Abstract
In a previous report, we presented longitudinal clinical, cognitive and anatomical data of a right-handed woman, whose clinical picture evolved from progressive nonfluent aphasia with apraxia of speech to corticobasal syndrome (CBS) in the last stage of the disease. The patient died at age 57 and pathological examination revealed severe atrophy in the left frontal operculum and left premotor area. On histological examination, there was diffuse tau-positive pathology in gray and white cortical hemispheric gray and white matter, basal ganglia and substantia nigra, compatible with corticobasal degeneration (CBD). This case demonstrates the clinical overlap between frontotemporal lobar degeneration and CBD. In this case, early motor speech impairment predicted earlier and more accurately than CBS the presence of underlying tau-pathology and CBD.
Publication types
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Case Reports
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Research Support, N.I.H., Extramural
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Research Support, Non-U.S. Gov't
MeSH terms
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Aphasia, Primary Progressive / diagnosis*
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Aphasia, Primary Progressive / etiology*
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Aphasia, Primary Progressive / physiopathology
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Basal Ganglia / pathology
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Basal Ganglia / physiopathology
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Brain / pathology*
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Brain / physiopathology*
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Cerebral Cortex / pathology
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Cerebral Cortex / physiopathology
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Dementia / diagnosis
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Dementia / pathology
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Dementia / physiopathology
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Diagnosis, Differential
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Disease Progression
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Fatal Outcome
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Female
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Humans
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Middle Aged
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Nerve Fibers, Myelinated / metabolism
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Nerve Fibers, Myelinated / pathology
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Neurofibrillary Tangles / metabolism
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Neurofibrillary Tangles / pathology
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Neurons / metabolism
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Neurons / pathology
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Predictive Value of Tests
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Substantia Nigra / pathology
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Substantia Nigra / physiopathology
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Tauopathies / complications*
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Tauopathies / diagnosis*
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Tauopathies / physiopathology
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tau Proteins / metabolism