The white matter and cortex are spared in NBIA type 1. MRI may also be helpful in distinguishing the other less common subtypes of NBIA [69,71,72]. IV contrast may be useful in the initial evaluation of suspected NBIA, considering the presence of limited inflammatory etiologies among the differential diagnoses. 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 suspected NBIA. Variant 5: Suspected motor neuron disease. Initial imaging. ALS is a progressive neurodegenerative disorder characterized by degeneration of the upper and lower motor neurons in the brain and spinal cord along the corticospinal tracts and is the most common motor neuron disease, representing ~85% of all cases [75]. The majority of cases are sporadic (85% to 90%) with an overall annual incidence of 1 to 2/100,000 and a median survival of 3 to 4 years after symptom onset [75]. Typically, patients present with hypertonicity and hyperreflexia (upper motor neuron degeneration) and muscle fasciculations, weakness, and atrophy (lower motor neuron degeneration). Electromyography and nerve conduction velocity are key tests in diagnosing ALS, with imaging relied upon mainly to exclude other conditions with similar clinical presentations rather than confirm or facilitate the diagnosis of ALS. CT Spine CT of the spine (either with, without, or without and with IV contrast) is not useful in making the diagnosis of ALS because of its limited soft-tissue characterization. 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 motor neuron disease. 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 motor neuron disease.
Movement Disorders and Neurodegenerative Diseases. The white matter and cortex are spared in NBIA type 1. MRI may also be helpful in distinguishing the other less common subtypes of NBIA [69,71,72]. IV contrast may be useful in the initial evaluation of suspected NBIA, considering the presence of limited inflammatory etiologies among the differential diagnoses. 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 suspected NBIA. Variant 5: Suspected motor neuron disease. Initial imaging. ALS is a progressive neurodegenerative disorder characterized by degeneration of the upper and lower motor neurons in the brain and spinal cord along the corticospinal tracts and is the most common motor neuron disease, representing ~85% of all cases [75]. The majority of cases are sporadic (85% to 90%) with an overall annual incidence of 1 to 2/100,000 and a median survival of 3 to 4 years after symptom onset [75]. Typically, patients present with hypertonicity and hyperreflexia (upper motor neuron degeneration) and muscle fasciculations, weakness, and atrophy (lower motor neuron degeneration). Electromyography and nerve conduction velocity are key tests in diagnosing ALS, with imaging relied upon mainly to exclude other conditions with similar clinical presentations rather than confirm or facilitate the diagnosis of ALS. CT Spine CT of the spine (either with, without, or without and with IV contrast) is not useful in making the diagnosis of ALS because of its limited soft-tissue characterization. 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 motor neuron disease. 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 motor neuron disease.