An autopsied case of corticobasal degeneration with onset of nonfluent aphasia revealing symmetrical cerebral involvement

Kenji Ishihara, Masaru Mimura, Seiichirou Ishigaki, Jun Ichi Shiota, Imaharu Nakano, Mitsuru Kawamura

Research output: Contribution to journalArticle

Abstract

Herein we describe a patient with established corticobasal degeneration with onset of nonfluent aphasia and showing symmetrical cerebral involvement. A 64-year-old man with a speech disorder for 2 years visited our hospital. He had nonfluent aphasia (reduced spontaneous speech, loss of intonation, anomia, repetition disorder, and difficulty in speaking short sentences). He also showed right-sided motor neglect, hypertonus of the left lower limb, a mask-like facial expression, and difficulty in closing his eyes. He was restless and walked around even during examination, suggesting frontotemporal dementia (FTD). Single-photon emission computed tomography (SPECT) revealed symmetrical reduction of cerebral blood flow in the bilateral fronto-temporo-parietal lobes. His neurological condition deteriorated gradually and a year later he could not speak comprehensive sentences. Magnetic resonance imaging (MRI) of the head at age 70 showed symmetrical atrophy of the bilateral fronto-temporal lobes. He died of respiratory failure after clinical problems lasting ten years. On pathological examination, the fixed brain weighed 1,010 g and showed bilateral symmetrical atrophy of the frontal lobes. Histopathological examination revealed neuronal loss and gliosis in the frontal lobes, especially in the frontal convexity, superior frontal gyrus and precentral gyrus. Gallyas-Braak silver staining showed astrocytic plaques, argyrophilic threads and coiled bodies mainly in the frontal lobes. The substantia nigra showed severe neuronal loss on both sides and presence of free melanin. Pathological diagnosis was corticobasal degeneration (CBD). We believe that the patient had nonfluent aphasia and FTD reflected in bilateral degeneration of the frontal lobes. Some cases of CBD may present with symmetrical degeneration of the brain, even though left-hemisphere symptoms such as aphasia reveal themselves at an early stage.

Original languageEnglish
Pages (from-to)943-949
Number of pages7
JournalBrain and Nerve
Volume64
Issue number8
Publication statusPublished - 2012 Aug 1

Keywords

  • Corticobasal degeneration
  • Frontotemporal dementia
  • Nonfluent aphasia
  • Pathology
  • Symmetrical degeneration

ASJC Scopus subject areas

  • Clinical Neurology

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