Although former writings have stressed the development of delusional systems, most dramatically in the direction of mania, such symptoms are exceptional in the early or preparalytic phase. More usual has been a simple dementia with reduction of intellectual capacities, forgetfulness, disorders of speaking and writing, and vague concerns about health. In a few patients the first hint of a syphilitic encephalitis, as mentioned earlier, may be facial quivering; tremulousness of the hands; indistinct, hurried speech; myoclonus; and seizures—reminiscent of delirium or acute viral encephalitis. As the deterioration continues into the paralytic stage, intellectual function progressively declines, and aphasias, agnosias, and apraxias intrude themselves.
Physical dissolution progresses concomitantly—impaired station and gait, debility, unsteadiness, dysarthria, and tremor of the tongue and hands. All these disabilities lead eventually to a bedridden state; hence the term paretic. Other symptoms are hemiplegia (hence the term “paresis”), hemianopia, aphasia, cranial-nerve palsies, and seizures with prominent focal signs of unilateral frontal or temporal lobe disease—a syndrome known pathologically as Lissauer cerebral sclerosis.
Agitated, delirious, depressive, and schizoid psychoses are special psychiatric syndromes that can be differentiated from general paresis by the lack of mental decline, neurologic signs, and CSF findings in the primary psychiatric disorders. The neuropsychiatric features of this disease create a picture unlike that of most degenerative diseases—with the notable exception that it may simulate the category of degenerative frontotemporal dementias as mentioned and discussed in Chap. 38. It is well to remember that many of our ideas about the brain and the mind were shaped historically by this disease.
Pathologic changes This consists of meningeal thickening, brain atrophy, ventricular enlargement, and granular ependymitis. Microscopically, the perivascular spaces are filled with lymphocytes, plasma cells, and mononuclear cells; nerve cells have disappeared; there are numerous rod-shaped microgliacytes and plump astrocytes in parts of the cortex devastated by neuronal loss; iron is deposited in mononuclear cells; and, with special stains, spirochetes are visible in the cortex. The changes are most pronounced in the frontal and temporal lobes. The ependymal surfaces of the ventricles are studded with granular elevations protruding between ependymal cells (granular ependymitis). Meningeal fibrosis with obstructive hydrocephalus is present in many cases.
Treatment The prognosis in early treated cases with antibiotics has in the past been fairly good; 35 to 40 percent of patients made some occupational readjustment; in another 40 to 50 percent, the disease was arrested but left the patient dependent. Without treatment as discussed below there is progressive mental decline, and death occurs within 3 to 4 years.