Neurodegeneration with Brain Iron Accumulation (NBIA): MRI

Discussion by Dr MGK Murthy, Dr A Satish& Dr GA prasad Neurodegeneration with brain iron accumulation (NBIA) -NBIA disorders have typically symmetrical accumulation of Iron in parts of grey matter which normally are rich in Iron content , i. e. Basal ganglia (Globi pallidi /Putamen) / Thalamus / Midbrain (Substantia nigra / Red nuclei) cerebellum (Dentate nuclei) with each of the appx 10 varieties demonstrating relatively unique pattern , Permitting imaging classificationThis group of inherited neurologic disorders in which iron accumulates in the basal ganglia resulting in progressive dystonia, spasticity, parkinsonism, neuropsychiatric abnormalities, and optic atrophy or retinal degeneration. At least ten types and their associated genes are recognized. The age of onset ranges from infancy to late adulthood; the rate of progression varies. Generalized cerebral atrophy and cerebellar atrophy are also frequently observed. The diagnosis is usually first suspected when brain MRI findings suggest abnormal brain iron accumulation.An important initial distinction between NBIA and other conditions that lead to both extrapyramidal findings and abnormal basal ganglia signal is the nature of the T2 signal. NBIA disorders produce a characteristic hypointensity of the basal ganglia, while other disorders, such as mitochondrial encephalopathies, organic acidurias, and abnormalities of cofactor metabolism, feature T...

Source: Sumer's Radiology Site - March 31, 2017 Category: Radiology Authors: Sumer Sethi Source Type: blogs