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A right handed 70 year old woman awakens to discover something wrong with her sight. When you examine her, you discover that although Snellen visual acuity is normal, she claims that she cannot read. Yet she completed high school and was employed as a cashier before retiring, and she seems to have no language problems, either in expression or comprehension. Surprisingly, she can spell and write to dictation.

  • Review Topic

    Where is the lesion?

    Correct!
    She is demonstrating the manifestations of “alexia without agraphia,” also known as “pure alexia.” It is a recognition disturbance for written language (and often other familiar symbols). It is not an aphasia (language disturbance). Written information cannot reach the posterior portion of the angular gyrus in the temporal lobe. This disconnection happens most often when a lesion—almost always an ischemic stroke in the domain of the posterior cerebral artery—damages the left medial occipital lobe, causing a right homonymous hemianopia, and extends anteriorly enough to damage the left forceps major of the splenium of the corpus callosum. The combination of a left medial occipital cortex and forceps major lesion interrupts transmission of visual information from the intact right visual cortex across the splenium and forceps major on its way to the angular gyrus. Indeed, this patient did have a right homonymous hemianopia.

    Patients with pure alexia do not have any features of an aphasia. They can can spell, understand spoken language, and express themselves normally, because those functions are generated in the angular gyrus, which is not lesioned. This patient had a left posterior cerebral artery stroke with forward extension into the splenial region.
  • Over time, her reading deficit gradually improved, but she still hesitated. The homonymous hemianopia was permanent.

    By the way, the “disconnection theory” of alexia without agraphia has been questioned. An alternative explanation is damage to a putative “visual word form” area near the angular gyrus. If that explanation is correct, it would explain why some patients with alexia without agraphia do not have homonymous hemianopia.