- Email: [email protected]
1.115 Benign tremulous parkinsonism: Clinical and pathological study
Poster Presentations: Parkinsonism 1.112 Olfactory dysfunction in Parkinson’s disease: Is extended testing useful to discriminate between patients and controls? S. Boesveldt1° , R.-J. de Muinck Keizer, E.Ch. Wolters, H.W. Berendse Netherlands
1 Amsterdam,
Objective: To investigate whether extended odour identification and discrimination tests are better at discriminating between Parkinson’s disease (PD) patients and controls than the standard 16-item tests. Method: Olfactory function was assessed using extended versions (32 vs. 16 items) of the Sniffin’ Sticks odour identification and discrimination tasks in 55 PD patients (Hoehn and Yahr stages I−III) and 51 control subjects, all aged between 50−78 years. Odour identification was measured by presenting 32 odorants in a multiple (4)-forced choice format. In the odour discrimination task, subjects were presented with 32 triplets of odorants and asked to select the odd odour out of each triplet. For each task, olfactory scores (defined as the number of correct answers) were calculated for the 16-item and 32-item versions of the task. Results: Receiver operating characteristic (ROC) curves based upon sensitivity and specificity estimates for each test will be presented at the meeting. Preliminary data suggests that the extended (32-item) odour discrimination test differentiates better between PD patients and controls than the 16-item odour discrimination test. Extended odour identification testing did not increase discriminating power compared to the 16-item identification test. Yet, both 32- and 16-item odour identification tests differentiated better between PD patients and controls than the extended discrimination test. The combination of the extended identification and discrimination tests was not better than the combination of both 16-item tests. Conclusion: Extended discrimination testing is better in differentiating between PD patients and controls than 16-item discrimination testing. However, extended identification testing or the combination of extended identification and discrimination tests does not have higher discriminating power than their 16-item counterparts.
S37
PD within five years. Olfactory testing may therefore become an interesting screening strategy for incipient PD.
1.114 Taste disorders in Parkinson’s disease and parkinsonian syndromes C. Lang1° , M. Krause, T. Hummel Germany
1 Erlangen,
Objective: The appearance of taste disorders in Parkinson’s disease is – other than disorders of smell – still a matter of debate. While one study described a mild increase in gustatory sensitivity in PD patients (Sienkiewicz-Jarosz et al. 2005), two other groups observed a decreased function (Lang et al. 2006, Shah et al. 2007). Method: Therefore we assessed unselected Parkinson patients diagnosed according to clinical criteria for disorders of taste using the so-called taste strips. Methods have been described in detail elsewhere (Lang et al. 2006). We included 39 patients suffering from idiopathic Parkinson’s disease (IPD) with a mean age of 70 ± 8 years who were not demented and compared to a control group matched for sex, age, education, drinking and smoking habits. Results: We found taste reductions for all qualities, most markedly for sweet and salty, whereas bitter was least reduced. This held also true for the non-idiopathic Parkinsonian syndromes (nIPD). Significant correlations were found for cognition (Mini Mental Status Test score), the Webster and Hoehn & Yahr scale, and the number of different drugs taken, but not for age, education, disease duration or L-Dopa dose. Conclusion: The results suggest that Parkinson patients independently of their disease duration, but dependent on their disease severity suffer from mild reductions of taste. These are apparently not quite as marked or frequent as reductions of smell but – with the exception of bitter – salient enough to be of possible clinical help with the diagnosis. The cause of this hypogeusia is not clear at present, but may be possibly sought in a CNS degeneration adjacent or linked to the nigrostriatal system such as the thalamus or frontal cortex.
1.113 Hyposmia, cognitive dysfunction and the future risk of Parkinson’s disease: A five-year prospective study
1.115 Benign tremulous parkinsonism: Clinical and pathological study
1 Amsterdam,
M. Ponsen1° , D. Stoffers, J. Booij, J. Twisk, E.Ch. Wolters, H.W. Berendse Netherlands
1 Saskatoon,
Objective: To determine whether olfactory dysfunction and/or subtle cognitive impairments are a sign of incipient Parkinson’s disease (PD). Method: In a cohort of 361 non-parkinsonian, non-demented first-degree relatives of PD patients, in whom alternative causes of olfactory dysfunction were excluded, we measured baseline performance on three olfactory and two cognitive tasks. Five years from baseline, eight relatives were lost to follow-up. In 74 individuals, that were selected for additional sequential [123 I]b-CIT SPECT scanning on the basis of their baseline olfactory scores, follow-up included a clinical neurological evaluation to determine whether they fulfilled the clinical diagnosis of PD. In the remaining 279 relatives, follow-up involved a validated mail questionnaire sensitive to early signs of parkinsonism, followed by a full clinical neurological evaluation in 17 relatives with a positive response to three or more questions. Survival analysis was used to calculate hazard ratios of developing PD for each of the olfactory and cognitive tasks. Results: Five years from baseline, five relatives, who also had reduced baseline SPECT binding, had developed clinical PD, as opposed to none of the other relatives in the cohort. In univariate analysis, the risk of developing PD significantly increased as the olfactory tasks were performed worse. In multivariate analysis, lower scores on the odor discrimination task and the detection task increased the risk of developing PD. Cognitive tasks were not associated with an increased risk of developing PD. Conclusion: In first degree relatives of PD patients, hyposmia, and not cognitive dysfunction, is associated with an increased risk of developing
Objective: Benign tremulous parkinsonism (BTP) is a rare entity with initial diagnosis of parkinsonism (PS) characterized by prominent tremor, mild or no other motor features, poor response to levodopa (LD), long survival and unknown pathology. We report clinical and autopsy study of two cases. Method: Patients were followed at Movement Disorder Clinic Saskatoon (MDCS) for 35 years (Case 1) and 24 years (Case 2). Case 1 had onset of right hand resting tremor (RT) at age 52 followed by left hand tremor. At age 54 she had bilateral “pill rolling” tremor and mild rigidity and bradykinesia on the right. She was treated with LD for 23 years with no benefit and no adverse effects. Discontinuing the LD produced no change. She died at age 88. Pathology revealed mild Alzheimer’s changes. Case 2 had onset of bilateral upper limb tremor at age 57. At age 67 she was on tranylcypromine and had bilateral upper and lower limb RT, upper limb action tremor, and mild bradykinesia and rigidity. The tranylcypromine was discontinued and she was started on LD. Six months later the tremor had improved, and this was attributed to stopping the tranylcypromine. Subsequently she had progressive worsening of tremor but little or no bradykinesia, and no adverse effects on levodopa. She died at age 92. The substantia nigra was normal and there was no pathology consistent with PS. Results: Both patients had tremor onset and were diagnosed as PS. More than 20 years of LD produced no benefit nor adverse effects and they survived 36 and 35 years, respectively, after tremor onset. Neither had
A. Rajput1° , C. Robinson, A. Rajput Sr. Canada
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Monday, 10 December 2007
pathology consistent with PS and each was diagnosed as atypical essential tremor. Conclusion: More than one disorder, including ET, may manifest as BTP. Further studies are needed to define the full spectrum.
1.118 An automated system for early and differential diagnostics of the clinical forms of Parkinson’s disease O. Khutorskaya1° Russia
1 Moscow,
1.116 Differential diagnosis and clinical profile in a large population of patients with parkinsonism R. Munhoz1° , L. Werneck, H. Teive Brazil
1 Curitiba,
Objective: To determine the etiologic diagnosis and clinical profile in a large population of patients with a syndromic diagnosis of parkinsonism followed at a tertiary movement disorders unit. Method: Cross sectional data collection of patients with a syndromic diagnosis of parkinsonism followed at the movement disorders unit, Federal University of Paran´a, Brazil. Data included demographic caracteristics, clinical profile including motor and non-motor symptoms/signs. Clinical etiologic diagnosis was based of published criteria. Results: The whole sample included 1097 subjects with 53.8% males, mean age 67.8±11.8 years (19−98), disease duration of 7.2±5.5 years (1−40), mean age of onset 60.7±12.8 years (15−96). The most common diagnosis was Parkinson’s disease (PD) found in 848 (77.3%) patients. A positive family history was reported in 11.2%. The other diagnoses found were drug induced parkinsonism in 79 (7.2%), vascular parkinsonism in 51 (4.6%), PSP in 35 (3.2%), Lewy body dementia in 33 (3%) and MSA in 31 (2.8%). Other less common diagnoses included 7 patients with normal pressure hydrocephalus, 6 each with cortico-basal degeneration and fronto-temporal dementia. 10 patients used 2 drugs, 1 used 3. The most common drugs identified as causal were flunarizine, cinnarizine, haloperidol, levomepromazine, valproate and risperidone among others. Conclusion: Drug induced parkinsonism continues to be the second most common form of parkinsonism in Brazil with some variation in regards to the causative agent. Vascular parkinsonism is being increasingly recognized as a cause of parkinsonism although clinical criteria are not fully established.
1.117 Mistakes at the beginning of Parkinson disease: Report of clinical cases G. Pavlic1° , I. Moldovanu, S. Odobescu, S. Albu 1 Chisinau, Moldova Objective: The objective was to emphasize the importance of painful symptoms in the context of motor, neuropsychological and affective disorders and the treatment influence on them. Method: We report three clinical cases of patients with Parkinson’s disease who experienced different types of pain, in two of them causing diagnostic difficulties at the beginning. Results: One patient has been treated for frozen shoulder syndrome and another for coxofemural osteoarthrosis for half a year before receiving the definite diagnosis; in contrast, the third one had pain in relation with motor fluctuations. All of them have been diagnosed by UK Parkinson Disease Society Data Bank criteria and assessed with UPDRS scale, MMSE, Hamilton depression scale, a detailed pain questionnaire and visual analogue scales. Conclusion: In conclusion, attention should be paid to early recognition of PD under its possible “masks”. Pain, besides motor symptoms, is also an important factor in patient assessment and might be reduced by achieving better control of the disease.
Objective: An automated system for early and differential diagnostics and control of treatment of Parkinson’s disease is developed for helping the physician in difficult situations (the absence of the specific diagnostic signs, symptoms similar for different diseases, etc.) to make the correct diagnosis. Method: The system is based on methods of spectral-statistical analysis of specially selected EMG signals. The method for analysis of motor function has been developed in the Institute of Control Sciences of the Russian Academy of Sciences. The method allows assessment of different parameters of EMG for further diagnostics. With four muscles (extensor carpi radialis longus and tibialis anterior of both right and left sides) the integrated EMGs are entered into the computer. For one checkup data arrays consisting of 20 spectra for each muscle studied are accumulated, the average spectra are built, that determines characteristic properties of the muscle, its “portrait”. The components determining the motion activity of the studied muscles are separated from the EMG signals on the PC using special software. Results: The automated system performs statistical analysis of the different EMG characteristics and reveals the main symptoms of Parkinson’s disease (akinesia, rigidity and tremor) for each muscle. The automated system also enables one to reveal and describe the criteria of the early stage of the PD, when it is not viewable yet. The database contains different diagnostic characteristics of the patients. Graph representation allows one to perform the assessment of the efficiency of the different treatments. Conclusion: The automated system allows apart form the early and differential diagnostics of the main symptoms of PD: – individual selection of medication for mono- or combined therapy; – control of effectiveness of antiparkinsonic medications; – assessment of the efficiency of the surgical treatment (neurotransplantation, stereotaxic operations and electrical stimulation. 1.119 New altered motion types in jerking, sympathetic, spasmlike movement disorders S.G. Echebarria1° 1 Spain
Objective: The role and function of advanced/magnified movement descriptors, producing altered motility in evolving conditions origined in peripheral and generalized diseases have been launched in extended and specific samples/casuistics (painful t. spasms, multifocal myoclonus, cortical myoclonus, jerk/postural trembling and neuromuscular/motion associated disorders). Method: Consulting chart/comparative with pre-grouping and disorders types (ICC – descriptives); Frequency peak in Hz/Coherence; Thixotropy grades; Peripheral nerve cond. association (RI–III reflexes); Startle reflex. Results: It would be applicable new phase-evolving movements in discrete sample sub-sizing considering: Active jerky spasm; Body/leg rolling; Distal limb flexion spasm; RSD; Action/postural trembling; Limb spreading contractions. Conclusion: These useful pinpoints may be described in current work-up steps in day-to-day movements disorders units/consulting. 1.120 Advanced screening tools in day-to-day movement disorders observed in ambulatory assistance S.G. Echebarria1° 1 Spain
Objective: Fruitful diagnostic casuistics have been obtained in nowadays methods employed in rigid-akinetic and trembling conditions prevalence–
1 Amsterdam,
Objective: To investigate whether extended odour identification and discrimination tests are better at discriminating between Parkinson’s disease (PD) patients and controls than the standard 16-item tests. Method: Olfactory function was assessed using extended versions (32 vs. 16 items) of the Sniffin’ Sticks odour identification and discrimination tasks in 55 PD patients (Hoehn and Yahr stages I−III) and 51 control subjects, all aged between 50−78 years. Odour identification was measured by presenting 32 odorants in a multiple (4)-forced choice format. In the odour discrimination task, subjects were presented with 32 triplets of odorants and asked to select the odd odour out of each triplet. For each task, olfactory scores (defined as the number of correct answers) were calculated for the 16-item and 32-item versions of the task. Results: Receiver operating characteristic (ROC) curves based upon sensitivity and specificity estimates for each test will be presented at the meeting. Preliminary data suggests that the extended (32-item) odour discrimination test differentiates better between PD patients and controls than the 16-item odour discrimination test. Extended odour identification testing did not increase discriminating power compared to the 16-item identification test. Yet, both 32- and 16-item odour identification tests differentiated better between PD patients and controls than the extended discrimination test. The combination of the extended identification and discrimination tests was not better than the combination of both 16-item tests. Conclusion: Extended discrimination testing is better in differentiating between PD patients and controls than 16-item discrimination testing. However, extended identification testing or the combination of extended identification and discrimination tests does not have higher discriminating power than their 16-item counterparts.
S37
PD within five years. Olfactory testing may therefore become an interesting screening strategy for incipient PD.
1.114 Taste disorders in Parkinson’s disease and parkinsonian syndromes C. Lang1° , M. Krause, T. Hummel Germany
1 Erlangen,
Objective: The appearance of taste disorders in Parkinson’s disease is – other than disorders of smell – still a matter of debate. While one study described a mild increase in gustatory sensitivity in PD patients (Sienkiewicz-Jarosz et al. 2005), two other groups observed a decreased function (Lang et al. 2006, Shah et al. 2007). Method: Therefore we assessed unselected Parkinson patients diagnosed according to clinical criteria for disorders of taste using the so-called taste strips. Methods have been described in detail elsewhere (Lang et al. 2006). We included 39 patients suffering from idiopathic Parkinson’s disease (IPD) with a mean age of 70 ± 8 years who were not demented and compared to a control group matched for sex, age, education, drinking and smoking habits. Results: We found taste reductions for all qualities, most markedly for sweet and salty, whereas bitter was least reduced. This held also true for the non-idiopathic Parkinsonian syndromes (nIPD). Significant correlations were found for cognition (Mini Mental Status Test score), the Webster and Hoehn & Yahr scale, and the number of different drugs taken, but not for age, education, disease duration or L-Dopa dose. Conclusion: The results suggest that Parkinson patients independently of their disease duration, but dependent on their disease severity suffer from mild reductions of taste. These are apparently not quite as marked or frequent as reductions of smell but – with the exception of bitter – salient enough to be of possible clinical help with the diagnosis. The cause of this hypogeusia is not clear at present, but may be possibly sought in a CNS degeneration adjacent or linked to the nigrostriatal system such as the thalamus or frontal cortex.
1.113 Hyposmia, cognitive dysfunction and the future risk of Parkinson’s disease: A five-year prospective study
1.115 Benign tremulous parkinsonism: Clinical and pathological study
1 Amsterdam,
M. Ponsen1° , D. Stoffers, J. Booij, J. Twisk, E.Ch. Wolters, H.W. Berendse Netherlands
1 Saskatoon,
Objective: To determine whether olfactory dysfunction and/or subtle cognitive impairments are a sign of incipient Parkinson’s disease (PD). Method: In a cohort of 361 non-parkinsonian, non-demented first-degree relatives of PD patients, in whom alternative causes of olfactory dysfunction were excluded, we measured baseline performance on three olfactory and two cognitive tasks. Five years from baseline, eight relatives were lost to follow-up. In 74 individuals, that were selected for additional sequential [123 I]b-CIT SPECT scanning on the basis of their baseline olfactory scores, follow-up included a clinical neurological evaluation to determine whether they fulfilled the clinical diagnosis of PD. In the remaining 279 relatives, follow-up involved a validated mail questionnaire sensitive to early signs of parkinsonism, followed by a full clinical neurological evaluation in 17 relatives with a positive response to three or more questions. Survival analysis was used to calculate hazard ratios of developing PD for each of the olfactory and cognitive tasks. Results: Five years from baseline, five relatives, who also had reduced baseline SPECT binding, had developed clinical PD, as opposed to none of the other relatives in the cohort. In univariate analysis, the risk of developing PD significantly increased as the olfactory tasks were performed worse. In multivariate analysis, lower scores on the odor discrimination task and the detection task increased the risk of developing PD. Cognitive tasks were not associated with an increased risk of developing PD. Conclusion: In first degree relatives of PD patients, hyposmia, and not cognitive dysfunction, is associated with an increased risk of developing
Objective: Benign tremulous parkinsonism (BTP) is a rare entity with initial diagnosis of parkinsonism (PS) characterized by prominent tremor, mild or no other motor features, poor response to levodopa (LD), long survival and unknown pathology. We report clinical and autopsy study of two cases. Method: Patients were followed at Movement Disorder Clinic Saskatoon (MDCS) for 35 years (Case 1) and 24 years (Case 2). Case 1 had onset of right hand resting tremor (RT) at age 52 followed by left hand tremor. At age 54 she had bilateral “pill rolling” tremor and mild rigidity and bradykinesia on the right. She was treated with LD for 23 years with no benefit and no adverse effects. Discontinuing the LD produced no change. She died at age 88. Pathology revealed mild Alzheimer’s changes. Case 2 had onset of bilateral upper limb tremor at age 57. At age 67 she was on tranylcypromine and had bilateral upper and lower limb RT, upper limb action tremor, and mild bradykinesia and rigidity. The tranylcypromine was discontinued and she was started on LD. Six months later the tremor had improved, and this was attributed to stopping the tranylcypromine. Subsequently she had progressive worsening of tremor but little or no bradykinesia, and no adverse effects on levodopa. She died at age 92. The substantia nigra was normal and there was no pathology consistent with PS. Results: Both patients had tremor onset and were diagnosed as PS. More than 20 years of LD produced no benefit nor adverse effects and they survived 36 and 35 years, respectively, after tremor onset. Neither had
A. Rajput1° , C. Robinson, A. Rajput Sr. Canada
S38
Monday, 10 December 2007
pathology consistent with PS and each was diagnosed as atypical essential tremor. Conclusion: More than one disorder, including ET, may manifest as BTP. Further studies are needed to define the full spectrum.
1.118 An automated system for early and differential diagnostics of the clinical forms of Parkinson’s disease O. Khutorskaya1° Russia
1 Moscow,
1.116 Differential diagnosis and clinical profile in a large population of patients with parkinsonism R. Munhoz1° , L. Werneck, H. Teive Brazil
1 Curitiba,
Objective: To determine the etiologic diagnosis and clinical profile in a large population of patients with a syndromic diagnosis of parkinsonism followed at a tertiary movement disorders unit. Method: Cross sectional data collection of patients with a syndromic diagnosis of parkinsonism followed at the movement disorders unit, Federal University of Paran´a, Brazil. Data included demographic caracteristics, clinical profile including motor and non-motor symptoms/signs. Clinical etiologic diagnosis was based of published criteria. Results: The whole sample included 1097 subjects with 53.8% males, mean age 67.8±11.8 years (19−98), disease duration of 7.2±5.5 years (1−40), mean age of onset 60.7±12.8 years (15−96). The most common diagnosis was Parkinson’s disease (PD) found in 848 (77.3%) patients. A positive family history was reported in 11.2%. The other diagnoses found were drug induced parkinsonism in 79 (7.2%), vascular parkinsonism in 51 (4.6%), PSP in 35 (3.2%), Lewy body dementia in 33 (3%) and MSA in 31 (2.8%). Other less common diagnoses included 7 patients with normal pressure hydrocephalus, 6 each with cortico-basal degeneration and fronto-temporal dementia. 10 patients used 2 drugs, 1 used 3. The most common drugs identified as causal were flunarizine, cinnarizine, haloperidol, levomepromazine, valproate and risperidone among others. Conclusion: Drug induced parkinsonism continues to be the second most common form of parkinsonism in Brazil with some variation in regards to the causative agent. Vascular parkinsonism is being increasingly recognized as a cause of parkinsonism although clinical criteria are not fully established.
1.117 Mistakes at the beginning of Parkinson disease: Report of clinical cases G. Pavlic1° , I. Moldovanu, S. Odobescu, S. Albu 1 Chisinau, Moldova Objective: The objective was to emphasize the importance of painful symptoms in the context of motor, neuropsychological and affective disorders and the treatment influence on them. Method: We report three clinical cases of patients with Parkinson’s disease who experienced different types of pain, in two of them causing diagnostic difficulties at the beginning. Results: One patient has been treated for frozen shoulder syndrome and another for coxofemural osteoarthrosis for half a year before receiving the definite diagnosis; in contrast, the third one had pain in relation with motor fluctuations. All of them have been diagnosed by UK Parkinson Disease Society Data Bank criteria and assessed with UPDRS scale, MMSE, Hamilton depression scale, a detailed pain questionnaire and visual analogue scales. Conclusion: In conclusion, attention should be paid to early recognition of PD under its possible “masks”. Pain, besides motor symptoms, is also an important factor in patient assessment and might be reduced by achieving better control of the disease.
Objective: An automated system for early and differential diagnostics and control of treatment of Parkinson’s disease is developed for helping the physician in difficult situations (the absence of the specific diagnostic signs, symptoms similar for different diseases, etc.) to make the correct diagnosis. Method: The system is based on methods of spectral-statistical analysis of specially selected EMG signals. The method for analysis of motor function has been developed in the Institute of Control Sciences of the Russian Academy of Sciences. The method allows assessment of different parameters of EMG for further diagnostics. With four muscles (extensor carpi radialis longus and tibialis anterior of both right and left sides) the integrated EMGs are entered into the computer. For one checkup data arrays consisting of 20 spectra for each muscle studied are accumulated, the average spectra are built, that determines characteristic properties of the muscle, its “portrait”. The components determining the motion activity of the studied muscles are separated from the EMG signals on the PC using special software. Results: The automated system performs statistical analysis of the different EMG characteristics and reveals the main symptoms of Parkinson’s disease (akinesia, rigidity and tremor) for each muscle. The automated system also enables one to reveal and describe the criteria of the early stage of the PD, when it is not viewable yet. The database contains different diagnostic characteristics of the patients. Graph representation allows one to perform the assessment of the efficiency of the different treatments. Conclusion: The automated system allows apart form the early and differential diagnostics of the main symptoms of PD: – individual selection of medication for mono- or combined therapy; – control of effectiveness of antiparkinsonic medications; – assessment of the efficiency of the surgical treatment (neurotransplantation, stereotaxic operations and electrical stimulation. 1.119 New altered motion types in jerking, sympathetic, spasmlike movement disorders S.G. Echebarria1° 1 Spain
Objective: The role and function of advanced/magnified movement descriptors, producing altered motility in evolving conditions origined in peripheral and generalized diseases have been launched in extended and specific samples/casuistics (painful t. spasms, multifocal myoclonus, cortical myoclonus, jerk/postural trembling and neuromuscular/motion associated disorders). Method: Consulting chart/comparative with pre-grouping and disorders types (ICC – descriptives); Frequency peak in Hz/Coherence; Thixotropy grades; Peripheral nerve cond. association (RI–III reflexes); Startle reflex. Results: It would be applicable new phase-evolving movements in discrete sample sub-sizing considering: Active jerky spasm; Body/leg rolling; Distal limb flexion spasm; RSD; Action/postural trembling; Limb spreading contractions. Conclusion: These useful pinpoints may be described in current work-up steps in day-to-day movements disorders units/consulting. 1.120 Advanced screening tools in day-to-day movement disorders observed in ambulatory assistance S.G. Echebarria1° 1 Spain
Objective: Fruitful diagnostic casuistics have been obtained in nowadays methods employed in rigid-akinetic and trembling conditions prevalence–