General paresis of the insane
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|Other names||General paralysis of the insane, paralytic dementia|
General paresis, also known as general paralysis of the insane (GPI) 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 approximately 7% of infected individuals. It is more common among men.
GPI was originally considered to be a type of madness due to a dissolute character, when first identified in the early 19th century. Then 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 curable and avoidable. Prior to these events, paresis was inevitably fatal unless another terminating illness intervened, and it accounted for as much as 25% of the primary diagnoses 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 occur. 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, 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-guilt, self-blame, or bizarre hypochondriacal complaints. Later, the patient experiences 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.
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.
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 experienced 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 would die, the process taking about three to five years on average.
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 20 and 40 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 12% of admissions and more than 2% of 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 19th 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.
Theo Van Gogh, brother of painter Vincent Van Gogh, died six months after Vincent in 1891 from "dementia parylitica" or what is now called syphilitic paresis.
The Chicago gangster Al Capone died of syphilitic paresis, having contracted syphilis in a brothel prior to Prohibition and the Volstead Act and not having been treated for it in time to prevent the development of syphilitic paresis in himself.
- PubMed.gov; Ned Tjdschr 2009; 153:B362