General paresis of the insane

General paresis, also known as general paralysis of the insane or paralytic dementia, is a severe neuropsychiatric disorder, classified as an organic mental disorder and caused by the chronic meningoencephalitis that leads to cerebral atrophy in late-stage syphilis. Degenerative changes are associated primarily with the frontal and temporal lobar cortex. The disease affects around 7 % of infected individuals; it also prevails among men.

GPI was originally considered to be a type of madness due to a dissolute character, when it was first identified in the eighteenth century, until the cause-effect connection with syphilis was discovered in the late 1880s. Subsequently, the discovery of penicillin and its use in the treatment of syphilis rendered paresis both curable and even avoidable. Prior to that, paresis was inevitably fatal unless some other terminating illness intervened, and accounted for as much as 25% of the primary diagnosis for residents in public psychiatric hospitals.

Signs and symptoms

Symptoms of the disease first appear from 10 to 30 years after infection. Incipient GPI is usually manifested by neurasthenic difficulties, such as fatigue, headaches, insomnia, dizziness, etc. As the disease progresses, mental deterioration and personality changes occurs. Typical symptoms include loss of social inhibitions, asocial behavior, gradual impairment of judgment, concentration and short-term memory, euphoria, mania, depression, or apathy. Subtle shivering, minor defects in speech and Argyll Robertson pupil may become noticeable.

Delusions, which are common as the illness progresses, tend to be poorly systematized and absurd. They can be grandiose, melancholic, or paranoid. These delusions include ideas of great wealth, immortality, thousands of lovers, unfathomable power, apocalypsis, nihilism, self-blame, or bizarre hypochondriacal complaints. Later still, the patient suffers from dysarthria, intention tremors, hyperreflexia, myoclonic jerks, confusion, seizures and severe muscular deterioration. Eventually, the paretic dies bedridden, cachectic and completely disoriented, frequently in a state of status epilepticus

Diagnosis

The diagnosis could be differentiated from other known psychoses and dementias by a characteristic abnormality in eye pupil reflexes (Argyll Robertson pupil), and, eventually, the development of muscular reflex abnormalities, seizures, memory impairment (dementia) and other signs of relatively pervasive neurocerebral deterioration. Definitive diagnosis is based on the analysis of cerebrospinal fluid and tests for syphilis.

Prognosis

Although there were recorded cases of remission of the symptoms, especially if they had not passed beyond the stage of psychosis, these individuals almost invariably suffered relapse within a few months to a few years. Otherwise, the patient was seldom able to return home because of the complexity, severity and unmanageability of the evolving symptom picture. Eventually, the patient would become completely incapacitated, bedfast, and die, the process taking about three to five years on average.

History

While retrospective studies have found earlier instances of what may have been the same disorder, the first clearly identified examples of paresis among the insane were described in Paris after the Napoleonic Wars. General paresis of the insane was first described as a distinct disease in 1822 by Antoine Laurent Jesse Bayle. General paresis most often struck people (men far more frequently than women) between twenty and forty years of age. By 1877, for example, the superintendent of an asylum for men in New York reported that in his institution this disorder accounted for more than twelve percent of the admissions and more than two percent of the deaths.

Originally, the cause was believed to be an inherent weakness of character or constitution. While Esmarch and Jessen had asserted as early as 1857 that syphilis caused general paresis, progress toward the general acceptance by the medical community of this idea was only accomplished later by the eminent nineteenth-century syphilographer Alfred Fournier (1832–1914). In 1913 all doubt about the syphilitic nature of paresis was finally eliminated when Hideyo Noguchi and J. W. Moore demonstrated the syphilitic spirochaetes in the brains of paretics.

In 1917 Julius Wagner-Jauregg discovered that infecting paretic patients with malaria could halt the progression of general paresis. He won a Nobel Prize for this discovery in 1927. After World War II the use of penicillin to treat syphilis made general paresis a rarity: even patients manifesting early symptoms of actual general paresis were capable of full recovery with a course of penicillin. The disorder is now virtually unknown outside third-world countries, and even there the epidemiology is substantially reduced.

Popular culture

In her 1915 novel, The Song of the Lark, Willa Cather referred to the insanity of the wife of a main character as having been caused by general paresis but there is no hint that the character had contracted syphilis. Wolf Larsen, in Jack London's "The Sea Wolf", is a paretic. In her 1953 novel, A Pocket Full of Rye, Agatha Christie used paresis as an explanation for the behavior of the victim, though she did not mention the syphilitic connection.

An account of various 20th Century mental institutions, A Mind That Found Itself, cites the acquaintance with a man who suffered from the condition, which at that time followed a predictable course towards death.^[1]

References

^ "A Mind That Found Itself". Another victim, forty-five years of age, was one who had formerly been a successful man of affairs. His was a forceful personality, and the traits of his sane days influenced his conduct when he broke down mentally. He was in the expansive phase of paresis, a phase distinguished by an exaggerated sense of well-being, and by delusions of grandeur which are symptoms of this form as well as of several other forms of mental disease.

[1] "A Mind That Found Itself". Another victim, forty-five years of age, was one who had formerly been a successful man of affairs. His was a forceful personality, and the traits of his sane days influenced his conduct when he broke down mentally. He was in the expansive phase of paresis, a phase distinguished by an exaggerated sense of well-being, and by delusions of grandeur which are symptoms of this form as well as of several other forms of mental disease.

[1]