Cases reported "Lewy Body Disease"

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1/6. Familial diffuse lewy body disease, eye movement abnormalities, and distribution of pathology.

    BACKGROUND: Familial diffuse lewy body disease (DLBD) is rare and not yet associated with a defect in the synuclein gene. In the differential diagnosis of the parkinsonian syndromes, defects in vertical gaze tend to be identified with progressive supranuclear palsy. False-positive diagnosis of progressive supranuclear palsy can occur, and defects in vertical gaze have been reported in DLBD, although so far a pure vertical gaze palsy associated with pathological abnormalities in the substrate for vertical gaze has not been described. OBJECTIVES: To report the clinical and pathological findings in 2 siblings with DLBD, and to relate the distribution of the pathological abnormalities in the brainstem to centers for vertical gaze. MATERIALS: For several years, 2 Irish siblings experienced a progressive parkinsonism-dementia complex associated in one with a defect in vertical gaze and in both with visual hallucinations. RESULTS: In both patients, results of pathological examination revealed (1) Lewy bodies positive for ubiquitin and alpha-synuclein together with cell loss and gliosis in the substantia nigra, locus ceruleus, and neocortex; and (2) similar findings in the rostral interstitial nucleus of the medial longitudinal fasciculus, the posterior commissure, and the interstitial nucleus of Cajal (substrates for vertical gaze). CONCLUSIONS: Familial DLBD (not shown to be genetically as distinct from environmentally transmitted) has been shown to exist in an Irish family. Caution should be enjoined in the interpretation of defects in vertical gaze in the differential diagnosis of the parkinsonian syndromes.
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ranking = 1
keywords = supranuclear, progressive supranuclear, supranuclear palsy, palsy
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2/6. An imaging study of parkinsonism among African-Caribbean and Indian london communities.

    We previously reported on 131 parkinsonian patients of African-Caribbean and Indian origin attending movement disorders clinics in six london hospitals, of whom approximately 20% manifested atypical parkinsonism with a late-onset, akinetic-rigid predominant syndrome, postural instability and minimal resting tremor refractory to levodopa therapy and dopamine agonists (see Hu et al., neurology 2000;54[Suppl.3]: A188 and Hu et al., Mov Disord 2000;15[Suppl.3]:S212). To better elucidate the phenotype of these atypical patients (18)FDG/(18)F-dopa positron emission tomography (PET) were performed in a subgroup to look for cortical and striatal metabolic changes suggestive of multiple system atrophy (MSA), progressive supranuclear palsy (PSP), corticobasal degeneration (CBD), or dementia with lewy bodies. magnetic resonance imaging (MRI) rating of cerebral vascular lesion load, putaminal atrophy, and neuropsychological testing were also performed. Discriminant function analysis of (18)F-dopa/(18)FDG striatal metabolism in 43 patients failed to separate atypical ethnic minority from typical Caucasian Parkinson's disease (PD) patients. Additionally, atypical Indian and African-Caribbean patients did not show cortical reductions in glucose metabolism suggestive of PSP, CBD, or DLB. Cerebral vascular lesion load rated in these patients did not differ between atypical and typical PD groups, and none of the atypical patients had MRI changes suggestive of MSA or PSP. Our results suggest the atypical parkinsonian phenotype seen in African-Caribbean and Indian patients represents a levodopa-refractory form of PD separate from MSA or PSP in most patients.
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ranking = 0.49997571411154
keywords = supranuclear, progressive supranuclear, supranuclear palsy, palsy
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3/6. An autopsied case of dementia with lewy bodies with supranuclear gaze palsy.

    A 66-year-old man had suffered from a slow and steady decline in both physical and cognitive function for four years. He showed bradykinesia and small step gait with supranuclear vertical gaze palsy, especially upward gaze palsy. He was started on levodopa therapy but without response. A diagnosis of progressive supranuclear palsy was clinically suspected. He died at age 69. Pathologically, many alpha-synuclein positive inclusions were detected both in the brain stem and cerebral cortices, and the diagnosis of dementia with lewy bodies was made. Scattered alpha-synuclein-positive inclusions and threads, which may be a pathological substrate for supranuclear gaze palsy, were identified in the rostal midbrain. From a review of five cases of dementia with lewy bodies with supranuclear gaze palsy including this case, the absence of falls in the early stage of the disease, fluctuation of cognition, hallucination and vertical gaze palsy with a more severe defect in the upward direction distinguished dementia with lewy bodies with vertical gaze palsy from progressive supranuclear palsy. In the differential diagnosis of parkinsonism with gaze palsy, clinicians should consider dementia with lewy bodies with gaze palsy as well as progressive supranuclear palsy.
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ranking = 3.1462968971908
keywords = supranuclear, progressive supranuclear, supranuclear palsy, palsy
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4/6. dementia with lewy bodies with supranuclear gaze palsy: a matter of diagnosis.

    Description of a case of probable dementia with lewy bodies featuring parkinsonism, dementia and supranuclear gaze palsy. This is the first patient to our knowledge affected with vertical gaze palsy receiving clinical diagnosis of DLB when alive and to be treated with cholinesterase inhibitors.
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ranking = 1.1758534974709
keywords = supranuclear, palsy
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5/6. A differential diagnosis of Parkinsonism.

    Parkinsonism is a common, age-related syndrome, characterized by resting tremor, bradykinesias, rigidity, and postural reflex impairment. Though Parkinsonism is not very difficult to recognize, all Parkinsonism is not created equal and it is important to distinguish among the most common identifiable syndromes. This review discusses the key clinical features of these various syndromes, including Parkinson's disease, progressive supranuclear palsy, multiple system atrophy, corticobasal ganglionic degeneration, lewy body disease, vascular Parkinsonism, and Parkinsonism with no clear etiology. Symptomatology and diagnostic testing for each syndrome are discussed and 4 typical cases are analyzed to offer clinicians guidance in making a differential diagnosis for Parkinsonism.
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ranking = 0.49997571411154
keywords = supranuclear, progressive supranuclear, supranuclear palsy, palsy
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6/6. Primary progressive freezing gait: a syndrome with many causes.

    Primary progressive freezing gait (PPFG) is characterized by early gait freezing and a stereotyped progression. Of nine patients followed up for 6 to 16 years, two were diagnosed pathologically: pallidonigroluysian degeneration (PNLD) and diffuse lewy body disease. Four others evolved clinically into progressive supranuclear palsy and corticobasal degeneration. PPFG is not a distinct disorder but a syndrome with diverse causes. Long-term follow-up (> or =10 years) and postmortem are required for accurate diagnosis. PNLD may be the primary form of disease.
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ranking = 0.49997571411154
keywords = supranuclear, progressive supranuclear, supranuclear palsy, palsy
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