RESUMEN
INTRODUCTION: Autonomic dysfunction is a well-known feature in neurodegenerative dementias, especially common in α-synucleinopathies like dementia with Lewy bodies and Parkinson's disease with dementia. The most common symptoms are orthostatic hypotension, incontinence and constipation, but its relevance in clinical practice is poorly understood. There are no earlier studies addressing the influence of autonomic dysfunction on clinical course and survival. The aim of this study was to investigate the frequency of the three most common features of autonomic dysfunction and analyze how it affects survival. METHODS: Thirty patients with dementia with Lewy bodies and Parkinson's disease with dementia were included in this prospective, longitudinal follow-up study. Presence of incontinence and constipation was recorded at baseline. Blood pressure was measured at baseline, after 3 months and after 6 months according to standardized procedures, with 5 measurements during 10 minutes after rising. Orthostatic hypotension was defined using consensus definitions and persistent orthostatic hypotension was defined as 5 or more measurements with orthostatic hypotension. Difference in survival was analyzed 36 months after baseline. RESULTS: There was a high frequency of persistent orthostatic blood pressure (50%), constipation (30%) and incontinence (30%). Patients with persistent orthostatic hypotension had a significantly shorter survival compared to those with no or non-persistent orthostatic hypotension (Log rank x(2)â=â4.47, pâ=â0.034). Patients with constipation and/or urinary incontinence, in addition to persistent orthostatic hypotension, had a poorer prognosis compared to those with isolated persistent orthostatic hypotension or no orthostatic hypotension (Log rank x(2)â=â6.370, pâ=â0.041). DISCUSSION: According to our findings, the identification of autonomic dysfunction seems to be of great importance in clinical practice, not only to avoid falls and other complications, but also as a possible predictor of survival.
Asunto(s)
Enfermedades del Sistema Nervioso Autónomo/complicaciones , Estreñimiento/fisiopatología , Enfermedad por Cuerpos de Lewy/complicaciones , Enfermedad de Parkinson/complicaciones , Síndrome de Shy-Drager/fisiopatología , Parálisis Supranuclear Progresiva/complicaciones , Incontinencia Urinaria/fisiopatología , Estreñimiento/etiología , Progresión de la Enfermedad , Estudios de Seguimiento , Humanos , Estimación de Kaplan-Meier , Estudios Longitudinales , Estudios Prospectivos , Síndrome de Shy-Drager/etiología , Incontinencia Urinaria/etiologíaRESUMEN
Patients with autonomic failure have disabling orthostatic hypotension because of impaired sympathetic activity. Norepinephrine transporter blockade with atomoxetine raises blood pressure in autonomic failure by increasing synaptic norepinephrine concentrations in postganglionic sympathetic neurons. This effect requires tonic release of norepinephrine, which is decreased in patients with low sympathetic tone. We hypothesized that increasing residual sympathetic outflow with the α-2 antagonist yohimbine would potentiate the pressor effect of norepinephrine transporter blockade with atomoxetine and improve orthostatic tolerance in peripheral autonomic failure. Seventeen patients received a single oral dose of either placebo, yohimbine 5.4 mg or atomoxetine 18.0 mg, and the combination yohimbine and atomoxetine in a single blind, crossover study. Blood pressure was assessed while patients were seated and standing for ≤10 minutes before and 1 hour postdrug. Neither yohimbine nor atomoxetine significantly increased seated systolic blood pressure or orthostatic tolerance compared with placebo. The combination, however, significantly increased seated systolic blood pressure and orthostatic tolerance (P<0.001 and P=0.016, respectively) in a synergistic manner. The maximal increase in seated systolic blood pressure seen with the combination was 31±33 mm Hg at 60 minutes postdrug. Only the combination showed a significant improvement in orthostatic symptoms. In conclusion, the combination of yohimbine and atomoxetine had a synergistic effect on blood pressure and orthostatic tolerance in peripheral autonomic failure, which may be explained by an increased release of norepinephrine in peripheral sympathetic neurons by α-2 antagonism combined with a reduced norepinephrine clearance by norepinephrine transporter blockade. Safety studies are required to address the clinical usefulness of this pharmacological approach.
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Inhibidores de Captación Adrenérgica/administración & dosificación , Antagonistas de Receptores Adrenérgicos alfa 2/administración & dosificación , Presión Sanguínea/efectos de los fármacos , Proteínas de Transporte de Noradrenalina a través de la Membrana Plasmática/antagonistas & inhibidores , Propilaminas/administración & dosificación , Síndrome de Shy-Drager/tratamiento farmacológico , Yohimbina/administración & dosificación , Anciano , Clorhidrato de Atomoxetina , Estudios Cruzados , Relación Dosis-Respuesta a Droga , Sinergismo Farmacológico , Quimioterapia Combinada , Femenino , Estudios de Seguimiento , Humanos , Masculino , Persona de Mediana Edad , Proteínas de Transporte de Noradrenalina a través de la Membrana Plasmática/metabolismo , Equilibrio Postural/efectos de los fármacos , Estudios Prospectivos , Síndrome de Shy-Drager/metabolismo , Síndrome de Shy-Drager/fisiopatología , Método Simple Ciego , Resultado del TratamientoRESUMEN
Braak's staging concept of Lewy body disease pathogenesis is based on a spatiotemporal sequence of alpha-synuclein deposition, with autonomic nervous system involvement before synucleinopathy in substantia nigra neurons. A patient with primary chronic autonomic failure underwent biennial brain 6-[(18)F]DOPA and myocardial 6-[(18)F]dopamine scanning over 4 years. Low myocardial radioactivity indicated cardiac noradrenergic denervation that persisted. Striatal 6-[(18)F]DOPA-derived radioactivity initially was normal, 2 years later was decreased subtly, and by 4 years was clearly decreased, accompanied by dementia and parkinsonism. In this case, neuroimaging evidence of cardiac noradrenergic denervation and subsequent progressive striatal dopaminergic denervation fit with Braak staging.
Asunto(s)
Desnervación Autonómica , Neuronas Dopaminérgicas/fisiología , Enfermedad por Cuerpos de Lewy/patología , Neostriado/fisiopatología , Norepinefrina/fisiología , Síndrome de Shy-Drager/fisiopatología , Sistema Nervioso Simpático/fisiopatología , alfa-Sinucleína/metabolismo , Anciano , Demencia/complicaciones , Demencia/fisiopatología , Demencia/psicología , Dihidroxifenilalanina/análogos & derivados , Progresión de la Enfermedad , Dopamina/análogos & derivados , Trastornos Neurológicos de la Marcha/etiología , Alucinaciones/etiología , Humanos , Masculino , Enfermedad de Parkinson/complicaciones , Enfermedad de Parkinson/fisiopatología , Radiofármacos , Síndrome de Shy-Drager/psicologíaRESUMEN
AIMS: This paper will review literature that examines the psychological and neuropsychological correlates of orthostatic blood pressure regulation. RESULTS: The pattern of change in systolic blood pressure in response to the shift from supine to upright posture reflects the adequacy of orthostatic regulation. Orthostatic integrity involves the skeletal muscle pump, neurovascular compensation, neurohumoral effects and cerebral flow regulation. Various physiological states and disease conditions may disrupt these mechanisms. Clinical and subclinical orthostatic hypotension has been associated with impaired cognitive function, decreased effort, reduced motivation and increased hopelessness as well as dementia, diabetes mellitus, and Parkinson's disease. Furthermore, inadequate blood pressure regulation in response to orthostasis has been linked to increased depression and anxiety as well as to intergenerational behavioral sequalae. CONCLUSIONS: Identifying possible causes and consequences of subclinical and clinical OH are critical in improving quality of life for both children and older adults.
Asunto(s)
Presión Sanguínea/fisiología , Circulación Cerebrovascular/fisiología , Trastornos del Conocimiento/fisiopatología , Trastornos del Humor/fisiopatología , Síndrome de Shy-Drager/fisiopatología , Animales , Sistema Nervioso Autónomo/crecimiento & desarrollo , Sistema Nervioso Autónomo/fisiopatología , Trastornos del Conocimiento/etiología , Humanos , Trastornos del Humor/etiología , Enfermedades Neurodegenerativas/complicaciones , Enfermedades Neurodegenerativas/fisiopatología , Síndrome de Shy-Drager/complicaciones , Síndrome de Shy-Drager/diagnósticoRESUMEN
BACKGROUND: There is no widely accepted validated scale to assess the comprehensive symptom burden and severity of neurogenic orthostatic hypotension (NOH). The Orthostatic Hypotension Questionnaire (OHQ) was developed, with two components: the six-item symptoms assessment scale and a four-item daily activity scale to assess the burden of symptoms. Validation analyses were then performed on the two scales and a composite score of the OHQ. METHODS: The validation analyses of the OHQ were performed using data from patients with NOH participating in a phase IV, double blind, randomized, cross over, placebo-controlled trial of the alpha agonist midodrine. Convergent validity was assessed by correlating OHQ scores with clinician global impression scores of severity as well as with generic health questionnaire scores. Test-retest reliability was evaluated using intraclass correlation coefficients at baseline and crossover in a subgroup of patients who reported no change in symptoms across visits on a patient global impression scores of change. Responsiveness was examined by determining whether worsening or improvement in the patients' underlying disease status produced an appropriate change in OHQ scores. RESULTS: Baseline data were collected in 137 enrolled patients, follow-up data were collected in 104 patients randomized to treatment arm. Analyses were conducted using all available data. The floor and ceiling effects were minimal. OHQ scores were highly correlated with other patient reported outcome measures, indicating excellent convergent validity. Test-retest reliability was good. OHQ scores could distinguish between patients with severe and patients with less severe symptoms and responded appropriately to midodrine, a pressor agent commonly used to treat NOH. CONCLUSION: These findings provide empirical evidence that the OHQ can accurately evaluate the severity of symptoms and the functional impact of NOH as well as assess the efficacy of treatment.
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Agonistas de Receptores Adrenérgicos alfa 1/uso terapéutico , Hipotensión Ortostática/diagnóstico , Hipotensión Ortostática/tratamiento farmacológico , Midodrina/uso terapéutico , Encuestas y Cuestionarios/normas , Anciano , Estudios Cruzados , Método Doble Ciego , Femenino , Encuestas Epidemiológicas/normas , Humanos , Hipotensión Ortostática/fisiopatología , Masculino , Persona de Mediana Edad , Placebos , Índice de Severidad de la Enfermedad , Síndrome de Shy-Drager/diagnóstico , Síndrome de Shy-Drager/tratamiento farmacológico , Síndrome de Shy-Drager/fisiopatología , Resultado del TratamientoRESUMEN
Idiopathic REM sleep behavior disorder (iRBD) has been suggested as an early "pre-motor" stage of Parkinson's disease (PD) in a significant proportion of cases. We investigated autonomic function in 15 consecutive iRBD patients and compared these findings to PD patients and healthy controls. All participants underwent cardiovascular autonomic function testing, and were rated on the COMPASS scale. Symptomatic orthostatic hypotension was present in two iRBD patients, two PD patients and none of the healthy controls. In the tilt table examination, blood pressure changes were similar between iRBD patients and healthy controls. In the PD group, blood pressure drops were more pronounced. In the orthostatic standing test, iRBD patients had higher blood pressure changes than healthy controls. Highest drops were found in PD. Valsalva ratio was lower in iRBD and PD compared to healthy controls. Total COMPASS score was higher in iRBD compared to healthy controls. Highest scores were found in PD. These results support the presence of autonomic dysfunction in iRBD. On several measures, dysfunction was intermediate between healthy controls and PD consistent with the concept that iRBD can be manifestation of synuclein-associated neurodegenerative disorders. Follow-up studies are needed to determine whether iRBD patients with dysfunction on several autonomic domains are at particular risk for developing one of these diseases.
Asunto(s)
Sistema Nervioso Autónomo/fisiología , Presión Sanguínea/fisiología , Trastorno de la Conducta del Sueño REM/fisiopatología , Anciano , Femenino , Humanos , Masculino , Persona de Mediana Edad , Pletismografía/métodos , Trastorno de la Conducta del Sueño REM/diagnóstico , Síndrome de Shy-Drager/diagnóstico , Síndrome de Shy-Drager/fisiopatología , Encuestas y Cuestionarios , Maniobra de Valsalva/fisiologíaRESUMEN
BACKGROUND: Orthostatic hypotension (OH) is a common problem among the elderly. It is associated with an increase in morbidity and mortality, but its prevalence in Mexico is unknown. METHODS: We conducted a cross-sectional prospective study of intern patients at several Mexican elderly assistance institutions. We carried out a history and took blood pressure readings in a seated position, immediately after standing up, and again after 3 min of standing up. RESULTS: We evaluated 132 patients, mean age 82.3 ± 9.5 years, 74.1% of them female. Thirty-nine (29.3%) subjects had OH. They had a higher prevalence of hypothyroidism, Parkinson's disease, depression and alcoholism. Their Minimental result was 15.45 ± 7.2 vs 16.12 ± 7.9 (p = 0.6) among those without OH, and their quality of life (Minnesota scale) was 12.1 ± 7.3 vs 9.15 ± 7.05 (p = 0.03). They used more ACEI, digoxin and levothyroxin. Hypertension and alcoholism showed respectively a RR of 2.6 (95% CI 0.9-7.6, p = 0.06) and 3.18 (95% CI 0.96-10.48, p = 0.05) to develop OH. CONCLUSIONS: OH was present in 29.3% of the studied population. A third of them had hypertension. The use of different medications does not solely explain OH, so it is necessary to look for different associations. Among those, chronic alcoholism stands out. OH is associated with a poorer quality of life and cognitive performance. OH is asymptomatic in most cases.
Asunto(s)
Alcoholismo/epidemiología , Pacientes Internos/estadística & datos numéricos , Enfermedad de Parkinson/epidemiología , Síndrome de Shy-Drager/epidemiología , Anciano , Anciano de 80 o más Años , Estudios Transversales , Femenino , Humanos , Masculino , México/epidemiología , Prevalencia , Estudios Prospectivos , Calidad de Vida , Síndrome de Shy-Drager/fisiopatologíaRESUMEN
BACKGROUND: Autonomic dysfunction (AD) is associated with morbidity and mortality in patients with systolic heart failure (SHF). The extent of AD when LV ejection fraction is preserved (HF-NEF), is unclear. Our objectives were: 1) quantitative assessment of autonomic function in SHF and HF-NEF; and 2) exploration of relationships among AD, symptoms and cardiac function. METHODS: This was an observational study of patients newly referred from primary care with a heart failure diagnosis; 21 SHF, 20 HF-NEF patients and 21 normal subjects were recruited. All subjects underwent clinical evaluation, 6-minute walk test (6 MWT), Minnesota Questionnaire (MLWHFQ) and echocardiography. Autonomic assessment included haemodynamic responses to standing, deep breathing and handgrip. Concomitant blood pressure variability (BPV) and heart rate variability (HRV) parameters were also derived. RESULTS: There were significant differences in all haemodynamic responses between SHF, HF-NEF and normal. Log transformed (ln) low frequency spectral component of BPV was lower in SHF (4.1 ± 0.3) than HF-NEF (4.2 ± 0.4) and normal (4.4 ± 0.1; p=0.001 SHF vs HF-NEF and vs normal). Ln LF/HF was greater in normal than HF-NEF and SHF (1.5 ± 0.7 vs 0.9 ± 1.0 vs 0.6 ± 0.6; p=0.003). Autonomic modulations correlated negatively with severity of heart failure. CONCLUSIONS: Autonomic responses in heart failure were blunted and the attenuation of responses correlated strongly with symptomatic and functional markers of disease severity. Autonomic dysfunction is a feature of the heart failure syndrome but is not dependent on ejection fraction.
Asunto(s)
Enfermedades del Sistema Nervioso Autónomo/complicaciones , Enfermedades del Sistema Nervioso Autónomo/fisiopatología , Insuficiencia Cardíaca Sistólica/complicaciones , Insuficiencia Cardíaca Sistólica/fisiopatología , Volumen Sistólico/fisiología , Adulto , Barorreflejo/fisiología , Presión Sanguínea/fisiología , Femenino , Fuerza de la Mano/fisiología , Insuficiencia Cardíaca Sistólica/diagnóstico por imagen , Frecuencia Cardíaca/fisiología , Humanos , Masculino , Persona de Mediana Edad , Pletismografía , Frecuencia Respiratoria/fisiología , Índice de Severidad de la Enfermedad , Síndrome de Shy-Drager/complicaciones , Síndrome de Shy-Drager/fisiopatología , Ultrasonografía , Maniobra de Valsalva/fisiologíaRESUMEN
Multiple system atrophy (MSA) is a Parkinson's Disease (PD)-like α-synucleinopathy clinically characterized by dysautonomia, parkinsonism, cerebellar ataxia, and pyramidal signs in any combination. We aimed to determine whether the clinical presentation of MSA as well as diagnostic and therapeutic strategies differ across Europe and Israel. In 19 European MSA Study Group centres all consecutive patients with a clinical diagnosis of MSA were recruited from 2001 to 2005. A standardized minimal data set was obtained from all patients. Four-hundred thirty-seven MSA patients from 19 centres in 10 countries were included. Mean age at onset was 57.8 years; mean disease duration at inclusion was 5.8 years. According to the consensus criteria 68% were classified as parkinsonian type (MSA-P) and 32% as cerebellar type (MSA-C) (probable MSA: 72%, possible MSA: 28%). Symptomatic dysautonomia was present in almost all patients, and urinary dysfunction (83%) more common than symptomatic orthostatic hypotension (75%). Cerebellar ataxia was present in 64%, and parkinsonism in 87%, of all cases. No significant differences in the clinical presentation were observed between the participating countries. In contrast, diagnostic work up and therapeutic strategies were heterogeneous. Less than a third of patients with documented orthostatic hypotension or neurogenic bladder disturbance were receiving treatment. This largest clinical series of MSA patients reported so far shows that the disease presents uniformly across Europe. The observed differences in diagnostic and therapeutic management including lack of therapy for dysautonomia emphasize the need for future guidelines in these areas.
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Atrofia de Múltiples Sistemas/diagnóstico , Atrofia de Múltiples Sistemas/terapia , Sistema de Registros , Edad de Inicio , Antiparkinsonianos/uso terapéutico , Ataxia Cerebelosa/diagnóstico , Ataxia Cerebelosa/fisiopatología , Europa (Continente) , Femenino , Humanos , Hipotensión Ortostática/diagnóstico , Hipotensión Ortostática/fisiopatología , Levodopa/uso terapéutico , Masculino , Persona de Mediana Edad , Atrofia de Múltiples Sistemas/fisiopatología , Trastornos Parkinsonianos/diagnóstico , Trastornos Parkinsonianos/tratamiento farmacológico , Trastornos Parkinsonianos/fisiopatología , Síndrome de Shy-Drager/diagnóstico , Síndrome de Shy-Drager/fisiopatologíaAsunto(s)
Anestésicos/farmacología , Enfermedades del Sistema Nervioso/fisiopatología , Sistema Nervioso/efectos de los fármacos , Enfermedades Neuromusculares/fisiopatología , Esclerosis Amiotrófica Lateral/fisiopatología , Esclerosis Amiotrófica Lateral/cirugía , Anestésicos/administración & dosificación , Sistema Nervioso Autónomo/efectos de los fármacos , Sistema Nervioso Autónomo/fisiología , Sistema Nervioso Autónomo/fisiopatología , Corea/fisiopatología , Corea/cirugía , Humanos , Esclerosis Múltiple/fisiopatología , Esclerosis Múltiple/cirugía , Sistema Nervioso/fisiopatología , Fenómenos Fisiológicos del Sistema Nervioso , Síndrome de Shy-Drager/fisiopatología , Síndrome de Shy-Drager/cirugíaRESUMEN
Survival of multiple system atrophy (MSA) depends on whether a variety of sleep-related breathing problems as well as autonomic failure (AF) occur. Since the brainstem lesions that cause respiratory and autonomic dysfunction overlap with each other, these critical manifestations might get worse in parallel. If so, the detection of AF, which is comparatively easy, might be predictive of a latent life-threatening breathing disorder. In 15 patients with MSA, we performed autonomic function tests composed of postural challenges and administered a questionnaire on bladder condition, as well as polysomnography and laryngoscopy during wakefulness and under anesthesia. Polysomnographic variables such as the apnea-hypopnea index (AHI) and oxygen saturation (SpO(2)) and the findings of laryngoscopy were compared with the degree of cardiac and urinary autonomic dysfunction. AHI, mean SpO(2) and the lowest SpO(2) showed significant correlations with urine storage dysfunction. In addition, patients with vocal cord abductor paralysis (VCAP) or central sleep apnea (CSA) contributing to nocturnal sudden death had more severe storage disorders than those without. On the other hand, no significant relationship between polysomnographic variables and orthostatic hypotension was observed except in the case of mean SpO(2). These results indicate that life-threatening breathing disorders have a close relationship with AF, and especially urine storage dysfunction. Therefore, longitudinal assessment of deterioration of the storage function might be useful for predicting the latent progress of VCAP and CSA.
Asunto(s)
Atrofia de Múltiples Sistemas/complicaciones , Insuficiencia Respiratoria/diagnóstico , Síndrome de Shy-Drager/diagnóstico , Síndrome de Shy-Drager/fisiopatología , Vejiga Urinaria Neurogénica/fisiopatología , Anciano , Anciano de 80 o más Años , Femenino , Humanos , Laringoscopía , Masculino , Persona de Mediana Edad , Examen Neurológico , Polisomnografía , Postura/fisiología , Insuficiencia Respiratoria/etiología , Síndrome de Shy-Drager/etiología , Vejiga Urinaria Neurogénica/etiologíaRESUMEN
OBJECTIVES: To report and characterize a dopamine agonist (DA) withdrawal syndrome (DAWS) in Parkinson disease. DESIGN: Retrospective cohort study. SETTING: Outpatient tertiary movement disorders clinic. Patients A cohort of 93 nondemented patients with Parkinson disease enrolled in a prospective study of nonmotor and motor disease manifestations. Main Outcome Measure The presence of DAWS, defined as a severe, stereotyped cluster of physical and psychological symptoms that correlate with DA withdrawal in a dose-dependent manner, cause clinically significant distress or social/occupational dysfunction, are refractory to levodopa and other Parkinson disease medications, and cannot be accounted for by other clinical factors. RESULTS: Of 40 subjects treated with a DA, 26 underwent subsequent DA taper. Of these 26 subjects, 5 (19%) developed DAWS and 21 (81%) did not. All subjects with DAWS had baseline DA-related impulse control disorders. Symptoms of DAWS resembled those of other drug withdrawal syndromes and included anxiety, panic attacks, agoraphobia, depression, dysphoria, diaphoresis, fatigue, pain, orthostatic hypotension, and drug cravings. Subjects with DAWS as compared with those without DAWS had higher baseline DA use (mean [SD], 420 [170] vs 230 [180] DA levodopa equivalent daily doses [DA-LEDD], respectively; P = .04) and higher cumulative DA exposure (mean [SD], 1800 [1200] vs 700 [900] DA-LEDD-years, respectively; P = .03). Subjects with DAWS also had considerably lower Unified Parkinson's Disease Rating Scale motor scores than those without DAWS (mean [SD], 21 [5] vs 31 [10], respectively; P = .007), despite comparable disease duration (mean [SD], 7.3 [7] vs 6.3 [4] years, respectively; P = .77) and similar total dopaminergic medication use (mean [SD], 830 [450] vs 640 [610] total LEDD, respectively; P = .52) in the 2 groups. CONCLUSIONS: Dopamine agonists have a stereotyped withdrawal syndrome that can lead to profound disability in a subset of patients. Physicians should monitor patients closely when tapering these medications.
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Antiparkinsonianos/efectos adversos , Encéfalo/efectos de los fármacos , Agonistas de Dopamina/efectos adversos , Enfermedad de Parkinson/tratamiento farmacológico , Síndrome de Abstinencia a Sustancias/fisiopatología , Anciano , Agorafobia/inducido químicamente , Agorafobia/fisiopatología , Ansiedad/inducido químicamente , Ansiedad/fisiopatología , Encéfalo/metabolismo , Encéfalo/fisiopatología , Estudios de Cohortes , Trastorno Depresivo/inducido químicamente , Trastorno Depresivo/fisiopatología , Evaluación de la Discapacidad , Relación Dosis-Respuesta a Droga , Femenino , Humanos , Masculino , Persona de Mediana Edad , Trastorno de Pánico/inducido químicamente , Trastorno de Pánico/fisiopatología , Estudios Prospectivos , Estudios Retrospectivos , Síndrome de Shy-Drager/inducido químicamente , Síndrome de Shy-Drager/fisiopatologíaRESUMEN
BACKGROUND: In autonomic failure (AF), blood pressure (BP) falls upon standing which is commonly ascribed to defective vasoconstriction and excessive pooling. Observations on the amount of pooling in AF are contradictory. METHODS: We evaluated pooling using strain-gauge plethysmography (SGP) during head-up tilt (HUT) with a parachute harness fixed to the tilt table to avoid muscle tension in the lower limbs and thus to maximise pooling. 23 healthy subjects and 12 patients with AF were tilted for 5 min. BP and calf volume changes, as measured by SGP, were measured continuously. Multiple regression analysis was used to examine the effect of AF on orthostatic fluid shifts after adjustment for potential confounders. RESULTS: Patients did not differ from controls with respect to the increase of calf volume after 5 min HUT. The acute (0-1 min) and the prolonged (1-5 min) phases of calf volume responses to HUT were also similar between patients and controls. No correlation was found between the degree of orthostatic hypotension and the orthostatic calf volume change in AF. In one patient an additional measurement was made before rising from bed in the early morning demonstrating a greater albeit small increase of calf volume upon HUT. CONCLUSION: Orthostatic fluid shifts at the level of the calf in AF are not augmented during the course of the day despite marked hypotension. However, a small increase of pooling may be expected when the patient first gets out of bed in the morning probably due to the absence of oedema.
Asunto(s)
Transferencias de Fluidos Corporales/fisiología , Pierna/irrigación sanguínea , Postura/fisiología , Síndrome de Shy-Drager/fisiopatología , Adulto , Anciano , Presión Sanguínea/fisiología , Ritmo Circadiano/fisiología , Interpretación Estadística de Datos , Femenino , Frecuencia Cardíaca/fisiología , Humanos , Pierna/fisiología , Masculino , Persona de Mediana Edad , Enfermedad de Parkinson/fisiopatología , Flujo Sanguíneo Regional/fisiología , Síncope/etiología , Síncope/fisiopatologíaRESUMEN
This paper summarizes the methods we devised for the treatment of psychosis, orthostatic hypotension, and mood disorders among the various non-motor complications of Parkinson's disease. Psychosis may not manifest when a patient believes in his/her delusions. If left untreated over a prolonged period, however, the delusions progress to paranoia that is very difficult to cure. Accordingly, enquiries should be made during routine examinations to detect the presence of psychosis and facilitate early discovery. Atypical antipsychotics are used when psychosis does not improve after reducing the doses of antiparkinson drugs. We achieved favorable results by using mianserin hydrochloride prior to this step, with efficacy being observed for hallucinations and mild delusions that often manifested at night. This drug does not act as a dopamine receptor blocker, so it has the advantage of not aggravating motor symptoms. With this therapy, it is also possible to improve motor symptoms without inducing psychosis by reducing the doses of antiparkinson drugs and locally stimulating the motor loop by deep brain stimulation of the subthalamic nucleus. We previously introduced leg-holding exercises for the treatment of orthostatic hypotension, through which blood pooled in the veins is returned to the systemic circulation by holding the knees. This can be done easily and is free of adverse reactions. Mood disorders are difficult to cope with in patients with Parkinson's disease, but may be treated by selecting an appropriate dopamine agonist while giving consideration to affinity for the dopamine D3 receptor. However, treatment becomes complicated when the dopamine receptor is overstimulated. Here we report on cases of successfully treated pathological gambling and dopamine dysregulation syndrome, which are considered difficult to manage. The solution may differ depending on a patient's environment, and it is not easy to prescribe therapy based on evidence-based medicine. The best therapy should be selected by maintaining communication with the patient and developing a relationship built on trust.
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Trastornos del Humor/tratamiento farmacológico , Trastornos del Humor/etiología , Enfermedad de Parkinson/complicaciones , Trastornos Psicóticos/tratamiento farmacológico , Trastornos Psicóticos/etiología , Síndrome de Shy-Drager/etiología , Síndrome de Shy-Drager/terapia , Adulto , Anciano , Antiparkinsonianos/efectos adversos , Antipsicóticos/farmacología , Antipsicóticos/uso terapéutico , Estimulación Encefálica Profunda/métodos , Dopaminérgicos/farmacología , Dopaminérgicos/uso terapéutico , Terapia por Ejercicio/métodos , Femenino , Humanos , Masculino , Persona de Mediana Edad , Trastornos del Humor/fisiopatología , Enfermedad de Parkinson/terapia , Trastornos Psicóticos/fisiopatología , Síndrome de Shy-Drager/fisiopatologíaAsunto(s)
Enfermedades del Sistema Nervioso Autónomo/fisiopatología , Sistema Nervioso Autónomo/fisiología , Trastornos del Sistema Nervioso Inducidos por Alcohol/prevención & control , Amiloidosis/complicaciones , Animales , Enfermedades Autoinmunes del Sistema Nervioso/complicaciones , Sistema Nervioso Autónomo/anatomía & histología , Enfermedades del Sistema Nervioso Autónomo/etiología , Enfermedades del Sistema Nervioso Autónomo/terapia , Complicaciones de la Diabetes/fisiopatología , Complicaciones de la Diabetes/prevención & control , Gastroparesia/fisiopatología , Gastroparesia/terapia , Humanos , Síndrome de Shy-Drager/fisiopatología , Síndrome de Shy-Drager/terapia , Vejiga Urinaria Neurogénica/fisiopatología , Vejiga Urinaria Neurogénica/terapiaRESUMEN
OBJECTIVE: To report preliminary results of a prospective ongoing study of multiple system atrophy (MSA) and Parkinson disease (PD), with a large subset of patients with PD with autonomic failure (25%), to evaluate autonomic indices that distinguish MSA from PD. METHODS: We used consensus criteria, detailed autonomic studies (Composite Autonomic Symptom Scale, Composite Autonomic Scoring Scale, thermoregulatory sweat test, and plasma catecholamines), and functional scales (Unified MSA Rating Scale [UMSARS] I-IV and Hoehn-Yahr grading) on a prospective, repeated, and ongoing basis. RESULTS: We report the results of a study on 52 patients with MSA (mean [SD], age, 61.1 [7.8] years; body mass index (calculated as weight in kilograms divided by height in meters squared), 27.2 [4.6]; Hoehn-Yahr grade, 3.2 [0.9]; UMSARS I score, 21.5 [7.4]; and UMSARS II score, 22.7 [9.0]) and 29 patients with PD, including PD with autonomic failure (mean [SD], age, 66.0 [8.1] years; body mass index, 26.6 [5.5]; Hoehn-Yahr grade, 2.2 [0.8]; UMSARS I score, 10.4 [6.1]; and UMSARS II score, 13.0 [5.9]). Autonomic indices were highly significantly more abnormal in MSA than PD (P < .001) for the Composite Autonomic Scoring Scale (5.9 [1.9] vs 3.3 [2.3], respectively), Composite Autonomic Symptom Scale (54.4 [21.8] vs 24.7 [20.5], respectively), and thermoregulatory sweat test (percentage anhidrosis, 57.4% [35.2%] vs 9.9% [17.7%], respectively). These differences were sustained and greater at 1-year follow-up, indicating a greater rate of progression of dysautonomia in MSA than PD. CONCLUSIONS: The severity, distribution, and pattern of autonomic deficits at study entry will distinguish MSA from PD, and MSA from PD with autonomic failure. These differences continue and are increased at follow-up. Our ongoing conclusion is that autonomic function tests can separate MSA from PD. Autonomic indices support the notion that the primary lesion in PD is ganglionic and postganglionic, while MSA is preganglionic.
Asunto(s)
Atrofia de Múltiples Sistemas/diagnóstico , Atrofia de Múltiples Sistemas/fisiopatología , Enfermedad de Parkinson/diagnóstico , Enfermedad de Parkinson/fisiopatología , Síndrome de Shy-Drager/diagnóstico , Síndrome de Shy-Drager/fisiopatología , Anciano , Regulación de la Temperatura Corporal/fisiología , Catecolaminas/análisis , Catecolaminas/sangre , Diagnóstico Diferencial , Errores Diagnósticos/prevención & control , Evaluación de la Discapacidad , Femenino , Ganglios Autónomos/fisiopatología , Humanos , Masculino , Persona de Mediana Edad , Atrofia de Múltiples Sistemas/complicaciones , Examen Neurológico/métodos , Enfermedad de Parkinson/complicaciones , Valor Predictivo de las Pruebas , Estudios Prospectivos , Sensibilidad y Especificidad , Índice de Severidad de la Enfermedad , Síndrome de Shy-Drager/complicaciones , Enfermedades de las Glándulas Sudoríparas/diagnóstico , Enfermedades de las Glándulas Sudoríparas/etiología , Enfermedades de las Glándulas Sudoríparas/fisiopatologíaRESUMEN
Acute autonomic, sensory and motor neuropathy (AASMN) is a rare peripheral nerve disorder characterized by prominent dysautonomia with somatic sensory and motor impairment. Dysautonomia in AASMN is intractable even with corticosteroid therapy or plasmapheresis. Here we report a case of AASMN with severe orthostatic hypotension. Although the effectiveness of corticosteroid was insufficient, high dose intravenous immunoglobulin therapy (IVIg) was effective for not only sensorimotor symptoms but also autonomic symptoms. This is the first case of AASMN showing favorable responses to IVIg treatment, suggesting that IVIg should be considered when corticosteroid therapy or plasmapheresis is ineffective or insufficient.
Asunto(s)
Enfermedades del Sistema Nervioso Autónomo/terapia , Inmunoglobulinas Intravenosas/uso terapéutico , Enfermedades del Sistema Nervioso Periférico/terapia , Enfermedad Aguda , Enfermedades del Sistema Nervioso Autónomo/diagnóstico , Enfermedades del Sistema Nervioso Autónomo/fisiopatología , Humanos , Masculino , Persona de Mediana Edad , Norepinefrina/sangre , Enfermedades del Sistema Nervioso Periférico/diagnóstico , Enfermedades del Sistema Nervioso Periférico/fisiopatología , Renina/sangre , Síndrome de Shy-Drager/diagnóstico , Síndrome de Shy-Drager/fisiopatología , Síndrome de Shy-Drager/terapia , Pruebas de Mesa InclinadaRESUMEN
We report the case of a 32-year-old man with an epidermoid tumor of the fourth ventricle. About 14 years later, he showed a tumor recurrence which was removed. After this procedure the patient complained of presyncopal and syncopal crisis while attempting to stand or walk. On examination, severe orthostatic hypotension was confirmed and autonomic tests were abnormal. The brain MRI showed a tetraventricular hydrocephalus predominating in the fourth ventricle. A ventriculo-peritoneal shunt was performed, and after surgery the orthostatic intolerance improved. We believe that hydrocephalus has probably been a contributory factor to orthostatic hypotension, and suggest expanding testing for dysautonomia in patients with hydrocephalus.
Asunto(s)
Carcinoma de Células Escamosas/complicaciones , Neoplasias del Ventrículo Cerebral/complicaciones , Hidrocefalia/complicaciones , Síndrome de Shy-Drager/etiología , Adulto , Vías Autónomas/fisiopatología , Barorreflejo/fisiología , Presión Sanguínea/fisiología , Tronco Encefálico/fisiopatología , Carcinoma de Células Escamosas/patología , Carcinoma de Células Escamosas/cirugía , Neoplasias del Ventrículo Cerebral/patología , Neoplasias del Ventrículo Cerebral/cirugía , Cuarto Ventrículo/patología , Cuarto Ventrículo/fisiopatología , Humanos , Hidrocefalia/diagnóstico , Hidrocefalia/fisiopatología , Imagen por Resonancia Magnética , Masculino , Recurrencia Local de Neoplasia/complicaciones , Recurrencia Local de Neoplasia/patología , Recurrencia Local de Neoplasia/cirugía , Examen Neurológico , Procedimientos Neuroquirúrgicos , Síndrome de Shy-Drager/diagnóstico , Síndrome de Shy-Drager/fisiopatología , Resultado del Tratamiento , Derivación Ventriculoperitoneal , VentriculostomíaRESUMEN
In this practically oriented review, we will outline the clinical approach of patients with falls due to an impairment or loss of consciousness. Following a set of definitions, we describe the salient clinical features of disorders leading to such falls. Among falls caused by true loss of consciousness, we separate the clinical characteristics of syncopal falls (due to reflex syncope, hypovolemia, orthostatic hypotension or cardiac syncope) from falls due to other causes of transient unconsciousness, such as seizures. With respect to falls caused by an apparent loss of consciousness, we discuss the presentation of cataplexy, drop attacks, and psychogenic falls. Particular emphasis will be laid upon crucial features obtained by history taking for distinguishing between the various conditions that cause or mimic a transient loss of consciousness.
Asunto(s)
Accidentes por Caídas/estadística & datos numéricos , Síncope/etiología , Síncope/fisiopatología , Inconsciencia/etiología , Inconsciencia/fisiopatología , Enfermedades del Sistema Nervioso Autónomo/complicaciones , Enfermedades del Sistema Nervioso Autónomo/diagnóstico , Enfermedades del Sistema Nervioso Autónomo/fisiopatología , Cataplejía/complicaciones , Cataplejía/diagnóstico , Cataplejía/fisiopatología , Diagnóstico Diferencial , Epilepsia/complicaciones , Epilepsia/diagnóstico , Epilepsia/fisiopatología , Humanos , Síndrome de Shy-Drager/complicaciones , Síndrome de Shy-Drager/diagnóstico , Síndrome de Shy-Drager/fisiopatología , Síncope/diagnósticoRESUMEN
Even with mild neurological signs, patients with botulism frequently complain of autonomic symptoms. This study aimed at the evaluation of sudomotor and cardiovascular reflex functions by quantitative autonomic testing (QAT), which may identify patients with autonomic involvement but otherwise benign clinical presentation. Five patients with food-borne botulism were subjected to a structured questionnaire on autonomic symptoms, cardiac and neurological examination, and QAT after a median of 2 weeks (baseline) and 12 weeks (follow-up) post intoxication. For calculation of haemodynamic and cardiovascular autonomic parameters, we used the Task Force((R)) Monitor (Version 2.1, CNSystems, Graz, Austria). Cardiovagal function was assessed by Ewing's test battery. Autonomic complaints were more pronounced than neurological symptoms. Baseline tests revealed widely abnormal sudomotor function and marked impairment of heart rate variation and blood pressure response to standing. Prominent features of cardiovascular failure were high resting heart rate, supine hypertension, orthostatic hypotension, and impaired baroreflex function. Three patients reported inability to keep up with their routine amount of physical work. Based on the baseline QAT results, these three patients were instructed to engage in physical activity but avoid physical strain until there was considerable improvement. On follow-up, fatigue was the most frequent residual complaint, sympathetic skin responses were present, and cardiovascular QAT results were significantly improved and did not differ from those of ten control subjects. QAT identified autonomic involvement in botulism patients with otherwise benign neurological presentation. Comprehensive evaluation of autonomic failure may provide useful information for the management of botulism.