Shy-Drager syndrome is a multisystem degenerative disorder of the central nervous system in which progressive autonomic failure such as orthostatic hypotension, urinary or fecal incontinence, impotence and anhidrosis is a main feature. The nonautonomic neurological abnormalities include parkinsonia, cerebellar and pyramidal signs. In 1984 we met 3 such cases at Seoul National University Hospital and present them here with a brief review of literatures.
Central Nervous System ; Erectile Dysfunction ; Fecal Incontinence ; Hypohidrosis ; Hypotension, Orthostatic ; Male ; Seoul ; Shy-Drager Syndrome*

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.
Anesthesia ; Anesthesia, Epidural ; Blood Pressure ; Female ; Humans ; Hypotension, Orthostatic ; Pressoreceptors ; Shy-Drager Syndrome

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.
Anesthesia ; Anesthesia, Epidural ; Blood Pressure ; Female ; Humans ; Hypotension, Orthostatic ; Pressoreceptors ; Shy-Drager Syndrome

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.
Anesthesia, Spinal* ; Ataxia ; Autonomic Nervous System ; Dysarthria ; Female ; Humans ; Hypotension, Orthostatic ; Libido ; Parkinsonian Disorders ; Postoperative Care ; Shy-Drager Syndrome* ; Sweat ; Sweating ; Tremor ; Vocal Cord Paralysis

Orthostatic hypotension with other signs of progressive neuronal degeneration particularly affecting the autonomic nervous system has been recognized independently and is called Shy-Drager syndrome. Degenerative changes in the autonomic ganglia, loss of neurons in the intermediolateral columns and degenerative changes in the neurons of the cortex and basal ganglia have been reported on examination of this condition. The authors report a case of Shy-Drager Syndrome with symptoms of orthosatatic hypotension, dysarthria, Parkinson's features, muscle wasting, sexual impotence, atonic bladder and loss of sweating.
Autonomic Nervous System ; Basal Ganglia ; Dysarthria ; Erectile Dysfunction ; Ganglia, Autonomic ; Hypotension ; Hypotension, Orthostatic ; Male ; Neurons ; Shy-Drager Syndrome* ; Sweat ; Sweating ; Urinary Bladder

Orthostatic hypotension with other signs of progressive neuronal degeneration particularly affecting the autonomic nervous system has been recognized independently and is called Shy-Drager syndrome. Degenerative changes in the autonomic ganglia, loss of neurons in the intermediolateral columns and degenerative changes in the neurons of the cortex and basal ganglia have been reported on examination of this condition. The authors report a case of Shy-Drager Syndrome with symptoms of orthosatatic hypotension, dysarthria, Parkinson's features, muscle wasting, sexual impotence, atonic bladder and loss of sweating.
Autonomic Nervous System ; Basal Ganglia ; Dysarthria ; Erectile Dysfunction ; Ganglia, Autonomic ; Hypotension ; Hypotension, Orthostatic ; Male ; Neurons ; Shy-Drager Syndrome* ; Sweat ; Sweating ; Urinary Bladder

We compared heavily T2W high field MR images of 18 Parkinson's disease (PD group), 8 Parkinsonian syndromes (PS group: progressive supranuclear palsy, OPCA, Shy-Drager syndrome, atypical' parkinsonism), and 20 control patients and exaimined the reported abnormalities (putaminal hypointensity, restoration of the signal intensity of the substantia nigra, narrowing of the pars compacta, brain atrophy) in our patients by 2.0 Tesla MRI. In this study, the narrowing of the signal band from the pars compacta of the substantia nigra was the most valuable index differentiating PD group or PS group from control group and the signal restoration of the substantia nirga was more common in PD than PS or control group. The frequency of putaminal hypointensity and brain atrophy increased with aging and brainstem atrophy was observed in only PS group.
Aging ; Atrophy ; Brain ; Brain Stem ; Humans ; Magnetic Resonance Imaging* ; Parkinson Disease* ; Parkinsonian Disorders* ; Shy-Drager Syndrome ; Substantia Nigra ; Supranuclear Palsy, Progressive