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P1.192 Anti-glutamic acid decarboxylase antibodies: a Brazilian neurological series
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Poster presentations / Parkinsonism and Related Disorders 15S2 (2009) S29–S199
Results: The patient was diagnosed as a “psychogenic tremor and parkinsonism”. Conclusion: Thus, this clinical case clearly demonstrates an example of a “psychogenic tremor and parkinsonism”. P1.190 Detection of Lewy body disease in patients with late-onset depression, anxiety and psychotic disorder with myocardial meta-iodobenzylguanidine scintigraphy K. Kobayashi1 , H. Sumiya2 , H. Nakano1 , N. Akiyama1 , K. Urata1 , Y. Koshino1 . 1 Psychiatry, Awazu Neuropsychiatric Sanatorium, Komatsu, 2 Nuclear Medicine, Kanazawa University Graduate School of Medical Sciences, Kanazawa, Japan Purpose: Lewy body disease (LBD) is an array of dementia and motor disorders including Parkinson’s disease (PD), PD dementia (PDD) and dementia with LBD (DLBD). Low uptake of myocardial meta-iodobenzylguanidine (MIBG) with decreased heart-mediastinum (HM) ratio validates diagnosis of LBD. Psychiatric symptoms sometimes precede atypical parkinsonian syndromes in LBD. Of 40 patients with low MIBG uptake, lateonset depressive, anxiety or psychotic symptoms were analyzed in term of clinical profiles. Method: Forty patients were classed into three groups according to main symptoms, 11 patients with visual hallucination (VH), 18 with depression-anxiety (DA) and 11 with psychosis with cognitive disturbance (PCD). Cutoff values of the HM ratio were set at 1.78 in early phase or 1.68 in late phase. Results: Group VH patients showed a trend toward higher age at onset and occipital lobe hypoperfusion. Group DA patients lacked central and core features of DLBD and five of ten showed frontal lobe hypoperfusion. Group PCD patients had the highest frequencies of suggestive symptoms and UPDRS scores and showed temporal lobe hypoperfusion. HM ratio was not associated with clinical profiles of three groups. Cognitive function was more severely disturbed in atypical parkinsonian syndrome cases at an initial visit. Conclusion: Group VH was considered to DLBD, and Group PCD was regarded as PDD or DLBD with early psychotic presentation. Group DA has a possibility of early depression or anxiety disorder of LBD although it lacked DLBD criteria. Atypical parkinsonian syndromes are associated with cognitive disturbance irrespective of psychiatric profiles. P1.191 Stiff Person Syndrome as the initial manifestation of systemic lupus erythematosus – case report R.P. Munhoz1 , H. Teive2 , L. Filla1 , M. Moschovich1 , H. Fameli1 . 1 Neurology Department, Pontifical University of Parana, 2 Neurology Department, Clinical Hospital, Curitiba – Paran´ a, Curitiba, Brazil Objective: To report a case of Stiff Person Syndrome (SPS) as the initial manifestation of systemic lupus erythematosus (SLE). Background: SPS is a rare neurological disorder, it is believed to be mediated by autoantibodies to glutamic acid decarboxylase (antiGAD). SPS with elevated serum anti-GAD levels may occur with other autoimmune disorders. Case report: A 48-year-old woman with an 8-month history of painful axial muscle spasm. Her symptoms were initially described as painful cramps in the thoracic and lumbar paravertebral muscles bilaterally. During the latest 2 months mild non-painful facial spasms were also noticed. Routine laboratory exams included Coombs-positive anemia and thrombocytopenia, plasma anti-GAD levels were elevated, 12.6 U/mL. Electromyography with nerve conduction studies revealed continuous activity of the lumbar paraspinal muscles. In the period required for investigation the patient presented photosensitive rash on the forehead, forearms and hands accompanied by metacarpo-phalangeal arthritis, fatigue, mental slowness and brief episodes of confusion. The additional
inflammatory parameters: positive anti-double-stranded-DNA, anti-nuclear, anti-RP, anti-Scl-70 antibodies and IgM isotype of anticardiolipin antibodies titers. A skin biopsy was also performed showing lymphocytic perivascular dermal infiltrates. A diagnosis of systemis lupus erithematosus was established and the treatment was. On follow up all immunological markers returned to normal levels, except for anti-GAD levels. Conclusion: SPS associated with SLE has been reported in a previous report of a 42 year old man with a similar presentation to the case presented here. Also, except for this previous case, anti-GAD antibodies have not been reported in SLE. P1.192 Anti-glutamic acid decarboxylase antibodies: a Brazilian neurological series H. Teive1 , R.P. Munhoz1 , W. Arruda1 , L.C. Werneck1 , M. Moscovich2 , L. Filla2 . 1 Clinical Hospital, Curitiba – Paran´ a, 2 Pontifical University of Paran´ a, Curitiba, Brazil Objective: To present the clinical and laboratory data of nine neurological patients with anti-glutamic acid decarboxylase antibodies (GAD-Ab) syndrome. Background: GAD-Ab has been described in patients with insulindependent (type 1) diabetes mellitus, stiff-person syndrome (SPS) and rarely in patients with cerebellar ataxia, epilepsy and progressive encephalomyelitis with rigidity. Methods: During a three year period, 11 patients with different neurological syndromes were assessed at our service presenting positive GAD-Ab. All underwent complete clinical and paraclinical investigations including immunological and imaging studies. Results: Six (54.5%) of the 11 cases were men, with mean age 42 years. SPS was diagnosed in 8 patients (2 with the classical form, 4 with stiff-lower-limbs, 2 with stiff-limb syndrome), and three with cerebellar ataxia. GAD-Ab titers were elevated in all cases, ranging from 1.4 U/ml to 85.8 U/ml. EMG testing showed the classical findings of SPS in two cases. Hypothyroidism with Hashimoto’s thyroiditis was found in 4 cases, three had epilepsy and one had diabetes mellitus type 2. Brain and spine imaging were normal and CSF analysis showed mild lymphocytic pleocytosis in two cases. Conclusions: In this series of 11 Brazilian cases of GAD-Ab syndrome we found 8 cases with the SPS, showing different presentations and three cases of cerebellar ataxia. Cases were associated with autoimmune hypothyroidism, epilepsy and DM. P1.193 Motor cortical excitatory plasticity is severely impaired in de novo Parkinson’s disease and is improved by chronic pramipexole treatment A. Kishore1 , T. Joseph2 , S. Meunier3 . 1 Neurology, Comprehensive Care Centre for Movement Disorders, 2 Comprehensive Care Centre for Movement Disorders, Sree Chitra Tirunal Institute for Medical Sciences and Technology, Trivandrum, India; 3 Neurophysiology, INSERM, ER 6, UPMC, Paris, France Background: Motor cortical plasticity is defective in advanced Parkinson’s disease (PD) and is restored by L-dopa in patients on chronic L-dopa treatment. Aims: To test: 1. the plasticity of motor cortex (M1) in denovo and pramipexoletreated cases of early PD with unilateral symptoms; 2. to examine the effect of single dose levodopa in the 2 groups; 3. differences in plasticity between the 2 sides of the brain in the 2 groups. Patients and Methods: Intermittent theta burst stimulation (iTBS) was used to induce excitatory plasticity in M1 in 10 controls, 10 denovo and 10 pramipexole treated patients (after 1–2 days of drug withdrawal) who were Ldopa naive. The effect of iTBS
Poster presentations / Parkinsonism and Related Disorders 15S2 (2009) S29–S199
Results: The patient was diagnosed as a “psychogenic tremor and parkinsonism”. Conclusion: Thus, this clinical case clearly demonstrates an example of a “psychogenic tremor and parkinsonism”. P1.190 Detection of Lewy body disease in patients with late-onset depression, anxiety and psychotic disorder with myocardial meta-iodobenzylguanidine scintigraphy K. Kobayashi1 , H. Sumiya2 , H. Nakano1 , N. Akiyama1 , K. Urata1 , Y. Koshino1 . 1 Psychiatry, Awazu Neuropsychiatric Sanatorium, Komatsu, 2 Nuclear Medicine, Kanazawa University Graduate School of Medical Sciences, Kanazawa, Japan Purpose: Lewy body disease (LBD) is an array of dementia and motor disorders including Parkinson’s disease (PD), PD dementia (PDD) and dementia with LBD (DLBD). Low uptake of myocardial meta-iodobenzylguanidine (MIBG) with decreased heart-mediastinum (HM) ratio validates diagnosis of LBD. Psychiatric symptoms sometimes precede atypical parkinsonian syndromes in LBD. Of 40 patients with low MIBG uptake, lateonset depressive, anxiety or psychotic symptoms were analyzed in term of clinical profiles. Method: Forty patients were classed into three groups according to main symptoms, 11 patients with visual hallucination (VH), 18 with depression-anxiety (DA) and 11 with psychosis with cognitive disturbance (PCD). Cutoff values of the HM ratio were set at 1.78 in early phase or 1.68 in late phase. Results: Group VH patients showed a trend toward higher age at onset and occipital lobe hypoperfusion. Group DA patients lacked central and core features of DLBD and five of ten showed frontal lobe hypoperfusion. Group PCD patients had the highest frequencies of suggestive symptoms and UPDRS scores and showed temporal lobe hypoperfusion. HM ratio was not associated with clinical profiles of three groups. Cognitive function was more severely disturbed in atypical parkinsonian syndrome cases at an initial visit. Conclusion: Group VH was considered to DLBD, and Group PCD was regarded as PDD or DLBD with early psychotic presentation. Group DA has a possibility of early depression or anxiety disorder of LBD although it lacked DLBD criteria. Atypical parkinsonian syndromes are associated with cognitive disturbance irrespective of psychiatric profiles. P1.191 Stiff Person Syndrome as the initial manifestation of systemic lupus erythematosus – case report R.P. Munhoz1 , H. Teive2 , L. Filla1 , M. Moschovich1 , H. Fameli1 . 1 Neurology Department, Pontifical University of Parana, 2 Neurology Department, Clinical Hospital, Curitiba – Paran´ a, Curitiba, Brazil Objective: To report a case of Stiff Person Syndrome (SPS) as the initial manifestation of systemic lupus erythematosus (SLE). Background: SPS is a rare neurological disorder, it is believed to be mediated by autoantibodies to glutamic acid decarboxylase (antiGAD). SPS with elevated serum anti-GAD levels may occur with other autoimmune disorders. Case report: A 48-year-old woman with an 8-month history of painful axial muscle spasm. Her symptoms were initially described as painful cramps in the thoracic and lumbar paravertebral muscles bilaterally. During the latest 2 months mild non-painful facial spasms were also noticed. Routine laboratory exams included Coombs-positive anemia and thrombocytopenia, plasma anti-GAD levels were elevated, 12.6 U/mL. Electromyography with nerve conduction studies revealed continuous activity of the lumbar paraspinal muscles. In the period required for investigation the patient presented photosensitive rash on the forehead, forearms and hands accompanied by metacarpo-phalangeal arthritis, fatigue, mental slowness and brief episodes of confusion. The additional
inflammatory parameters: positive anti-double-stranded-DNA, anti-nuclear, anti-RP, anti-Scl-70 antibodies and IgM isotype of anticardiolipin antibodies titers. A skin biopsy was also performed showing lymphocytic perivascular dermal infiltrates. A diagnosis of systemis lupus erithematosus was established and the treatment was. On follow up all immunological markers returned to normal levels, except for anti-GAD levels. Conclusion: SPS associated with SLE has been reported in a previous report of a 42 year old man with a similar presentation to the case presented here. Also, except for this previous case, anti-GAD antibodies have not been reported in SLE. P1.192 Anti-glutamic acid decarboxylase antibodies: a Brazilian neurological series H. Teive1 , R.P. Munhoz1 , W. Arruda1 , L.C. Werneck1 , M. Moscovich2 , L. Filla2 . 1 Clinical Hospital, Curitiba – Paran´ a, 2 Pontifical University of Paran´ a, Curitiba, Brazil Objective: To present the clinical and laboratory data of nine neurological patients with anti-glutamic acid decarboxylase antibodies (GAD-Ab) syndrome. Background: GAD-Ab has been described in patients with insulindependent (type 1) diabetes mellitus, stiff-person syndrome (SPS) and rarely in patients with cerebellar ataxia, epilepsy and progressive encephalomyelitis with rigidity. Methods: During a three year period, 11 patients with different neurological syndromes were assessed at our service presenting positive GAD-Ab. All underwent complete clinical and paraclinical investigations including immunological and imaging studies. Results: Six (54.5%) of the 11 cases were men, with mean age 42 years. SPS was diagnosed in 8 patients (2 with the classical form, 4 with stiff-lower-limbs, 2 with stiff-limb syndrome), and three with cerebellar ataxia. GAD-Ab titers were elevated in all cases, ranging from 1.4 U/ml to 85.8 U/ml. EMG testing showed the classical findings of SPS in two cases. Hypothyroidism with Hashimoto’s thyroiditis was found in 4 cases, three had epilepsy and one had diabetes mellitus type 2. Brain and spine imaging were normal and CSF analysis showed mild lymphocytic pleocytosis in two cases. Conclusions: In this series of 11 Brazilian cases of GAD-Ab syndrome we found 8 cases with the SPS, showing different presentations and three cases of cerebellar ataxia. Cases were associated with autoimmune hypothyroidism, epilepsy and DM. P1.193 Motor cortical excitatory plasticity is severely impaired in de novo Parkinson’s disease and is improved by chronic pramipexole treatment A. Kishore1 , T. Joseph2 , S. Meunier3 . 1 Neurology, Comprehensive Care Centre for Movement Disorders, 2 Comprehensive Care Centre for Movement Disorders, Sree Chitra Tirunal Institute for Medical Sciences and Technology, Trivandrum, India; 3 Neurophysiology, INSERM, ER 6, UPMC, Paris, France Background: Motor cortical plasticity is defective in advanced Parkinson’s disease (PD) and is restored by L-dopa in patients on chronic L-dopa treatment. Aims: To test: 1. the plasticity of motor cortex (M1) in denovo and pramipexoletreated cases of early PD with unilateral symptoms; 2. to examine the effect of single dose levodopa in the 2 groups; 3. differences in plasticity between the 2 sides of the brain in the 2 groups. Patients and Methods: Intermittent theta burst stimulation (iTBS) was used to induce excitatory plasticity in M1 in 10 controls, 10 denovo and 10 pramipexole treated patients (after 1–2 days of drug withdrawal) who were Ldopa naive. The effect of iTBS