Henrique Ballalai Ferraz

Chronic exposure to the fungicide maneb may produce symptoms and signs of CNS manganese intoxication.

Manganese (Mn) poisoning, a well-known hazard in miners and industrial workers, shares many features with Parkinsons disease. Two young agricultural workers with a parkinsonian syndrome, who mentioned exposure to the fungicide maneb (manganese ethylene-bis-dithiocarbamate), led us to investigate a new possible source of Mn intoxication. Fifty male rural workers with occupational exposure to maneb were compared with 19 rural workers without fungicide exposure. We noted significantly higher prevalence of plastic rigidity with cogwheel phenomenon, headache, fatigue, nervousness, memory complaints, and sleepiness in the exposed group. In addition, we saw other neurologic signs, such as postural tremor, cerebellar signs, and bradykinesia, although without statistical significance. The data suggest that occupational exposure to pesticides containing Mn is a possible source of Mn intoxication of the CNS.

Effects of external cues on gait parameters of Parkinson's disease patients: A systematic review

OBJECTIVES A systematic review to evaluate the benefits of external cues on the gait of PD patients and their impact on the quality of life, freezing and psychomotor performance was performed. The types of cues that could lead to more significant gains were analyzed. METHODS We searched for randomized clinical trials (RCTs) and quasi-randomized clinical trial (QRCTs) that assessed the influence of different external cues on gait, freezing, quality of life and psychomotor performance. RESULTS Of 259 articles collected, seven (six RCTs and one QRCT) were included in the methodological quality criteria (two consider visual cues, two consider auditory cues, one considers verbal instructions, one considers combined cues and one considers sensory cues). All of the data regarding the methodology, interventions, population and bias were described. Cues generally led to a statistically significant improvement in the step and stride length, speed of gait, cadence and UPDRS. None of these studies assessed the quality of life, and one study analyzed freezing. CONCLUSION Our review could show that external cues are effective for improving the gait parameters and psychomotor performance of PD patients. We need more studies to verify if the impact of this type of treatment could improve the quality of life of patients with PD.

Quality of life scale in parkinson's disease PDQ-39 - (Brazilian Portuguese version) to assess patients with and without levodopa motor fluctuation

A qualidade de vida (QdV) e um item importante para se mensurar o sucesso do tratamento na doenca de Parkinson (DP). O objetivo deste estudo foi o de avaliar a utilidade do questionario sobre a doenca de Parkinson - PDQ-39 (versao em lingua portuguesa falada no Brasil) para mensurar a QdV dos pacientes parkinsonianos com e sem flutuacao motora. Nos avaliamos 56 pacientes com DP com tempo medio da doenca de 7,4 anos, e destes 41 (73,3%) apresentavam flutuacao motora. A PDQ-39 tem oito dominios que variam de 0 a 100 e quanto maior o escore pior a QdV. A comparacao dos grupos de pacientes com e sem flutuacao motora mostrou que os dominios: mobilidade, atividades de vida diaria, comunicacao e desconforto corporal tinham escores maiores nos flutuadores. Quanto maiores os estagios de Hoehn e Yahr (HY) da doenca, maiores os escores da PDQ-39. Pacientes com mais de 5 anos de evolucao da doenca mostraram escores piores da PDQ39 apenas nos itens atividades da vida diaria e comunicacao se comparados a pacientes com 5 anos ou menos de doenca. A PDQ-39 e um instrumento capaz de detectar declinio da QdV de pacientes com DP e a presenca de flutuacao motora e um preditor para reducao na QdV.Quality of life (QoL) is an important treatment outcome indicator in Parkinsons disease (PD). The aim of this study is to assess the usefulness of the Parkinsons disease questionnaire--PDQ-39 (Brazilian Portuguese Version) in measuring QoL of PD patients with or without motor fluctuations. Fifty-six PD patients with mean disease duration of 7.4 years were assessed and 41 of them (73.3%) had motor fluctuations. The PDQ-39 has eight dimensions ranging from 0 to 100; being the higher the score, the worse the QoL. Comparing groups with and without motor fluctuations showed that the dimensions mobility, activities of daily living (ADL), communication and bodily discomfort scored higher in the fluctuating group. There was a tendency to see that the higher the Hoehn and Yahr (HY) scale stages, the higher the PDQ-39 scores. Patients suffering from the disease for more than five years had worse PDQ-39 scores only in the items ADL and communication, when compared with those with the disease for < 5 years. The PDQ-39 is an instrument that detects decrease in QoL of PD patients and the presence of motor fluctuations predicts QoL reduction.

Effect of riluzole on dyskinesia and duration of the on state in Parkinson disease patients: a double-blind, placebo-controlled pilot study.

The objective of this study was to evaluate the effect of riluzole on dyskinesia and the duration of the ON state in patients with Parkinson disease (PD). The authors studied 16 PD patients with levodopa-induced dyskinesia. All patients initially received an apomorphine dose intended to induce the motor function benefit (ON state) generally accompanied by dyskinesia. They evaluated the patients during the OFF and ON states using the UPDRS-III, UPDRS-IV, and Larsen scales, and measured the duration of the ON state. Patients were randomly assigned to receive either riluzole (50 mg bid) or placebo for 7 consecutive days (8 patients in each group). The authors did not interrupt previously prescribed medication. Following the 7-day period, they carried out similar evaluation procedures before and after another apomorphine challenge. Mean UPDRS-IV scores were 6.1 points and 6.0 points before and after riluzole therapy respectively. For the placebo group, the scores were 6.9 points and 6.6 points for the initial and final evaluations respectively. Larsen scale had mean scores of 9.2 points and 9.9 points for the pre- and postriluzole periods, and 10.2 points and 9.6 points for pre- and post-placebo evaluations respectively. The ON state was 33.5% lengthier after 7 days of riluzole and 28.0% lengthier after placebo. They could not find any statistical differences between the 2 groups. Short-term riluzole administration in PD patients was not able to reduce apomorphine-induced dyskinesia but could extend the ON state duration, although this did not reach statistical significance.

Higher dopamine transporter density in Parkinson’s disease patients with depression

RationaleDepression 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.ObjectivesTo further examine the role of DAT in PD depression, this study compared thoroughly matched depressed vs. non-depressed PD patients (ndPD).Materials and methodsTwenty PD patients (n = 10 ndPD; n = 10 dPD) matched for age and disease severity were submitted to brain SPECT imaging with [99mTc]-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).ResultsMean 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.ConclusionOur 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.

Effect of voice rehabilitation on oral communication of Parkinson's disease patients

Voice and speech disorders are common in Parkinsons disease patients and may lead to social isolation. We employed routine clinical voice therapy measures to evaluate the effect of voice rehabilitation. Twenty patients with a stable drug regimen participated in this study. The patients were assessed before and after a program of voice rehabilitation consisting of 13 group therapy sessions during 1 month, with emphasis on the increase in laryngeal sphincteric activity. Voice rehabilitation produced an increase in maximal phonation times, decrease in the values of s/z ratio and air flow, increase in vocal intensity, decrease in the complaints of weak and strained‐strangled voice and monotonous and unintelligible speech and elimination of complaints of swallowing alterations. These data indicate a greater glottic efficiency after voice rehabilitation reflecting a more functional oral communication.

Molecular imaging studies in Parkinson disease: reducing diagnostic uncertainty.

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.

Brain SPECT imaging in Sydenham's chorea

The objective of the present study was to determine whether brain single-photon emission computed tomography (SPECT) imaging is capable of detecting perfusional abnormalities. Ten Sydenhams chorea (SC) patients, eight females and two males, 8 to 25 years of age (mean 13.4), with a clinical diagnosis of SC were submitted to brain SPECT imaging. We used HMPAO labeled with technetium-99m at a dose of 740 MBq. Six examinations revealed hyperperfusion of the basal ganglia, while the remaining four were normal. The six patients with abnormal results were females and their data were not correlated with severity of symptoms. Patients with abnormal brain SPECT had a more recent onset of symptoms (mean of 49 days) compared to those with normal SPECT (mean of 85 days) but this difference did not reach statistical significance. Brain SPECT can be a helpful method to determine abnormalities of the basal ganglia in SC patients but further studies on a larger number of patients are needed in order to detect the phase of the disease during which the examination is more sensitive.

Validation of the brazilian version of the berg balance scale for patients with parkinson's disease

BACKGROUND Changes in balance occur with the progression of Parkinsons disease (PD). OBJECTIVE To validate the Brazilian version of the Berg Balance Scale (BBS) for PD patients, determining its reliability and internal consistency and correlating it with PD-specific instruments. METHOD We evaluated 53 patients (M/F 37/16, mean age+/-SD, 62+/-7.9 years) with PD (mean+/-SD, 7.8+/-4.4 years). Unified Parkinsons Disease Rating Scale (UPDRS), Schwab and England Scale (SandE , Hoehn and Yahr Staging Scale (HY) and BBS were used to assess patients. Statistical analyses for inter-rater reliability, internal consistency and correlations among BBS, UPDRS, SandE and HY were performed. RESULTS The mean scores+/-SD on UPDRS and BBS were, respectively, 41.6+/-17.8 and 47.2+/-8.2. The median on SandE and HY scales were 80% and 2.5, respectively. The BBS presented a high intra-class correlation coefficient (ICC=0.84) and internal consistency (Cronbrachs alpha=0.92). There was a statistically significant correlation between BBS and disease duration (r(s)= -0.520, p<0.001), UPDRS subscales II and III (r(s)= -0.467, p=0.011; r(s)= -0.374, p=0.046, respectively), stage of disease (HY; r(s)= -0.507, p<0.001) and the activities of daily living (SandE; r(s)=0.492, p<0.001). CONCLUSION The BBS is a promising tool for the assessment of balance in PD, correlating with the stage of disease and the level of independence.

Genetic and environmental findings in early‐onset Parkinson's disease Brazilian patients

Parkinsons disease (PD) etiology has been attributed both to genetic and environmental factors. In this study, we investigated Brazilian early‐onset PD (EOPD) patients for mutations in PARK2 and PARK8, exposure to environmental factors and possible correlations between PARK2 polymorphisms, environmental exposure, and disease age of onset. We enrolled 72 EOPD index patients and 81 healthy volunteers. Both groups were investigated for environmental exposure. EOPD patients were screened for PARK2 and PARK8 mutations. PARK2 coding polymorphisms Ser167Asn and Val380Leu were investigated in both groups. Mutations were present in 18% of the patients and in 32% of those with a positive family history. PARK2 mutations represented 12.5% and PARK8 mutations accounted for 5.5% of the mutations. A novel PARK2 mutation (D53X) was identified in 2 patients. A positive correlation was found between EOPD and well water drinking. In patients exposed to well water, a later age of onset was observed for those who carried at least one PARK2 380Leu allele. PARK2 mutations have an important role in EOPD Brazilian patients and PARK8 might be the second most important disease causing gene in this group. Well water drinking exposure represents a risk factor for EOPD and the PARK2 coding polymorphism Val380Leu might be interacting with environmental factors acting as a disease modifier.