ABSTRACT
La atrofia multisistémica constituye un trastorno neurodegenerativo esporádico de etiología no precisada que se caracteriza por parkinsonismo, trastornos cerebelosos, disfunción autonómica y piramidalismo; los hallazgos patológicos comprenden pérdida celular y gliosis en las neuronas estriatonígricas, olivopontocerebelosas y autonómicas; y la presencia de inclusiones intracitoplasmáticas oligodendrogliales y neuronales, ubiquitina, tau y alfasinucleína positivas. Afecta tanto a hombres como a mujeres, con inicio en la sexta década de la vida como promedio y una prevalencia de 4/100 000. Se presentaron los últimos criterios diagnósticos de atrofia multisistémica y el caso clínico de un paciente de 65 años con un cuadro progresivo, de 4 años de evolución, ataxia cerebelosa progresiva, síndrome rígido acinético, disfunción autonómica, signos piramidales y mala respuesta a la levodopa, con imágenes de resonancia magnética que muestran atrofia de vermis, hemisferios cerebelosos, tallo cerebral (puente) e hipointensidad de ambas regiones putaminales en t2. Se concluyó el caso con el diagnóstico de atrofia multisistémica tipo C
The multisystem atrophy is a sporadic neurodegenerative disorder of unknown origin characterized by parkinsonism, cerebellar disorders, autonomic dysfunction and pyramidal disease, provoked by a cellular loss and gliosis in the nigrostriatal, olivopontocerebellar and autonomic neurons and the presence of oligodendroglia and neuronal intracytoplasmic positive inclusions, ubiquitin, tau and alpha-sinuclein affecting men and women starting as average during the sixth decade of life and a prevalence of 4/100 000. The last diagnostic criteria of multisystem atrophy were showed as well as the clinical case of a patient aged 65 with a progressive picture of 4 years of evolution, progressive cerebellar ataxia, a rigid akinetic syndrome autonomic dysfunction, pyramidal signs and a poor response to levodopa with magnetic resonance images showing vermis atrophy, cerebellar hemispheres, cerebral stem (bridge) and hipointensity of both putamen regions in T2. We conclude that case was diagnosed with type C multisystem atrophy
ABSTRACT
We report a patient with Multiple system atrophy that was successfully treated with the herbal formulation Hachimijiougan.A 79-year-old man was hospitalized with Multiple system atrophy. He showed slow movement, frozen gait, finger tremor, dizziness on standing up with slowly progressive characteristics. Various medications had been administered for orthostatic hypotension, but the effect was insufficient.So we administered Hachimijiougan without changing the other oral medications. After administration, orthostatic hypotension was improved, and he became able to perform various activities of daily living.In this case, we thought that Hachimijiougan improved autonomic nervous system disorders such as the orthostatic hypotension in a patient with Multiple system atrophy.
ABSTRACT
Orthostatic hypotension is a frequent symptom in patients with multiple system atrophy and it has an important impact on their quality of life. We report a case of idiopathic orthostatic hypotension, in a patient with multiple system atrophy (Shy-Drager syndrome), treated with pacemaker implantation resulting in a substantial improvement in the quality of his life.
Hipotensão ortostática é um sintoma freqüente em pacientes com atrofia de múltiplos sistemas e tem importante impacto na sua qualidade de vida. Relatamos um paciente com hipotensão ortostática idiopática e atrofia de múltiplos sistemas (Síndrome de Shy-Drager), tratado com implantação de marca-passo resultando numa evidente melhora na qualidade de vida.
Subject(s)
Humans , Male , Middle Aged , Multiple System Atrophy/diagnosis , Hypotension, Orthostatic/diagnosis , Hypotension, Orthostatic/therapy , Pacemaker, Artificial , Shy-Drager Syndrome , Neurodegenerative DiseasesABSTRACT
Shy-Drager syndrome (SDS) is a chronic progressive disease with central autonomic nervous system defect, Parkinsonism, and cerebellar defect. The clinical maifestations of this syndrome are orthostatic hypotension, urinary and bowel dysfunction, impaired sexual potency, impaired libido, decreased sweating, ataxia, dysarthria, intension tremor, and vocal cord palsy. Anesthesiologists should consider SDS as having major implications during surgery when choosing the anesthetic technique, monitoring, and postoperative care because it causes loss of autonomic regulation of vascular tone. We report a successful spinal anesthesia for left salphingo-oophorectomy in a female patient with Shy-Drager syndrome.
Subject(s)
Female , Humans , Anesthesia, Spinal , Ataxia , Autonomic Nervous System , Dysarthria , Hypotension, Orthostatic , Libido , Parkinsonian Disorders , Postoperative Care , Shy-Drager Syndrome , Sweat , Sweating , Tremor , Vocal Cord ParalysisABSTRACT
The Shy-Drager syndrome is a very rare chronic progressive disease characterised by autonomic failure and multiple system atropy. The main clinical manifestations of this syndrome are orthostatic hypotension, urinary and bowel dysfunction, impaired sexual potency and parkinsonean symptoms. A key in the management of anesthesia is the maintenance of cardiovascular atability. These patient are suffered from defective baroreceptor regulation of the blood pressure as a result of autonomic failure. We report a successful epidural anesthesia for vwicolithotomy in a female patient with Shy-Drager syndrome.
Subject(s)
Female , Humans , Anesthesia , Anesthesia, Epidural , Blood Pressure , Hypotension, Orthostatic , Pressoreceptors , Shy-Drager SyndromeABSTRACT
The Shy-Drager syndrome is a very rare chronic progressive disease characterised by autonomic failure and multiple system atropy. The main clinical manifestations of this syndrome are orthostatic hypotension, urinary and bowel dysfunction, impaired sexual potency and parkinsonean symptoms. A key in the management of anesthesia is the maintenance of cardiovascular atability. These patient are suffered from defective baroreceptor regulation of the blood pressure as a result of autonomic failure. We report a successful epidural anesthesia for vwicolithotomy in a female patient with Shy-Drager syndrome.