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Objective: Moyamoya disease (MMD) is a rare degenerative cerebrovascular disorder that leads to recurrent strokes and cerebral hypoperfusion. Seizures are not uncommon in MMD especially following surgical treatment for the disease. Cognitive changes that are largely executive in nature have been documented following MMD and surgical treatment, although research is limited in adults. Methods: The present case report details the comprehensive neurocognitive evaluation of a patient with MMD and concomitant epilepsy. Results: Neurocognitive findings revealed a prominent dysexecutive pattern and atypically poor performance in areas such as visual and verbal memory. The patient reported significant affective symptoms and functional decline. Conclusions: This case offers insight into unique neurocognitive results that may present in adult MMD cases and underscores the importance of interpreting results in the context of neurological comorbidities in this rare disease.

Primary care integration of Down syndrome (DS)-specific dementia screening is strongly advised. The current study employed principal components analysis (PCA) and classification and regression tree (CART) analyses to identify an abbreviated battery for dementia classification. Scale- and subscale-level scores from 141 participants (no dementia n = 68; probable Alzheimer’s disease n = 73), for the Severe Impairment Battery (SIB), Dementia Scale for People with Learning Disabilities (DLD), and Vineland Adaptive Behavior Scales—Second Edition (Vineland-II) were analyzed. Two principle components (PC1, PC2) were identified with the odds of a probable dementia diagnosis increasing 2.54 times per PC1 unit increase and by 3.73 times per PC2 unit increase. CART analysis identified that the DLD sum of cognitive scores (SCS < 35 raw) and Vineland-II community subdomain (p = 0.57; PCA AUC = 0.87; CART AUC = 0.91). The PCA sensitivity was 80% and specificity was 70%; CART was 100% and specificity was 81%. These results support an abbreviated dementia screening battery to identify at-risk individuals with DS in primary care settings to guide specialized diagnostic referral.

Anti-N-methyl-D-aspartate receptor encephalitis is a neurological, autoimmune disorder tightly conceptualized only as recently as the mid-2000s. It presents itself in a combination of psychiatric, neurological, and autonomic features. We observe a unique case with probable earlier episode (prior to the mid-2000s conceptualization of the disease) and a later relapse, accompanying a comprehensive neuropsychological profile tracked after the relapse and subsequent improvement. Neurocognitive findings revealed residual frontal deficits with mood changes even in the state after plasmapheresis. This case is the first to describe posttreatment cognition in anti-NMDAR encephalitis after probable serial autoimmune episodes.