Clinical Reasoning: a woman with rapidly progressive apraxia

Abstract

A 56-year-old woman presented with changes in balance, handwriting, and thinking. Approximately 1 year before her first visit, the patient developed difficulty walking, which caused multiple falls without serious injury. She also developed bilateral upper-extremity tremors that worsened with movement. At the time of her visit, she could barely sign her name.

Figure 1. Fluid-attenuated inversion recovery MRI of the patient's brain in the axial plane

There are confluent T2 hyperintensities involving subcortical white matter of the superior frontal gyri bilaterally and central to the motor strip, with additional T2 hyperintensities in periventricular white matter and atrophy of the paracentral lobules bilaterally.

Figure 2. Coronal section at the level of the anterior commissure

Note marked attenuation of subcortical white matter.

Figure 3. Marked white-matter rarefaction with neuroaxonal spheroid (open arrow) and glial cell with brownish pigment (arrow)

Hematoxylin & eosin, 600× magnification.

Figure 4. Neuroaxonal spheroids highlighted by neurofilament immunostain

Neurofilament, 100× magnification.