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(B) 18F-FDG PET, axial slice, shows marked bilateral striatal hypometabolism, slightly more prominent in caudates. (C) Right anteromesial temporal hypometabolism was also observed (arrow), which might be related to the epileptic seizures and the EEG abnormalities, although its precise physiopathological explanation remains unknown. 3D-SSP maps comparing our PET image with healthy controls (Cortex ID software, General Electric) also reveal the above described striatal (medial view, D) and right temporal (inferior view, E) metabolic alterations." ] ] ] "textoCompleto" => "A 32-year-old man with 1–2 year history of possible cognitive and behavioral decline, in the form of verbal and visual memory loss and disinhibition, was referred to our center. He had also been diagnosed of probable epilepsy 5 years ago, because of several episodes of loss of consciousness. On his admission, he showed inappropriate behavior with disinhibition and perseverative features, as well as an attentional-executive deficit and mild memory impairment. Electroencephalogram showed possible left temporal subclinical seizures.Magnetic resonance imaging showed a very slight unspecific hyperintensity in amygdaline nucleus and hippocampus bilaterally, along with a certain loss of size of both caudate nuclei heads. Serum autoimmunity markers (including antineuronal antibodies) and serology for HIV, lues and Brucella were negative. Peripheral blood smear showed isolated acanthocytes. A remarkable elevation of serum creatine-kinase (CK), which had already been observed in previous blood tests, was also found. An electromyography and a muscle tissue biopsy were performed, and revealed denervation atrophy with mild collateral reinnervation.Genetic testing revealed 28 and 30 trinucleotide CAG repeats, which were considered negative for Huntington disease (HD). Such a diagnostic uncertainty led clinicians to request a cerebral 18F-FDG PET, which showed a severe bilateral, diffuse and symmetric striatal hypometabolism, highly suggestive of neurodegenerative striatal neuronal loss. A much less prominent right temporal anteromesial hypometabolism was also noticed. After having ruled out HD, and taking into consideration the other clinical and complementary data, a neuroacanthocytosis type disorder was suspected.The term “neuroacanthocytosis” accounts for a group of rare hereditary disorders that share the features of basal ganglia neurodegeneration, neuromuscular manifestations and spiculated erythrocytes (acanthocytes) in peripheral blood.<a class="elsevierStyleCrossRef" href="#bib0020">1</a> One of these entities is chorea-acanthocytosis, which is inherited in an autosomal recessive manner and is caused by mutations in the gene encoding the chorein, a protein being expressed in many tissues including the erythrocyte membrane. Despite not exhibiting an evident movement disorder, our patient had other chorea-acanthocytosis typical features, as epileptic seizures, frontal cognitive decline, suggestive electromyographical and histopathological findings, and elevated serum CK levels. The 18F-FDG PET findings suggesting striatal neurodegeneration were crucial in the course of the diagnostic work-up (<a class="elsevierStyleCrossRef" href="#fig0005">Fig. 1</a>).<a class="elsevierStyleCrossRefs" href="#bib0025">2,3</a> A Western Blot analysis with antichorein antisera was finally performed, showing absence of chorein on patient's erythrocyte membranes, which was considered a confirmatory finding of chorea-acanthocytosis.<elsevierMultimedia ident="fig0005"></elsevierMultimedia>" "pdfFichero" => "main.pdf" "tienePdf" => true "NotaPie" => array:1 [ 0 => array:2 [ "etiqueta" => "☆" "nota" => "Please cite this article as: Rubí S, Oporto M, García A, Legarda I, Picado MJ, Peña C. Hipometabolismo estriatal detectado por PET-FDG cerebral en un caso de corea-acantocitosis. Rev Esp Med Nucl Imagen Mol. 2016;35:205–206." ] ] "multimedia" => array:1 [ 0 => array:7 [ "identificador" => "fig0005" "etiqueta" => "Figure 1" "tipo" => "MULTIMEDIAFIGURA" "mostrarFloat" => true "mostrarDisplay" => false "figura" => array:1 [ 0 => array:4 [ "imagen" => "gr1.jpeg" "Alto" => 1931 "Ancho" => 2500 "Tamanyo" => 346000 ] ] "descripcion" => array:1 [ "en" => "(A) MRI Fast Spin Echo T2, axial slice showing minimal size decrease of caudate heads, without other striatal abnormalities. (B) 18F-FDG PET, axial slice, shows marked bilateral striatal hypometabolism, slightly more prominent in caudates. (C) Right anteromesial temporal hypometabolism was also observed (arrow), which might be related to the epileptic seizures and the EEG abnormalities, although its precise physiopathological explanation remains unknown. 3D-SSP maps comparing our PET image with healthy controls (Cortex ID software, General Electric) also reveal the above described striatal (medial view, D) and right temporal (inferior view, E) metabolic alterations." ] ] ] "bibliografia" => array:2 [ "titulo" => "References" "seccion" => array:1 [ 0 => array:2 [ "identificador" => "bibs0005" "bibliografiaReferencia" => array:3 [ 0 => array:3 [ "identificador" => "bib0020" "etiqueta" => "1" "referencia" => array:1 [ 0 => array:2 [ "contribucion" => array:1 [ 0 => array:2 [ "titulo" => "Neuroacanthocytosis syndromes orphanet" "autores" => array:1 [ 0 => array:2 [ "etal" => false "autores" => array:3 [ 0 => "H.H. Jung" 1 => "A. Danek" 2 => "R.H. Walker" ] ] ] ] ] "host" => array:1 [ 0 => array:1 [ "Revista" => array:4 [ "tituloSerie" => "J Rare Dis" "fecha" => "2011" "volumen" => "6" "paginaInicial" => "68" ] ] ] ] ] ] 1 => array:3 [ "identificador" => "bib0025" "etiqueta" => "2" "referencia" => array:1 [ 0 => array:2 [ "contribucion" => array:1 [ 0 => array:2 [ "titulo" => "Reduction of glucose metabolism in basal ganglia diagnosed with FDG-PET scan: a neuroacanthocytosis case" "autores" => array:1 [ 0 => array:2 [ "etal" => false "autores" => array:2 [ 0 => "N.A. Selcuk" 1 => "A. Fenercioglu" ] ] ] ] ] "host" => array:1 [ 0 => array:2 [ "doi" => "10.1097/RLU.0b013e3181e05d3f" "Revista" => array:6 [ "tituloSerie" => "Clin Nucl Med" "fecha" => "2010" "volumen" => "35" "paginaInicial" => "557" "paginaFinal" => "558" "link" => array:1 [ 0 => array:2 [ "url" => "https://www.ncbi.nlm.nih.gov/pubmed/20548161" "web" => "Medline" ] ] ] ] ] ] ] ] 2 => array:3 [ "identificador" => "bib0030" "etiqueta" => "3" "referencia" => array:1 [ 0 => array:2 [ "contribucion" => array:1 [ 0 => array:2 [ "titulo" => "Chorea-acanthocytosis in monozygotic twins: clinical findings and neuropathological changes as detected by diffusion tensor imaging, FDG-PET and 123I-β-CIT-SPECT" "autores" => array:1 [ 0 => array:2 [ "etal" => false "autores" => array:4 [ 0 => "K.R. Müller-Vahl" 1 => "G. Berding" 2 => "H.M. Emrich" 3 => "T. Peschel" ] ] ] ] ] "host" => array:1 [ 0 => array:2 [ "doi" => "10.1007/s00415-006-0492-5" "Revista" => array:6 [ "tituloSerie" => "J Neurol" "fecha" => "2007" "volumen" => "254" "paginaInicial" => "1081" "paginaFinal" => "1088" "link" => array:1 [ 0 => array:2 [ "url" => "https://www.ncbi.nlm.nih.gov/pubmed/17294064" "web" => "Medline" ] ] ] ] ] ] ] ] ] ] ] ] ] "idiomaDefecto" => "en" "url" => "/22538089/0000003500000003/v2_201703300105/S2253808916000288/v2_201703300105/en/main.assets" "Apartado" => array:4 [ "identificador" => "7927" "tipo" => "SECCION" "en" => array:2 [ "titulo" => "Interesting images" "idiomaDefecto" => true ] "idiomaDefecto" => "en" ] "PDF" => "https://static.elsevier.es/multimedia/22538089/0000003500000003/v2_201703300105/S2253808916000288/v2_201703300105/en/main.pdf?idApp=UINPBA00004N&text.app=https://www.elsevier.es/" "EPUB" => "https://multimedia.elsevier.es/PublicationsMultimediaV1/item/epub/S2253808916000288?idApp=UINPBA00004N"]

ISSN: 2253-8089

The Revista Española de Medicina Nuclear e Imagen Molecular (Spanish Journal of Nuclear Medicine and Molecular Imaging), was founded in 1982, and is the official journal of the Spanish Society of Nuclear Medicine and Molecular Imaging, which has more than 700 members. The Journal, which publishes 6 regular issues per year, has the promotion of research and continuing education in all fields of Nuclear Medicine as its main aim. For this, its principal sections are Originals, Clinical Notes, Images of Interest, and Special Collaboration articles. The works may be submitted in Spanish or English and are subjected to a peer review process. In 2009, it became the leading Spanish journal in the field of Medical Imaging on having an Impact Factor , awarded by the Journal Citation Reports.

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Impact factor

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1.6

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1.1

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0.208

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SNIP 2023

0.273

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