The Distinguished Career Contributions Award
The Distinguished Career Contributions (DCC) award honors senior cognitive neuroscientists for their distinguished career, leadership and mentoring in the field of cognitive neuroscience. The recipient of this prize will give a lecture at our annual meeting.
Congratulations to Dr. Marsel Mesulam for being awarded this honor!
Dr. Mesulam, will accept this prestigious award and deliver his lecture on Sunday, April 6, 2014, 5:00 – 6:00 pm, in the Grand Ballroom Salon A-F. Reception to follow 6:00 – 7:00 pm in the Back Bay Conference and Exhibition Hall.
Primary Progressive Aphasia and the Language Network
Marsel Mesulam, MD, Ruth Dunbar Davee Professor of Neuroscience and Director of the Cognitive Neurology and Alzheimer’s Disease Center at Northwestern University
Primary progressive aphasia (PPA) is a clinical syndrome diagnosed when three core criteria are met. First, there should be a language impairment (i.e., aphasia) that interferes with the usage or comprehension of words. Second, the neurological work-up should determine that the disease is neurodegenerative, and therefore progressive. Third, the aphasia should arise in relative isolation, without equivalent deficits of comportment or episodic memory. The language impairment can be fluent or non-fluent and may or may not interfere with word comprehension. Memory for recent events is preserved although memory scores obtained in verbally mediated tests may be abnormal. Minor changes in personality and behavior may be present but are not the leading factors that bring the patient to medical attention or that limit daily living activities.
This distinctive clinical pattern is most conspicuous at the initial stages of the disease, and reflects a relatively selective atrophy of the language network, usually located in the left hemisphere. Asymmetry of neuronal loss is the biological hallmark of PPA and persists until death. Its determinants are unknown but may include a familial vulnerability of the language network, as reflected by the higher prevalence of learning disability in patients and their first degree relatives.
There are different clinical variants of PPA, each with a characteristic pattern of atrophy. The underlying neuropathological diseases are heterogeneous and can include Alzheimer’s disease as well as frontotemporal lobar degenerations. Each PPA variant has a preferential (but not absolute) association with a specific cellular type of pathology. The relationship of clinical variant to underlying pathology is probabilistic so that novel biomarkers are playing an increasingly more important role in surmising the nature of the underlying neuropathology.
Imaging studies in PPA have shown that the left anterior temporal lobe, which is not part of the classic language network, plays a critical role in word comprehension and object naming. The nature of this specialization has shed new light on the functional organization of temporal cortex. Research on PPA can therefore offer unique opportunities for exploring the biology of selective neurodegeneration as well as the cognitive architecture of language function.
2013 Robert T. Knight, M.D., University of California, Berkeley
2012 Morris Moscovitch, Ph.D., University of Toronto