Objective: Our objective is to report a rare and unusual association and assess the plausibility of sporadic Creutzfeldt‑Jakob disease as an underlying cause of corticobasal syndrome in this patient.

Background: Corticobasal syndrome (CBS) is an atypical parkinsonian syndrome that may result from diverse underlying pathologies, most commonly corticobasal degeneration (CBD), but also Alzheimer’s disease, progressive supranuclear palsy, and less frequently prion diseases. Creutzfeldt‑Jakob disease (CJD) is a rare, rapidly progressive prion disease that can unusually present with CBS and thus be initially misdiagnosed as CBD.

Method: Case report

Results: Here we report the case of right-handed man, 71 years old who presented to our department with a 2-month-long history of paresthesia of the left hand, in addition to a maintained abnormal posture of the hand and breve paroxysmic jerks along with alien hand phenomenon.

On examination, he fulfilled criteria for CBS with asymmetric cortical sensory loss of the left hand, distal dystonia and myoclonus of the same limb, and ocular apraxia, without initial cognitive decline, his cognitive evaluation was normal.

Brain MRI showed: bilateral and symmetric cortical diffusion restriction and corresponding flair hyper-intensity predominantly along the cortical sulci.

The EEG was normal

The lumbar punction was normal for routine explorations and the examination of the cerebral spinal fluid for the 14-3-3 protein was negative.

Systematic work‑up for alternative etiologies was negative, including thoraco‑abdominal CT scan (no malignancy) and serum onconeural antibodies and infectious serologies. Over three months, the patient exhibited rapid clinical progression with worsening myoclonus and functional decline, while follow‑up MRI showed persistence of the characteristic cortical diffusion restriction pattern.

was concerning for the diagnosis of the CFD miming CBD

Conclusion: This case illustrates sporadic CJD presenting as a CBS, initially mimicking CBD. Clinicians should consider CJD in patients with rapidly progressive CBS, especially when MRI shows persistent cortical ribboning, even in the absence of typical EEG changes or positive CSF 14‑3‑3 protein.

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MDS Abstracts - https://www.mdsabstracts.org/abstract/creutzfeldt-jakob-disease-miming-a-cortico-basal-syndrome-does-it-exist/