No abstract available.
Multiple System Atrophy

No abstract available.
Multiple System Atrophy ; Respiratory Sounds*

OBJECTIVE: To clarify the specificity of the ‘hot cross bun’ sign (HCBS) for multiple system atrophy (MSA) in adult cerebellar ataxia or parkinsonism. METHODS: The radiologic information systems at an academic center and affiliated veterans' hospital were queried using the keywords ‘hot cross bun,’ ‘pontocerebellar,’ ‘cruciate,’ ‘cruciform,’ ‘MSA,’ ‘multiple system atrophy,’ and ‘multisystem atrophy.’ Scans were reviewed by a neurologist and neuroradiologist to identify the HCBS. Subjects with the HCBS were reviewed by 2 neurologists to identify the most likely etiology of the patient's neurologic symptoms. RESULTS: Eleven cases were identified. Etiologies included MSA (4 probable, 2 possible), hereditary cerebellar ataxia (3/11), probable dementia with Lewy bodies (1/11), and uncertain despite autopsy (1/11). CONCLUSION: MSA was the most common etiology. However, 5 of the 11 patients did not have MSA. The most common alternate etiology was an undefined hereditary cerebellar ataxia (3/11).
Adult ; Autopsy ; Cerebellar Ataxia ; Dementia ; Hexachlorobenzene ; Humans ; Lewy Bodies ; Magnetic Resonance Imaging ; Multiple System Atrophy ; Neurologic Manifestations ; Olivopontocerebellar Atrophies ; Parkinsonian Disorders ; Radiology Information Systems ; Sensitivity and Specificity

No abstract available.
Anesthesia, General* ; Humans ; Multiple System Atrophy*

No abstract available.
Anesthesia, General* ; Humans ; Multiple System Atrophy*

Previous studies showed that 37-71% of the patients with idiopathic Parkinson's disease (IPD) or parkinsonism of other causes had urinary problems. There are several possible reasons for such wide range of frequency of urinary problems in Parkinson's disease or parkinsonism; (1) They used different questionnaires on the urinary problems; (2) they did not try to differentiate the patients with idiopathic Parkinson's disease and those with multiple system atrophy (MSA), in which condition severe urinary problems occur frequently early in the clinical course or even before the onset of parkinsonian symptoms. However, exact nature of urinary problems in MSA and IPD has never been compared. Using Boyarsky score, we compared the frequency and severity of urinary symptoms between 32 patients with IPD and 28 patients with probable MSA. All except one with MSA (96.5%) had urinary symptoms. Although 24 of the 32 with IPD (75%) also had urinary problems, the severity was milder than those with MSA. In 8 with MSA, urinary symptoms preceeded the onset of parkinsonian symptoms. No one with IPD developed urinary symptoms before the onset of parkinsonian symptoms. Seven out of the 28 patients with MSA voided more than 8 times during the day, 10 woke up more than 2 times to void during sleep, 20 wet their clothes more than 2 times per a day. However no one with IPD had such severe urinary problems. Careful history taking about the urinary had such severe urinary problems. Careful history taking and MSA problems seems to be a helpful way in differentiating IPD and MSA.
Humans ; Multiple System Atrophy* ; Parkinson Disease* ; Parkinsonian Disorders ; Surveys and Questionnaires

Multiple system atrophy (MSA) is a progressive neurodegenerative disorder. Widespread presence of glial cytoplasmic inclusions is the neuropathologic hallmark of MSA. The disease has long been considered as a sporadic disorder. However, in recent years, a few familial cases of MSA have been reported, and researches have verified certain genetic variants could increase the risk of MSA. These indicated genetic factors may play an imported role in the pathogenesis of MSA. In this review, the emerging evidence in favor of genetic players in MSA is discussed.
Animals ; Gene Dosage ; Genetic Research ; Humans ; Multiple System Atrophy ; genetics

Female ; Humans ; Middle Aged ; Multiple System Atrophy ; diagnosis ; pathology

BACKGROUND AND PURPOSE: Parkinson's disease (PD) and multiple-system atrophy of the parkinsonian type (MSA-P) are progressive neurodegenerative disorders that in addition to dysfunction of the motor system also present with features of dysautonomia, frequently manifesting as orthostatic hypotension (OH). The pathophysiology of OH has been proposed to differ between these two disorders. This study investigated the spontaneous and cardiovagal baroreflex sensitivity (BRS) in Parkinson's disease patients with orthostatic hypotension (PD(OH)) and multiple system atrophy of Parkinsonian type with orthostatic hypotension in an attempt to differentiate the two disorders. METHODS: Two methods were used for determining the BRS: a spontaneous method (spontaneous BRS) and the reflexive baroreflex gain (cardiovagal BRS) from phases II and IV of the Valsalva maneuver (VM) in PD(OH) and MSA-P(OH). RESULTS: The spontaneous BRS (5.04±0.66 ms/mm Hg vs. 4.78±0.64 ms/mm Hg, p=0.54) and the cardiovagal BRS from phase II of the VM (0.96±0.75 ms/mm Hg vs. 1.34±1.51 ms/mm Hg, p=0.76) did not differ between PD(OH) and MSA-P(OH), but the cardiovagal BRS from phase IV of the VM (0.03±0.07 ms/mm Hg vs. 2.86±2.39 ms/mm Hg, p=0.004) was significantly lower in PD(OH). CONCLUSIONS: The cardiovagal BRS from phase IV of the VM has potential for differentiating PD(OH) and MSA-P(OH), indicating a difference in the pathophysiological mechanisms underlying the autonomic dysfunction in the two disorders.
Atrophy* ; Baroreflex* ; Humans ; Hypotension, Orthostatic ; Multiple System Atrophy ; Neurodegenerative Diseases ; Parkinson Disease* ; Primary Dysautonomias ; Reflex ; Valsalva Maneuver

A 41-year-old man was admitted due to altered mentality and confusion. He had showed progressive cerebellar ataxia, dysarthria, gait disturbance from his age of 33 years old. Brain MRI revealed high signal lesions in periaqueductal gray matter, mammillary bodies, median thalami and postcentral gyri bilaterally on T2-weighted images. Severe cerebellar atrophy was noted, too. We report a case of Wernicke's encephalopathy in a patient with probable multiple system atrophy. As far as we know, there have been no published report on this kind of case.
Adult ; Atrophy ; Brain ; Cerebellar Ataxia ; Dysarthria ; Gait ; Humans ; Magnetic Resonance Imaging ; Mamillary Bodies ; Multiple System Atrophy* ; Periaqueductal Gray ; Wernicke Encephalopathy*