j.jns.2013.07.629 Abstract WCN 2013 No: 1699 Topic: 2 Movement Disorders Effects of botulinum toxin treatment on subjective visual vertical perception in cervical dystonia K. Elwischger, G. Kranz, T. Sycha, P. Rommer, C. Müller, E. Auff, G. Wiest. Medical University of Vienna, Vienna, Austria Background: Previous data suggest that we might abandon the idea of a single reference framefor verticality perception. When the head or body is tilted, otolithic and somatosensory signals can have opposite sign effects during perceiving verticality. When cervical dystonia (CD) is treated with botulinum toxin, abnormal head posture improves within 3 weeks. Objective: This dynamic alteration of head posture is a unique model to study effects of altered somatosensory and otolithic input on static graviceptive function. Patients and methods: Static graviceptive function was assessed by means of subjective visual vertical (SVV) judgement. Thirty patients suffering from idiopathic CD and, for control, thirteen healthy individuals were investigated. At baseline and 3 weeks after injection, patients were investigated at 6 different head positions (no xation, xed upright (0°), xed deviation at 15° and 30° to the left and right, respectively). The control group was investigated at 5 head positions (0°, 15° and 30° to the left and right, respectively). Results: At baseline, CD's SVV judgement in habitual head position vs. control's at 0° was signicantly deviated (p = 0,0166). Three weeks after injection, there was no signicant difference. Thus, disease severity and SVV aberration correlated positively (Pearson correlation: 0.61). We noticed a general trend of major SVV errors of CD patients in contrast to healthy subjects, and a trend of contraversive deviation of SVV in xed head positions (at 30°: p = 0.052). Conclusion: Verticality perception of CD differs from healthy subjects and improves after BoNT therapy. Thereby, increased neck muscle proprioception has a major inuence on verticality perception. doi:10.1016/j.jns.2013.07.630 Abstract WCN 2013 No: 1674 Topic: 2 Movement Disorders Anatomic localization of secondary dystonias: Analysis of 21 patients M.F. Oztekin a , N. Oztekin b . a Neurology, MOH Ankara Yıldırım Beyazit Education and Research Hospital, Ankara, Turkey; b Neurology, MOH Ankara Numune Education and Research Hospital, Ankara, Turkey Objective: To investigate anatomical distribution, etiology and symptomatology of secondary dystonia. Background: Although lesions of basal ganglia, especially putamen and globus pallidus are widely accepted sites for the development of secondary dystonias, structural lesions located in the centrum semiovale, thalamus, brainstem, cerebellum and peripheral nervous system are also reported sites. Analysis of lesion localization and correlation of symptoms may contribute to the relation of phenotype and pathophysiology of dystonia. Patients and methods: 21 patients with structural lesions veried with magnetic resonance imaging (MRI) or computerized tomogra- phy were selected among 365 dystonic patients referred to our movement disorder clinic between 2001 and 2013. The patients were classied according to age onset, distribution and etiology. Results: There were 13 patients with childhood onset and 8 patients with adult-onset.4 patients had focal, 4 patients had segmental, 3 Abstracts / Journal of the Neurological Sciences 333 (2013) e65e108 e104

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context. This phenomenon is called attentional boost, which is impairedin individuals with hippocampal atrophy. In this study, we examinedthe effect of l-DOPA in attentional boost. We report data from 20patients with Parkinson's disease (PD) before and after l-DOPA therapy.Participants received a letter detection task, while also viewing a seriesof briefly presented natural and urban scenes in the background of theletters. Before l-DOPA treatment (unmedicated state), the performanceof PD patients was similar to that of the controls: they exhibited higherlevels of scene recognition performance when scenes were presentedwith target letters relative to baseline (scenes alone) and distractors.After l-DOPA treatment, target-associated scene recognition wasfurther enhanced, but PD patients also improved recognition whenscenes were presented with distractor letters outperforming con-trols. l-DOPA did not affect baseline scene recognition. Changes insubthreshold impulsive traits and psychosis-like experiences (follow-upminus baseline) correlated with paradoxically enhanced scene recogni-tion at distractor letters. These results suggest that l-DOPA provides anattentional boost at both behaviorally relevant and irrelevant points oftime when distractors are presented. A behaviorally irrelevant boostmay account for subthreshold psychiatric symptoms detectable in PD.

doi:10.1016/j.jns.2013.07.628

Abstract — WCN 2013No: 889Topic: 2 — Movement DisordersRelapsing polychondritis and lymphocytic meningitis with variedneurological symptoms

R.D.-P. Duccia, F.M. Branco Germiniania, L.E. Augustin Czeckoa, E. Paivab,H.A.G. Teivea. aNeurology, Hospital de Clínicas — Universidade Federaldo Paraná, Curitiba, Brazil; bRheumatology, Hospital de Clínicas —

Universidade Federal do Paraná, Curitiba, Brazil

Background: Relapsing polychondritis is a rheumatological diseasecharacterized by bilateral auricular chondritis, vestibular compromiseand varied neurological symptoms.Objective: To report the case of a patient with relapsing polychondritisand several neurological manifestations.Patient and method: We report the case of a patient with relapsingpolychondritis with several neurological manifestations.Results: A 69 year-old male was admitted with a 20-day history ofataxia, paraparesis, tinnitus, vertigo and confusion. Two months beforehe started with bilateral auricular chondritis and arthritis ofmetacarpophalangeal joints and ankles. He had been previously seenat another hospital, where hehad been treated for herpetic encephalitis,with improvement of confusion. On examination he had downwardnistagmus, rigidity of upper limbs, paraparesis, absent reflexes, tactilehypoesthesia, dismetric movements, gross postural and action tremor,bradikynesia and truncal ataxia. He also had swelling and a purplisherythema of both ear lobes and arthritis in the metacarpophalangealjoints of the right hand. Brain and cervical MRI disclosed a mildthickening of the dura. A new lumbar puncture confirmed the presenceof elevated leukocytes and laboratory exams disclosed augmentedinflammatory activity. A diagnosis of relapsing polychondritis wasmade based on the association of chondritis, arthritis and vestibularataxia with predominant neurological symptoms. Following a course ofPrednisone 1 mg/kg qid there was major improvement of chondritis,arthritis, ataxia and paraparesis, but the tremor remained unchanged.Conclusion: Relapsing polychondritis is a multisystemic disease thatcan manifest not only with bilateral auricular chondritis andvestibular compromise, but also with varied neurological symptoms.

doi:10.1016/j.jns.2013.07.629

Abstract — WCN 2013No: 1699Topic: 2 — Movement DisordersEffects of botulinum toxin treatment on subjective visual verticalperception in cervical dystonia

K. Elwischger, G. Kranz, T. Sycha, P. Rommer, C. Müller, E. Auff, G. Wiest.Medical University of Vienna, Vienna, Austria

Background: Previous data suggest thatwemight abandon the idea of asingle “reference frame” for verticality perception. When the head orbody is tilted, otolithic and somatosensory signals can have oppositesign effects during perceiving verticality.When cervical dystonia (CD) istreated with botulinum toxin, abnormal head posture improves within3 weeks.Objective: This dynamic alteration of head posture is a unique modelto study effects of altered somatosensory and otolithic input on staticgraviceptive function.Patients and methods: Static graviceptive function was assessed bymeans of subjective visual vertical (SVV) judgement. Thirty patientssuffering from idiopathic CD and, for control, thirteen healthyindividuals were investigated. At baseline and 3 weeks afterinjection, patients were investigated at 6 different head positions(no fixation, fixed upright (0°), fixed deviation at 15° and 30° to theleft and right, respectively). The control group was investigated at 5head positions (0°, 15° and 30° to the left and right, respectively).Results: At baseline, CD's SVV judgement in habitual head position vs.control's at 0° was significantly deviated (p= 0,0166). Three weeksafter injection, there was no significant difference. Thus, diseaseseverity and SVV aberration correlated positively (Pearson correlation:0.61). We noticed a general trend of major SVV errors of CD patients incontrast to healthy subjects, and a trend of contraversive deviation ofSVV in fixed head positions (at 30°: p = 0.052).Conclusion: Verticality perception of CD differs from healthysubjects and improves after BoNT therapy. Thereby, increasedneck muscle proprioception has a major influence on verticalityperception.

doi:10.1016/j.jns.2013.07.630

Abstract — WCN 2013No: 1674Topic: 2 — Movement DisordersAnatomic localization of secondary dystonias: Analysis of21 patients

M.F. Oztekina, N. Oztekinb. aNeurology, MOH Ankara Yıldırım BeyazitEducation and Research Hospital, Ankara, Turkey; bNeurology, MOHAnkara Numune Education and Research Hospital, Ankara, Turkey

Objective: To investigate anatomical distribution, etiology andsymptomatology of secondary dystonia.Background: Although lesions of basal ganglia, especially putamenand globus pallidus are widely accepted sites for the development ofsecondary dystonias, structural lesions located in the centrumsemiovale, thalamus, brainstem, cerebellum and peripheral nervoussystem are also reported sites. Analysis of lesion localization andcorrelation of symptoms may contribute to the relation of phenotypeand pathophysiology of dystonia.Patients and methods: 21 patients with structural lesions verifiedwith magnetic resonance imaging (MRI) or computerized tomogra-phy were selected among 365 dystonic patients referred to ourmovement disorder clinic between 2001 and 2013. The patientswere classified according to age onset, distribution and etiology.Results: There were 13 patients with childhood onset and 8 patientswith adult-onset.4 patients had focal, 4 patients had segmental, 3

Abstracts / Journal of the Neurological Sciences 333 (2013) e65–e108e104