ABSTRACT
BACKGROUND AND OBJECTIVES: To evaluate residual symptoms after all-cause autoimmune encephalitis in a real-life outpatient setting and compare long-term outcome measures. A secondary objective was to identify correlates of poor outcomes. METHODS: We analyzed patients referred to the Neuroimmunology clinic for evaluation of autoimmune encephalitis for whom standardized data were collected. We compared the prevalence of symptoms at the latest follow-up to presentation and calculated symptom improvement rates. We compared the Modified Rankin Scale (mRS) to the Clinical Assessment Scale for Autoimmune Encephalitis (CASE). Non-parametric Wilcoxon rank sum tests and Fisher's exact tests were used to compare clinical attributes between patients with and without poor outcomes. RESULTS: We evaluated 54 patients from 2017 to 2021 of whom 33 met inclusion criteria (average age 47±20 years, 57% females, 55% seropositive). By latest follow-up, 94% improved compared to presentation but six patients (18%) had poor outcomes as defined by an mRS ≥3. The most common residual symptoms were cognitive and mood dysfunction. The highest improvement rates were in alertness and psychosis while the lowest were in motor function and ataxia. CASE had moderate correlation with mRS (r2 = 0.53 [95%CI:0.23,0.74, p = 0.0015) but it captured more nuances than mRS at both presentation and follow-up. Older age and higher post-treatment CASE score correlated with poor outcomes. DISCUSSION: Most autoimmune encephalitis patients experience symptom improvement post-treatment. The CASE score was more representative of the wide symptomatic spectrum of autoimmune encephalitis and correlated with poor outcomes. However, CASE did not capture patients with dysautonomia, sleep dysfunction, or death.
Subject(s)
Encephalitis , Hashimoto Disease , Adult , Aged , Disease Progression , Encephalitis/complications , Encephalitis/diagnosis , Encephalitis/therapy , Female , Hashimoto Disease/complications , Hashimoto Disease/diagnosis , Humans , Male , Middle Aged , Statistics, NonparametricABSTRACT
This paper describes the diagnostic and treatment utility of visual art therapy in a case of sporadic Creutzfeldt-Jakob disease. Visual art therapy was compared longitudinally with clinical and neuroimaging data over five-month period in an autopsy-confirmed case of sporadic Creutzfeldt-Jakob disease of MM2-cortical subtype. Art therapy sessions and content were useful in ascertaining neuropsychiatric symptoms during the course of her illness. Art therapy offered a unique emotional and cognitive outlet as illness progressed. Patients and families affected by sporadic Creutzfeldt-Jakob disease may benefit from art therapy despite the rapidly progressive nature of the illness. Art therapy can also be useful for assessment of patients with sporadic Creutzfeldt-Jakob disease by healthcare professionals.
Subject(s)
Art Therapy/methods , Creutzfeldt-Jakob Syndrome/rehabilitation , Photic Stimulation/methods , Adult , Cerebral Cortex/diagnostic imaging , Cerebral Cortex/pathology , Creutzfeldt-Jakob Syndrome/diagnostic imaging , Creutzfeldt-Jakob Syndrome/pathology , Diffusion Magnetic Resonance Imaging , Female , Fluorodeoxyglucose F18/pharmacokinetics , Humans , Positron-Emission Tomography , Treatment Outcome , Visual Perception/physiologyABSTRACT
Rapidly progressive dementia (RPD) is roughly defined as neurocognitive decline resulting in dementia or death within 2 years. Although RPDs affect all age groups, many occur in patients with young-onset dementia. Although prion disease (eg, Creutzfeldt-Jakob disease) is often thought to be the prototypic rapidly progressive young-onset dementia, the differential diagnosis is broad and some etiologies may be treatable. Hence, an appropriate workup to determine the etiology of RPD is crucial to planning the appropriate management. This article reviews the differential diagnosis, diagnostic workup, and management considerations for this unique patient population.
