'Tiger eye' appearance on MRI brain is characteristic feature of:
Ans. B. Hallervorden Spatz diseaseHallervorden- Spatz Disease (are neurodegenerative disorder of basal ganglia)DefinitionHallervorden- Spatz disease (HSD) is a rare disorder characterized by progressive extrapyramidal dysfunction and dementia. It's an iron metabolic disorder.Genetics1. The disease can be familial or sporadic. When familial, it is inherited recessively and has been linked to chromosome 20.2. Recently, a mutation in the pantothenate kinase (PANK2) gene on band 20pl3 has been described in patients with typical HSD.Clinical features: -1. Onset is most commonly in late childhood or early adolescence.2. progressive extrapyramidal dysfunction and dementia.3. Visual impairment from optic atrophy or retinal degeneration is not uncommon and can be the presenting symptom of the disease, although this is rare.MRIbilaterally symmetric hyperintense signal changes in anterior medial globus pallidus with surrounding hypointensity in the globus pallidus on T2-weighted images (Eye-of-the-tiger sign).
Refsum's disease
Hallervorden Spatz disease
NARP mitochondrial disease
Abetalipoproteinemia
Nervous System