Objective: To explore the expanding indications of dopamine transporter imaging (DaT Spect) beyond the established indications.

Background: DaTSPECT is a well-established method to rule out presynaptic dopaminergic deficit, hence to rule out degenerative Parkinsonism. However, recent research has shed light on its diagnostic value in rare neurodegenerative conditions, which might cause presynaptic dopaminergic deficit.

Method: Review of literature in PubMed to evaluate the findings in DaT SPECT in different neurodegenerative conditions.

Results: Premotor Parkinsonism

Recognizing the prodromal stage of a-synucleinopathies can be challenging. Idiopathic REM sleep behavior (iRBD) has been identified as the strongest risk factor for phenoconversion, with putaminal dopaminergic dysfunction (found in 30% of iRBD), constipation and age over 70 years being the strongest predictors (1).

NBIA

Besides a clinical parkinsonism, many publications report a normal nigrostriatal radiotracer uptake (2, 3) or just a slight uniform asymmetric decrement (4) in patients with neurodegeneration with brain iron accumulation (NBIA) as well as phantotenate kinase-associated neurodegeneration (PKAN). These findings are in line with neuropathologic findings, demonstrating involvement mainly of central pallidum and substantia nigra pars reticulata.

Autoimmune encephalitis

The only case report in Anti-Iglon 5 encephalitis with Lewy body dementia also described a reduced bilateral concentration of dopamine transporter (5)

HSP

Disruption of presynaptic dopaminergic pathways is a widespread phenomenon in patients with hereditary spastic paraplegia (HSP) typ 7 and 11, even in the absence of parkinsonism (6-10). Faber observed a correlation between the nigral degeneration and disease duration, motor and cognitive symptoms in patients with a SPG 11 mutation (10).

Ataxias

Reduced uptake has been described in SCA 2, 3, 6 and 17 (11). Some case series exists for the use of dopaminergic imaging in FXTAS showing some alterations in the dopaminergic pathway for patients with a parkinsonian phenotype also in premutation carriers (13, 14)

Conclusion: Dopamine transporter imaging is a sensitive tool to detect nigrostriatal degeneration. This can add important information regarding diagnosis and treatment options in above mentioned degenerative diseases

References: 1. Arnaldi D, Chincarini A, Hu MT, Sonka K, Boeve B, Miyamoto T, et al. Dopaminergic imaging and clinical predictors for phenoconversion of REM sleep behaviour disorder. Brain. 2021;144(1):278-87.
2. Fekete R. Late onset neurodegeneration with brain-iron accumulation presenting as parkinsonism. Case Rep Neurol Med. 2012;2012:387095.
3. Castelnau P, Zilbovicius M, Ribeiro MJ, Hertz-Pannier L, Ogier H, Evrard P. Striatal and pontocerebellar hypoperfusion in Hallervorden-Spatz syndrome. Pediatr Neurol. 2001;25(2):170-4.
4. Antonini A, Goldwurm S, Benti R, Prokisch H, Ebhardt M, Cilia R, et al. Genetic, clinical, and imaging characterization of one patient with late-onset, slowly progressive, pantothenate kinase-associated neurodegeneration. Mov Disord. 2006;21(3):417-8.
5. Seniaray N, Verma R, Ranjan R, Belho E, Mahajan H. Lewy Body Dementia Associated with Anti-IgLON 5 Encephalitis Detected on (18)F Fluorodeoxyglucose Positron Emission Tomography/Computed Tomography and 99mTc-TRODAT Single-Photon Emission Computed Tomography/Computed Tomography. Indian J Nucl Med. 2022;37(1):94-6.
6. Kim JS, Kim JM, Kim YK, Kim SE, Yun JY, Jeon BS. Striatal dopaminergic functioning in patients with sporadic and hereditary spastic paraplegias with parkinsonism. J Korean Med Sci. 2013;28(11):1661-6.
7. Bellini G, Del Prete E, Unti E, Frosini D, Siciliano G, Ceravolo R. Positive DAT-SCAN in SPG7: a case report mimicking possible MSA-C. BMC Neurol. 2021;21(1):328.
8. De la Casa-Fages B, Fernandez-Eulate G, Gamez J, Barahona-Hernando R, Moris G, Garcia-Barcina M, et al. Parkinsonism and spastic paraplegia type 7: Expanding the spectrum of mitochondrial Parkinsonism. Mov Disord. 2019;34(10):1547-61.
9. Pedroso JL, Vale TC, Bueno FL, Marussi VHR, Amaral L, Franca MC, Jr., et al. SPG7 with parkinsonism responsive to levodopa and dopaminergic deficit. Parkinsonism Relat Disord. 2018;47:88-90.
10. Faber I, Martinez ARM, Martins CR, Jr., Maia ML, Souza JP, Lourenco CM, et al. SPG11-related parkinsonism: Clinical profile, molecular imaging and l-dopa response. Mov Disord. 2018;33(10):1650-6.
11. Varrone A, Salvatore E, De Michele G, Barone P, Sansone V, Pellecchia MT, et al. Reduced striatal [123 I]FP-CIT binding in SCA2 patients without parkinsonism. Ann Neurol. 2004;55(3):426-30.
12. Shinotoh H, Thiessen B, Snow BJ, Hashimoto S, MacLeod P, Silveira I, et al. Fluorodopa and raclopride PET analysis of patients with Machado-Joseph disease. Neurology. 1997;49(4):1133-6.
13. Madeo G, Alemseged F, Di Pietro B, Schillaci O, Pisani A. Early abnormalities in 123I-ioflupane (DaTSCAN) imaging in the fragile X-associated tremor ataxia syndrome (FXTAS): a case report. Neurol Sci. 2013;34(8):1475-7.
14. Paucar M, Beniaminov S, Paslawski W, Svenningsson P. PSP-CBS with Dopamine Deficiency in a Female with a FMR1 Premutation. Cerebellum. 2016;15(5):636-40.

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