Retinal function in patients with the neuronal ceroid lipofuscinosis phenotype.

PURPOSE:: To analyze the clinical features, visual acuity, and full-field electroretinogram (ERG) findings of 15 patients with the neuronal ceroid lipofuscinosis (NCL) phenotype and to establish the role of ERG testing in NCL diagnosis. METHODS:: The medical records of five patients with infantile NCL, five with Jansky-Bielschowsky disease, and five with juvenile NCL who underwent full-field ERG testing were retrospectively analyzed. RESULTS:: Progressive vision loss was the initial symptom in 66.7% of patients and was isolated or associated with ataxia, epilepsy, and neurodevelopmental involution. Epilepsy was present in 93.3% of patients, of whom 86.6% presented with neurodevelopmental involution. Fundus findings ranged from normal to pigmentary/atrophic abnormalities. Cone-rod, rod-cone, and both types of dysfunction were observed in six, one, and eight patients, respectively. CONCLUSION:: In our study, all patients with the NCL phenotype had abnormal ERG findings, and the majority exhibited both cone-rod and rod-cone dysfunction. We conclude that ERG is a valuable tool for the characterization of visual dysfunction in patients with the NCL phenotype and is useful for diagnosis.

Retinal function in patients with the neuronal ceroid lipofuscinosis phenotype / Estudo da função retiniana em pacientes com o fenótipo da lipofuscinose ceróide neuronal

ABSTRACT Purpose: To analyze the clinical features, visual acuity, and full-field electroretinogram (ERG) findings of 15 patients with the neuronal ceroid lipofuscinosis (NCL) phenotype and to establish the role of ERG testing in NCL diagnosis. Methods: The medical records of five patients with infantile NCL, five with Jansky-Bielschowsky disease, and five with juvenile NCL who underwent full-field ERG testing were retrospectively analyzed. Results: Progressive vision loss was the initial symptom in 66.7% of patients and was isolated or associated with ataxia, epilepsy, and neurodevelopmental involution. Epilepsy was present in 93.3% of patients, of whom 86.6% presented with neurodevelopmental involution. Fundus findings ranged from normal to pigmentary/atrophic abnormalities. Cone-rod, rod-cone, and both types of dysfunction were observed in six, one, and eight patients, respectively. Conclusion: In our study, all patients with the NCL phenotype had abnormal ERG findings, and the majority exhibited both cone-rod and rod-cone dysfunction. We conclude that ERG is a valuable tool for the characterization of visual dysfunction in patients with the NCL phenotype and is useful for diagnosis.

RESUMO Objetivo: Analisar o quadro clínico, a acuidade visual e o eletrorretinograma de campo total (ERG) de 15 pacientes com o fenótipo da lipofuscinose ceróide neuronal (LCN), estabelecendo o papel do eletrorretinograma no seu diagnóstico. Métodos: Eletrorretinograma foi realizado em 5 pacientes com lipofuscinose ceróide neuronal infantil, 5 com doença de Jansky-Bielschowsky e 5 com lipofuscinose ceróide neuronal juvenil sendo feita uma análise retrospectiva dos registros médicos. Resultados: A perda progressiva da acuidade visual foi o sintoma inicial em 66,7%; isolada ou associada à ataxia, epilepsia e involução do desenvolvimento neuropsico motor. Epilepsia foi o sintoma inicial em 93,3% e 86,6% apresentaram involução do desenvolvimento neuropsicomotor. Achados fundoscópicos variaram de normal a alterações pigmentares/atróficas. Disfunção de cone-bastonete foi constatada em 6 pacientes, bastonete-cone em 1 e em 8 pacientes observou-se disfunção proporcional de ambos os sistemas. Conclusão: O eletrorretinograma foi alterado em todos os pacientes, e o achado mais frequente foi o comprometimento de cones e bastonetes. O eletrorretinograma constitui, portanto, uma ferramenta valiosa para caracterizar a disfunção visual em pacientes com o fenótipo da lipofuscinose ceróide neuronal, contribuindo para seu diagnóstico.

Applications of visual evoked potentials and Fourier-domain optical coherence tomography in Parkinson's disease: a controlled study.

PURPOSE: The goal of this cross-sectional observational study was to quantify the pattern-shift visual evoked potentials (VEP) and the thickness as well as the volume of retinal layers using optical coherence tomography (OCT) across a cohort of Parkinson's disease (PD) patients and age-matched controls. METHODS: Forty-three PD patients and 38 controls were enrolled. All participants underwent a detailed neurological and ophthalmologic evaluation. Idiopathic PD cases were included. Cases with glaucoma or increased intra-ocular pressure were excluded. Patients were assessed by VEP and high-resolution Fourier-domain OCT, which quantified the inner and outer thicknesses of the retinal layers. VEP latencies and the thicknesses of the retinal layers were the main outcome measures. RESULTS: The mean age, with standard deviation (SD), of the PD patients and controls were 63.1 (7.5) and 62.4 (7.2) years, respectively. The patients were predominantly in the initial Hoehn-Yahr (HY) disease stages (34.8% in stage 1 or 1.5, and 55.8 % in stage 2). The VEP latencies and the thicknesses as well as the volumes of the retinal inner and outer layers of the groups were similar. A negative correlation between the retinal thickness and the age was noted in both groups. The thickness of the retinal nerve fibre layer (RNFL) was 102.7 µm in PD patients vs. 104.2 µm in controls. CONCLUSIONS: The thicknesses of retinal layers, VEP, and RNFL of PD patients were similar to those of the controls. Despite the use of a representative cohort of PD patients and high-resolution OCT in this study, further studies are required to establish the validity of using OCT and VEP measurements as the anatomic and functional biomarkers for the evaluation of retinal and visual pathways in PD patients.

Applications of visual evoked potentials and Fourier-domain optical coherence tomography in Parkinson's disease: a controlled study / Potenciais evocados visuais e tomografia de coerência óptica com domínio de Fourier na doença de Parkinson: um estudo controlado

Purpose: The goal of this cross-sectional observational study was to quantify the pattern-shift visual evoked potentials (VEP) and the thickness as well as the volume of retinal layers using optical coherence tomography (OCT) across a cohort of Parkinson's disease (PD) patients and age-matched controls. Methods: Forty-three PD patients and 38 controls were enrolled. All participants underwent a detailed neurological and ophthalmologic evaluation. Idiopathic PD cases were included. Cases with glaucoma or increased intra-ocular pressure were excluded. Patients were assessed by VEP and high-resolution Fourier-domain OCT, which quantified the inner and outer thicknesses of the retinal layers. VEP latencies and the thicknesses of the retinal layers were the main outcome measures. Results: The mean age, with standard deviation (SD), of the PD patients and controls were 63.1 (7.5) and 62.4 (7.2) years, respectively. The patients were predominantly in the initial Hoehn-Yahr (HY) disease stages (34.8% in stage 1 or 1.5, and 55.8 % in stage 2). The VEP latencies and the thicknesses as well as the volumes of the retinal inner and outer layers of the groups were similar. A negative correlation between the retinal thickness and the age was noted in both groups. The thickness of the retinal nerve fibre layer (RNFL) was 102.7 μm in PD patients vs. 104.2 μm in controls. Conclusions: The thicknesses of retinal layers, VEP, and RNFL of PD patients were similar to those of the controls. Despite the use of a representative cohort of PD patients and high-resolution OCT in this study, further studies are required to establish the validity of using OCT and VEP measurements as the anatomic and functional biomarkers for the evaluation of retinal and visual pathways in PD patients. .

Objetivo: Este estudo observacional transversal controlado visou quantificar os potenciais evocados visuais com estímulo de padrões alternantes (PEV), a espessura e o volume das camadas retinianas com tomografia de coerência óptica (TCO) num grupo de pacientes com doença de Parkinson (DP). Métodos: Quarenta e três pacientes com DP e 38 controles. Procedimentos: pacientes e controles foram submetidos a exames neurológico e oftalmológico detalhados, sendo incluídos pacientes com DP idiopática e excluídos casos com glaucoma ou aumento da pressão intraocular. Todos os participantes foram estudados com PEV e TCO de Fourier, sendo quantificadas as latências de P100, a espessura e o volume das camadas retinianas interna e externa. Resultados: A média das idades e desvio-padrão dos pacientes com DP e controles foram respectivamente 63,1(7,5) e 62,4 (7,2) anos. Os pacientes com DP situaram-se predominantemente nos estágios iniciais de Hoehn-Yahr (34.8% no estágio 1 ou 1.5; 55.8 % no estágio 2). Não foram observadas diferenças entre os dois grupos quanto às latências dos PEV, a espessura e o volume das camadas retinianas. Observamos uma correlação negativa entre a espessura das camadas retinianas e a idade em ambos os grupos. Não se observou diferença significativa quanto à espessura da camada retiniana das fibras do nervo óptico (CRFNO), que foi de 102,7 μm nos pacientes e 104,2 μm nos controles. Conclusões: A espessura das camadas retinianas, os PEV e a espessura da CRFNO foi semelhante nos pacientes com DP e controles. Apesar desse grupo representativo de pacientes com DP e da alta resolução da TCO utilizada neste estudo, mais estudos são necessários para estabelecer o papel da TCO e dos PEV como biomarcadores anatômico e funcional na avaliação ...

Urinary dysfunction with detrusor hyperactivity in women with Parkinson's disease cannot be blamed as a factor of worsening motor performance.

INTRODUCTION: Detrusor hyperactivity is the leading cause of urinary dysfunction in Parkinson's disease (PD). There are few studies correlating PD clinical aspects with this autonomic feature. METHODS: A cohort of 63 women with PD were prospectively examined for assessment of clinical aspects and disease severity using unified Parkinson's disease rating scale and Hoehn-Yahr scale, respectively. The urologic function was evaluated by the urodynamic study. Two groups were categorized at this time - groups with and without detrusor hyperactivity. After seven years, the same parameters were re-evaluated. RESULTS: Progression of the disease on mental scores was found in the group with detrusor hyperactivity. On follow-up, clinical symptoms and severity did not show significant worsening between the groups. CONCLUSION: Detrusor hyperactivity is a frequent urodynamic finding in PD, and even though it is associated with dopaminergic dysfunction, it cannot be blamed as a factor of worsening motor performance, but is probably associated with poor cognitive and mental prognosis.

Urinary dysfunction with detrusor hyperactivity in women with Parkinson's disease cannot be blamed as a factor of worsening motor performance / Disfuncao urinaria com hiperatividade detrusora nao e fator de risco para progressao do desempenho motor em mulheres com doenca de Parkinson

Introduction Detrusor hyperactivity is the leading cause of urinary dysfunction in Parkinson's disease (PD). There are few studies correlating PD clinical aspects with this autonomic feature. Methods A cohort of 63 women with PD were prospectively examined for assessment of clinical aspects and disease severity using unified Parkinson's disease rating scale and Hoehn-Yahr scale, respectively. The urologic function was evaluated by the urodynamic study. Two groups were categorized at this time - groups with and without detrusor hyperactivity. After seven years, the same parameters were re-evaluated. Results Progression of the disease on mental scores was found in the group with detrusor hyperactivity. On follow-up, clinical symptoms and severity did not show significant worsening between the groups. Conclusion Detrusor hyperactivity is a frequent urodynamic finding in PD, and even though it is associated with dopaminergic dysfunction, it cannot be blamed as a factor of worsening motor performance, but is probably associated with poor cognitive and mental prognosis. .

Introdução Hiperatividade detrusora (HD) é a principal causa de disfunção urinária na doença de Parkinson e poucos estudos correlacionam aspectos clínicos da doença com este componente autonômico. Métodos Foi avaliada uma coorte de 63 pacientes com DP quanto aos aspectos clínicos e gravidade global da doença utilizando as escalas UPDRS e Hoehn-Yahr. A função urológica foi avaliada através de estudo urodinâmico. Foram então categorizados dois grupos: pacientes com e sem HD. Após sete anos os mesmos parâmetros foram reavaliados. Resultados Houve progressão da doença quanto aos escores mentais no grupo com HD. Na reavaliação dos grupos os sintomas motores não evidenciaram piora significante. Conclusão HD é um achado urodinâmico frequente em pacientes com DP. Embora associada à disfunção dopaminérgica, HD não pode ser considerada fator de risco para piora do desempenho motor, mas provavelmente está associada com pior prognóstico mental e cognitivo. .

Topiramate-associated acute, bilateral, angle-closure glaucoma: case report.

This paper describes a topiramate induced acute bilateral angle-closure glaucoma. This rare adverse effect is an idiosyncratic reaction characterized by uveal effusion and lens forward displacement, leading to increased intraocular pressure and vision loss. We describe a 55 year-old white woman with migraine, spasmodic torticollis and essential tremor, who developed bilateral acute angle-closure glaucoma, one week after starting topiramate 25 mg/day. She was seen at the Ophthalmology Emergency Department of the Fundação João Penido Burnier (Campinas, SP, Brazil) with a 4 hours history of blurry vision, ocular pain and bright flashes vision. Slit lamp examination revealed moderate conjunctival injection and corneal edema, and shallow anterior chambers. Intraocular pressure was 48 mmHg in both eyes. Fundoscopic examination findings were normal. She was treated with timolol, brimonidine, dorzolamide, pilocarpine, prednisone acetate eye drops and acetazolamide. One hour after those measures, as the intraocular pressure was 30 mmHg, she received a manitol intravenous injection and the intraocular pressure normalized. After 24 hours an iridotomy with Yag laser was performed. Topiramate was discontinued and she was totally recovered after one week.

Topiramate-associated acute, bilateral, angle-closure glaucoma: case report / Glaucoma agudo de ângulo fechado, bilateral, desencadeado pelo topiramato: relato de caso

This paper describes a topiramate induced acute bilateral angle-closure glaucoma. This rare adverse effect is an idiosyncratic reaction characterized by uveal effusion and lens forward displacement, leading to increased intraocular pressure and vision loss. We describe a 55 year-old white woman with migraine, spasmodic torticollis and essential tremor, who developed bilateral acute angle-closure glaucoma, one week after starting topiramate 25 mg/day. She was seen at the Ophthalmology Emergency Department of the Fundação João Penido Burnier (Campinas, SP, Brazil) with a 4 hours history of blurry vision, ocular pain and bright flashes vision. Slit lamp examination revealed moderate conjunctival injection and corneal edema, and shallow anterior chambers. Intraocular pressure was 48 mmHg in both eyes. Fundoscopic examination findings were normal. She was treated with timolol, brimonidine, dorzolamide, pilocarpine, prednisone acetate eye drops and acetazolamide. One hour after those measures, as the intraocular pressure was 30 mmHg, she received a manitol intravenous injection and the intraocular pressure normalized. After 24 hours an iridotomy with Yag laser was performed. Topiramate was discontinued and she was totally recovered after one week.

Relato de um caso de glaucoma bilateral de ângulo fechado induzido pelo topiramato. Este raro efeito colateral é uma idiosincrasia causada por efusão uveal e deslocamento do cristalino para frente, causando aumento da pressão intraocular e perda visual. Descrevemos o caso de uma paciente de 55 anos com migrânea, torcicolo espasmódico e tremor essencial, que desenvolveu glaucoma bilateral de ângulo fechado uma semana após iniciar o uso de topiramato, 25 mg/dia. A paciente foi atendida no setor de Emergências Oftalmológicas da Fundação Penido Burnier (Campinas, SP, Brasil), com história de 4 horas de embaçamento visual, dor ocular e visão de flashes brilhantes. O exame com lâmpada de fenda revelou injeção conjuntival moderada, edema corneano e câmara anterior rasa em ambos os olhos. A pressão intraocular era de 48 mmHg bilateralmente e a fundoscopia era normal. Foi tratada com colírios de timolol, brimonidina, dorzolamida, pilocarpina e acetato de prednisona e acetazolamida via oral. Uma hora após essas medidas, a pressão intraocular era 30 mmHg, e a paciente recebeu uma injeção intravenosa de manitol, ocorrendo normalização da pressão intraocular após essa medida. Após 24 horas foi realizada iridectomia com Yag laser. O topiramato foi interrompido e ela se recuperou totalmente após uma semana.

Prospective, randomized, double-blind study, comparing botulinum toxins type a botox and prosigne for blepharospasm and hemifacial spasm treatment.

Botulinum toxin A (BTA) is considered an effective treatment of blepharospasm and hemifacial spasm, but there are few studies to permit a comparison of its different formulations. This prospective, randomized, double-blind study compared Prosigne, a BTA of Chinese origin, with Botox to establish safety, efficacy, and equivalence of doses between those 2 formulations in blepharospasm and hemifacial spasm treatment. Fifty-seven patients participated in this study: 21 blepharospasm (from whom 11 were treated with Botox; and 10, with Prosigne) and 36 hemifacial spasm patients (17 were treated with Botox; and 19, with Prosigne). All patients were similar in age, disease time span, number of previous shots, and time elapsed since the last BTA application. Pain and burning during the injection and the result of the treatment were similar in both groups. There were no systemic adverse events, and the local ones were observed with similar intensity and frequency for both groups. The mean effect time length was similar for both blepharospasm (11.3 weeks for both toxins) and hemifacial spasm patients (12.8 weeks for Botox and 12.9 weeks for Prosigne). In both blepharospasm groups, only the 36-Item Short-Form Health Survey emotional aspects domain showed improvement from baseline after 16 weeks. There were no differences between the 36-Item Short-Form Health Survey scores before and after the treatment of all hemifacial spasm patients. Therefore, it has been concluded that Botox and Prosigne have similar efficacy, safety, and tolerability profiles, so that a dose equivalence of 1:1 may be considered for blepharospasm and hemifacial spasm treatments.

A prospective, randomized, double-blind study comparing the efficacy and safety of type a botulinum toxins botox and prosigne in the treatment of cervical dystonia.

Botulinum toxin A (BTA) is considered an effective treatment of cervical dystonia. The aim of this prospective, randomized, double-blind study was to compare Botox and Prosigne, a BTA of Chinese origin, with a view to establish the safety, the efficacy, and the equivalence of doses of the 2 formulations in the treatment of cervical dystonia. Twenty-four patients were randomized to receive 300 U of Botox or Prosigne (12 patients in each group). The patients were assessed before the injection and after 4 and 16 weeks by the Toronto Western Spasmodic Torticollis Rating Scale and the Short-Form 36 for quality of life before and 16 weeks after the injection. All patients were comparable in age, time since onset, number of previous injections, and time since last BTA application. According to the Toronto Western Spasmodic Torticollis Rating Scale scores, the patients improved after injection and the scores increased after 16 weeks, without returning to baseline values. Both pain and burning during the injection and the treatment outcomes were similar in both groups. No systemic adverse events occurred, and the severity and frequency of local events were comparable in both groups. Average duration of effect was similar in both groups (11 weeks). The quality-of-life evaluations before and after the injections were comparable in both groups. Social aspects, pain, and vitality improved after 16 weeks in both groups. In conclusion, Botox and Prosigne were determined to have equivalent efficacy, safety, and tolerability profiles and dose equivalence for cervical dystonia treatment is 1:1.

Olfaction and essential tremor.

OBJECTIVE: To characterize the olfactory identification in 40 essential tremor (ET) patients, with the University of Pennsylvania 12 Smell Identification Test (UPSIT), to correlate UPSIT scores to clinical and epidemiological data and to compare it to 89 aged matched controls. METHOD: Patients were assessed using ET Clinical Scale of Evaluation and UPSIT. RESULTS: In patients with ET, the UPSIT medium score was 9.10, similar to the control group (9.11), which was also observed in all age groups. ET severity did not correlate to UPSIT scores. CONCLUSION: This study demonstrated normality of olfactory identification on ET, qualifying UPSIT to be an important tool on tremor differential diagnosis of undetermined origin.

Olfaction and essential tremor / Olfato no tremor essencial

OBJECTIVE: To characterize the olfactory identification in 40 essential tremor (ET) patients, with the University of Pennsylvania 12 Smell Identification Test (UPSIT), to correlate UPSIT scores to clinical and epidemiological data and to compare it to 89 aged matched controls. METHOD: Patients were assessed using ET Clinical Scale of Evaluation and UPSIT. RESULTS: In patients with ET, the UPSIT medium score was 9.10, similar to the control group (9.11), which was also observed in all age groups. ET severity did not correlate to UPSIT scores. CONCLUSION: This study demonstrated normality of olfactory identification on ET, qualifying UPSIT to be an important tool on tremor differential diagnosis of undetermined origin.

OBJETIVO: Caracterizar a identificação olfatória em 40 pacientes com tremor essencial, através do Teste de Identificação de 12 Cheiros da Universidade de Pensilvânia (TICUP), correlacioná-la aos dados clínicos e epidemiológicos e compará-la com 89 indivíduos normais. MÉTODO: Os pacientes foram avaliados com a Escala Clínica de Avaliação do TE e com o TICUP. RESULTADOS: A média de acertos no TICUP nos pacientes com TE foi 9,10, semelhante à do grupo controle (9,11), sendo isso observado em todas as faixas etárias. A gravidade do TE não se correlacionou com o resultado do TICUP. CONCLUSÃO: Este estudo demonstrou normalidade da identificação olfatória no TE, qualificando o TICUP como ferramenta importante no diagnóstico diferencial dos tremores de causa indeterminada.

Pain in Parkinson's disease: analysis of 50 cases in a clinic of movement disorders.

INTRODUCTION: Pain is a common symptom in Parkinson's disease (PD), and is often related to the illness itself. OBJECTIVE: To prospectively establish the occurrence of pain in PD patients. METHOD: This study was conducted within a population composed of 50 patients with PD to evaluate the presence of pain. RESULTS: Twenty-eight patients reported pain; comparing the group with pain and the group without pain, there were no differences related to the beginning of the illness and the motor symptoms of PD. However, many patients related an improvement of pain when antiparkinsonian therapy was initiated or adjusted. CONCLUSION: The use of techniques for analgesia and the adjustment of PD medication contribute to improve the manifestations of pain and the life quality of patients with PD.

Pain in Parkinson’s disease: analysis of 50 cases in a clinic of movement disorders

INTRODUCTION: Pain is a common symptom in Parkinson's disease (PD), and is often related to the illness itself. OBJECTIVE: To prospectively establish the ocurrence of pain in PD patients. METHOD: This study was conducted within a population composed of 50 patients with PD to evaluate the presence of pain. RESULTS: Twenty-eight patients reported pain; comparing the group with pain and the group without pain, there were no differences related to the beginning of the illness and the motor symptoms of PD. However, many patients related an improvement of pain when antiparkinsonian therapy was initiated or adjusted. CONCLUSION: The use of techniques for analgesia and the adjustment of PD medication contribute to improve the manifestations of pain and the life quality of patients with PD.

INTRODUÇÃO: Dor é um sintoma comum na doença de Parkinson (DP) e, às vezes, está relacionada à própria patologia de base. OBJETIVOS: Estabelecer prospectivamente a ocorrência de dor em pacientes com DP. MÉTODO: Foram avaliados consecutivamente 50 pacientes com DP, para comparação entre os que referiam e os que negavam quadro álgico. RESULTADOS: Entre os entrevistados, 28 referiam episódios dolorosos, não havendo diferenças quanto ao início da doença e os sintomas motores da DP, na comparação entre os dois grupos. Porém, muitos pacientes referiam melhora da dor com a introdução ou ajuste da terapia antiparkinsoniana. CONCLUSÃO: O uso de analgesia e ajuste da terapia para a DP ajudam na melhora do quadro álgico e na qualidade de vida dos pacientes.

Dementia and functional decline in patients with Parkinson's disease.

Functional decline in Parkinson's disease (PD), characterized by reduced ability to carry out activities of daily living, usually results from typical motor impairment and may be aggravated by concomitant cognitive impairment. OBJECTIVE: To compare the functional decline in Parkinson's disease between patients with dementia and cognitively preserved patients. METHODS: From an original sample composed of 50 patients with a clinical diagnosis of idiopathic PD seen in a consecutive series, 33 non-depressed patients were selected comprising 13 with dementia and 20 cognitively preserved individuals. All patients enrolled in this study were drawn from a public outpatient clinic, specialized in movement disorders. The clinical stage of PD was determined by the Hoehn & Yahr scale, and the functional capacity was verified using the Unified Parkinson's Disease Rating Scale UPDRS ADL (subscale II: activities of daily living) and the Schwab & England scale. The two last scales measure the functional degree of independence in activities of daily living. The neuropsychological assessment was performed using The Cambridge Examination for Mental Disorders of the Elderly - CAMCOG, Cognitive Section and the Stroop Color Word Test. RESULTS: As expected, in comparison with cognitively preserved patients, the group with dementia presented significantly lower scores throughout the neuropsychological evaluation. The patients with dementia were found to have a longer period of disease, a more advanced clinical staging according to the Hoehn & Yahr, and greater functional decline according both to the UPDRS ADL and Schwab & England, with statistically significant difference between the groups. CONCLUSION: Patients with dementia were at a more advanced clinical stage of Parkinson's disease and evidenced greater functional decline in comparison with patients without dementia.

O declínio funcional na doença de Parkinson (DP), caracterizado pela redução da capacidade para as atividades de vida diária, usualmente resulta do comprometimento motor típico, podendo ainda ser agravado pelo declínio cognitivo concomitante. OBJETIVOS: Comparar o declínio funcional de pacientes com e sem demência na doença de Parkinson. MÉTODOS: De uma amostra original composta por 50 pacientes com diagnóstico clínico de DP idiopática, avaliados em uma série consecutiva, foram selecionados 33 pacientes não deprimidos, sendo 13 com demência e 20 cognitivamente preservados. Todos os pacientes eram acompanhados em um ambulatório público especializado em distúrbios do movimento. Aplicamos a Unified Parkinson's Disease Rating Scale ­ UPDRS (sub-escala II: atividades de vida diária ­ UPDRS ADL), a escala Schwab & England, a escala Hoehn & Yahr, o Cambridge Examination for Mental Disorders of the Elderly ­ Seção Cognitiva (CAMCOG) e o Stroop Color Word Test. RESULTADOS: Como esperado, em comparação com pacientes cognitivamente preservados, o grupo com demência apresentou escores menores na avaliação neuropsicológica. Os pacientes com demência tinham, também, um tempo maior de doença, pertenciam a um estágio clínico mais avançado segundo a Hoehn & Yahr, e seu declínio funcional, segundo a UPDRS ADL e a Schwab & England, mostrava-se mais acentuado, com diferença estatisticamente significativa entre os grupos. CONCLUSÃO: Os pacientes com demência encontravam-se em um estágio clínico mais avançado da doença e seu declínio funcional mostrou-se mais acentuado em comparação com os pacientes sem demência.

[Olfactory dysfunction in Parkinson's disease]. / Alterações do olfato na doença de Parkinson.

OBJECTIVE: To characterize the olfactory dysfunction in 50 Parkinson's disease (PD) patients with the University of Pennsylvania 12 smell identification test (UPSIT), establishing a comparison with 76 age-matched healthy controls, and associate with clinical and epidemiologic picture. METHOD: The PD group was evaluated in phase "on" through United Parkinson's disease rating scale, UPSIT, and Hoehn and Yahr stage and the control group with the UPSIT. RESULTS: The mean UPSIT score was 5.7 in PD patients and 9 in the control group. Patients that presented initially resting tremor and those that currently have tremor, rigidity and bradykinesia had a significant lower scores. There were negative correlation between patients' age and PD stage with the UPSIT scores. There were no correlation between olfactory scores, age at the initial PD symptoms and disease duration. CONCLUSION: Among PD patients 80% had olfactory deficit and, therefore, smell evaluation may be a tool to make PD differential diagnosis.

Alterações do olfato na doença de Parkinson / Olfactory dysfunction in Parkinson's disease

OBJETIVO: Caracterizar o comprometimento olfatório em 50 pacientes com doença de Parkinson (DP) utilizando o teste de identificação de 12 cheiros da Universidade de Pensilvânia (TICUP), comparando-os com 76 indivíduos normais e associá-lo ao quadro clínico e epidemiológico. MÉTODO: Os pacientes foram avaliados na fase "on" com as escalas unificada da doença de Parkinson (UPDRS), Hoehn e Yahr e TICUP e o grupo controle com o TICUP. RESULTADOS: A média geral do número de acertos foi 5,7 nos parkinsonianos e 9 nos controles, com pontuação menor nos que apresentaram como sintoma inicial tremor e naqueles que atualmente apresentavam tremor, rigidez e bradicinesia. A idade e o estágio da DP correlacionaram-se negativamente com o número de acertos, não havendo correlação da perda olfatória com idade de início do quadro e pontuação da UPDRS. CONCLUSÃO: Apresentaram comprometimento olfatório 80 por cento dos pacientes com DP, sendo essa avaliação ferramenta importante no diagnóstico diferencial.

OBJECTIVE: To characterize the olfactory dysfunction in 50 Parkinson's disease (PD) patients with the University of Pennsylvania 12 smell identification test (UPSIT), establishing a comparison with 76 age-matched healthy controls, and associate with clinical and epidemiologic picture. METHOD: The PD group was evaluated in phase "on" through United Parkinson's disease rating scale, UPSIT, and Hoehn and Yahr stage and the control group with the UPSIT. RESULTS:The mean UPSIT score was 5.7 in PD patients and 9 in the control group. Patients that presented initially resting tremor and those that currently have tremor, rigidity and bradykinesia had a significant lower scores. There were negative correlation between patients' age and PD stage with the UPSIT scores. There were no correlation between olfactory scores, age at the initial PD symptoms and disease duration. CONCLUSION:Among PD patients 80 percent had olfactory deficit and, therefore, smell evaluation may be a tool to make PD differential diagnosis.

Evaluation of Taenia solium and Taenia crassiceps cysticercal antigens for immunodiagnosis of neurocysticercosis using ELISA on cerebrospinal fluid samples.

The efficacy of whole parasite and vesicular fluid antigen extracts from Taenia solium and Taenia crassiceps cysticerci for immunodiagnosis of neurocysticercosis was evaluated using ELISA on cerebrospinal fluid samples. Anticysticercal IgG antibodies were assayed in cerebrospinal fluid samples from 23 patients with neurocysticercosis and 35 patients with other neurological disorders. The ELISA reaction for the whole Taenia solium cysticercal extract showed 91.3% sensitivity and 94.3% specificity, whereas the sensitivity and specificity of the ELISA for the whole Taenia crassiceps cysticercal extract were 87% and 94.3%, respectively. The ELISA reactions for vesicular fluid from Taenia solium or Taenia crassiceps showed 91.3% sensitivity and 97.1% specificity. Considering the results obtained from the four antigen preparations, vesicular fluid from Taenia solium and Taenia crassiceps cysticerci may be useful as a source of antigens for immunological reactions that are used for detecting specific antibodies in cerebrospinal fluid samples from patients with neurocysticercosis.

Evaluation of Taenia solium and Taenia crassiceps cysticercal antigens for immunodiagnosis of neurocysticercosis using ELISA on cerebrospinal fluid samples

The efficacy of whole parasite and vesicular fluid antigen extracts from Taenia solium and Taenia crassiceps cysticerci for immunodiagnosis of neurocysticercosis was evaluated using ELISA on cerebrospinal fluid samples. Anticysticercal IgG antibodies were assayed in cerebrospinal fluid samples from 23 patients with neurocysticercosis and 35 patients with other neurological disorders. The ELISA reaction for the whole Taenia solium cysticercal extract showed 91.3 percent sensitivity and 94.3 percent specificity, whereas the sensitivity and specificity of the ELISA for the whole Taenia crassiceps cysticercal extract were 87 percent and 94.3 percent, respectively. The ELISA reactions for vesicular fluid from Taenia solium or Taenia crassiceps showed 91.3 percent sensitivity and 97.1 percent specificity. Considering the results obtained from the four antigen preparations, vesicular fluid from Taenia solium and Taenia crassiceps cysticerci may be useful as a source of antigens for immunological reactions that are used for detecting specific antibodies in cerebrospinal fluid samples from patients with neurocysticercosis.

A eficácia de extratos antigênicos de parasitas totais e líquido vesicular de cisticercos de Taenia solium e Taenia crassiceps para o imunodiagnóstico da neurocisticercose foi avaliada por meio de reações de ELISA em amostras de líquido cefalorraquidiano. Anticorpos IgG anti-cisticercos foram pesquisados em amostras de líquido cefalorraquidiano de 23 pacientes com neurocisticercose e 35 pacientes com outras doenças neurológicas. A reação ELISA com o extrato bruto total de cisticercos de Taenia solium apresentou 91,3 por cento de sensibilidade e 94,3 por cento de especificidade, enquanto a sensibilidade e a especificidade da reação ELISA com o extrato total de cisticercos de Taenia crassiceps foram 87 por cento e 94,3 por cento, respectivamente. As reações ELISA com o líquido vesicular de Taenia solium ou Taenia crassiceps mostraram 91,3 por cento de sensibilidade e 97,1 por cento de especificidade. Considerando os resultados obtidos com as quatro preparações antigênicas, o liquido vesicular de cisticercos de Taenia solium e Taenia crassiceps pode ser útil como fonte de antígenos em reações imunológicas usadas para detectar anticorpos específicos em amostras de líquido cefalorraquidiano de pacientes com neurocisticercose.

Vascular parkinsonism: analysis of seven cases.

INTRODUCTION: Neuroimaging studies of elderly individuals reveal alterations in the white matter that are incompatible with the patients parkinsonism, mistakenly classified as vascular parkinsonism (VP). METHOD: This study was conducted on a population composed of 20 patients with Parkinsons disease (PD) whose neuroimaging exams revealed vascular alterations in the white matter and seven patients with VP in order to compare diagnostic criteria. RESULTS: Age at disease onset of patients with PD was 55+/-12 years and patients with VP it was 62+/-13 years. Twelve patients with PD and five patients with VP presented arterial hypertension; three patients with VP and two patients with PD presented gait impairment; all patients with VP presented rigidity and bradykinesia, six of them presented resting tremor; 19 patients with PD presented tremor and 19 of them presented rigidity, while 17 presented bradykinesia. When the symptoms and evolution of both diseases were compared, the vascular alterations in the white matter were considered unspecific. CONCLUSION: Since clinical symptoms are unspecific, a differential diagnosis requires neuroimaging, good response to levodopa and clinical evolution.