General paralysis of the insane* and psychiatry’s only Nobel Prize
General paralysis of the insane, a syndrome of mental disorder and weakness occurring in tertiary syphilis, is also known as dementia paralytica, Bayle disease, parenchymal syphilis and symptomatic neurosyphilis. It is a rare disease in western general adult psychiatry these days but, at the height of its powers, it is thought that it accounted for up to 20% of patients in asylums.
In brief: syphilis is a chronic sexually transmitted disease, caused by the bacterium Treponema pallidum, resulting in the formation of lesions throughout the body. As well as being sexually transmitted the infection can also be transmitted from mother to the developing fetus (congenital syphilis).
The primary symptom is a hard ulcer (chancre) at the site of infection (i.e the penis or vagina) and this forms 2-4 weeks following exposure. Secondary symptoms develop about two months after this infection and include fever, malaise general enlargement of lymph nodes and a faint red rash on the chest. Then, after a period of time somewhere between some months to many years the disease enters its tertiary phase with widespread formation of tumour like masses (gummas). These can cause serious damage to the heart or blood vessels (cardiovascular syphilis) or to the brain (neurosyphilis) resulting in tabes dorsalis, blindness and general paralysis of the insane.
The onset of GPI is usually gradual with depression as the dominant symptom. There is then a slowly progressive memory and intellectual impairment. Frontal lobes are particularly involved, resulting in characteristic personality change with disinhibition, uncontrolled excitement and over activity which may be mistaken for hypomania. Grandiose delusions are present in 10%. Physically there is slurred speech, a tremor of the lips and tongue, and Argyll Robinson pupil** in 50%. As the condition progresses there is increased leg weakness leading to spastic paralysis. Patients become completely incapacitated, bedridden, and die, the process taking about three to five years on average.
The first clearly identified examples of paresis among the insane were described in Paris after the Napoleonic wars and general paresis of the insane was first described as a distinct disease in 1822 by Antoine Laurent Jesse Bayle. Originally, the cause was (charitably) 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 Alfred Fournier. In 1913 all doubt about the syphilitic nature of paresis was finally eliminated when Noguchi and Moore demonstrated the syphillitic spirochaetes in the brains of paretics.
In 1927 Julius Wagner-Jauregg was given the first and only Nobel Prize awarded to a psychiatrist. This was for work done in 1917 by which time he had been selflessly studying the the relationship between fever and psychosis 30 years. Wagner-Jauregg had exposed three neurosyphilitic patients to malaria drawn from the blood of a wounded soldier. The resulting high fever killed the syphilis spirochetes, leading to their recovery (the fate of the soldier is not recorded). Given that there were few cures for anything in 1917, Wagner-Jauregg’s achievement was a milestone in psychiatric and medical science. There was now a reliable, albeit risky, cure for neurosyphilis.
After WWII the use of penicillin to treat syphilis has made general paresis a rarity and now even patients manifesting early symptoms of actual general paresis are capable of full recovery with a course of penicillin. The disorder is now virtually unknown outside third world countries, although it has been reported in western HIV sufferers. I was recently told by someone who should know about these things, that it is no longer routine to test for syphilis in sufferers of dementia.
If you can get hold of it Hare (1959) has written an interesting historical analysis.
Trivia:
Murderous Ugandan dictator Idi Amin is thought to have died from neurosyphilis
Also check out:
Tuskegee Syphilis Study - a clinical study, conducted between 1932 and 1972 in Tuskegee Alabama in which 399 poor and mostly illiterate African Americans were studied to observe the natural progression of the syphilis if left untreated. Wikipedia page / List Universe: Top ten evil human experiments #3
* I’ve always thought this is an extremely evocative name for a disease (although I grant the use of the word ‘insane’ is archaic and potentially un-PC)
** non medics - these are known, rather tastelessly, as prostitute’s pupils as they accommodate, but don’t react (to light)…
If you enjoyed this post you can buy me a coffee!
June 6th, 2008 at 10:07 pm
Love the ‘prostitute’s pupils’… definately a little nugget of knowledge to be stored :>
Would it be fair to say that the symptoms of syphilis could be similar to those experienced by some people with brain tumours caused by, say, cancer? On reading your comments, it sounded like the physical problem within the brain - i.e. a mass that shouldn’t be there - is presumably the same in both cases. But I don’t think I’ve ever heard about people with brain tumors becoming insane. Or is that because it would be ‘un-PC’ to talk about it?
June 8th, 2008 at 6:57 am
It is interesting how many people from history are now being retrospectively diagnosed with neurosyphilis, like Hitler.
June 10th, 2008 at 3:25 pm
You are a charlatan. Give up psychiatry and do something ethical.
You are a pseudoscientist and a fascist. Shame on you.
June 10th, 2008 at 5:13 pm
northernirelandexile:
Different parts of the brain do different things, therefore the effect of a brain tumour will be related to the part of the brain that is affected. A tumour of the frontal lobes can cause psychiatric symptoms, with tumours of other parts of the brain this is less likely. Syphilis used to be known as ‘The Great Mimicker’ for its wide variety of clinic manifestations, so without modern scanning techniques (and a syphilis test) it would be difficult to tell GPI and a brain tumour apart.
Sceptic:
I am sympathetic to people who dislike psychiatrists, but there is no need to be rude. Please explain your objections more fully.
June 10th, 2008 at 7:35 pm
Well I don’t see anything “pseudoscientific” about a thoughtful piece on the history of organic psychiatry in GPI - one of the few conditions that has a solid aetiology.
I suspect “Sceptic”’s agitation stems from wasting too much money on “courses”.
Have you thought about doing a piece on cults FP?
June 12th, 2008 at 4:35 pm
deClerambault…
I think that you may be referring to the rabid bunch who accosted a friend and I on Tottenham Court Rd on Saturday, trying to have us attend a free screening of their own take on ’science.’ From their pushy behaviour, I doubt that they have ever actually taken the time to consider others, so in my view - shame on them!!!
FP - thanks for the explanation. (and for what it’s worth, I don’t think your posts are fascist, I think they are kind of fun. I only happened across this page when I was looking for the song by the Avalanches, but I enjoy reading it!)
September 6th, 2008 at 11:23 pm
Actually, I rarely test for neurosyphilis. If one suspects it, do not use the screening test for primary and secondary syphilis—proceed directly to the MHA-TP, as the VDRL will be false 50% of the time in tertiary neurosyphilis.
September 6th, 2008 at 11:26 pm
In repsone to one of the posts, yes, people with brain tumors can have psychotic features, psychosis being defined as a breach of reality testing—voices, visions, paranoid delusions, etc. It is, therefore, not always dramatic.
In my experience, it does not always come at you in the media’s portrayal of psychosis, just as my work as a psychiatrist has nothing to do with the leather creaking, grandfather clock ticking portrayal of movies.
September 6th, 2008 at 11:28 pm
You forgot the work on neurotransmission in giant squids—psychiatry’s second Nobel Prize.
September 8th, 2008 at 10:15 am
I think that you could argue that Moniz’s Nobel Prize in 1949 for his work on lobotomy was a Nobel Prize for psychiatry but I don’t think that neurotransmission in giant squids has had any effect on understanding of psychiatric disease…..