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5 Cards in this Set
- Front
- Back
Hallervorden Spatz
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Hallervorden-Spatz Syndrome
Preferred terms: Pantothenate kinase-associated neurodegeneration (PKAN) or NBIA-1 Progressive neurodegenerative disorder with brain iron accumulation "Eye of the tiger": Symmetric GP T2 hyperintensity surrounded by hypointensity May see T1 hyperintensity in T2 hypointense areas (iron accumulation) |
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Fahr dz
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Rare degenerative neurological disorder
Extensive bilateral basal ganglia (BG) calcifications (Ca++) Can lead to - progressive dystonia - parkinsonism - neuropsychiatric manifestations Also known as cerebrovascular ferrocalcinosis AD (sometimes AR) |
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McCune-Albright
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McCune-Albright syndrome (MAS)
Subtype of polyostotic fibrous dysplasia (FD) Classic triad - polyostotic FD - endocrine dysfunction (precocious puberty most common) - cutaneous hyperpigmentation (café au lait skin pigmentation) Female > > male Represents 3-5% of FD cases |
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Joubert's Syndrome
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Vermian hypoplasia
AR elnlarged fourth ventricle lack of midline fusion of the cerebellum heterotopic or dysplastic cerebellar nucleii |
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Jakob-Creutzfeld Dz
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Prion transmission
Symmetric increased signal on T2W in BG, cortex Cortical atrophy Restricted diffusion in BG and involved cortex p/w: - progressive dementia - myoclonus - periodic sharp wave complexes |