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Tânia M Bovolenta,1 Sônia Maria Cesar de Azevedo Silva,2 Roberta Arb Saba,3 Vanderci Borges,2 Henrique Ballalai Ferraz,2 Andre C Felicio1 1Hospital Israelita Albert Einstein, São Paulo, 2Neurology Department, Federal University of São Paulo, UNIFESP, São Paulo, 3Neurology Department, Federal University of São Paulo, UNIFESP, São Paulo, Brazil Background: Although Parkinson’s disease is the second most prevalent neurodegenerative disease worldwide, its cost in Brazil – South America’s largest country – is unknown.Objective: The goal of this study was to calculate the average annual cost of Parkinson’s disease in the city of São Paulo (Brazil), with a focus on disease-related motor symptoms.Subjects and methods: This was a retrospective, cross-sectional analysis using a bottom-up approach (ie, from the society’s perspective). Patients (N=260) at two tertiary public health centers, who were residents of the São Paulo metropolitan area, completed standardized questionnaires regarding their disease-related expenses. We used simple and multiple generalized linear models to assess the correlations between total cost and patient-related, as well as disease-related variables.Results: The total average annual cost of Parkinson’s disease was estimated at US$5,853.50 per person, including US$3,172.00 in direct costs (medical and nonmedical) and US$2,681.50 in indirect costs. Costs were directly correlated with disease severity (including the degree of motor symptoms), patients’ age, and time since disease onset.Conclusion: In this study, we determined the cost of Parkinson’s disease in Brazil and observed that disease-related motor symptoms are a significant component of the costs incurred on the public health system, patients, and society in general. Keywords: Parkinson’s disease, health economics, cost of illness, health evaluation, cost analysis

Tânia M Bovolenta, Andre C Felicio R. Neurology Program, Hospital Israelita Albert Einstein, São Paulo, Brazil Abstract: Brazil is currently experiencing a significant demographic transition characterized by a decrease in fertility rates and an exponential increase in the number of elderly citizens, which presents a special challenge for the health care professionals. More than other portions of the population, the elderly are most commonly affected by chronic diseases such as Parkinson’s disease. Policymakers contend that Brazil is reasonably well-prepared regarding elderly health care, with policies that aim to ensure the quality of life and the well-being of this portion of the population. However, what happens in practice falls short of what the Brazilian Constitution sets forth. Specifically, there is a clear contradiction between what the law recognizes as being a citizen’s rights and the implementation of guidelines. Because health financing in Brazil remains relatively low, the civil society tries to fill in the gaps as much as possible in the treatment of elderly patients suffering from chronic diseases such as Parkinson’s disease. In this review, we outline the current legislation in Brazil regarding the elderly and in particular, patients with Parkinson’s disease, in the context of a rapidly aging population. Keywords: Parkinson’s disease, demographic transition, public health, health assistance financing

Introduction: With the current demographic transition, it is estimated that by 2050 Brazil will have a population of 90 million people aged 60 years or more, and in parallel Parkinson's disease (PD) will bring a considerable economic burden to our society. Brazil is considered multiracial due to its colonization, generating important social and regional inequalities. Knowing the costs of the PD may aid to improve local public policies. However, in Brazil, no estimates of these values have been made so far., Objectives: To evaluate direct, indirect, and out-of-pocket costs in Brazilian people with PD (PwP)., Methods: Categorical and numerical data were collected through a customized and standardized cost-related-questionnaire from 1055 PwP nationwide, from 10 tertiary movement disorders centers across all Brazilian regions., Results: The estimated average annual cost of PwP was US$ 4020.48. Direct and indirect costs accounted for 63% and 36% of the total, respectively, and out-of-pocket costs were 49%. There were no evidence of differences in the total cost of PD across the regions of the country; however, differences were reported between the stages of the Hoehn and Yahr scale (H&Y)., Conclusion: This data suggests a considerable burden of PD for Brazilian society in general, not only for the public health system, but mainly for those with PD., Competing Interests: Declaration of competing interest We wish to confirm that there are no known conflicts of interest associated with this publication and there has been no significant financial support for this work that could have influenced its outcome. We confirm that the manuscript has been read and approved by all named authors and that there are no other persons who satisfied the criteria for authorship but are not listed. We further confirm that the order of authors listed in the manuscript has been approved by all of us. We confirm that we have given due consideration to the protection of intellectual property associated with this work and that there are no impediments to publication, including the timing of publication, with respect to intellectual property. In so doing we confirm that we have followed the regulations of our institutions concerning intellectual property. We understand that the Corresponding Author is the sole contact for the Editorial process (including Editorial Manager and direct communications with the office). He is responsible for communicating with the other authors about progress, submissions of revisions and final approval of proofs. We confirm that we have provided a current, correct email address which is accessible by the Corresponding Author, and which has been configured to accept email from cf.andre@gmail.comandre.felicio@einstein.br (Andre Carvalho Felício)., (Copyright © 2023 The Authors. Published by Elsevier Ltd.. All rights reserved.)

Objectives: This study aimed to investigate the accuracy of TCS combined with the Sniffin' sticks olfactory test (SST-16) for differentiation between idiopathic PD patients and healthy controls compared to that of 99 m Tc-TRODAT-1 SPECT (TRODAT)., Methods: A cross-sectional study included PD patients diagnosed in accordance with United Kingdom PD Society Brain Bank criteria and a control group of age and sex- matched healthy subjects. All patients were examined by a movement disorder specialist and underwent brain SPECT using TRODAT, TCS examination and SST-16 test. Receiver Operating Characteristic (ROC) curves were used to calculate cut-off points for TCS, striatal TRODAT binding potentials and SST-16. The area under the ROC curve determined the diagnostic accuracy of the method., Results: Twenty patients with PD (13 males and 7 females) and nine healthy subjects were included. Median age of PD onset was 56.5 years with median disease duration of 5 years. A larger substantia nigra (SN) echogenic area was observed in the PD group (p = 0.013). SN echogenic area cut-off point of 0.22 cm 2 was obtained from a ROC curve for PD diagnosis. Considering this cut-off point, TCS diagnostic accuracy was estimated at 79.2% for PD diagnosis. The cut-off value of 0.90 for striatal TRODAT binding was associated with 99% diagnostic accuracy for the diagnosis of PD. SST-16 values equal or less than 9 points showed an 85.8% diagnostic accuracy for PD diagnosis. Combination of both SST-16 and TCS improved the diagnostic accuracy to 95% for PD diagnosis., Conclusion: Combined SST-16 and TCS assessment was indicated as accurate for distinguishing PD patients from healthy controls. The diagnostic accuracy of TCS combined with SST-16 for differentiation between idiopathic PD patients and healthy controls is similar to that of SPECT TRODAT., Competing Interests: Declarations of interest None., (Copyright © 2022 Elsevier B.V. All rights reserved.)

Background: The serotonergic system is known to contribute to levodopa-derived dopamine release in advanced Parkinson's disease., Objective: We investigated the role of the serotonergic system in determining response to treatment in early disease and risk for complications concurrently with dopaminergic alterations., Methods: Eighteen patients with early and stable Parkinson's disease underwent multitracer positron emission tomography using [ 11 C]dihydrotetrabenazine (vesicular monoamine transporter 2 marker), [ 11 C]methylphenidate (dopamine transporter marker), [ 11 C]-3-amino-4-(2-dimethylaminomethylphenylsulfanyl)-benzonitrile (DASB, serotonin transporter marker), and [ 11 C]raclopride (D2 marker) to investigate relationships between striatal dopaminergic and serotonergic alterations and levodopa-induced dopamine release, related to motor response to treatment and risk for dyskinesias, using a novel joint pattern analysis., Results: The joint pattern analysis revealed correlated spatial patterns conceptually related to abnormal dopamine turnover in the putamen (higher dopamine release associated with dopaminergic and serotonergic denervation); response to treatment significantly inversely correlated with turnover-related dopamine release (P < 10 -5 ). Patterns identified without inclusion of the DASB data showed no correlation with clinical data, indicating an important contribution from the serotonergic system to a clinically relevant abnormal dopamine release in early disease. Subjects who experienced dyskinesia 3 years after baseline scans showed higher turnover-related dopamine release compared with subjects who remained stable (P < 0.01)., Conclusions: Joint analysis of dopaminergic and serotonergic data identified a turnover-related dopamine release component, strongly related to motor response to levodopa in early disease and contributing to higher risk for dyskinesia. These findings suggest that the contribution of the serotonergic system to dopamine release not only increases the risk for motor complications but also fails to provide sustained therapeutic advantage in early disease. © 2020 International Parkinson and Movement Disorder Society., (© 2020 International Parkinson and Movement Disorder Society.)

BACKGROUND Studies on the routine clinical use of dopamine transporter (DAT) imaging have largely been conducted in Europe and the United States. In this real-world study, we investigated the use of cerebral 99mTc-TRODAT-1 SPECT imaging of DAT in patients with Parkinson disease (PD) at a tertiary hospital in Brazil. MATERIAL AND METHODS We included 119 patients with suspected PD or clinically unclear parkinsonism who underwent brain scintigraphy with 99mTc-TRODAT-1 during a 3-year period. Additionally, a brief interview was conducted with the physician who requested the scan to determine the usefulness of the method in clinical decision-making. RESULTS Regarding the scan requests, most were intended to evaluate or confirm dopaminergic denervation (69%), distinguish PD from essential tremor (10%), or distinguish degenerative parkinsonism from drug-induced parkinsonism (6%). Data analysis showed that scintigraphy with 99mTc-TRODAT-1 was useful in 85% of cases, changing the management of 75% of the patients who underwent a scan. The majority of physicians who requested the scan were neurologists, and 54% were self-reported movement disorder specialists. An inappropriate use of DAT imaging was seen in 5% of cases. CONCLUSIONS This study demonstrated that brain scintigraphy with the DAT ligand 99mTc-TRODAT-1 may influence diagnostic or therapeutic interventions, meaning that Brazilian physicians who requested the exam have taken in vivo DAT results into account at the time of clinical decision-making.

Purpose: "Chemobrain" is a medical secondary effect of cancer chemotherapy treatment characterized by a general decline in cognition affecting visual and verbal memory, attention, complex problem-solving skills, and motor function. Dopamine (DA) central nervous system neurotransmitters serve an important role in cognition, and changes in DA could potentially explain impaired cognition associated with chemotherapy. Therefore, our objective was to assess in vivo dopaminergic dysfunction in the central nervous system (CNS) of a group of female breast cancer survivors with cognitive impairment following chemotherapy., Methods: Twenty-eight women reporting chemobrain were recruited for this study and compared to 22 healthy reference women. Striatal dopamine transporter (DAT) binding ratio was determined by 99m Tc-TRODAT-1 (a highly selective radiotracer for DAT in the dorsal striatum) single-photon emission computed tomography and a quantitative evaluation was obtained by DatQUANT™ software (GE Healthcare). The DAT binding ratio (BR DAT ) in the patient and control groups was compared using the Student's t test, a multivariate analysis of variance (MANOVA) was used to compare age, years of schooling and BR DAT . The relationship between continuous variables, such as cognitive impairment and BR DAT was assessed using Pearson correlation test., Results: There was a difference in BR DAT between the chemobrain patients and control group. Patients had statistically significant (p < 0.05) lower concentrations of the radiopharmaceutical in the striatum., Conclusions: We identified a significant dopaminergic decrease in all regions of the dorsal striatum within the patients reporting cognitive dysfunction after chemotherapy. Therefore, our results indicate a possible role of dopamine transporter in the physiopathology of chemobrain, even out of the acute phase of symptoms.

The liver is the organ most commonly affected by colorectal cancer metastasis. Currently, liver resection is the treatment of choice, but in cases where it is not possible, intensity-modulated radiation therapy is an alternative. We report the case of a 57-year-old woman whose colorectal carcinoma metastasized to the liver. She was referred for implantation of fiducial markers for intensity-modulated radiation therapy planning. PET-CT stands out as an additional tool for distinguishing metabolically active tumor tissue from other tissues, ensuring effective introduction of percutaneous fiducial markers, especially in cases where use of conventional imaging techniques is limited.

Parkinson's disease (PD) is the second most prevalent neurodegenerative disease worldwide, affecting more than four million people. Typically, it affects individuals above 45, when they are still productive, compromising both aging and quality of life. Therefore, the cost of the disease must be identified, so that the use of resources can be rational and efficient. Additionally, in Brazil, there is a lack of research on the costs of neurodegenerative diseases, such as PD, a gap addressed in this study. This systematic review critically addresses the various methodologies used in original research around the world in the last decade on the subject, showing that costs are hardly comparable. Nonetheless, the economic and social impacts are implicit, and important information for public health agents is provided., Competing Interests: The authors declare that they have no competing interests.

Background: Although the decrease in striatal dopamine transporter (DAT) density has been described in North American, European, and Asian Parkinson's disease (PD) patients, studies on this issue are required in the rest of the world. This study examined the diagnostic utility of DAT imaging in Brazilian PD patients., Material/methods: Twenty PD patients (13 males, 7 females, median age: 62 years, median age at disease onset: 56 years, median disease duration: 5 years, and median UPDRS-III score: 29) and 9 age- and sex-matched healthy subjects underwent single-photon emission computerized tomography (SPECT) using 99mTc-TRODAT-1., Results: PD patients showed a significant decrease in the striatum, caudate nucleus, and putamen DAT densities compared with data from healthy subjects. Striatal 99mTc-TRODAT-1 bindings had the highest diagnostic accuracy compared to those estimates from caudate nucleus and putamen. For the diagnosis of PD, a striatal 99mTc-TRODAT-1 binding cut-off value of 0.90 was associated with a sensitivity of 100% and a specificity of 89%. There was no significant difference between striatal 99mTc-TRODAT-1 binding values provided by different readers, contrary to 99mTc-TRODAT-1 binding estimates in the caudate nucleus., Conclusions: Striatal DAT imaging using 99mTc-TRODAT-1 can be considered a marker for differentiating PD patients from healthy individuals, with a good interobserver reproducibility.

Introduction: We used positron emission tomography (PET) to assess dopaminergic and serotonergic terminal density in three subjects carrying a mutation in the DCT1 gene, two clinically affected with Perry syndrome., Methods: All subjects had brain imaging using 18F-6-fluoro-l-dopa (FDOPA, dopamine synthesis and storage), (+)-11C-dihydrotetrabenazine (DTBZ, vesicular monoamine transporter type 2), and 11C-raclopride (RAC, dopamine D2/D3 receptors). One subject also underwent PET with 11C-3-amino-4-(2-dimethylaminomethyl-phenylsulfanyl)-benzonitrile (DASB, serotonin transporter)., Results: FDOPA-PET and DTBZ-PET in the affected individuals showed a reduction of striatal tracer uptake. Also, RAC-PET showed higher uptake in these area. DASB-PET showed significant uptake changes in left orbitofrontal cortex, bilateral anterior insula, left dorsolateral prefrontal cortex, left orbitofrontal cortex, left posterior cingulate cortex, left caudate, and left ventral striatum., Conclusions: Our data showed evidence of both striatal dopaminergic and widespread cortical/subcortical serotonergic dysfunctions in individuals carrying a mutation in the DCTN1 gene., (© 2014 International Parkinson and Movement Disorder Society.)

Approximately 10% of patients with a presumed diagnosis of Parkinson's disease (PD) remain misdiagnosed despite recent advances in neuroimaging. The current study addresses the use of transcranial sonography and single-photon emission computed tomography (SPECT) using (99m)Tc-TRODAT-1 to evaluate the echogenicity of the substantia nigra (SN) and the density of striatal presynaptic dopamine transporters, respectively, in a sample of 20 PD patients (13 males and 7 females) and 9 healthy subjects. The median age of the PD patients was 62 years. The median age at disease onset was 56 years, and the median disease duration was 5 years. The SN echogenic area was larger in PD patients than healthy subjects. The cut-off value of 0.22 cm(2) for the SN echogenic area was associated with 100% sensitivity and 78% specificity for the diagnosis of PD. Striatal and putaminal (99m)Tc-TRODAT-1 binding was lower in PD patients than healthy subjects. The cut-off value of 0.90 for the striatal (99m)Tc-TRODAT-1 binding was associated with 100% sensitivity and an 89% specificity for the diagnosis of PD, and the cut-off value of 0.76 for putaminal (99m)Tc-TRODAT-1 binding was associated with an 85% sensitivity and an 89% specificity. The size of the SN echogenic area did not correlate with the degree of striatal (99m)Tc-TRODAT-1 binding in PD patients. In conclusion, both SN hyperechogenicity and decreased striatal or putaminal (99m)Tc-TRODAT-1 binding constitute surrogate markers for differentiating PD patients from healthy individuals with a slightly higher diagnostic specificity of (99m)Tc-TRODAT-1 SPECT., (Copyright © 2014 Elsevier Ltd. All rights reserved.)

Diagnosing oncogenic osteomalacia is still a challenge. The disorder is characterized by osteomalacia caused by renal phosphate wasting and low serum concentration of 1,25-dihydroxyvitamin D3 occurring in the presence of a tumor that produces high levels of fibroblast growth factor 23. However, it is possible that the disease is much more misdiagnosed than rare. We present the case of a 42-year-old man with a long-term history of undiagnosed progressive muscle weakness. His laboratory results mainly showed low serum phosphate. Surgical removal of a nasal hemangiopericytoma that had been diagnosed five years earlier, brought him to a symptom-free condition. Even though knowing the underlying etiology would explain his osteomalacia, the patient sought medical help from countless physicians for five consecutive years, and only after adequate treatment a rewarding outcome was achieved.

Objective: Hyperechogenicity of the substantia nigra is a frequent observation on transcranial sonography in Parkinson's disease and Machado-Joseph disease patients. Additionally, restless legs syndrome is a sleep disorder that is also frequently found in both diseases. Autopsy studies have demonstrated increased SN iron content in hyperechogenic substantia nigra. Iron storage is also known to be involved in restless legs syndrome. We formally compared echogenicity of the substantia nigra with restless legs syndrome in Parkinson's disease and Machado-Joseph disease patients., Methods: Transcranial brain sonography was performed in a sample of Parkinson's disease and Machado-Joseph disease patients, and findings then correlated with the presence and severity of restless legs syndrome., Results: There was a continuum of substantia nigra echogenicity among groups (Parkinson's disease versus Machado-Joseph disease versus controls) and sub-groups (Parkinson's disease with and without restless legs syndrome versus Machado-Joseph disease with and without restless legs syndrome) as well as a statistically significant negative correlation between restless legs syndrome severity and substantia nigra echogenicity (p<0.001)., Conclusions: These preliminary observations demonstrate that the severity of RLS may be influenced by nigral iron load reflected by substantia nigra echogenicity in different neurodegenerative movement disorders., (Copyright © 2012 Elsevier B.V. All rights reserved.)

Objective: To determine the clinical correlates of the restless legs syndrome (RLS) in a Brazilian sleep disorders center., Methods: We retrospectively studied 118 patients with RLS from January, 2004, to December, 2010. The analyzed variables were: age at disease onset, gender, race, years of school instruction, primary and secondary RLS, and treatment options., Results: Among the studied patients, 83.9% were women with a female/male sex ratio of 5:1. Mean age of the patients at symptom onset ± standard deviation was 41.7±17.9 years-old. The primary RLS was found in 85% of patients. The other 15% remainders consisted of secondary forms, and they were associated with neuropathy, iron deficiency anemia, end-stage renal disease, or Parkinson's disease. Drug therapy for RLS was introduced in 67% of patients., Conclusions: Most patients presented primary RLS with an early disease onset. Further epidemiological studies are welcomed to provide better information on secondary RLS in Brazil.

Social Anxiety Disorder (SAD) is more common among PD patients than in the general population. This association may be explained by psychosocial mechanisms but it is also possible that neurobiological mechanism underlying PD can predispose to SAD. The aim of this study was to investigate a possible dopaminergic mechanism involved in PD patients with SAD, by correlating striatal dopamine transporter binding potential (DAT-BP) with intensity of social anxiety symptoms in PD patients using SPECT with TRODAT-1 as the radiopharmaceutical. Eleven PD patients with generalized SAD and 21 PD patients without SAD were included in this study; groups were matched for age, gender, disease duration and disease severity. SAD diagnosis was determined according to DSM IV criteria assessed with SCID-I and social anxiety symptom severity with the Brief Social Phobia Scale (BSPS). Demographic and clinical data were also collected. DAT-BP was significantly correlated to scores on BSPS for right putamen (r=0.37, p=0.04), left putamen (r=0.43, p=0.02) and left caudate (r=0.39, p=0.03). No significant correlation was found for the right caudate (r=0.23, p=0.21). This finding may reinforce the hypothesis that dopaminergic dysfunction might be implicated in the pathogenesis of social anxiety in PD., (Copyright © 2011 Elsevier B.V. All rights reserved.)

Olfactory dysfunction is a very common and early sign in neurodegenerative disorders, but few data are already available in hereditary ataxias. Our aim was to evaluate the sense of smell in patients with molecular-proven spinocerebellar ataxia type 3 (SCA3). Forty-one patients with SCA3 and 46 control subjects were studied. The sense of smell was tested using the Sniffin's Sticks (SS-16). We also evaluated Mini-Mental State Examination (MMSE) and non-cerebellar symptoms, such as parkinsonism, dystonia, and restless legs syndrome (RLS). The SCA3 group had significantly lower SS-16 scores than controls (11.5 ± 2.4 vs 12.8 ± 1.5, p = 0.003). Multiple linear regression analyses, controlling for age, sex, education, cigarette smoking, and MMSE scores, showed that SCA3 (p = 0.021), sex (p = 0.003) and MMSE scores (p = 0.002) had significant regression coefficients. All the variables taken together were significantly associated with the SS-16 scores (p ≤ 0.001). Although MMSE scores and female sex were stronger predictors of the SS-16 scores than SCA3, subjects with SCA3 had lower scores on the SS-16, regardless of sex or MMSE scores. Additionally, MMSE scores, sex and presence of RLS were the best predictors of SS-16 scores. Overall, our results strengthen that the sense of smell is significantly reduced in patients with SCA3 and that sex, MMSE scores and RLS also influence the SS-16 scores., (Crown Copyright © 2011. Published by Elsevier Ltd. All rights reserved.)

LRRK2 mutations can cause familial and sporadic Parkinson's disease (PD) with Lewy-body pathology at post-mortem. Studies of olfaction in LRRK2 are sparse and incongruent. We applied a previously validated translation of the 16 item smell identification test from Sniffin' Sticks (SS-16) to 14 parkinsonian carriers of heterozygous G2019S LRRK2 mutation and compared with 106 PD patients and 118 healthy controls. The mean SS-16 score in LRRK2 was higher than in PD (p < 0.001, 95% CI for β = -4.7 to -1.7) and lower than in controls (p = 0.007, 95% CI for β = +0.6 to +3.6). In the LRRK2 group, subjects with low scores had significantly more dyskinesia. They also had younger age of onset, longer disease duration, and reported less frequently a family history of PD, but none of these other differences reached significance. Odor identification is diminished in LRRK2 parkinsonism but not to the same extent as in idiopathic PD., (© 2010 Movement Disorder Society.)

Rationale: Depression is a frequent non-motor symptom in Parkinson's disease (PD) with increasing rates with the progression of the disease. Molecular imaging studies have shown a reduction of dopamine transporter (DAT) density in depressed PD patients (dPD); however, DAT role in the pathophysiology of PD depression is not clear since clinical matching was inappropriate and DAT reduction could be attributed to PD severity., Objectives: To further examine the role of DAT in PD depression, this study compared thoroughly matched depressed vs. non-depressed PD patients (ndPD)., Materials and Methods: Twenty PD patients (n = 10 ndPD; n = 10 dPD) matched for age and disease severity were submitted to brain SPECT imaging with [(99m)Tc]-TRODAT-1, a DAT radioligand. DAT-binding potential was calculated using regions of interest bilaterally drawn in the striatum, caudate, and putamen. Depression was defined according to Beck Depression Inventory (BDI; cut-off >18)., Results: Mean BDI scores were higher in dPD (25.0 +/- 5.6) than in ndPD patients (8.0 +/- 1.9, p < 0.0001). DAT density was greater on dPD especially in the left caudate (dPD 0.87 +/- 0.19 vs. ndDP 0.69 +/- 0.18, p = 0.02) and right putamen (dPD 0.37 +/- 0.07 vs. ndPD 0.28 +/- 0.13, p = 0.03) than in ndPD patients., Conclusion: Our results suggest that in vivo DAT density is increased in dPD patients as compared to ndPD, suggesting that DAT is implicated in the pathophysiology of PD depression.

Objectives: To evaluate patients with "clinically established" psychogenic parkinsonism (PsyP) using single-photon emission computer tomography (SPECT) with the technetium-99m labeled tracer TRODAT-1, a dopamine transporter (DAT) ligand, and investigate whether these patients have an underlying degenerative parkinsonism., Patients and Methods: Five patients with PsyP were assessed using demographic data, standard clinical scales for Parkinson's Disease (PD), and a neuropsychiatric interview. DAT imaging using SPECT with TRODAT-1 was performed, and values for caudate/putamen DAT binding potentials (BP) registered. Patients with PsyP were matched with PD (n=5) and healthy control subjects (n=5)., Results: The mean age (years-old) at first evaluation in the PsyP group was 37.4+/-3.7, and the mean disease duration (years) was 3.9+/-1.2. DAT BPs (means+/-standard deviations) on right/left caudate were, respectively, 0.69+/-0.18 and 0.70+/-0.18 in the PD group versus 1.17+/-0.06 and 1.12+/-0.10 in the control group. DAT BPs on right/left putamen were, respectively, 0.48+/-0.10 and 0.45+/-0.06 in the PD group versus 1.10+/-0.10 and 1.21+/-0.43 in the control group. Two out of five patients from the PsyP group had values for DAT BP in the putamen under the cut-off (< or =0.70) for controls, implying pre-synaptic dopaminergic deficit., Conclusions: Our data in this small group of patients suggest that DAT imaging is a tool that may help in the identification of underlying degenerative parkinsonism in PsyP., (Copyright 2009 Elsevier B.V. All rights reserved.)

Despite its relatively high prevalence Parkinson's Disease (PD) is still misdiagnosed in approximately 25% of cases. In this study our aim was to evaluate patients with Clinically Unclear Parkinsonian Syndromes (CUPS) submitted to brain SPECT imaging using the technetium-99m labeled Dopamine Transporter (DAT) tracer TRODAT-1. We recruited 15 subjects with CUPS and matched them with 13 patients with probable PD and 13 healthy control subjects (HCS). A SPECT with TRODAT-1 was performed at the baseline evaluation and patients from the CUPS were followed-up for 2-years to ensure or not PD diagnosis ("gold-standard"). The mean+/-SD results from Right and Left striatum Binding Potential (BP) were, respectively, 1.08+/-0.20 and 1.04+/-0.16 in the HCS group, 0.47+/-0.16 and 0.53+/-0.17 in the PD group, and 0.68+/-0.11 and 0.84+/-0.17 in the CUPS group. The rate of disagreement between baseline SPECT in the CUPS group as compared to the "gold standard" diagnosis (clinical diagnosis of PD on follow-up) was of 20%. The sensitivity of the SPECT with TRODAT-1 was 100%, while specificity was 70%. In conclusion, our data provided further information about the role of the technetium-99m labeled tracer TRODAT-1 as a biomarker of DAT reduction that can also be used in the diagnosis of patients with CUPS., (Copyright 2010 Elsevier B.V. All rights reserved.)

Unlabelled: The origin of amyotrophic lateral sclerosis (ALS) remains unknown, although it seems to be multifactorial. The role of environmental factors has been frequently investigated and suspicion of its influence can be obtained when clusters of a rare disease are described., Objective: To describe conjugal cases of ALS in Brazil., Method: We describe 2 couples in which both spouses were affected by ALS. Both couples had lived in southeast Brazil and were married for at least 20 years., Results: There was a great variability in clinical presentation of ALS in our patients. In both couples the interval between disease onsets was short. No precise environmental factors could be identified at the origin of these conjugal cases., Conclusion: The occurrence of ALS in couples living in the same area may be epidemiologically important, but we cannot exclude that cases may be due to a chance association.

We retrospectively evaluated 118 patients with cervical dystonia (CD) treated with botulinum toxin type A (BTX-A) for the following variables: gender, age at evaluation, age at symptom onset, disease duration, presence of head/neck pain and/or tremor, pattern of head deviation, disease progression (spreading of symptoms), etiology (primary vs. secondary), pretreatment with oral medication, and Tsui score. We investigated whether these parameters could predict the clinical outcome in a short- (<30 days) and long-term basis. On short-term treatment, there were no clinically significant differences between BTX-A responsive and non-responsive patients. On long-term treatment, however, intake of oral medication previously to BTX-A injection and higher Tsui scores were predictors of favorable response. These results suggest that the greater CD severity the more likely patients will respond to BTX-A.

Pituitary tumor apoplexy is a medical emergency due to acute infarction or hemorrhage in the pituitary gland. In this review, the authors discuss the sellar anatomy, the pituitary gland and adenomas' vascularization and the general aspects of the syndrome such as its ethiopatogenesis, predisposing factors, clinical features, treatment and prognosis.

Clinical features of pontine infarction depend on the topography of vascular lesion and most remarkably sometimes the same topographic region can lead to different clinical syndromes (e.g., dorsal pontine tegmentum). In this report we describe an elderly patient with acute dorsal pontine infarction leading to a unique syndrome of bilateral horizontal gaze palsy and unilateral peripheral facial paralysis. We propose that this syndrome could be included as a part of a continuum that involves one-and-a-half syndrome, eight-and-a-half syndrome, and other variants of pontine tegmentum infarction.

Background: The diagnosis of Parkinson disease (PD) is based on clinical criteria but misdiagnosis is as high as 25% of cases as confirmed by anatomic-pathologic studies. Since the introduction of in vivo molecular imaging techniques using Single-Photon Emission Computed Tomography and Positron Emission Tomography, the diagnosis of PD became more reliable by assessing dopaminergic and even nondopaminergic systems., Review Summary: The purpose of this article is to critically review the current data on molecular neuroimaging focusing on the nigrostriatal circuitry and providing useful information on the role of these new imaging techniques in the management of clinically unclear cases of PD., Conclusions: Patients with essential tremor, psychogenic Parkinsonism or drug-induced Parkinsonism can be differentiated from PD in doubtful situations using molecular imaging techniques evaluating striatal dopamine transporters (DAT). However, in patients with vascular Parkinsonism, atypical Parkinsonism and Parkinsonism associated with dementia DAT scans have less diagnostic usefulness. Scans with non-DAT tracers (ie, D2 dopamine receptors) are necessary together with long-term clinical follow-up, and rescans to improve diagnostic accuracy.

Objective: The association of cervical dystonia (CD) with other movement disorders have been already described, but data on clinical outcome regarding these patients are scant. The aim of this paper was to investigate whether patients with CD and head tremor (HT) would have a different outcome regarding to botulinum toxin type-A (BTX-A) treatment response and clinical and demographic parameters., Method: We retrospectively evaluated 118 medical charts of patients with CD and divided them into two groups: with (HT+) and without (HT-) head tremor. We compared the following clinical and demographic parameters: age at onset, disease duration, progression of symptoms, etiology, familial history, presence of hand tremor. We also analyzed the response to BTX-A according to Tsui score in both groups., Results: The occurrence of head tremor in our sample was of 38.2%. The occurrence of postural hand tremor in the patients from the HT+ group was higher than in the HT- one (p=0.015) and if we compare BTX-A response in each group, we observe that patients with HT present a better outcome in a setting of longer follow-up. In HT+ group, Tsui score pre treatment was 10 (6-12.5) and after follow-up was 8 (5.5-10.5); p<0.001. In HT- group there was no significant difference 9 (7-12) in pre treatment and after follow-up; p=0.07., Conclusion: According to our data it seems that head tremor may influence the clinical outcome or treatment response with BTX-A in patients with CD.

Craniopharyngiomas and germ cell tumors (GCT) may affect the pituitary-hypothalamic region during childhood. Although different in origin, their clinical and radiological features may be similar. In this article we present a 5-year-old girl with clinical and radiological findings (computer tomography calcification) that were initially considered as craniopharyngioma. However clinical outcome, blood and cerebral spinal fluid tumoral markers, and results from anatomopathology and immunohistochemistry disclosed a mixed GCT. This case report highlights that some clinical features and radiological findings of pituitary-hypothalamic tumors may be misdiagnosed as craniopharyngioma mainly when there is a mature teratoma with cartilaginous tissue differentiation.

Background: Kleine-Levin syndrome (KLS) is a rare disorder whose pathophysiological mechanisms remain unknown., Patients and Methods: To investigate dopamine abnormalities in KLS, a [99mTc]-TRODAT-1 single photon emission computerized tomography (SPECT) was performed in a patient with KLS during the asymptomatic period and compared with three matched healthy controls., Results: The patient had 14% lower striatal dopamine transporter binding potential (DAT-BP) compared to the mean DAT-BP of three healthy controls., Conclusion: This study provides in vivo evidence for abnormalities in the DAT-BP, suggesting an involvement of the dopaminergic system in the pathophysiology of KLS.

Behr syndrome is an autosomal recessive disease characterized by early-onset ataxia, optic atrophy and other signs such as pyramidal tract dysfunction. Autosomal dominant inheritance has also been described. In this case report we present a family pedigree of patients with an inherited autosomal dominant Behr syndrome-like phenotype emphasizing their clinical and neuroimaging features.

We report a 30-year-old man with moving ear syndrome caused by focal myoclonic jerks of the right temporal muscle. This focal myoclonus would disappear while the patient was sleeping, swallowing, or speaking. He was treated with botulinum toxin type A with a favorable outcome. Previous reports of this condition and possible therapeutic approaches are discussed., (2007 Movement Disorder Society)

Objective: To compared the clinical features of 373 patients with primary and postparalytic hemifacial spasm (HFS)., Method: Data analyzed were gender, ethnicity, age at symptom onset, disease duration, affected side, distribution of facial spasm at onset, hypertension, family history of HFS, previous history of facial palsy and latency between facial palsy and HFS., Results: The prevalence of patients with Asian origin was similar in both groups such as female/male ratio, mean age at symptom onset, disease duration, affected side and distribution at onset of facial twitching. The upper left side of the face was the main affected region at onset. Almost 40% of the patients in both groups had hypertension. A prevalence of vascular abnormalities on the posterior fossa was seen in 7% and 12.5% of both groups., Conclusion: The clinical profile and radiological findings of patients with primary and postparalytic HFS are similar. The association of hypertension with vascular abnormalities and HFS was not frequent.

Early-onset Parkinson's disease (EOPD) is distinct from the classic late-onset PD (LOPD) because of its slower disease progression. The aim of this study was to compare dopamine neuronal loss in EOPD with that of LOPD with the same disease duration, through dopamine transporter (DAT) estimation. Fourteen patients, seven EOPD (<50 years) and seven LOPD, matched for disease duration were scanned with [(99m)Tc]-TRODAT-1-SPECT (INER-Taiwan), and were assessed with standard PD scales. EOPD patients had 34% lower striatal DAT binding potential (BP) compared with that of LOPD patients (BP = 0.29 +/- 0.12, BP = 0.44 +/- 0.12, P < 0.02) with similar PD severity. These results suggest that EOPD patients have greater dopamine density loss than LOPD patients without motor-symptom worsening.

The development of in vivo molecular imaging to evaluate the dopamine (DA) system with positron-emission tomography and single photon emission computed tomography has been of key importance on monitoring in vivo nigrostriatal neuronal loss in Parkinson's disease (PD), mostly through assessments of pre- and post-synaptic DA receptors. The discoveries of genes related to hereditary forms of parkinsonism (PARK1, PARK2, PARK6, PARK7 and PARK8) have increased our understanding either of distinct subtypes of clinical expression in PD or its etiology. This article revises current data on molecular neuroimaging of genetic forms of parkinsonism comparing and contrasting its main features with the classical sporadic forms. Awareness of the spectrum variance in the genotype and its respective PD phenotype are useful to distinguish different pathophysiological mechanisms of PD.

Chorea and ballism are movement disorders that result from a variety of conditions. Hyperglycemia is an unusual recognized cause of these movement disorders. We report 3 cases of new-onset chorea-ballism induced by nonketotic hyperglycemia in elderly patients, highlighting that chorea may be the first manifestation of undiagnosed decompensated diabetes mellitus.