Behavioral Neurology Takes You on a Tour of the Brain

Abstract

  For years Behavioral Neurology studies of focal syndromes has depended on patients with stroke and tumor lesions. With the development of imaging tools such as voxel based morphometry degenerative brain disease has expanded anatomical related syndromes such as the primary progressive aphasias. This paper describes degenerative brain disease cases illustrating focal syndrome onset related to the anatomy of the initial and most severe degeneration. The paper will start in the frontal area describing a patient with behavioral variant frontotemporal dementia relating the published criteria and different pathologies to this syndrome. The paper then describes a patient with posterior cortical atrophy affecting the occipital and parietal lobes and pointing out that these patients have preserved anterograde memory and other clinical features with which the anatomy correlates. Alzheimer pathology is typical but the syndrome may also be caused by corticobasal syndrome. The third case is one with corticobasal degeneration affecting the parietal lobe which has published criteria and a myriad of pathologies that may cause this syndrome. The next two case illustrations will be semantic dementia from the left more than right temporal lobe and prosopagnosia affecting the right more than left temporal lobe. The paper will discuss the diagnostic criteria and typical pathology of these cases. The paper describes a patient with non-fluent primary progressive aphasia and demonstrates that Progranulin mutation patients may present this way. To round out the causes of primary progressive aphasia the paper will describe a case with logopenic aphasia. The published criteria and typical pathology will be added. Lastly the paper describes an Alzheimer patient that demonstrates different memory pathways with the patient having retained “know how” (procedural memory) but not “know what” (episodic memory) and the underlying pathological anatomy that causes this. In conclusion Behavioral Neurology syndromes have expanded with the study of degenerative dementia.