Experiencia con la enfermedad de Creutzfeldt-Jakob de un único centro de referencia en México. Análisis de una serie de casos / Experience with Creutzfeldt-Jakob disease in a single referral center in Mexico. Case series

Resumen Introducción: La enfermedad de Creutzfeldt-Jakob (ECJ) es una enfermedad del sistema nervioso central rápidamente progresiva y mortal causada por priones. Objetivo: Presentar las principales características clínicas y paraclínicas de pacientes con probable ECJ en un centro de referencia de América Latina. Métodos: Estudio retrospectivo de pacientes diagnosticados con demencia rápidamente progresiva entre 2014 y 2019. Se incluyeron características clínicas, demográficas, del electroencefalograma, imágenes por resonancia magnética, proteína 14-3-3 y tomografía por emisión de positrones (PET), cuando estaba disponible. Resultados: Veinticuatro pacientes cumplieron con los criterios de ECJ esporádica (75 % mujeres), la edad media fue de 59.29 ± 11.67 años, la duración de la enfermedad desde el inicio de los síntomas hasta el ingreso hospitalario fue de 7.41 ± 6.54 meses y las primeras manifestaciones más comunes fueron las alteraciones del comportamiento (41.7 %). Los complejos de ondas delta prevalecieron en el electroencefalograma (54.2 %), la hiperintensidad cortical en la resonancia magnética (83.3 %) y el hipometabolismo frontal en la PET (37.5 %). En el análisis del líquido cefalorraquídeo, siete casos mostraron proteína tau total positiva; cinco, proteína 14-3-3 positiva; y tres, proteína tau hiperfosforilada positiva. Conclusiones: Existe importante heterogeneidad clínica en cuanto a los síntomas iniciales. Los hallazgos de las pruebas auxiliares coincidieron con los de otras series.

Abstract Introduction: Creutzfeldt-Jakob disease (CJD) is a rapidly progressive and fatal central nervous system disease caused by prions. Objective: To present the main clinical and paraclinical characteristics of patients with probable CJD in a referral center of Latin America. Methods: Retrospective study of patients diagnosed with rapidly progressive dementia between 2014 and 2019. Clinical, demographic, electroencephalogram, magnetic resonance imaging, and 14-3-3 protein characteristics were included, as well as positron-emission tomography (PET) data when available. Results: Twenty-four patients met the criteria for sporadic CJD (75% were women). Mean age was 59.29 ± 11.67 years, while mean disease duration from symptom onset to hospital admission was 7.41 ± 6.54 months. The most common first symptom was behavioral changes (41.7%). Delta wave complexes prevailed (54.2%) on electroencephalogram, cortical hyperintensity (83.3%) on magnetic resonance and frontal hypometabolism (37.5%) on PET. Seven cases showed positive total Tau; five, positive 14-3-3 protein; and three, positive phosphorylated tau on cerebrospinal fluid analysis. Conclusions: There is significant clinical heterogeneity regarding initial symptoms. Auxiliary test findings were consistent with those of other series.

Assessment of therapeutic strategies for management of impulse control disorder in Parkinson's disease / Evaluación de las estrategias terapéuticas en trastornos del control de impulsos en enfermedad de Parkinson

ABSTRACT Background: Impulse control disorders (ICD) occur frequently in individuals with Parkinson's disease. So far, prevention is the best treatment. Several strategies for its treatment have been suggested, but their frequency of use and benefit have scarcely been explored. Objective: To investigate which strategy is the most commonly used in a real-life setting and its rate of response. Methods: A longitudinal study was conducted. At the baseline evaluation, data on current treatment and ICD status according to QUIP-RS were collected. The treatment strategies were categorized as "no-change", dopamine agonist (DA) dose lowering, DA removal, DA switch or add-on therapy. At the six-month follow-up visit, the same tools were applied. Results: A total of 132 individuals (58.3% men) were included; 18.2% had at least one ICD at baseline. The therapeutic strategy most used in the ICD group was no-change (37.5%), followed by DA removal (16.7%), DA switch (12.5%) and DA lowering (8.3%). Unexpectedly, in 20.8% of the ICD subjects the DA dose was increased. Overall, nearly 80% of the subjects showed remission of their ICD at follow-up. Conclusions: Regardless of the therapy used, most of the subjects presented remission of their ICD at follow-up Further research with a longer follow-up in a larger sample, with assessment of decision-making processes, is required in order to better understand the efficacy of strategies for ICD treatment.

Resumen Antecedentes: Los trastornos del control de impulsos (TCI) son frecuentes en personas con enfermedad de Parkinson. A la fecha, la prevención es el mejor tratamiento. Existen varias estrategias sugeridas para su tratamiento, pero su frecuencia de uso y beneficio ha sido escasamente explorada. Objetivo: Investigar qué estrategia es la más utilizada en un entorno de la vida real y su tasa de respuesta. Métodos: Se realizó un estudio longitudinal. En la evaluación inicial, se recopiló el tratamiento actual y el estado del TCI de acuerdo con el QUIP-RS. La estrategia de tratamiento se clasificó como "sin cambios", reducción de la dosis de agonista de la dopamina (AD), eliminación de AD, cambio de AD o terapia complementaria. En la visita de seguimiento a los 6 meses, se aplicaron las mismas herramientas. Resultados: Se incluyeron un total de 132 (58.3% hombres) personas. El 18.2% tenía al menos un TCI al inicio del estudio. La estrategia terapéutica más utilizada en el grupo de TCI fue sin cambios (37.5%), seguida de eliminación de DA (16.7%), cambio de AD (12.5%) y reducción de DA (8.3%). En el 20.8% de los sujetos con TCI se aumentó la dosis de AD. Casi el 80% de los sujetos tuvieron una remisión del TCI al seguimiento. Conclusiones: Independientemente de la terapia utilizada, la mayoría de los sujetos tuvieron una remisión del TCI. Se requiere más investigación con un seguimiento y una muestra mayor para evaluar l proceso de toma de decisiones para comprender mejor la eficacia de las estrategias.

Malnutrition and Associated Motor and Non-motor Factors in People with Parkinson's Disease

Background: People with Parkinson’s disease (PwP) are at higher risk of developing malnutrition. Several factors have been suggested to be involved including motor symptoms, non-motor symptoms, and treatment-related complications. Objective: The objective of the study was to analyze the combined effect of motor, non-motor, and pharmacological factors in the risk of malnutrition in PwP. Methods: Eighty-seven consecutive PwP were included in the study. Clinical data and pharmacological treatment were collected. Nutritional status was assessed using the Mini-Nutritional Assessment (MNA) questionnaire. Movement Disorder Society Unified Parkinson’s Disease Rating Scale (MDS-UPDRS), Non-motor Symptoms Scale (NMSS), Hamilton Depression Rating Scale HAM-D, and Montreal Cognitive Assessment were applied. Results: Thirty (34.4%) PwP were at risk of malnutrition and seven had malnutrition (8%). Abnormal nutritional status was associated with lower education, higher MDS-UPDRS Parts I, II, and III and total scores, and higher scores in the NMSS domain of sleep disorders and fatigue. MDS-UPDRS motor score remained as a determinant of abnormal nutritional status, defined as MNA <23.5, with an odds ratio 1.1 (95% confidence interval 1.01-1.10, p = 0.02). Conclusion: The main factor associated with nutritional status was severity of the motor symptoms as assessed by the MDS-UPDRS Part III. Non-motor symptoms and treatment-related complications were not associated with malnutrition. (REV INVEST CLIN. 2020;72(5):293-9)

Neurological manifestations in coronavirus disease 2019 / Manifestaciones neurológicas en la enfermedad del coronavirus 2019

Abstract Coronavirus disease 2019 (COVID-19), an infection caused by severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2), is currently hitting the world in the form of a pandemic. Given that some reports suggest that this infection can also occur with neurologic manifestations, this narrative review addresses the basic and clinical aspects concerning the nervous system involvement associated with this disease. More than one third of patients hospitalized for COVID-19 can present with both central and peripheral neurological manifestations. The former includes dizziness and headache, while the latter includes taste and smell disturbances. Other reported neurological manifestations are cerebrovascular disease and epileptic seizures. According to published reports, neurological disorders are not uncommon in COVID-19 and can sometimes represent the first manifestation of the disease; therefore, neurologists should consider this diagnostic possibility in their daily practice. Since maybe not all COVID-19 neurological manifestations are due to SARS-CoV-2 direct effects, it is important to monitor the rest of the clinical parameters such as, for example, oxygen saturation. Similarly, follow-up of patients is advisable, since whether neurological complications may develop lately is thus far unknown.

Resumen La enfermedad del coronavirus 2019 (COVID-19), infección causada por el coronavirus 2 del síndrome respiratorio agudo grave (SARS-CoV-2), actualmente afecta al mundo en forma de una pandemia. Debido a que algunos reportes apuntan a que esta infección puede cursar también con manifestaciones neurológicas, en esta revisión narrativa se abordan los aspectos básicos y clínicos concernientes a la afectación del sistema nervioso por esta enfermedad. Más de un tercio de los pacientes hospitalizados por COVID-19 pueden presentar manifestaciones neurológicas, tanto centrales como periféricas. Entre las primeras se encuentran el mareo y la cefalea; y entre las segundas, las alteraciones del gusto y el olfato. Otras manifestaciones neurológicas reportadas son la enfermedad vascular cerebral y las crisis epilépticas. Según los informes publicados, los padecimientos neurológicos no son infrecuentes en COVID-19 y en ocasiones pueden representar la primera manifestación de la enfermedad, de modo que los neurólogos deberán considerar esta posibilidad diagnóstica en su práctica cotidiana. Dado que no todas las manifestaciones neurológicas de COVID-19 pudieran deberse a efectos directos de SARS-CoV-2, es importante monitorear el resto de los parámetros clínicos, por ejemplo, la oxigenación. De igual forma, es recomendable el seguimiento de los pacientes, ya que hasta el momento se ignora si las complicaciones neurológicas pueden desarrollarse tardíamente.

Falls in persons with Parkinson's disease: Do non-motor symptoms matter as much as motor symptoms? / Caídas en personas con enfermedad de Parkinson: ¿Los síntomas no motores importan tanto como los síntomas motores?

ABSTRACT Falls are common among persons with Parkinson's disease (PD). On the other hand, predicting falls is complex as there are both generic and PD-specific contributors. In particular, the role of non-motor symptoms has been less studied. Objective: The objective of this study was to identify the role of non-motor predictors of falling in persons with PD (PwP). Methods: A cross-sectional study was carried out in PwP recruited from a movement disorders clinic. Clinical and demographical data were collected. All PwP were assessed using the Movement Disorders Society Unified Parkinson's Disease Rating Scale (MDS-UPDRS) and the Non-Motor Symptoms Scale (NMSS). Variables were assessed at the bivariate level. Significant variables were put into a logistic regression model. Results: A total of 179 PwP were included. Overall, 16.8% of PwP had fallen in the past 12 months, with 53.3% of them being recurrent fallers. The mean number of monthly falls was 2.5 ± 3.3. Factors associated with falling in the bivariate analysis included the disease duration, Hoehn and Yahr stage, MDS-UPDRS part I and II, postural instability/gait disturbance (PIGD) subtype, NMSS urinary domain, NMSS miscellaneous domain, and non-motor severity burden (all p-values < 0.05). After multivariate analysis, only the disease duration (p = 0.03) and PIGD (p = 0.03) remained as independent risk factors. Conclusion: Disease duration and the PIGD subtype were identified as relevant risk factors for falls in PwP Non-motor symptoms appear to have a less important role as risk factors for falls.

RESUMEN Las caídas son frecuentes entre las personas con Parkinson (EP). La predicción de caídas es compleja ya que existen contribuyentes genéricos y específicos. El papel de los síntomas no motores ha sido menos estudiado. Objetivo: Identificar el papel de los factores no motores en caídas en personas con EP (PcP). Métodos: Estudio transversal en PcP reclutadas en una clínica de trastornos del movimiento. Se incluyeron datos clínicos y demográficos. Todos los PcP se evaluaron con la Escala Unificada de Enfermedad de Parkinson modificada por la Sociedad Internacional de Trastornos del Movimiento (MDS-UPDRS) y la Escala de Síntomas No Motores (NMSS). Se incluyeron variables significativas en un modelo de regresión logística. Resultados: Se incluyeron un total de 179 PcP El 16.8% había presentado una caída en los últimos doce meses y el 53.3% de forma recurrente. El número medio de caídas mensuales fue de 2.5 ± 3.3. Los factores asociados con la caída en el análisis bivariado fueron la duración de la enfermedad, Hoehn e Yahr, MDS-UPDRS parte I y II, subtipo de alteración de la marcha/inestabilidad postural (PIGD), dominio urinario del NMSS, dominio misceláneo del NMSS y carga de severidad no motora (todos los valores de p < 0.05). Después del análisis multivariado, solo la duración de la enfermedad (p = 0.03) y PIGD (p = 0.03) permanecieron como un factor de riesgo independiente. Conclusión: La duración de la enfermedad y PIGD se identificaron como factores de riesgo para caídas. Los síntomas no motores parecen tener un papel menos relevante en las caídas.

Frecuencia De Casos Juveniles Con Enfermedad De Huntington En Población Mexicana / Frequency Of Juvenile Huntington´S Disease In A Mexican Population

Resumen Objetivo: El propósito de este estudio es determinar la prevalencia de casos juveniles en una muestra de sujetos mexicanos con enfermedad de Huntington (EH) confirmada molecularmente. Métodos: Se incluyeron pacientes con inicio clínico antes de los 21 años de edad que acudieron a la clínica de trastornos del movimiento del Instituto Nacional de Neurología y Neurocirugía. La información demográfica y clínica se obtuvo de la revisión de expedientes. Resultados: Se revisaron un total de 198 casos de pacientes con diagnóstico de EH, de los cuales el 6.5% (n=13) correspondió a formas juveniles. La media de edad para el inicio de síntomas fue de 17.8 ± 3.9 años. La puntuación media del UHDRS-motor fue de 46.2 ± 17.4 puntos. El síntoma motor predominante fue corea en el 53.8% de los casos. El 84.6% de los afectados presentó al menos una alteración neuropsiquiátrica. Conclusión: Se detectó que el fenotipo motor de estos pacientes fue dominantemente corea, contrario a lo reportado mundialmente hasta ahora, es decir, nuestro grupo presentó clínica motora atípica de EHJ.

Abstract Objective: The purpose of this study is to know the prevalence of juvenile cases in a sample of mexican subjects with confirmed Huntington Disease (HD). Methods: Patients with clinical debut before 21 years of age were included who attended at movement disorders clinic of the National Institute of Neurology and Neurosurgery. The demographic and clinical information was obtained from the review of files. Results: A total of 198 cases of patients diagnosed with HD were reviewed, of which 6.5% (n = .13) corresponded to juvenile forms. The mean age for the onset of symptoms was 17.8 ± 3.9 years. The mean score of the UHDRS-motor was 46.2 ± 17.4 points. The predominant motor symptom was chorea in (53.8%) of the cases. 84.6% of those affected presented at least one neuropsychiatric disorder. Conclusion: It was detected that the dominant motor phenotype of these patients was chorea compared to the world reports until now, accordingly to that, our group of juvenile HD shows atypical motor clinical.

Pruebas Terapéuticas Con Fines Diagnósticos En Trastornos Del Movimiento / Therapeutic Tests For The Diagnosis Of Movement Disorders

Resumen El diagnóstico en medicina tiene su piedra angular en la obtención de la historia clínica y la exploración física; sin embargo, en algunas de las patologías el uso de pruebas diagnósticas es de gran utilidad como apoyo para confirmar o descartar una enfermedad. Las pruebas diagnósticas incluyen una amplia variedad de procedimientos y dentro de estas se incluyen las pruebas terapéuticas, también llamadas desafíos o retos, en los que se administra una sustancia activa para evaluar la respuesta y apoyar la impresión diagnóstica. En el contexto de los trastornos del movimiento las pruebas terapéuticas más frecuentemente utilizadas son la de levodopa, apomorfina y etanol. En esta revisión se presenta, describe y discute el alcance y utilidad de estas.

Abstract The diagnosis in medicine has its cornerstone in obtaining the clinical history and in the physical examination. However, in some of the pathologies the use of diagnostic tests is very useful as a support to confirm or rule out a disease. Diagnostic tests include a wide variety of procedures and these include therapeutic tests, also called challenges, in which an active substance is administered to evaluate the response and support the presumptive diagnosis. In the context of movement disorders, the most frequently used therapeutic tests are those with levodopa, apomorphine and ethanol. In this review we present, describe and discuss the scope and usefulness of these challenges.

Motor and non-motor wearing-off and its impact in the quality of life of patients with Parkinson's disease / Flutuações motoras e não-motoras e seu impacto na qualidade de vida de pacientes com doença de Parkinson

ABSTRACT The wearing-off phenomenon is common in patients with Parkinson's disease. Motor and non-motor symptoms can fluctuate in relation to the "on/off" periods. Objective: To assess the impact of motor and non-motor wearing-off on activities of daily living and quality of life of patients with PD. Methods: A cross-sectional study was carried out. All patients were evaluated using the Movement Disorders Society Unified Parkinson's Disease Rating Scale. Wearing-off was assessed using the Wearing-Off Questionnaire-19, and quality of life was assessed using the Parkinson's Disease Questionnaire-8. Results: A total of 271 patients were included; 73.4% had wearing-off; 46.8% had both motor and non-motor fluctuations. Patients with both motor and non-motor wearing-off had a worst quality of life compared with those with only motor fluctuations (p = 0.047). Conclusions: Motor and non-motor fluctuations have an impact on activities of daily living and quality of life. Non-motor wearing-off may have a higher impact.

RESUMO O fenômeno de encurtamento do fim de dose é comum em pacientes com doença de Parkinson. Tanto os sintomas motores quanto os não motores podem flutuar em relação aos períodos de "on/off". Objetivo: Avaliar o impacto das flutuações motoras e não-motoras nas atividades da vida diária e qualidade de vida em pacientes com doença de Parkinson. Métodos: Um estudo transversal foi realizado. Todos os sujeitos foram avaliados utilizando a escala unificada para a doença de Parkinson da Sociedade de Distúrbios do Movimento. O encurtamento do fim de dose foi avaliado através do questionário WOQ-19 e a qualidade de vida foi avaliada através do PDQ-8. Resultados: Um total de 271 pacientes foram incluídos, 73,4% tiveram deterioração de fim de dose. A maioria dos pacientes tiveram tanto flutuações motoras quanto não-motoras (46,8%). Os pacientes com ambos os tipos de flutuações motoras e não-motoras tiveram pior qualidade de vida do que pacientes apenas com flutuações motoras (p = 0.047). Conclusões: Pacientes com flutuações motoras e não-motoras tiveram impacto significativo nas atividades da vida diária e na qualidade de vida. As flutuações não-motoras parecem ter um impacto maior que as flutuações motoras sobre a qualidade de vida.

Electroconvulsive therapy in Parkinson´s disease / A eletroconvulsoterapia na doença de Parkinson

Purpose To analyze the effectiveness of electroconvulsive therapy for the management of depression and/or psychosis refractory to drug therapy in patients with Parkinson disease.Methods A retrospective study was carried out including patients treated with electroconvulsive therapy during the period between 2002 and 2013. A review of the literature was performed.Results A total of 27 patients were included. In regards to the neuropsychiatric diagnosis, 14 patients had major depression, 12 patients had both psychosis and depression, and only one patient had isolated psychosis. The mean number of electroconvulsive therapy sessions was 12 ± 2.8. After electroconvulsive therapy, all patients showed a statistically significant improvement in the Brief Psychiatric Rating scale (reduction of 52% points) and Hamilton Depression Rating Scale (reduction of 50% points) independent of the presence of psychosis, depression or both.Conclusion Electroconvulsive therapy is effective for the treatment of refractory neuropsychiatric symptoms in Parkinson’s disease.

Propósito Analisar a eficácia da eletroconvulsoterapia para o tratamento da depressão e/ou psicoses refratária ao tratamento medicamentoso em pacientes com doença de Parkinson.Métodos Um estudo retrospectivo foi realizado com pacientes tratados com a eletroconvulsoterapia, durante o período entre 2002 e 2013. Uma revisão da literatura foi realizada.Resultados Um total de 27 pacientes foram incluídos. Em relação ao diagnóstico neuropsiquiátrico, 14 pacientes tinham depressão maior, 12 pacientes tiveram tanto psicoses e depressão, e apenas um paciente tinha isolado psicoses. O número médio de sessões de eletroconvulsoterapia foi de 12 ± 2,8. Após a eletroconvulsoterapia, todos os pacientes apresentaram uma melhora estatisticamente significativa no Brief Psychiatric Rating Scale (redução de 52% de pontos) e Hamilton Depression Rating Scale (redução de 50% de pontos), independente da presença de psicose, depressão ou ambos.Conclusão Eletroconvulsoterapia é eficaz para o tratamento de sintomas neuropsiquiátricos refractários na doença de Parkinson.

Prevalence and correlates of sleep disorders in Parkinson’s disease: a polysomnographic study / Prevalência e correlatos de distúrbios do sono na doença de Parkinson: estudo polissonográfico

Objective Sleep disorders in Parkinson’s disease are very common. Polysomnography (PSG) is considered the gold standard for diagnosis. The aim of the present study is to assess the prevalence of nocturnal sleep disorders diagnosed by polysomnography and to determine the associated clinical factors. Method A total of 120 patients with Parkinson’s disease were included. All patients underwent a standardized overnight, single night polysomnography. Results Ninety-four (78.3%) patients had an abnormal PSG. Half of the patients fulfilled criteria for sleep apnea-hypopnea syndrome (SAHS); rapid eye movement behavior disorder (RBD) was present in 37.5%. Characteristics associated with SAHS were age (p = 0.049) and body mass index (p = 0.016). Regarding RBD, age (p < 0.001), left motor onset (p = 0.047) and levodopa equivalent dose (p = 0.002) were the main predictors. Conclusion SAHS and RBD were the most frequent sleep disorders. Higher levodopa equivalent dose and body mass index appear to be risk factors for RBD and SAHS, respectively. .

Objetivo Os distúrbios do sono na doença de Parkinson são muito comuns. A polissonografia é considerada o padrão-ouro para o diagnóstico. O objetivo do presente estudo é avaliar a prevalência de distúrbios de sono noturno diagnosticados por polissonografia. Método 120 pacientes com doença de Parkinson foram incluídos. Todos os pacientes foram submetidos a uma única noite, polissonografia de noite. Resultados 94 (78,3%) pacientes tiveram uma polissonografia anormal e 50% preencheram a síndrome da apneia e hipopneia do sono (SAHOS); distúrbio de comportamento do movimento rápido dos olhos (RBD) esteve presente em 37,5%. As características associadas com SAHOS foram idade (p = 0,049) e índice de massa corporal (p = 0,016). Quanto RBD, idade (p < 0,001), deixou início motor (p = 0,047) e levodopa dose equivalente (p = 0,002) foram os preditores. Conclusão SAHOS e RBD foram os distúrbios do sono mais frequente. Dose superior equivalente de levodopa e índice de massa corporal parecem ser fatores de risco, respectivamente. .

Cardiovascular variability in Mexican patients with Parkinson's disease / Variabilidade cardiovascular em pacientes mexicanos com doença de Parkinson

Cardiovascular variability (CVV) has been evaluated in patients with Parkinson's disease (PD) in other countries and exhibit ethnic differences. Objective We investigated heart rate variability (HRV) and blood pressure variability (BPV) in Mexican patients with PD. Method We further compared HRV and BPV between this group and young healthy controls (YHC) in order to estimate, for the first time in our country, the magnitude of the difference. Twenty patients were examined. Time- and frequency-domain CVV parameters were studied during supine rest (SR), active standing (AS) and controlled breathing. These measurements were compared to those of 20 YHC. Results In the three conditions tested, our study showed a decrease in almost all HRV parameters in PD patients; on the contrary, decreased BPV parameters were found less frequently and only during SR and AS. Conclusion Our results indicate that HRV is impaired in PD. Some BPV parameters are also diminished. .

A variabilidade cardiovascular (VCV) tem sido avaliada em doentes parkinsonianos em vários países e apresenta diferenças étnicas. Objetivo Investigar a variabilidade da frequência cardíaca (VFC) e a variabilidade da pressão arterial (VPA) em doentes parkinsonianos mexicanos. Método Comparamos VFC e VPA entre o grupo de estudo e controles jovens (CJ), a fim de estimar a magnitude da diferença. CVV foi estudada no domínio do tempo e da frequência durante repouso supino (RS), ortostase ativa (OA) e respiração controlada. Estas medições foram comparadas com aquelas de 20 CJ. Resultados Nosso estudo mostrou diminuição em quase todos os parâmetros da VFC em doentes parkinsonianos. A diminuição dos parâmetros da VPA foi encontrada com menos frequência. Conclusão Nossos resultados indicam que a VFC é prejudicada na doença de Parkinson. Alguns parâmetros do VPA também são reduzidos. .

Initial circulatory response to active standing in Parkinson's disease without typical orthostatic hypotension / Resposta circulatoria inicial apos ortostatismo ativo na doenca de Parkinson sem hipotensao ortostatica tipica

While the circulatory response to orthostatic stress has been already evaluated in Parkinson's disease patients without typical orthostatic hypotension (PD-TOH), there is an initial response to the upright position which is uniquely associated with active standing (AS). We sought to assess this response and to compare it to that seen in young healthy controls (YHC). Method In 10 PD-TOH patients (8 males, 60±7 years, Hoehn and Yahr ≤3) the changes in systolic blood pressure (SBP) and heart rate that occur in the first 30 seconds (sec) of standing were examined. Both parameters were non-invasively and continuously monitored using the volume-clamp method by Peñáz and the Physiocal criteria by Wesseling. The choice of sample points was prompted by the results of previous studies. These sample points were compared to those of 10 YHC (8 males, 32±8 years). Results The main finding of the present investigation was an increased time between the AS onset and SBP overshoot in PD-TOH group (24±4 vs. 19±3 sec; p<0.05). Conclusion This delay might reflect a prolonged latency in the baroreflex-mediated vascular resistance response, but more studies are needed to confirm this preliminary hypothesis. .

Apesar da resposta circulatória ao estresse ortostático já foi estudada em pacientes com doença de Parkinson sem hipotensão ortostática típica (PD-TOH), não há uma resposta inicial que é exclusivamente associada com o ortostase ativa (AS). Portanto, buscou-se avaliar esta resposta e compará-la à observada em jovens saudáveis (YHC). Método Em 10 PD-TOH pacientes (8 homens, 60±7 anos, Hoehn e Yahr ≤3) as mudanças na pressão arterial sistólica (PAS) e da frequência cardíaca que ocorrem nos primeiros 30 segundos (seg) de pé foram examinados. Ambos parâmetros foram monitorizados continuamente através do método Peňáz e os critérios de Wesseling. Os pontos de amostragem foram escolhidos com base em estudos anteriores. Estes pontos foram comparados com os de 10 YHC (32±8 anos). Resultados O principal achado deste estudo foi o aumento do tempo entre o início de AS e rebote sistólica no grupo PD-TOH (24±4 vs 19±3 seg, p<0,05). Conclusão Este atraso pode refletir uma latência prolongada na resposta da resistência vascular mediado pelo barorreflexo, mas outros estudos são necessários para confirmar esta hipótese preliminar. .

Low specificity and sensitivity of smell identification testing for the diagnosis of Parkinsons disease / Baixa sensibilidade e especificidade do teste de identificacao de odores para o diagnostico da doenca de Parkinson

Objective: The aim of this study is to determine if the University of Pennsylvania’s Smell Identification Test (UPSIT) is an accurate diagnostic tool for olfactory dysfunction in Parkinson’s disease (PD). Method: We included 138 non-demented PD subjects and 175 control subjects matched by gender. Smell identification was tested using UPSIT. Results: The mean number of UPSIT items correctly identified by controls was 27.52±5.88; the mean score for PD subjects was 19.66±6.08 (p=<0.001). UPSIT sensitivity was 79.7% with a specificity of 68.5% using a cut-off score of ≤25. The overall accuracy for the diagnosis of PD was of 75.3%. Conclusion: UPSIT accuracy and specificity were lower than what has been previously reported. Our data demonstrates that 17.5% of items of the UPSIT were not well identified by healthy controls. Further research of the identification of a truly cross-cultural test is warranted. .

Objetivo: O objetivo deste estudo é determinar se o University of Pennsylvania Smell Identification Test (UPSIT) é uma ferramenta diagnóstica útil para a caracterizar disfunção olfativa na doença de Parkinson (DP). Método: Foram incluídos 138 indivíduos não dementes assuntos PD e 175 indivíduos controle pareados por sexo. Identificação cheiro foi testada usando UPSIT. Resultados: O número médio de itens UPSIT corretamente identificados pelos controles foi de 27,52±5,88; para sujeitos com DP foi de 19,66±6,08 (p=<0,001). A sensibilidade do UPSIT foi de 79,7%, com especificidade de 68,5%, utilizando um ponto de corte de ≤25. A exatidão global para o diagnóstico de DP foi de 75,3%. Conclusão: A precisão e a especificidade do UPSIT foram menores do que o que foi relatado anteriormente. Nossos dados demonstram que 17,5% dos itens da UPSIT não foram adequadamente identificados pelos controles saudáveis. São necessárias outras pesquisas para a identificação de um teste verdadeiramente cross-cultural nessa área. .

Respuesta ortostática de la tensión arterial de pacientes con enfermedad de Parkinson inicial / Orthostatic response of blood pressure in patients with early Parkinson's disease

Objetivo: Evaluar la respuesta ortostática de la tensión arterial de forma no invasiva en pacientes con enfermedad de Parkinson inicial leve a moderada atendidos en el Instituto Nacional de Neurología y Neurocirugía. Método: Se realizó un estudio de corte transversal con grupo control. Se incluyeron pacientes con enfermedad de Parkinson de menos de 5 años de evolución y severidad leve a moderada así como controles sanos. Se excluyeron sujetos con diabetes mellitus, hipertensión arterial o diagnóstico de disautonomía. Se realizaron mediciones de la tensión arterial en posición supina, y posteriormente a los3y5minde asumir bipedestación. Resultados: Se incluyeron 70 sujetos con enfermedad de Parkinson y 145 controles. No se encontraron diferencias estadísticamente significativas en género y edad entre ambos grupos. No hubo diferencias en el cambio de la tensión arterial sistólica entre los grupos a los 3 min pero sí a los 5min (p = 0.03). En el caso de la tensión diastólica existió diferencia entre ambos grupos a los 3 y 5min (p = 0.02 y p = 0.007). Conclusión: Los pacientes con enfermedad de Parkinson inicial presentan diferencias en la respuesta ortostática con respecto a controles sanos, siendo de mayor relevancia a los 5 min de bipedestación.

Objective: To evaluate orthostatic blood pressure response in patients with early Parkinson's disease and mild to moderate disease treated at the National Institute of Neurology and Neurosurgery. Method: A cross-sectional study with control group was carried out. Patients with Parkinson's disease with a disease duration of less than 5 years and mild to moderate severity along with healthy controls of the same age group were included. Those subjects with diabetes mellitus, hypertension or diagnosis of dysautonomia were excluded. Blood pressure was measured in supine position as well as after 3 and 5 min of standing. Results: Seventy subjects with Parkinson's disease and 145 controls were included. There were no statistically significant differences in gender and age between both groups. There was no difference in systolic blood pressure change between groups at 3 minutes but at 5 minutes a statistically significant difference was found (P = .03). For diastolic blood pressure a difference between both groups was present at three and five minutes (P = .02 and P = .007). Conclusion: Patients with early Parkinson's disease have differences in orthostatic responses compared to healthy controls, these differences are more relevant at 5 minutes of standing.

Overweight is more prevalent in patients with Parkinson's disease / Excesso de peso é mais frequente em pacientes com doença de Parkinson

Underweight and malnutrition are well documented in Parkinson's disease (PD), while overweight has been less reported. We carried out a cross-sectional study including 177 healthy controls and 177 PD patients attending a tertiary care center. We recorded weight and height for all participants. A statistically significant difference was found in body mass index (BMI) between controls and PD patients (29.1±5.4 versus 27.2±4.7, p<0.001). In the PD Group, two patients were underweight, 32.7% were within normal range, 46.9% had overweight, and 19.2% were obese. Overweight and normal weight were more prevalent in the PD Group (p=<0.01 and <0.001, respectively) when compared to controls. In conclusion, overweight/obesity are common among patients with PD, while underweight is almost negligible.

Baixo peso e desnutrição são muito documentadas na doença de Parkinson (DP), enquanto que o excesso de peso tem sido menos relatado. Foi realizado um estudo transversal com 177 controles saudáveis e 177 pacientes com DP que frequentavam um centro terciário. O peso e a altura de todos os participantes foram arquivados. Uma diferença estatisticamente significativa no índice de massa corporal (IMC) foi encontrada entre controles e pacientes com DP (29,1±5,4 versus 27,2±4,7, p<0,001). No Grupo DP, dois pacientes estavam abaixo do peso, 32,7% estavam dentro do intervalo normal, 46,9% apresentavam sobrepeso e 19,2% eram obesos. Peso normal e excesso de peso foram mais prevalentes no Grupo DP (p=<0,01 e <0,001, respectivamente) em relação aos controles. Em conclusão, o sobrepeso/obesidade são comuns entre os pacientes com DP, enquanto baixo peso nessa população é quase insignificante.

Detección y manejo de síntomas no motores en la enfermedad de Parkinson: impacto en su prevalencia / Detection and management of non-motor symptoms in Parkinson's disease: impact on their prevalence

Background: Non-motor symptoms are common among patients with Parkinson disease and include cognitive, psychiatric, sleep and autonomic dysfunctions. Aim: To determine if the detection of non-motor symptoms along with the appropriate referral to an specialist has an impact on their prevalence after one year follow-up. Material and Methods: We included 60 patients diagnosed with Parkinson´ s disease attending to the Movement Disorder Clinic. The presence of non-motor symptoms was determined by the non-motor symptom questionnaire (NMSQuest). Results: The mean NMSQuest scores at baseline and at one year follow up were 12.6 ± 6.2 and 9.9 ± 5.6, respectively (p < 0.01). The non-motor symptoms that showed a statistically significant differences in frequency between baseline and the final assessment, were constipation (p = 0.04), urinary urgency (p = 0.02), hallucinations (p = 0.04), dizziness (p = 0.02) and vivid dreams (p = 0.04). Conclusions: Intentional search for non-motor symptoms in patients with Parkinson´ s disease along with a multidisciplinary approach has an impact on their prevalence. The change in specific symptoms is probably related to adjustments in dopaminergic management.