Parkinsonism Associated with Pathological 123I-FP-CIT SPECT (DaTSCAN) Results as the Initial Manifestation of Sporadic Creutzfeldt-Jakob Disease

Abstract

Sporadic Creutzfeldt-Jakob disease (sCJD) is a type of progressive, subacute encephalopathy associated with spongiform degeneration of the central nervous system. sCJD includes a broad and heterogeneous spectrum of clinical variants, but extrapyramidal symptoms and signs at disease onset were rarely reported. We describe a case of unilateral parkinsonism associated with pathological 123I-ioflupane SPECT (DaTSCAN) results as the initial manifestation of M129V subtype sCJD patient. To the best of our knowledge, only 2 cases of Creutzfeldt-Jakob disease demonstrating nigrostriatal dopaminergic deficits in vivo using DaTSCAN have been published in the literature.

18F-FP-CIT PET/CT in a case of probable sporadic Creutzfeldt-Jakob disease with parkinsonism as initial symptom

“…To the best of our knowledge, cases about DAT PET/CT in CJD are rarely reported. The finding in our case, presynaptic dopaminergic deficit in CJD patients presenting parkinsonism at onset, is consistent with previous reports using 123I-FP-CIT single photon emission computed tomography (SPECT) [ 9–11 ]. Some previous studies have shown that no definite presynaptic dopaminergic deficit was observed in their DAT-scan despite the existence of rapidly progressive parkinsonism [ 12 , 13 ].…”

DaT Scan “Abnormality” in Hyperglycemic-Hemichorea

“…6 However, when MRI signal abnormalities are present in the basal ganglia, DaT scan results must be interpreted with caution. 8 Some reported cases in the literature [9][10][11][12][13] attribute parkinsonism due to abnormal DaT scan despite abnormality on structural imaging. It is therefore imperative that the results of DaT scan are interpreted in the context of structural imaging findings.…”

I123‐FP‐CIT (DaTSCAN) SPECT beyond the Most Common Causes of Parkinsonism: A Systematic Review

“…Prion diseases in general, and Creutzfeldt‐Jakob disease (CJD) in particular, can sometimes include parkinsonian symptoms as part of their heterogeneous phenotype (Table 2). These symptoms might be a consequence of the involvement of subcortical structures such as the caudate and putamen and would be associated with the presence of hyperintensities in the basal ganglia in MRI diffusion‐weighted sequences in approximately 70% of the patients 28 …”

“…These symptoms might be a consequence of the involvement of subcortical structures such as the caudate and putamen and would be associated with the presence of hyperintensities in the basal ganglia in MRI diffusionweighted sequences in approximately 70% of the patients. 28 However, the potential secondary damage to the dopaminergic nigrostriatal pathway has not been studied in depth. DaTSCAN could be a useful tool to confirm the presence of dopaminergic striatal degeneration in vivo.…”