Psychosis in Machado-Joseph Disease: Clinical Correlates, Pathophysiological Discussion, and Functional Brain Imaging. Expanding the Cerebellar Cognitive Affective Syndrome

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dc.citation.issue4
dc.citation.volume15
dc.contributor.authorBraga-Neto, Pedro [UNIFESP]
dc.contributor.authorPedroso, Jose Luiz [UNIFESP]
dc.contributor.authorGadelha, Ary [UNIFESP]
dc.contributor.authorLaureano, Maura Regina [UNIFESP]
dc.contributor.authorNoto, Cristiano de Souza [UNIFESP]
dc.contributor.authorGarrido, Griselda Jara
dc.contributor.authorPovoas Barsottini, Orlando Graziani [UNIFESP]
dc.coverageNew York
dc.date.accessioned2020-08-14T13:43:55Z
dc.date.available2020-08-14T13:43:55Z
dc.date.issued2016
dc.description.abstractMachado-Joseph disease (MJD) is the most common spinocerebellar ataxia worldwide with a broad range of clinical manifestations, but psychotic symptoms were not previously characterized. We investigated the psychiatric manifestations of a large cohort of Brazilian patients with MJD in an attempt to characterize the presence of psychotic symptoms. We evaluated 112 patients with clinical and molecular diagnosis of MJD from February 2008 to November 2013. Patients with psychotic symptoms were referred to psychiatric evaluation and brain perfusion single-photon emission computed tomography (SPECT) analysis. A specific scale-Positive and Negative Syndrome Scale (PANSS)-was used to characterize psychotic symptoms in MJD patients. We also performed an autopsy from one of the patients with MJD and psychotic symptoms. Five patients presented psychotic symptoms. Patients with psychotic symptoms were older and had a late onset of the disease (p<0.05). SPECT results showed that MJD patients had significant regional cerebral blood flow (rCBF) decrease in the cerebellum bilaterally and vermis compared with healthy subjects. No significant rCBF differences were found in patients without psychotic symptoms compared to patients with psychotic symptoms. The pathological description of a patient with MJD and psychotic symptoms revealed severe loss of neuron bodies in the dentate nucleus and substantia nigra. MJD patients with a late onset of the disease and older ones are at risk to develop psychotic symptoms during the disease progression. These clinical findings may be markers for an underlying cortical-cerebellar disconnection or degeneration of specific cortical and subcortical regions that may characterize the cerebellar cognitive affective syndrome.en
dc.description.affiliationUniv Fed Sao Paulo, Dept Neurol & Neurosurg, Div Gen Neurol, 650 Pedro de Toledo St, BR-04039002 Sao Paulo, SP, Brazil
dc.description.affiliationUniv Fed Sao Paulo, Dept Neurol & Neurosurg, Ataxia Unit, 650 Pedro de Toledo St, BR-04039002 Sao Paulo, SP, Brazil
dc.description.affiliationUniv Estadual Ceara, Ctr Hlth Sci, Fortaleza, Ceara, Brazil
dc.description.affiliationUniv Fed Sao Paulo, Dept Psychiat, Sao Paulo, Brazil
dc.description.affiliationUniv Western Australia, Med Res Ctr, Western Australian Ctr Hlth & Ageing, Crawley, WA, Australia
dc.description.affiliationUnifespUniv Fed Sao Paulo, Dept Neurol & Neurosurg, Div Gen Neurol, 650 Pedro de Toledo St, BR-04039002 Sao Paulo, SP, Brazil
dc.description.affiliationUnifespUniv Fed Sao Paulo, Dept Neurol & Neurosurg, Ataxia Unit, 650 Pedro de Toledo St, BR-04039002 Sao Paulo, SP, Brazil
dc.description.affiliationUnifespUniv Fed Sao Paulo, Dept Psychiat, Sao Paulo, Brazil
dc.description.sourceWeb of Science
dc.format.extent483-490
dc.identifierhttp://dx.doi.org/10.1007/s12311-015-0716-2
dc.identifier.citationCerebellum. New York, v. 15, n. 4, p. 483-490, 2016.
dc.identifier.doi10.1007/s12311-015-0716-2
dc.identifier.issn1473-4222
dc.identifier.urihttps://repositorio.unifesp.br/handle/11600/57444
dc.identifier.wosWOS:000382928400009
dc.language.isoeng
dc.publisherSpringer
dc.relation.ispartofCerebellum
dc.rightsinfo:eu-repo/semantics/restrictedAccess
dc.subjectMachado-Joseph diseaseen
dc.subjectSpinocerebellar ataxiaen
dc.subjectPsychosisen
dc.subjectCerebellar cognitive affective syndromeen
dc.titlePsychosis in Machado-Joseph Disease: Clinical Correlates, Pathophysiological Discussion, and Functional Brain Imaging. Expanding the Cerebellar Cognitive Affective Syndromeen
dc.typeinfo:eu-repo/semantics/article
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