Cases reported "Multiple System Atrophy"

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1/7. Mixed multiple system atrophy and progressive supranuclear palsy: a clinical and pathological report of one case.

    We report a patient who showed pathological features of both multiple system atrophy (MSA) and progressive supranuclear palsy (PSP) at autopsy. The clinical features included severe cerebellar ataxia, autonomic failure, and rigid-akinetic parkinsonism. The clinical diagnosis was MSA. Pathological examination showed severe neuronal loss with gliosis in the putamen, substantia nigra, inferior olive, and the pontine nucleus, and numerous glial cytoplasmic inclusions. In addition, moderate neuronal loss with gliosis was observed in the globus pallidus and subthalamic nucleus, and neurofibrillary tangles and tufted astrocytes were seen in the basal ganglia and the brain stem. These findings indicate that the patient had both MSA and PSP. Double-labeling immunofluorescence in the brain stem showed alpha-synuclein immunoreactivity localized in the oligodendrocytes and phosphorylated tau immunoreactivity in the neurons and the glia. Co-existence of synucleinopathy and tauopathy is rare.
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2/7. association of lewy bodies and glial cytoplasmic inclusions in the brain of Parkinson's disease.

    We report the histopathological and immunohistochemical findings from the brain of an elderly patient diagnosed with Parkinson's disease (PD). Neuropathological examination revealed moderate neuronal cell loss and astrocytosis in the substantia nigra. lewy bodies were found in many sites characteristic for PD, including the substantia nigra, locus coeruleus, hypothalamus, substantia innominata, pontine raphe nucleus, and dorsal motor vagal nucleus, cingulate and insular cortices. Furthermore, argyrophilic glial intracytoplasmic inclusions were found predominantly in the ventral pons, cerebellar white matter, precentral and frontal white matter, internal and external capsule, claustrum, and putamen. Inclusions were triangular in shape, and immunopositive for ubiquitin and alpha-synuclein. In view of these histopathological and immunohistochemical findings and patterns of distribution, the inclusions were suggested to be glial cytoplasmic inclusions (GCIs) in multiple system atrophy (MSA). These findings suggested that our case might have experienced two pathological processes; PD and the early stage of MSA (striatonigral degeneration) that had not progressed to striatal involvement. Alternatively a common pathological background including abnormal processing of alpha-synuclein could contribute to widespread accumulation of lewy bodies and GCIs in a single condition accompanied by nigral degeneration.
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3/7. Atypical parkinsonism combining alpha-synuclein inclusions and polyglucosan body disease.

    adult polyglucosan body disease (APGBD) is a rare disorder affecting the central and peripheral nervous systems and in which parkinsonism is unusual. A 71-year-old man presented levodopa-unresponsive parkinsonism with urinary incontinence and recurrent syncopes of 6 years standing masquerading as atypical parkinsonism of the multiple system atrophy (MSA-P) type. Brain histopathology demonstrated massive accumulation of polyglucosan bodies particularly in the putamen. In addition, there were dense alpha-synuclein-positive cytoplasmic oligodendroglial inclusions in the pons and in the middle cerebellar peduncle. These inclusions may be either due to the chance association of MSA-P with APGBD, or pathologically related to APGBD.
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4/7. Camptocormia or Pisa syndrome in multiple system atrophy.

    Although a mild stooped posture is a hallmark of parkinsonism, extreme trunk forward flexion is not common. This phenomenon was described in different etiological entities and called camptocormia. Other similar presentations called Pisa syndrome and antecollis were described mainly in extrapyramidal disorders. Authors present two cases of probable multiple system atrophy (MSA) with predominant parkinsonism and Pisa syndrome (or camptocormia). Both of them were previously misdiagnosed as idiopathic Parkinson's disease (PD) and one was reported 1 year earlier. The typical clinical presentation fulfilling the diagnostic criteria for multiple system atrophy, rapid progression with lack of responsiveness to L-DOPA and apomorphine and typical MRI putaminal pathology observed in both cases allowed us to make a diagnosis. Accuracy of clinical diagnosis in multiple system atrophy is still very poor. Therefore, unusual or rare clinical presentations may support the final diagnosis. The camptocormia, Pisa syndrome and antecollis may represent the continuum of the same motor phenomenon and most of the authors refer them to unusual form of axial dystonia. According to many clinical presentations on different forms of camptocormia/Pisa syndrome authors conclude that not etiology, but the localization of specific lesion, probably within putamen is responsible for that form of dystonia. In cases of parkinsonism and severe forward flexion of trunk multiple system atrophy, diagnosis should be considered.
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5/7. Increased basal ganglia iron in striatonigral degeneration: in vivo estimation with magnetic resonance.

    BACKGROUND: As many as 20% of individuals with the clinical diagnosis of Parkinson's disease (PD) do not have the characteristic neuropathologic features of PD at post mortem. The striatonigral degeneration (SND) subtype of multiple system atrophy is one of the categories of pathology which may be incorrectly diagnosed as PD on the basis of clinical presentation. SND may be associated with increased iron deposition in the putamen which can be detected with magnetic resonance imaging. methods: We have estimated regional brain iron content in a patient with probable SND, using a novel imaging method developed in our laboratory, and have compared the results in this patient to those which we have previously reported in patients with PD and in age-matched controls. RESULTS: We observed that putamenal iron content was increased in our SND patient, beyond the 95% confidence limit for inclusion in the PD group, even when considering clinical severity. In contrast, pallidal and thalamic iron were within the PD range. CONCLUSIONS: The demonstration of increased putamenal iron content may be a useful adjunctive investigative procedure in patients with suspected SND.
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6/7. Correlative studies of MR findings with neuropathology in shy-drager syndrome and striatonigral degeneration.

    OBJECTIVE: To determine the pathologic bases of the abnormal signal intensities detected on magnetic resonance imaging (MRI) in shy-drager syndrome (SDS) and striatonigral degeneration (SND). methods: The correlation of MRI and pathological findings was prospectively evaluated by postmortem scanning of the brain in a case of SDS and another of SND. MRI was performed by using a 1.0 T superconductive magnetic unit, with spin echo (SE) pulse sequences. The brain sections were prepared parallel to the MRI planes. The gross and microscopic pathological studies were conducted according to the corresponding abnormal signal intensities on MRI. RESULTS: In SDS, abnormal hypointense signals on T2 weighted MR images were symmetrically demonstrated in bilateral putamen, where a remarkable positive Prussian blue stain reaction was revealed on pathological examinations. In SND, MRI showed isointense signals on T1 weighted images and hyperintense signals on T2 weighted images in bilateral putamen, and the microscopic findings included necrosis, abundant reactive fibrillary astrocytes and prominence of capillary vascular networks, as well as marked lipofuscin. The number of neurons decreased in both cases. CONCLUSIONS: The demonstration of the abnormal signals in the putamen on MRI is of important value in establishing an antemortem diagnosis of SDS and SND.
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7/7. multiple system atrophy: the putative causative role of environmental toxins.

    BACKGROUND: Whereas a number of studies have investigated the putative role of environmental toxins in the pathogenesis of idiopathic parkinson disease, the possibility of such a role in multiple system atrophy has received little attention. DESIGN AND SETTING: review of records of patients examined in the Parkinson's Disease Center and movement Disorder Clinic, Baylor College of medicine, Houston, Tex, from July 1, 1977, to February 4, 1998. patients: We reviewed 100 consecutive medical records of patients who satisfied the diagnostic criteria for multiple system atrophy formulated by the consensus Committee of the American Autonomic Society and the American Academy of neurology. INTERVENTION: The type and amount of toxin exposure were verified by history and examination of records whenever possible. Severity of parkinsonism was assessed by clinical rating scales. MAIN OUTCOME MEASURE: Development of multiple system atrophy after environmental toxin exposure. RESULTS: Eleven patients had a notable history of heavy exposure to environmental toxins. One patient with multiple system atrophy confirmed by postmortem evaluation was exposed to high concentrations of malathion, diazinon, and formaldehyde, while the other patients with multiple system atrophy had well-documented high exposures to agents including n-hexane, benzene, methyl isobutyl ketone, and pesticides. The case studied pathologically demonstrated extensive advanced glial changes, including glial cytoplasmic inclusions in deep cerebellar white matter, brainstem, cortex (superior frontal, insula) and putamen, with notable cell loss and depigmentation of the substantia nigra and locus ceruleus. CONCLUSION: While many studies report a possible role of environmental toxins in parkinson disease, such a role is even more likely in multiple system atrophy, as this is a sporadic disease.
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