Pathology

Extensive Purkinje cell degeneration characterizes the cerebellar pathology (Fig. 5.33). Residual cells may contain eosinophilic cytoplasmic inclusions. Cerebral lesions include cortical neuronal atrophy, neuronal

FIGURE 5.33

Ataxia telangiectasia. A 22-year-old man, diagnosed with AT before the age of 2 years, had bilateral conjunctival telangiectasia and nystagmus. From the age of 9 years, he was confined to a wheelchair because of progressing gait ataxia. His medical history was remarkable for multiple episodes of infection. He died of pneumonia following resection of a pulmonary abscess. The cerebellar cortex shows Purkinje cell losses, empty basket cells and replacement Bergmann astrocytosis (Bodian stain).

FIGURE 5.33

Ataxia telangiectasia. A 22-year-old man, diagnosed with AT before the age of 2 years, had bilateral conjunctival telangiectasia and nystagmus. From the age of 9 years, he was confined to a wheelchair because of progressing gait ataxia. His medical history was remarkable for multiple episodes of infection. He died of pneumonia following resection of a pulmonary abscess. The cerebellar cortex shows Purkinje cell losses, empty basket cells and replacement Bergmann astrocytosis (Bodian stain).

losses in the basal ganglia, and gliosis in the hemispheric white matter. The spinal cord, in advanced cases, shows degeneration of the posterior columns and atrophy of the motor neurons. Pathologic changes outside the nervous system include hypoplasia of the thymus and lymphoid tissues, ovarian or testicular dysgenesis, and large bizarre cells in the anterior pituitary gland.

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