Parkinsonismo precoce associado a lesões palidais de tipo "eye-of-the-tiger"

Abstract

RESUMO -Relata-se o caso de uma paciente com quadro parkisoniano instalado aos 26 anos de idade, 30 anos de evolução, boa resposta à levodopa e desenvolvimento precoce de discinesias induzidas por essa droga. Essas características associadas à ausência de outras manifestações neurológicas ao longo de todo o curso da moléstia sugeriam substrato anátomo-patológico superponível ao da forma clássica da doença de Parkinson. Entretanto, as imagens de ressonância magnética mostraram lesões palidais do tipo "eye-of-the-tiger", semelhantes às observadas na doença de Hallervorden-Spatz. O presente caso ilustra a heterogeneidade patológica dos casos de parkinsonismo de instalação precoce e a possibilidade do encontro desse padrão de lesões palidais em outras doenças degenerativas dos gânglios da base.PALAVRAS-CHAVE: parkinsonismo precoce, doença de Hallervorden-Spatz, imagem de ressonância magnética."Eye-of-the-tiger" sign at MRI in a patient with early-onset parkinsonism SUMMARY -We report the case of a 56-years-old woman patient, bom to unrelated parents, who since 26-years-old gradually developed bradykinesia, rigidity, tremor of both hands, and speech and gait difficulties. Her past history was unremarkable. There was no family history of neurologic disease. She was admitted to our Hospital at age 39 and at that time she presented a full parkinsonian syndrome. The following tests were normal or negative: routine blood studies, serum copper, ceruloplasmin and cerebrospinal fluid examination. There was not Kayser-Fleicher ring, and fundoscopic examination revealed no abnormalities. Levodopa was introduced and response was good for more than ten years, despite early-onset of dyskinesias (three months after the introduction of the drug). After 30 years under levodopa she still presents a moderate response but with severe fluctuations of the motor performance. Except for slowness of cognition she developed no other neuropsychological impairments, and a recent neurological examination disclosed no abnormalities besides a parkinsonian syndrome. One year ago, a magnetic resonance imaging (MRI) was perfomed and showed bilateral, symmetrical lesions with "eye-of-the-tiger" pattern. This case illustrates the pathological heterogenity of earlyonset parkinsonism and suggests the possibility to find the typical MRI lesions seen in Hallervorden-Spatz disease in other degenerative affections involving globus pallidus.

Neurodegeneration with brain iron accumulation: A case report

“… 4 , 20 This same sign has been described in other pathologies, such as neuroferritinopathy, corticobasal degeneration, multiple system atrophy 18 and in a case report with syndromic diagnosis of early onset parkinsonism, with response to dopaminergic therapy. 21 …”

Relaxation and susceptibility MRI characteristics in Hallervorden‐Spatz syndrome

“…These processes lengthen T2, which produces high SI on T2WI, as was seen in 12 of our patients (5, 15, 16). The eye‐of‐the‐tiger sign is most commonly described in association with HSS, but it has also been reported in other extrapyramidal disorders, including cortical‐basal degeneration (17), early‐onset levodopa‐responsive parkinsonism (18), and Steele‐Richardson‐Olszewski syndrome (19).…”