Movement Disorders and Neurodegenerative Diseases

Variant: 1 Rapidly progressive dementia; suspected Creutzfeldt-Jakob disease. Initial imaging.

Procedure	Appropriateness Category	Relative Radiation Level
MRI head without and with IV contrast	Usually Appropriate	O
MRI head without IV contrast	Usually Appropriate	O
CT head without IV contrast	May Be Appropriate	☢☢☢
FDG-PET/CT brain	May Be Appropriate	☢☢☢
SPECT or SPECT/CT brain perfusion	May Be Appropriate	☢☢☢
MR spectroscopy head without IV contrast	Usually Not Appropriate	O
MRI functional (fMRI) head without IV contrast	Usually Not Appropriate	O
CT head with IV contrast	Usually Not Appropriate	☢☢☢
CT head without and with IV contrast	Usually Not Appropriate	☢☢☢

Variant: 2 Chorea; suspected Huntington disease. Initial imaging.

Procedure	Appropriateness Category	Relative Radiation Level
MRI head without IV contrast	Usually Appropriate	O
MRI head without and with IV contrast	May Be Appropriate	O
CT head without IV contrast	May Be Appropriate	☢☢☢
MR spectroscopy head without IV contrast	Usually Not Appropriate	O
MRI functional (fMRI) head without IV contrast	Usually Not Appropriate	O
CT head with IV contrast	Usually Not Appropriate	☢☢☢
CT head without and with IV contrast	Usually Not Appropriate	☢☢☢
FDG-PET/CT brain	Usually Not Appropriate	☢☢☢
SPECT or SPECT/CT brain perfusion	Usually Not Appropriate	☢☢☢

Variant: 3 Parkinsonian syndromes. Initial imaging.

Procedure	Appropriateness Category	Relative Radiation Level
MRI head without IV contrast	Usually Appropriate	O
MRI head without and with IV contrast	May Be Appropriate	O
CT head without IV contrast	May Be Appropriate	☢☢☢
FDG-PET/CT brain	May Be Appropriate	☢☢☢
SPECT or SPECT/CT brain striatal	May Be Appropriate	☢☢☢
MR spectroscopy head without IV contrast	Usually Not Appropriate	O
MRI functional (fMRI) head without IV contrast	Usually Not Appropriate	O
Amyloid PET/CT brain	Usually Not Appropriate	☢☢☢
CT head with IV contrast	Usually Not Appropriate	☢☢☢
CT head without and with IV contrast	Usually Not Appropriate	☢☢☢
SPECT or SPECT/CT brain perfusion	Usually Not Appropriate	☢☢☢

Variant: 4 Suspected neurodegeneration with brain iron accumulation. Initial imaging.

Procedure	Appropriateness Category	Relative Radiation Level
MRI head without IV contrast	Usually Appropriate	O
MRI head without and with IV contrast	May Be Appropriate	O
CT head without IV contrast	May Be Appropriate	☢☢☢
MR spectroscopy head without IV contrast	Usually Not Appropriate	O
MRI functional (fMRI) head without IV contrast	Usually Not Appropriate	O
CT head with IV contrast	Usually Not Appropriate	☢☢☢
CT head without and with IV contrast	Usually Not Appropriate	☢☢☢
FDG-PET/CT brain	Usually Not Appropriate	☢☢☢
SPECT or SPECT/CT brain perfusion	Usually Not Appropriate	☢☢☢

Variant: 5 Suspected motor neuron disease. Initial imaging.

Procedure	Appropriateness Category	Relative Radiation Level
MRI head without IV contrast	Usually Appropriate	O
MRI head without and with IV contrast	May Be Appropriate	O
MRI spine without and with IV contrast	May Be Appropriate	O
MRI spine without IV contrast	May Be Appropriate (Disagreement)	O
CT head without IV contrast	May Be Appropriate	☢☢☢
MR spectroscopy head without IV contrast	Usually Not Appropriate	O
MRI functional (fMRI) head without IV contrast	Usually Not Appropriate	O
CT head with IV contrast	Usually Not Appropriate	☢☢☢
CT head without and with IV contrast	Usually Not Appropriate	☢☢☢
CT spine with IV contrast	Usually Not Appropriate	☢☢☢
CT spine without IV contrast	Usually Not Appropriate	☢☢☢
FDG-PET/CT brain	Usually Not Appropriate	☢☢☢
SPECT or SPECT/CT brain perfusion	Usually Not Appropriate	☢☢☢
CT spine without and with IV contrast	Usually Not Appropriate	☢☢☢☢

Panel Members

H Benjamin. Harvey, MD, JD^a; Laura C. Watson, MD^b; Rathan M. Subramaniam, MD, PhD, MPH^c; Judah Burns, MD^d; Julie Bykowski, MD^e; Santanu Chakraborty, MBBS, MSc^f; Luke N. Ledbetter, MD^g; Ryan K. Lee, ^h; Jeffrey S. Pannell, MDⁱ; Jeffrey M. Pollock, MD^j; William J. Powers, MD^k; Joshua M. Rosenow, MD^l; Robert Y. Shih, MD^m; Konstantin Slavin, MDⁿ; Pallavi S. Utukuri, MD^o; Amanda S. Corey, MD^p.

Summary of Literature Review

Introduction/Background

Discussion of Procedures by Variant

Variant 1: Rapidly progressive dementia; suspected Creutzfeldt-Jakob disease. Initial imaging.

Variant 1: Rapidly progressive dementia; suspected Creutzfeldt-Jakob disease. Initial imaging.
A. CT Head

Variant 1: Rapidly progressive dementia; suspected Creutzfeldt-Jakob disease. Initial imaging.
B. FDG-PET/CT Brain

Variant 1: Rapidly progressive dementia; suspected Creutzfeldt-Jakob disease. Initial imaging.
C. MR Spectroscopy Head

Variant 1: Rapidly progressive dementia; suspected Creutzfeldt-Jakob disease. Initial imaging.
D. MRI Functional (fMRI) Head

Variant 1: Rapidly progressive dementia; suspected Creutzfeldt-Jakob disease. Initial imaging.
E. MRI Head

Variant 1: Rapidly progressive dementia; suspected Creutzfeldt-Jakob disease. Initial imaging.
F. HMPAO SPECT or SPECT/CT Brain

Variant 2: Chorea; suspected Huntington disease. Initial imaging.

Variant 2: Chorea; suspected Huntington disease. Initial imaging.
A. CT Head

Variant 2: Chorea; suspected Huntington disease. Initial imaging.
B. FDG-PET/CT Brain

Variant 2: Chorea; suspected Huntington disease. Initial imaging.
C. MR Spectroscopy Head

Variant 2: Chorea; suspected Huntington disease. Initial imaging.
D. MRI Functional (fMRI) Head

Variant 2: Chorea; suspected Huntington disease. Initial imaging.
E. MRI Head

Variant 2: Chorea; suspected Huntington disease. Initial imaging.
F. HMPAO SPECT or SPECT/CT Brain

Variant 3: Parkinsonian syndromes. Initial imaging.

Variant 3: Parkinsonian syndromes. Initial imaging.
A. CT Head

Variant 3: Parkinsonian syndromes. Initial imaging.
B. Amyloid PET/CT Brain

Variant 3: Parkinsonian syndromes. Initial imaging.
C. FDG-PET/CT Brain

Variant 3: Parkinsonian syndromes. Initial imaging.
D. Ioflupane SPECT/CT Brain

Variant 3: Parkinsonian syndromes. Initial imaging.
E. MR Spectroscopy Head

Variant 3: Parkinsonian syndromes. Initial imaging.
F. MRI Functional (fMRI) Head

Variant 3: Parkinsonian syndromes. Initial imaging.
G. MRI Head

Variant 3: Parkinsonian syndromes. Initial imaging.
H. HMPAO SPECT or SPECT/CT Brain

Variant 4: Suspected neurodegeneration with brain iron accumulation. Initial imaging.

Variant 4: Suspected neurodegeneration with brain iron accumulation. Initial imaging.
A. CT Head

Variant 4: Suspected neurodegeneration with brain iron accumulation. Initial imaging.
B. FDG-PET/CT Brain

Variant 4: Suspected neurodegeneration with brain iron accumulation. Initial imaging.
C. MR Spectroscopy Head

Variant 4: Suspected neurodegeneration with brain iron accumulation. Initial imaging.
D. MRI Functional (fMRI) Head

Variant 4: Suspected neurodegeneration with brain iron accumulation. Initial imaging.
E. MRI Head

Variant 4: Suspected neurodegeneration with brain iron accumulation. Initial imaging.
F. HMPAO SPECT or SPECT/CT Brain

Variant 5: Suspected motor neuron disease. Initial imaging.

Variant 5: Suspected motor neuron disease. Initial imaging.
A. CT Head

Variant 5: Suspected motor neuron disease. Initial imaging.
B. CT Spine

Variant 5: Suspected motor neuron disease. Initial imaging.
C. FDG-PET/CT Brain

Variant 5: Suspected motor neuron disease. Initial imaging.
D. MR Spectroscopy Head

Variant 5: Suspected motor neuron disease. Initial imaging.
E. MRI Functional (fMRI) Head

Variant 5: Suspected motor neuron disease. Initial imaging.
F. MRI Head

Variant 5: Suspected motor neuron disease. Initial imaging.
G. MRI Spine

Variant 5: Suspected motor neuron disease. Initial imaging.
H. HMPAO SPECT or SPECT/CT Brain

Summary of Recommendations

Supporting Documents

The evidence table, literature search, and appendix for this topic are available at https://acsearch.acr.org/list. The appendix includes the strength of evidence assessment and the final rating round tabulations for each recommendation.

For additional information on the Appropriateness Criteria methodology and other supporting documents, please go to the ACR website at https://www.acr.org/Clinical-Resources/Clinical-Tools-and-Reference/Appropriateness-Criteria.

Appropriateness Category Names and Definitions

Appropriateness Category Name	Appropriateness Rating	Appropriateness Category Definition
Usually Appropriate	7, 8, or 9	The imaging procedure or treatment is indicated in the specified clinical scenarios at a favorable risk-benefit ratio for patients.
May Be Appropriate	4, 5, or 6	The imaging procedure or treatment may be indicated in the specified clinical scenarios as an alternative to imaging procedures or treatments with a more favorable risk-benefit ratio, or the risk-benefit ratio for patients is equivocal.
May Be Appropriate (Disagreement)	5	The individual ratings are too dispersed from the panel median. The different label provides transparency regarding the panel’s recommendation. “May be appropriate” is the rating category and a rating of 5 is assigned.
Usually Not Appropriate	1, 2, or 3	The imaging procedure or treatment is unlikely to be indicated in the specified clinical scenarios, or the risk-benefit ratio for patients is likely to be unfavorable.

Relative Radiation Level Information

Potential adverse health effects associated with radiation exposure are an important factor to consider when selecting the appropriate imaging procedure. Because there is a wide range of radiation exposures associated with different diagnostic procedures, a relative radiation level (RRL) indication has been included for each imaging examination. The RRLs are based on effective dose, which is a radiation dose quantity that is used to estimate population total radiation risk associated with an imaging procedure. Patients in the pediatric age group are at inherently higher risk from exposure, because of both organ sensitivity and longer life expectancy (relevant to the long latency that appears to accompany radiation exposure). For these reasons, the RRL dose estimate ranges for pediatric examinations are lower as compared with those specified for adults (see Table below). Additional information regarding radiation dose assessment for imaging examinations can be found in the ACR Appropriateness Criteria^® Radiation Dose Assessment Introduction document.

Relative Radiation Level Designations
Relative Radiation Level*	Adult Effective Dose Estimate Range	Pediatric Effective Dose Estimate Range
O	0 mSv	0 mSv
☢	<0.1 mSv	<0.03 mSv
☢☢	0.1-1 mSv	0.03-0.3 mSv
☢☢☢	1-10 mSv	0.3-3 mSv
☢☢☢☢	10-30 mSv	3-10 mSv
☢☢☢☢☢	30-100 mSv	10-30 mSv
*RRL assignments for some of the examinations cannot be made, because the actual patient doses in these procedures vary as a function of a number of factors (e.g., region of the body exposed to ionizing radiation, the imaging guidance that is used). The RRLs for these examinations are designated as “Varies.”

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Disclaimer

The ACR Committee on Appropriateness Criteria and its expert panels have developed criteria for determining appropriate imaging examinations for diagnosis and treatment of specified medical condition(s). These criteria are intended to guide radiologists, radiation oncologists and referring physicians in making decisions regarding radiologic imaging and treatment. Generally, the complexity and severity of a patient’s clinical condition should dictate the selection of appropriate imaging procedures or treatments. Only those examinations generally used for evaluation of the patient’s condition are ranked. Other imaging studies necessary to evaluate other co-existent diseases or other medical consequences of this condition are not considered in this document. The availability of equipment or personnel may influence the selection of appropriate imaging procedures or treatments. Imaging techniques classified as investigational by the FDA have not been considered in developing these criteria; however, study of new equipment and applications should be encouraged. The ultimate decision regarding the appropriateness of any specific radiologic examination or treatment must be made by the referring physician and radiologist in light of all the circumstances presented in an individual examination.