Diffuse Lewy body disease presenting with supranuclear gaze palsy

National Hospital for Nervous Diseases, Queen Square, London, UK.
Journal of Neurology Neurosurgery & Psychiatry (Impact Factor: 6.81). 03/1991; 54(2):159-61. DOI: 10.1136/jnnp.54.2.159
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A patient with diffuse Lewy body disease presented with supranuclear vertical and horizontal ophthalmoplegia, dementia, axial rigidity and falls, bradykinesia and pyramidal signs. This broadens the clinical presentation of this pathological diagnosis and re-emphasises the heterogeneity of patients diagnosed clinically as progressive supranuclear palsy (Steele-Richardson-Olszewski syndrome).

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Available from: David J Brooks, Aug 04, 2014
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    • "The frequency of LBs in our PSP population was lower than in previous reports (Table 2). Besides case reports describing the incidental presence of LBs in PSP (Mori et al., 1986; Fearnley et al., 1991; Judkins et al., 2002; Abhinav et al., 2011) the percentages of LBs in necropsy series of PSP-diagnosed patients ranged between 10.7% to 31.5% (Tsuboi et al., 2001; Mori et al., 2002; Uchikado et al., 2006; Keith-Rokosh and Ang, 2008). "
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    ABSTRACT: Objective: To analyze the frequency and distribution of α-synuclein deposits in progressive supranuclear palsy (PSP). Methods: The brains of 25 cases of pathologically confirmed PSP were evaluated with immunohistochemistry for α-synuclein and tau. Multiple immunofluorescent stains were applied to analyze the expression of tau and α-synuclein aggregates in catecholaminergic neurons. Patients' clinical symptoms were retrospectively recorded. Results: Deposits α-synuclein in the form of typical Lewy bodies (LBs) were only found in two PSP cases (8%) that fulfilled the clinical subtype of PSP known as Richardson's syndrome (RS). LBs were present in the locus ceruleus (LC), substantia nigra pars compacta (SNc), basal forebrain, amygdala and cingulated cortex in a distribution mimicking that of Parkinson's disease (PD). Triple-immunolabeling revealed co-expression of α-synuclein and tau proteins in some tyrosine hydroxilase (TH)-positive neurons of the LC and SNc. Conclusions: There is no apparent clinical correlation between the presence of LBs in PSP. Tau protein co-aggregate with α-synuclein in catecholaminergic neurons of PSP brains suggesting a synergistic interaction between the two proteins. This is in keeping with the current view of neurodegenerative disorders as "misfolded protein diseases".
    Frontiers in Neuroanatomy 03/2015; 9. DOI:10.3389/fnana.2015.00025 · 3.54 Impact Factor
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    • "There is a widening spectrum of clinical phenotypes documented in PSP and pathologically confirmed cases without an eye movement disorder have been described (Daniel et al., 1995; Revesz et al., 1996; Morris et al., 2002). A number of other neurodegenerative and vascular pathologies have been associated with a clinical picture of PSP including diffuse cerebrovascular disease (Winikates and Jankovic, 1994; Josephs et al., 2002), cerebral amyloid angiopathy with MND (Weeks et al., 2003), CBD (Litvan et al., 1997), FTDP-17 (Morris et al., 2003; Soliveri et al., 2003), subcortical gliosis with prion protein deposition (Will et al., 1988; Revesz et al., 1995) and diffuse Lewy body disease (Fearnley et al., 1991; de Bruin et al., 1992; Nakashima et al., 2003). Midbrain tumours (Silbert et al., 1993), Whipple's disease (Averbuch-Heller et al., 1999), neuro-syphilis (Murialdo et al., 2000) and neuroleptics (Campdelacreu et al., 2004) are other rare causes of a similar clinical picture. "
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    ABSTRACT: The frontotemporal lobar degenerations (FTLDs) are a group of disorders in which the clinical picture is not necessarily predictive of the underlying neuropathology. The FTLD with ubiquitin-only-immunoreactive neuronal changes (FTLD-U) subtype is pathologically characterized by ubiquitin-positive, tau and alpha-synuclein-negative neuronal cytoplasmic inclusions in the frontotemporal cortex and hippocampal dentate fascia. When similar pathological changes are accompanied by histological features of motor neuron disease (MND), the term FTLD-MND is used. The latter pathological changes may be found in patients with or without clinical evidence of MND. We retrospectively reviewed the clinical details of three patients with a rapidly progressive, levodopa-unresponsive bradykinetic-rigid syndrome and frontal cognitive impairment. A diagnosis of progressive supranuclear palsy (PSP) had been considered in all three cases at initial presentation. Two of the cases fulfilled clinical diagnostic criteria for PSP, which was the final clinical diagnosis during life. Pathological analysis showed typical histological appearances of FTLD-MND in two cases and of FTLD-U in one case. Semi-quantitative analysis of pathological load seemed to correlate with the clinical phenotype. FTLD-U or FTLD-MND should be considered in the differential diagnosis of progressive frontal dementia with an akinetic rigid syndrome and supranuclear gaze palsy or Steele-Richardson-Olszewski disease.
    Brain 12/2004; 127(Pt 11):2441-51. DOI:10.1093/brain/awh265 · 9.20 Impact Factor
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    ABSTRACT: A 67-year-old man with a family history of parkinsonism had visual complaints due to difficulty in convergence, which was followed 2 years later by development of bradykinesia and rigidity. The diagnosis of Steele-Richardson-Olszewski syndrome was made on the basis of a supranuclear gaze palsy, bradykinesia, rigidity, and poor response to levodopa. However, subsequent neuropathological examination revealed diffuse Lewy body disease with no evidence of neurofibrillary tangles involving either subcortical or brain stem structures.
    Movement Disorders 01/1992; 7(4):355-8. DOI:10.1002/mds.870070410 · 5.68 Impact Factor
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