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P1.094 Radiation risks: a test of perception
Poster presentations / Parkinsonism and Related Disorders 15S2 (2009) S29–S199
4. 5. 6. 7.
Putaminal hypointensity, Slit like putaminal hyperintensity, Dentate nuclear hypointensity, Cortical subcortical hypointensity DTI – Thickness of transverse pontocerebellar fibers. Results: 15 patients [IPD – 3, MSA – 7, CBD – 4, DLBD – 1] and 15 age matched controls of age range 55–75 years with M:F ratio of 3:2 were included in this study. Diagnosis was based on clinical findings. Mean disease duration was 2.8±1.3 years. Mean H&Y score was 2.9. On imaging, brain iron rich areas in normal controls and patients with IPD, MSA-P, MSA-C and others (CBD and DLBD) showed 4 distinctive striatonigral patterns. Conclusions: MR siderotractography using 3T MRI with the 4 patterns of suceptibility signal characterstics of striatonigral pathways may help in differentiating between idiopathic Parkinson’s disease and atypical parkinsonian syndromes. P1.094 Radiation risks: a test of perception
J. McKenzie, R. de la Fuente Fernandez, J. Gavinio, S. Appel Cresswell. Pacific Parkinson’s Research Centre, University of British Columbia, Vancouver, BC, Canada The Pacific Parkinson’s Research Centre (PPRC) at the University of British Columbia conducts neuroimaging studies for investigation of Parkinson’s disease and associated complications. In its >25 year history, PPRC has completed over 5000 positron emission tomography (PET) scans on human subjects using various radiotracers and observed no adverse events directly or indirectly related to the radiotracer administration. The risk of side effects associated with PET scans is 1 in 27,000 [1]. There has been a recent increased level of attention from government and research ethics boards about the risks of radiation to which subjects are exposed while participating in PET studies. We are curious as to what is driving the elevated level of concern. The acceptance of risk by research subjects implies a degree of trust in health research. If the general population perceives that there is significant risk associated with research involving radiation exposure, we might expect that people who choose to participate in PET studies show a higher level of trust in health research than people who decline PET but participate in other imaging (e.g. fMRI) studies in which no radiation is used. By implementing trust scales about healthcare and research participation for control subjects participating in PET & fMRI research, we can gauge the degree of concern from the population perspective. If there is no significant difference in trust between the groups, one might deduce that there is no real perception of risk associated with radiation exposure. Reference(s) [1] J Nucl Med 1998; 39: 2190–2192.
P1.095 Spinocerebellar ataxia 3 presenting as Parkinson disease – case series N. Mendonca ¸ 1 , M.J. Cunha2 , C. Januario ´ 1 . 1 Department of Neurology, 2 Department of Nuclear Medicine, Hospitais da Universidade de Coimbra, Coimbra, Portugal Machado-Joseph disease (MJD) is a dominantly-inherited ataxia first described in people of Azorean descent. MJD results from polyglutamine repeat expansion in the MJD-1 gene, conferring a toxic gain of function to the ataxin-3 protein. Distinct phenotypes appear to reflect the clinical spectrum of a single mutant gene. New imaging techniques using radioactive tracers such as 123 I-ioflupane are used to provide earlier and more accurate diagnosis of Parkinson’s disease by binding to dopamine transporter (DAT) in the striatum and assessing dopamine deficits. We present seven cases positive for the Ataxin-3 gene mutation that presented initially as Parkinsonian Syndrome. They were submitted
S53
to 123 I-ioflupane SPECT that showed absent uptake in putamen nuclei and severe decrease in the caudate nuclei. The results were compatible with bilateral Parkinsonian Syndrome. Most of the patients were also responsive to levodopa in the intial symptomatic stages. The youngest of the patients (73 CAG repeats) was the most striking example and at age 27 began experiencing rigidity, bradykinesia and rest tremor with a good response to levodopa and the development of peak dose diskinesia. The other cases presented with a clinical Parkisonian syndrome and afterwards began the typical cerebellar signs of MJD. We wish to underline the different phenotypical presentations of MJD and in these cases the undeniable envolvement of basal ganglia, showing a clear dopaminergic abnormality. In fact in some cases MJD mutation can actually be considered one of the genetic causes of Parkinson’s syndrome. P1.096 Selective frontal atrophy of the inferior fronto-occipital fasciculus in progressive supranuclear palsy (PSP) P. Kvickstrom ¨ 1 , B. Eriksson1 , D. van Westen2 , J. Latt ¨ 3 , C. Elfgren1 , C. Nilsson1 . 1 Department of Cognitive Medicine, 2 Department of Diagnostic Radiology, 3 Department of Medical Radiation Physics, Lund University Hospital, Lund, Sweden Background: The clinical presentation in PSP includes varying degrees of frontal dysexecutive symptoms. Using diffusion tensor imaging (DTI) and tractography (DTT), we investigated whether atrophy of the inferior fronto-occipital fasciculus (IFO) occurs in PSP and if this atrophy correlates with disease stage and clinical phenotype. Methods: DTI was performed of the whole brain in a 3T MR scanner using a single shot-EPI sequence with diffusion encoding in 48 directions. Scans were obtained in patients with PSP (n = 14) and healthy age-matched controls (n = 12). DTT of the IFO and corticospinal tract (CST) was performed with the PRIDE fibre tracking tool (Philips). Mean fractional anisotropy (FA) and apparent diffusion coefficient (ADC) were calculated and correlated with disease stage and clinical phenotype. Results: In patients with PSP, selective atrophy of the frontal part of IFO was seen with significantly decreased FA and increased ADC, compared with the medial and occipital parts of IFO, as well as compared with controls. The frontal atrophy of IFO was most pronounced in patients with severe frontal cognitive symptoms, such as dysexecutive problems, apathy and personality change. There was a strong correlation between disease stage and both FA and ADC values in the CST. Conclusions: Atrophy of the IFO occurs in PSP, especially in patients showing a frontal clinical phenotype. DTT is a useful tool for demonstrating selective patterns of neuronal tract atrophy in neurodegenerative disease. In selected tracts, FA/ADC might act as surrogate markers for disease stage. P1.097 Brain functional correlates of geriatric assessment in dementia with Lewy bodies R. Perneczky1 , A. Drzezga2 , H. Boecker3 , H. Foerstl1 , A. Kurz1 , P. Haeussermann4 . 1 Department of Psychiatry and Psychotherapy, 2 Department of Nuclear Medicine, Technische Universitaet Muenchen, Munich, 3 Department of Radiology, Rheinische-Friedrich-WilhelmsUniversitaet Bonn, Bonn, 4 Centre for Integrative Psychiatry, Christian-Albrechts-Universitaet Kiel, Kiel, Germany Background and Aims: Geriatric assessment is crucial for the planning and execution of therapeutic strategies in elderly people. A good appraisal of the individual’s current functional level and factors with a negative impact on his autonomy can have great effects on his everyday competence and may reduce mortality. Nevertheless, investigations on the neurobiological substrates of geriatric assessment scales are scarce. The present studies
4. 5. 6. 7.
Putaminal hypointensity, Slit like putaminal hyperintensity, Dentate nuclear hypointensity, Cortical subcortical hypointensity DTI – Thickness of transverse pontocerebellar fibers. Results: 15 patients [IPD – 3, MSA – 7, CBD – 4, DLBD – 1] and 15 age matched controls of age range 55–75 years with M:F ratio of 3:2 were included in this study. Diagnosis was based on clinical findings. Mean disease duration was 2.8±1.3 years. Mean H&Y score was 2.9. On imaging, brain iron rich areas in normal controls and patients with IPD, MSA-P, MSA-C and others (CBD and DLBD) showed 4 distinctive striatonigral patterns. Conclusions: MR siderotractography using 3T MRI with the 4 patterns of suceptibility signal characterstics of striatonigral pathways may help in differentiating between idiopathic Parkinson’s disease and atypical parkinsonian syndromes. P1.094 Radiation risks: a test of perception
J. McKenzie, R. de la Fuente Fernandez, J. Gavinio, S. Appel Cresswell. Pacific Parkinson’s Research Centre, University of British Columbia, Vancouver, BC, Canada The Pacific Parkinson’s Research Centre (PPRC) at the University of British Columbia conducts neuroimaging studies for investigation of Parkinson’s disease and associated complications. In its >25 year history, PPRC has completed over 5000 positron emission tomography (PET) scans on human subjects using various radiotracers and observed no adverse events directly or indirectly related to the radiotracer administration. The risk of side effects associated with PET scans is 1 in 27,000 [1]. There has been a recent increased level of attention from government and research ethics boards about the risks of radiation to which subjects are exposed while participating in PET studies. We are curious as to what is driving the elevated level of concern. The acceptance of risk by research subjects implies a degree of trust in health research. If the general population perceives that there is significant risk associated with research involving radiation exposure, we might expect that people who choose to participate in PET studies show a higher level of trust in health research than people who decline PET but participate in other imaging (e.g. fMRI) studies in which no radiation is used. By implementing trust scales about healthcare and research participation for control subjects participating in PET & fMRI research, we can gauge the degree of concern from the population perspective. If there is no significant difference in trust between the groups, one might deduce that there is no real perception of risk associated with radiation exposure. Reference(s) [1] J Nucl Med 1998; 39: 2190–2192.
P1.095 Spinocerebellar ataxia 3 presenting as Parkinson disease – case series N. Mendonca ¸ 1 , M.J. Cunha2 , C. Januario ´ 1 . 1 Department of Neurology, 2 Department of Nuclear Medicine, Hospitais da Universidade de Coimbra, Coimbra, Portugal Machado-Joseph disease (MJD) is a dominantly-inherited ataxia first described in people of Azorean descent. MJD results from polyglutamine repeat expansion in the MJD-1 gene, conferring a toxic gain of function to the ataxin-3 protein. Distinct phenotypes appear to reflect the clinical spectrum of a single mutant gene. New imaging techniques using radioactive tracers such as 123 I-ioflupane are used to provide earlier and more accurate diagnosis of Parkinson’s disease by binding to dopamine transporter (DAT) in the striatum and assessing dopamine deficits. We present seven cases positive for the Ataxin-3 gene mutation that presented initially as Parkinsonian Syndrome. They were submitted
S53
to 123 I-ioflupane SPECT that showed absent uptake in putamen nuclei and severe decrease in the caudate nuclei. The results were compatible with bilateral Parkinsonian Syndrome. Most of the patients were also responsive to levodopa in the intial symptomatic stages. The youngest of the patients (73 CAG repeats) was the most striking example and at age 27 began experiencing rigidity, bradykinesia and rest tremor with a good response to levodopa and the development of peak dose diskinesia. The other cases presented with a clinical Parkisonian syndrome and afterwards began the typical cerebellar signs of MJD. We wish to underline the different phenotypical presentations of MJD and in these cases the undeniable envolvement of basal ganglia, showing a clear dopaminergic abnormality. In fact in some cases MJD mutation can actually be considered one of the genetic causes of Parkinson’s syndrome. P1.096 Selective frontal atrophy of the inferior fronto-occipital fasciculus in progressive supranuclear palsy (PSP) P. Kvickstrom ¨ 1 , B. Eriksson1 , D. van Westen2 , J. Latt ¨ 3 , C. Elfgren1 , C. Nilsson1 . 1 Department of Cognitive Medicine, 2 Department of Diagnostic Radiology, 3 Department of Medical Radiation Physics, Lund University Hospital, Lund, Sweden Background: The clinical presentation in PSP includes varying degrees of frontal dysexecutive symptoms. Using diffusion tensor imaging (DTI) and tractography (DTT), we investigated whether atrophy of the inferior fronto-occipital fasciculus (IFO) occurs in PSP and if this atrophy correlates with disease stage and clinical phenotype. Methods: DTI was performed of the whole brain in a 3T MR scanner using a single shot-EPI sequence with diffusion encoding in 48 directions. Scans were obtained in patients with PSP (n = 14) and healthy age-matched controls (n = 12). DTT of the IFO and corticospinal tract (CST) was performed with the PRIDE fibre tracking tool (Philips). Mean fractional anisotropy (FA) and apparent diffusion coefficient (ADC) were calculated and correlated with disease stage and clinical phenotype. Results: In patients with PSP, selective atrophy of the frontal part of IFO was seen with significantly decreased FA and increased ADC, compared with the medial and occipital parts of IFO, as well as compared with controls. The frontal atrophy of IFO was most pronounced in patients with severe frontal cognitive symptoms, such as dysexecutive problems, apathy and personality change. There was a strong correlation between disease stage and both FA and ADC values in the CST. Conclusions: Atrophy of the IFO occurs in PSP, especially in patients showing a frontal clinical phenotype. DTT is a useful tool for demonstrating selective patterns of neuronal tract atrophy in neurodegenerative disease. In selected tracts, FA/ADC might act as surrogate markers for disease stage. P1.097 Brain functional correlates of geriatric assessment in dementia with Lewy bodies R. Perneczky1 , A. Drzezga2 , H. Boecker3 , H. Foerstl1 , A. Kurz1 , P. Haeussermann4 . 1 Department of Psychiatry and Psychotherapy, 2 Department of Nuclear Medicine, Technische Universitaet Muenchen, Munich, 3 Department of Radiology, Rheinische-Friedrich-WilhelmsUniversitaet Bonn, Bonn, 4 Centre for Integrative Psychiatry, Christian-Albrechts-Universitaet Kiel, Kiel, Germany Background and Aims: Geriatric assessment is crucial for the planning and execution of therapeutic strategies in elderly people. A good appraisal of the individual’s current functional level and factors with a negative impact on his autonomy can have great effects on his everyday competence and may reduce mortality. Nevertheless, investigations on the neurobiological substrates of geriatric assessment scales are scarce. The present studies