Teaching NeuroImages: Nonfluent variant primary progressive aphasia: A distinctive clinico-anatomical syndrome

A 66-year-old woman presented with 4 years of progressive speech difficulty. She had nonfluent speech with phonemic errors but intact single-word comprehension and object knowledge. Her grammar was impaired in both speech and writing, and she exhibited orofacial apraxia. A clinico-radiologic (see figure) diagnosis of nonfluent variant primary progressive aphasia was made

Teaching Neuro Images: Nonfluent variant primary progressive aphasia: A distinctive clinico-Anatomical syndrome

A 66-year-old woman presented with 4 years of progressive speech difficulty. She had nonfluent speech with phonemic errors but intact single-word comprehension and object knowledge. Her grammar was impaired in both speech and writing, and she exhibited orofacial apraxia. A clinico-radiologic (see figure) diagnosis of nonfluent variant primary progressive aphasia was made

Attorneys\u27 and Judges\u27 Needs for Continuing Legal Education on Mental Disability Law: Findings from a Survey

Attorneys leave law school with limited knowledge and skillsconcerning the issues that arise in mental disability law. Yetpsychiatrists and psychologists are appearing with increasingfrequency as witnesses in the nation\u27s courts, and more attorneysand judges can therefore expect to have to deal with testimony frommental health professionals. To our knowledge, this article is thefirst published assessment of practicing attorneys\u27 and judges\u27needs for continuing legal education (CLE) on mental disabilityissues. The 267 Dayton-area attorneys and 41 southwestern Ohio judgeswho responded to our mailed survey said that one-seventh of theircases raise issues related to mental health or mental disability.Most responders had not taken any law school courses that dealtwith mental disability issues; those who had said their courses wereonly modestly helpful. CLE was the attorneys\u27 and judges\u27principal source of information about mental disability law. Forpracticing attorneys, perceived need for CLE was related to therate at which psychological issues arose in their practices;practicing lawyers and judges were interested primarily in CLEtopics that related to the types of cases they handled or heard.Three-fourths of the attorneys and 95% of the judges said theywould probably or definitely attend locally offered CLE on at leastone subject. Our findings are consistent with the hypothesis that traditionallaw school course work relating to mental disability does not givefuture attorneys and judges the skills and knowledge necessary totheir practices (e.g., the ability to challenge expert witnesses);CLE might help remedy this deficiency. Legal educators should useour findings when thinking about law school course content andpostgraduate legal education

Conditional survival with increasing duration of ICU admission: an observational study of three intensive care databases.

OBJECTIVES: Prolonged admissions to an ICU are associated with high resource utilization and personal cost to the patient. Previous reports suggest increasing length of stay may be associated with poor outcomes. Conditional survival represents the probability of future survival after a defined period of treatment on an ICU providing a description of how prognosis evolves over time. Our objective was to describe conditional survival as length of ICU stay increased. DESIGN: Retrospective observational cohort study of three large intensive care databases. SETTING: Three intensive care databases, two in the United States (Medical Information Mart for Intensive Care III and electronic ICU) and one in United Kingdom (Post Intensive Care Risk-Adjusted Alerting and Monitoring). PATIENTS: Index admissions to intensive care for patients 18 years or older. INTERVENTIONS: None. MEASUREMENTS AND MAIN RESULTS: A total of 11,648, 38,532, and 165,125 index admissions were analyzed from Post Intensive Care Risk-Adjusted Alerting and Monitoring, Medical Information Mart for Intensive Care III and electronic ICU databases respectively. In all three cohorts, conditional survival declined over the first 5-10 days after ICU admission and changed little thereafter. In patients greater than or equal to 75 years old conditional survival continued to decline with increasing length of stay. CONCLUSIONS: After an initial period of 5-10 days, probability of future survival does not appear to decrease with increasing length of stay in unselected patients admitted to ICUs in United Kingdom and United States [corrected]. These findings were consistent between the three populations and suggest that a prolonged admission to an ICU is not a reason for a pessimism in younger patients but may indicate a poor prognosis in the older population

C9orf72 mutations and the puzzle of cerebro-cerebellar network degeneration

The recent work of Guo and colleagues (2016) underlines the important and specific involvement of the cerebellum in canonical dementia syndromes of Alzheimer’s disease and frontotemporal dementia. This new evidence poses the puzzle of how molecular pathology, regional cerebellar atrophy and phenotype are linked in these diseases

A sediment-based reconstruction of Caribbean effective precipitation during the Little Ice Age from Freshwater Pond, Barbuda

Contemporary climate dynamics of the circum-Caribbean region are characterised by significant precipitation variability on interannual and interdecadal timescales controlled primarily by El Niño Southern Oscillation (ENSO) and the Atlantic Multidecadal Oscillation (AMO). However, our understanding of pre-industrial climate variability in the region is hampered by the sparse geographic distribution of palaeoclimate archives. Here, we present a high-resolution reconstruction of effective precipitation for Barbuda since the mid-16th century, based on biostratigraphic and stable isotope analyses of fossil ostracods and gastropods recovered from lake sediment cores from Freshwater Pond, the only freshwater lake on the island. We interpret episodic fluctuations in shell accumulation in the sediment record to represent changes in the balance between precipitation and evaporation during the ‘Little Ice Age’ (LIA; ~1400–1850 CE) and Industrial (1850–present) periods. Comparisons between indices of reconstructed ENSO and AMO variability, the abundance of the freshwater gastropod Pyrgophorus parvulus and the δ18O records from ostracod calcite suggest that the relative influence of ENSO and AMO on long-term rainfall patterns in Barbuda has changed over the last 400 years. Our findings are in agreement with other high-resolution palaeoclimate studies that suggest that long-term changes in effective precipitation during the LIA were much more variable, temporally and spatially, than previously suggested

Frontotemporal Dementia: A Clinical Review.

Frontotemporal dementias are a clinically, neuroanatomically, and pathologically diverse group of diseases that collectively constitute an important cause of young-onset dementia. Clinically, frontotemporal dementias characteristically strike capacities that define us as individuals, presenting broadly as disorders of social behavior or language. Neurobiologically, these diseases can be regarded as "molecular nexopathies," a paradigm for selective targeting and destruction of brain networks by pathogenic proteins. Mutations in three major genes collectively account for a substantial proportion of behavioral presentations, with far-reaching implications for the lives of families but also potential opportunities for presymptomatic diagnosis and intervention. Predicting molecular pathology from clinical and radiological phenotypes remains challenging; however, certain patterns have been identified, and genetically mediated forms of frontotemporal dementia have spearheaded this enterprise. Here we present a clinical roadmap for diagnosis and assessment of the frontotemporal dementias, motivated by our emerging understanding of the mechanisms by which pathogenic protein effects at the cellular level translate to abnormal neural network physiology and ultimately, complex clinical symptoms. We conclude by outlining principles of management and prospects for disease modification

Hearing and dementia: from ears to brain

The association between hearing impairment and dementia has emerged as a major public health challenge, with significant opportunities for earlier diagnosis, treatment and prevention. However, the nature of this association has not been defined. We hear with our brains, particularly within the complex soundscapes of everyday life: neurodegenerative pathologies target the auditory brain, and are therefore predicted to damage hearing function early and profoundly. Here we present evidence for this proposition, based on structural and functional features of auditory brain organization that confer vulnerability to neurodegeneration, the extensive, reciprocal interplay between ‘peripheral’ and ‘central’ hearing dysfunction, and recently characterized auditory signatures of canonical neurodegenerative dementias (Alzheimer’s disease, Lewy body disease and frontotemporal dementia). Moving beyond any simple dichotomy of ear and brain, we argue for a reappraisal of the role of auditory cognitive dysfunction and the critical coupling of brain to peripheral organs of hearing in the dementias. We call for a clinical assessment of real-world hearing in these diseases that moves beyond pure tone perception to the development of novel auditory ‘cognitive stress tests’ and proximity markers for the early diagnosis of dementia and management strategies that harness retained auditory plasticit