2004
DOI: 10.1590/s0004-282x2004000400031
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Síndrome de Hallervorden Spatz: achados na resonância magnética. Relato de caso

Abstract: RESUMO -A síndrome de Hallervorden-Spatz é afecção neurodegenerativa, autossômica recessiva com duas apresentações clínicas: precoce e tardia. Esta última é caracterizada pelo acometimento psiquiátrico e a presença de sinais piramidais e extrapiramidais. Relatamos o caso de mulher de 41 anos, com história de alterações extrapiramidais. O exame de ressonância magnética (RM) mostrou o sinal dos olhos-de-tigre, lesão dos globos pálidos mediais com deposição de ferro periférica (hipo-sinal) e gliose central (hiper… Show more

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Cited by 1 publication
(2 citation statements)
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“…Pantothenate kinase-associated neurodegeneration (PKAN) is a rare autosomal recessive disorder, and a form of neurodegeneration with brain iron accumulation (NBIA). [1][2][3] PKAN cases can be either classical, with onset in the first decade, or atypical, with late-onset, both presenting extrapyramidal dysfunction. 1,3 The 'eye of the tiger' sign is the characteristic MRI finding.…”
Section: Pantothenate Kinase Associated Neurodegeneration In Two Brotmentioning
confidence: 99%
See 1 more Smart Citation
“…Pantothenate kinase-associated neurodegeneration (PKAN) is a rare autosomal recessive disorder, and a form of neurodegeneration with brain iron accumulation (NBIA). [1][2][3] PKAN cases can be either classical, with onset in the first decade, or atypical, with late-onset, both presenting extrapyramidal dysfunction. 1,3 The 'eye of the tiger' sign is the characteristic MRI finding.…”
Section: Pantothenate Kinase Associated Neurodegeneration In Two Brotmentioning
confidence: 99%
“…[1][2][3] PKAN cases can be either classical, with onset in the first decade, or atypical, with late-onset, both presenting extrapyramidal dysfunction. 1,3 The 'eye of the tiger' sign is the characteristic MRI finding. [1][2][3][4]…”
Section: Pantothenate Kinase Associated Neurodegeneration In Two Brotmentioning
confidence: 99%