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A case of a rapidly progressive degeneration of the visual, auditory and corticospinal tract in a patient with a HIV infection is presented.The HIV-infected patient suffered from severe and rapidly progressive sensorineural hearing loss, blindness, dysarthria, dysphagia and tetraparesis. MRI showed degeneration of the visual, auditory and corticospinal tract. Diffusion tensor imaging showed reduced fractional anisotropy of the corticospinal tract. HAART and other salvage therapies did not save the patient from death 5 months after the onset of the symptoms.The triad of auditory, visual and corticospinal lesions has been described in rare syndromes and hereditary diseases. This is the first case of this syndrome associated with a HIV infection.