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Prion Diseases and Protein Misfolding / Autoimmune Neurological Disorders and Treatments / Peripheral Neuropathies and Disorders · Journal article
Cureus · August 20, 2026
Early or partial results. Treat as a signal, not a conclusion.
This is a case report of a 53-year-old man with probable sporadic CJD confirmed by positive CSF 14-3-3 protein, presenting with rapidly progressive dementia, cerebellar ataxia, pyramidal signs, and extrapyramidal features over approximately one month before progressive decline to death at seven months. The report emphasizes diagnostic challenges and the importance of considering CJD in the differential diagnosis of rapidly progressive dementia with mixed neurological features, but provides no efficacy data on intervention (empirical high-dose methylprednisolone produced no improvement).
Case report. A 53-year-old male from Georgia presenting with one month of progressive gait difficulty, dysarthria, behavioral changes, and memory impairment. Autoimmune and paraneoplastic antibody testing was negative; PET showed no malignancy.. Intervention: Empirical high-dose methylprednisolone. n = 1. Georgia.
Progressive gait difficulty, dysarthria, behavioral changes, and memory impairment developed over approximately one month MRI showed restricted diffusion involving the head and body of the left caudate nucleus CSF analysis showed mildly elevated protein level and positive CSF 14-3-3 protein result
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This case illustrates the need for CJD to be considered in the differential diagnosis of rapidly progressive dementia, especially when accompanied by cerebellar, pyramidal, extrapyramidal, and peripheral neurological features. CSF 14-3-3 protein positivity supports probable sporadic CJD diagnosis when other infectious, autoimmune, and paraneoplastic causes have been excluded.
A single case report of probable sporadic CJD with clinical and CSF findings; descriptive only, without comparative data or intervention efficacy, and raises diagnostic awareness rather than testing a therapeutic hypothesis.
As stated by the source record.
Quoted from the source exactly as published.
This case illustrates the need for CJD to be considered in the differential diagnosis of rapidly progressive dementia, especially when accompanied by cerebellar, pyramidal, extrapyramidal, and peripheral neurological features. CSF 14-3-3 protein positivity supports probable sporadic CJD diagnosis when other infectious, autoimmune, and paraneoplastic causes have been excluded.
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Creutzfeldt-Jakob disease (CJD) is a rare, fatal prion disorder that commonly presents as rapidly progressive dementia and may initially mimic autoimmune, paraneoplastic, infectious, or metabolic encephalopathies. We report a case of probable sporadic CJD in a 53-year-old man from Georgia who developed approximately one month of progressive gait difficulty, dysarthria, behavioral changes, and memory impairment. Neurological examination revealed hypophonic dysarthric speech, horizontal nystagmus, mild-to-moderate tetraparesis, dystonic hyperkinesia, distal sensory impairment, and severe gait ataxia with astasia-abasia. Brain computed tomography showed no acute intracranial lesion, while magnetic resonance imaging demonstrated restricted diffusion involving the head and body of the left caudate nucleus. Electroencephalography (EEG) demonstrated nonspecific findings. Testing for a defined set of autoimmune and paraneoplastic antibodies was negative, and positron emission tomography showed no evidence of malignancy. Electroneuromyography demonstrated mixed demyelinating and axonal peripheral nerve involvement. Cerebrospinal fluid (CSF) analysis showed no pleocytosis, a mildly elevated protein level, and a positive CSF 14-3-3 protein result, supporting the diagnosis of probable sporadic CJD. Real-time quaking-induced conversion (RT-QuIC) testing was not performed because it was unavailable in the treating clinical setting. Empirical high-dose methylprednisolone produced no meaningful neurological improvement. The patient subsequently progressed to a bedbound, nonverbal state with severe dependence and recurrent respiratory complications. Despite prolonged intensive care, mechanical ventilation, tracheostomy, and broad-spectrum antimicrobial therapy, he died approximately seven months after symptom onset from pneumonia complicated by acute respiratory failure and shock, culminating in cardiac arrest. This case highlights the diagnostic challenges of rapidly progressive dementia and the importance of considering sporadic CJD when cognitive decline is accompanied by cerebellar, pyramidal, extrapyramidal, and peripheral neurological features.
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