Additionally, recent studies in patients with PD have shown decreased magnetization transfer ratio and functional anisotropy within the substantia nigra (particularly caudally) when compared with normal controls [67]. HMPAO SPECT or SPECT/CT Brain There is no relevant literature to support the use of Tc-99m HMPAO SPECT/CT of the brain in the initial imaging evaluation of a patient with Parkinsonian syndrome. Variant 4: Suspected neurodegeneration with brain iron accumulation. Initial imaging. Although some iron accumulation within the basal ganglia and dentate nuclei can normally be seen as we age, NBIA is a distinct group of conditions characterized by excess iron deposition in the basal ganglia with progressive neuronal degeneration. Four subtypes have been defined, the most common of which is NBIA type 1, also known as pantothenate kinase-associated neurodegeneration and formerly known as Hallervorden-Spatz disease. NBIA type 1 is a rare autosomal recessive disease (annual incidence of 1/1,000,000), which classically presents in the first decade with slowly progressive gait disturbances, dystonia, dysarthria, spasticity, and pyramidal tract signs [68]. FDG-PET/CT Brain There is no relevant literature to support the use of FDG-PET/CT of the brain in the initial imaging evaluation of a patient with suspected NBIA. MR Spectroscopy Head There is no relevant literature to support the use of MR spectroscopy of the brain in the initial imaging evaluation of a patient with suspected NBIA. MRI Functional (fMRI) Head There is no relevant literature to support the use of fMRI of the brain in the initial imaging evaluation of a patient with suspected NBIA. Movement Disorders and Neurodegenerative Diseases associated enhancement or restricted diffusion in these areas of iron deposition, although diffusion tensor imaging has shown increased fractional anisotropy in both the globus pallidus and substantia nigra in patients with NBIA [74].
Movement Disorders and Neurodegenerative Diseases. Additionally, recent studies in patients with PD have shown decreased magnetization transfer ratio and functional anisotropy within the substantia nigra (particularly caudally) when compared with normal controls [67]. HMPAO SPECT or SPECT/CT Brain There is no relevant literature to support the use of Tc-99m HMPAO SPECT/CT of the brain in the initial imaging evaluation of a patient with Parkinsonian syndrome. Variant 4: Suspected neurodegeneration with brain iron accumulation. Initial imaging. Although some iron accumulation within the basal ganglia and dentate nuclei can normally be seen as we age, NBIA is a distinct group of conditions characterized by excess iron deposition in the basal ganglia with progressive neuronal degeneration. Four subtypes have been defined, the most common of which is NBIA type 1, also known as pantothenate kinase-associated neurodegeneration and formerly known as Hallervorden-Spatz disease. NBIA type 1 is a rare autosomal recessive disease (annual incidence of 1/1,000,000), which classically presents in the first decade with slowly progressive gait disturbances, dystonia, dysarthria, spasticity, and pyramidal tract signs [68]. FDG-PET/CT Brain There is no relevant literature to support the use of FDG-PET/CT of the brain in the initial imaging evaluation of a patient with suspected NBIA. MR Spectroscopy Head There is no relevant literature to support the use of MR spectroscopy of the brain in the initial imaging evaluation of a patient with suspected NBIA. MRI Functional (fMRI) Head There is no relevant literature to support the use of fMRI of the brain in the initial imaging evaluation of a patient with suspected NBIA. Movement Disorders and Neurodegenerative Diseases associated enhancement or restricted diffusion in these areas of iron deposition, although diffusion tensor imaging has shown increased fractional anisotropy in both the globus pallidus and substantia nigra in patients with NBIA [74].