Sporadic Creutzfeldt-Jakob disease (sCJD) can initially present with focal neurological symptoms, delaying diagnosis when a plausible structural lesion is present.A 70-year-old woman presented with progressive right arm weakness and shoulder pain, clinically suggesting a C5 palsy-like pattern.Cervical magnetic resonance imaging (MRI) showed C5/6 disc herniation with right foraminal stenosis, prompting anterior cervical discectomy and fusion.Postoperatively, her weakness and gait disturbance progressed, and she subsequently developed confusion, gaze abnormalities, and status epilepticus requiring intensive care.Serial electroencephalography demonstrated evolving focal and multifocal epileptiform abnormalities with diffuse cerebral dysfunction.Initial brain MRI showed no acute diffusion restriction, but follow-up MRI revealed evolving cortical diffusion abnormalities in the left hemisphere.Real-time quaking-induced conversion testing of cerebrospinal fluid was positive, and total tau was elevated, supporting a diagnosis of probable sCJD.Rapid neurological progression accompanied by encephalopathy or seizures should prompt reconsideration of an apparently plausible cervical diagnosis.
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Oh et al. (2026) studied this question.
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