We present a patient with rapidly progressive dementia (RPD) clinically suspicious for prion disease who met clinical criteria for probable and proposed MRI criteria for definite sporadic Creutzfeldt-Jakob disease (sCJD) but was found to have autoimmune encephalitis (AE). 1,2Case report A 69-year-old man with a history of smoking and tremor presented with RPD. During the prior 3 weeks, he lost his job and became increasingly confused, with worsening tremor and difficulty ambulating. At admission, he was oriented to self and setting, had poor recent and remote memory, and could not follow complex commands. There was moderate expressive aphasia with frequent paraphasic errors. Diffuse paratonia, a right parietal drift, tremor of the hands, poor coordination of rapid alternating movements, and brisk right deep tendon stretch reflexes were noted. He listed to the right when walking.Testing revealed hyponatremia (133 mEq/L) and CSF studies showed 1 leukocyte, protein 68 mg/dL, and glucose 91 mg/dL, without evidence of infection. CSF 14-3-3 and tau protein assay results were pending during hospitalization.Admission diffusion-weighted image (DWI) brain MRI demonstrated hyperintense cortical ribboning throughout the left hemisphere with corresponding hypointensity on apparent diffusion coefficient (ADC) mapping, sparing bilateral caudate nuclei and putamen ( figure, A and B). Admission EEG was low voltage with slight left hemispheric slowing in the delta/theta range, without periodic sharp-wave complexes commonly associated with prion disease.On day 6 the patient followed no commands, and had right facial myoclonus, right hemineglect, and severe ataxia.Antithyroperoxidase antibody level was 528 WHO units/mL blood. CT demonstrated a thyroid nodule and a 4-mm right upper lung nodule with 1 prominent hilar lymph node. Whole-body FDG-PET on day 8 demonstrated increased uptake in the left posterior cingulate Practical ImplicationsIn patients presenting with rapidly progressive dementia, brain FDG-PET imaging may be helpful in distinguishing patients with autoimmune encephalitis from those with CreutzfeldtJakob disease.

Aims of the study: We aimed to define the cognitive burden of the largest pseudotumor cerebri syndrome (PTCS) population to date, compare objective to subjective cognitive dysfunction, and determine clinical predictors of cognitive dysfunction amongst an array of previously unstudied factors.Clinical rationale: Patients with PTCS commonly report cognitive dysfunction, a factor associated with poor quality of life. It is not definitively known whether cognitive impairment is present in these patients, and what features of the syndrome predict impairment. Materials and methods:We administered a cognitive battery consisting of the National Adult Reading Test, Mini-Mental Status Exam, Digit Span, Boston Naming Test, Rey Auditory Verbal Learning Test, Clock Drawing, Trail Making Test, Controlled Oral Word Association, and Category Fluency. Cognitive impairment was defined as mild-single domain with one test score, and mild-multiple domain with two scores, more than two standard deviations below the mean for age-, gender-, and education-adjusted norms.Results: One-hundred and one prospectively recruited PTCS patients were enrolled. The objective testing showed 30 patients had mild-single domain impairment, and 25 had mild-multi domain impairment. More patients without objective cognitive impairment had transverse venous sinus stenosis, but otherwise the groups did not differ. Two measures of headache severity, the Headache Impact Test and pain on the Numeric Rating Scale, were negatively associated with the composite cognitive score, as was ocular pain, vision-related disability, and mental health. Opening pressure and visual function were not associated with objective cognitive impairment. We found no association between subjective and objective cognitive impairment. Conclusions and clinical implications:Patients with PTCS may be cognitively impaired, and this correlates with measures of headache burden. Studies evaluating cognitive impairment before and after remission of the headache disorder would have to be performed to investigate this relationship further. Patients with self-perception of cognitive burden are no more likely to be cognitively impaired.