Patterns of breakdown in spelling in primary progressive aphasia.

INTRODUCTION: The objective of this study is to determine which cognitive processes underlying spelling are most affected in the three variants of primary progressive aphasia (PPA): Logopenic variant primary progressive aphasia (lvPPA), Semantic variant primary progressive aphasia (svPPA), and Nonfluent variant primary progressive aphasia (nfvPPA). METHODS: 23 PPA patients were administered The Johns Hopkins Dysgraphia Battery to assess spelling. Subtests evaluate for effects of word frequency, concreteness, word length, grammatical word class, lexicality (words vs pseudowords), and "regularity" by controlling for the other variables. Significant effects of each variable were identified with chi square tests. Responses on all spelling to dictation tests were scored by error type. 16 of the 23 subjects also had a high resolution MRI brain scan to identify areas of atrophy. RESULTS: We identified 4 patterns of spelling that could be explained by damage to one or more cognitive processes underlying spelling. Nine patients (3 unclassifiable, 4 with lvPPA, 2 with svPPA) had dysgraphia explicable by impaired access to lexical representations, with reliance on sublexical phonology-to-orthography conversion (POC). Two patients (with nfvPPA) showed dysgraphia explicable by impaired access to lexical representations and complete disruption of sublexical POC. Seven patients (4 with lvPPA, 1 with svPPA, 2 unclassifiable) showed dysgraphia explicable by impaired access to lexical-semantic representations and/or lexical representations with partially spared sublexical POC mechanisms. Five patients (1 with nfvPPA, 2 with svPPA, 1 with lvPPA, and 1 unclassifiable) showed dysgraphia explicable by impairment of the graphemic buffer. CONCLUSIONS: Any cognitive process underlying spelling can be affected in PPA. Predominance of phonologically plausible errors, more accurate spelling of regular words than irregular words, and more accurate spelling of pseudowords than words (indicating spared POC mechanisms) may indicate a low probability of progression to nfvPPA.

Athletics, minor trauma, and pediatric arterial ischemic stroke.

Pediatric arterial ischemic stroke may occur as the result of trivial head or neck trauma sustained during a sports activity. We describe three cases of sports-related stroke in previously healthy school-age children and discuss acute and long-term stroke care. Possible mechanisms of sports-related stroke are addressed, as is evaluation for cause of stroke in children. In one of the reported cases, the child was found to have a vertebral artery dissection as the cause of his stroke, but no definitive cause of stroke was identified in the other two cases despite extensive evaluation. The advisability and timing of returning to athletic activities after stroke is also discussed. Many children with sports-related stroke are initially seen by a sports trainer, a pediatrician, or an ER physician. Thus, it is particularly important that these professionals are aware of the possibility of ischemic stroke occurring after even mild athletic injury. Childhood stroke may result from injuries sustained during athletic activities and should be considered when a child has acute focal neurologic signs.