To describe unusual case of CJD presenting as acute neuropathy and Ophthalmoplegia.

Progressive CJD is a devastating disease with rapidly progressive neurological symptoms and poor prognosis.

It is rare but still the most frequent human prion disease with incidence of one per 1,000,000 population per year.

A 59 years old with hypertension presented with progressive diplopia and distal upper and lower extremities numbness. She was found to have impaired finger to nose test bilaterally and normal MMSE.

NCS/EMG showed no evidence of demyelinating or axonal peripheral neuropathy, CSF analysis was normal with no evidence of infection or albuminocytogenic dissociation. She was treated with plasma exchange for possible Miller Fischer syndrome without improvement. Her symptoms rapidly progressed within few weeks as she became severely ataxic and unable to walk, MRI Brain showed no diffusion restriction or FLAIR changes. No periodic sharp waves seen in EEG.

Metabolic, paraneoplastic and autoimmune workup came back negative.

Because of the rapidly progressive symptoms CSF was sent and tested positive for tau, protein 14-3-3 and RT- Qu1C. CJD diagnosis was confirmed and patient died within few weeks.

We report an unusual presentation of CJD. The initial picture made neuropathy more likely. The continuous progression of symptoms despite adequate treatment raised the possibility of neurodegenerative disease.

Diagnosis of CJD is quite challenging. The gold standard for diagnosis is brain biopsy, CSF 14-3-3 can be seen in other diseases as infectious and paraneoplastic encephalitis, T.Tau is the best biomarker, however the novel Real Time Quaking Induced Conversion assays of CSF and olfactory Mucosa RT- Qu1C is more than 98 % specific and 90% sensitive.