AMYOTROPHIC-LATERAL-SCLEROSIS WITH NUMEROUS AXONAL SPHEROIDS IN THE CORTICOSPINAL TRACT AND MASSIVE DEGENERATION OF THE CORTEX

A 53-year-old woman developed bulbar palsy, spastic quadriplegia, amyo trophy, and supranuclear ophthalmoplegia, and thereafter her condition was managed with mechanical ventilation for 1 year, Her total clinica l course was 6 years, The autopsy examination revealed neuronal loss w ith reactive astrocytosis in the precentral cortex, thalamus, mammilla ry body, amygdala, putamen, globus pallidus, subthalamic nucleus, and the substantia nigra, in addition to degeneration of lower motor neuro ns, some of which contained Bunina bodies, Alone the corticospinal tra ct, there were severe axonal loss and numerous axonal spheroids, which were positive for phosphorylated neurofilament, ubiquitin, and synapt ophysin. and lipid-laden macrophages in the centrum semiovale to the c rus cerebri. Ballooned neurons, which were positive for phosphorylated neurofilament. were occasionally seen in the frontal cortex. Although in the common form of amyotrophic lateral sclerosis (ALS) both upper and lower motor neurons are mainly involved, the corticospinal tract d egeneration cannot be traced rostral to the pens. The noteworthy featu res in our patient were the precentral cortical degeneration and axona l spheroids in the corticospinal tract rostral to the pens. It remains unclear why axonal spheroids in the corticospinal tract and precentra l cortical degeneration are not observed in most ALS cases, Whether th eir development depends on the clinical duration, mechanical ventilato r management, or some ether factors remains an open question.