Marfan syndrome

Any remarks or questions on the life of Sergei Vasilyevich Rachmaninoff can be posted here.
Lea
Posts: 4
Joined: Mon Feb 01, 2010 2:46 am

Marfan syndrome

Postby Lea » Mon Feb 08, 2010 1:43 am

I was actually kind of surprised that no one had mentioned this yet! Have you all heard of all the speculation that SVR had Marfan syndrome? I don't think there's enough evidence to prove it... what do you think?

Lea
Posts: 4
Joined: Mon Feb 01, 2010 2:46 am

Re: Marfan syndrome

Postby Lea » Mon Jun 28, 2010 12:31 am

Zane, that is very interesting and I think you are right. I just don't understand why people insist on trying to diagnose him with such things (this isn't even the only one!), especially with such insufficient evidence to support it.

But I'm so glad to see that some people are making sense in this world :D

StewH
Posts: 94
Joined: Thu Sep 25, 2008 9:42 pm

Re: Marfan syndrome

Postby StewH » Fri Jul 23, 2010 11:57 pm

Zane,

That's certainly as thorough an analysis as one could hope for these many years later. This should put this particular rumor to rest, one would hope!
StewH

Big Mac
Posts: 2
Joined: Mon May 01, 2006 7:53 pm
Location: New York

Re: Marfan syndrome

Postby Big Mac » Mon Oct 21, 2013 9:30 pm

I have known three genuine Marfan syndrome cases. All were tall, but not unusually so, but all three had incredibly thin arm and leg bones, and spidery fingers. The contrast between these people and Rachmaninoff's big thick fingers and powerful wrists could not be greater. Acromegaly is highly doubtful also: there is no trace that I can see of the characteristic overgrowth of facial bones.

Incidentally, there has never been to my knowledge an explanation of why silicon in trace amounts has a role in metabolism. Normal bones do contain tiny amounts of silica. Whether this is just accidental or necessary, I do not know. It's pure speculation that some enzyme involving silicon may be inoperative in Marfan's.

Zane
Posts: 10
Joined: Wed Jul 15, 2009 6:46 pm

Re: Marfan syndrome

Postby Zane » Wed Sep 07, 2016 11:07 pm

Interesting idea, Lea, and this is the first speculation I've heard that Rachmaninoff could have had Marfan's syndrome. There are many Internet sources on Marfan's, and I think Wikipedia's is good enough for our review purposes: http://en.wikipedia.org/wiki/Marfan_syndrome.

HERE ARE A FEW SALIENT SUMMARY POINTS FROM WIKIPEDIA:

1. [Marfan] "is carried by a gene called FBN1...Because it is dominant, people who have inherited one affected FBN1 gene from either parent will have Marfan's."

2. "Most of the readily visible signs are associated with the skeletal system. Many individuals with Marfan syndrome grow to above average height. Some have long slender limbs with long fingers and toes (arachnodactyly). This condition of elongated limbs is known as dolichostenomelia. An individual's arms may be disproportionately long, with thin, weak wrists."

3. "Marfan syndrome can also seriously affect the eyes and vision. Nearsightedness and astigmatism are common, but farsightedness can also result. Subluxation (dislocation) of the crystalline lens in one or both eyes (ectopia lentis) (in 80% of patients) also occurs..."


RACHMANINOFF'S BODY VERSUS THE ABOVE SYMPTOMS

1. With Marfan's being passed on genetically and dominantly from either parent, the first ones to investigate are Lyoubov and Vasili Rachmaninoff.

(a) Neither of SVR's parents was extremely tall, and available photos show neither wearing spectacles.

(b) The 10 November 1918 Ellis Island, NY records of SVR and family's immigration list SVR's children's height: "Irene" (5'-8" at age 15), and Tatiana (4'-10" at age 11). And the 30 October 1924 Ellis immigration records show Tatiana's height at 5'-4" at age 17 (“Irene” or Irina is not listed on that arrival). Marfan's dominant-gene genetics, from a single parent, suggests both of SVR's children would be very tall.

2. (a) SVR's immigration files (from repeated entries into the United States at Ellis Island) list his height as 6'-1", although Eugene Ormandy recalled SVR's height was "about six feet, three" [in a conversation with Ed Cunningham, in Philadelphia; radio station KUSC, of the USA's National Public Radio (NPR), broadcast excerpts of this conversation in 1979].

(b) On the same 10 November 1918 Ellis Island NY ship manifests as SVR's, another man's height was shown as 6-6 1/2"; so there appears no reason to suspect extreme understatements of a person's height by Ellis Island immigration.

(c) Photographs of SVR's hands do not show "long (slender) fingers” and "thin, weak wrists." Instead, SVR had BIG hands with husky fingers and huge strong wrists. As Adolph Altman (who photographed SVR for ASCAP in 1938) told me on 6 August 1987, “[Rachmaninoff's] fingers looked almost like those of a butcher...not beautiful [and slender] like those of Frank Black."

3. Photographs of SVR show him wearing glasses only while reading later in life, and never otherwise or while giving concerts. Reading glasses suggest typical farsightedness upon advancing age, rather than Marfan (where Rachmaninoff most likely would have been nearsighted, and less likely farsighted, at an early age).

(a) Photo of SVR as a young man proofing a score (no reading glasses):
Image


(b) SVR in later years at his desk. Note partial view of glasses on the left:
Image

c) Never did Rachmaninoff experience: "Subluxation (dislocation) of the crystalline lens in one or both eyes (ectopia lentis) (in 80% of patients) also occurs..."

The Marfan speculation was proposed by Dr. D. A. B. Young (formerly principal scientist of the Wellcome Foundation) in a 1986 British Medical Journal article. Twenty years later, an article in the Journal of the Royal Society of Medicine, by Ramachandran and Aronson differed greatly from Young’s speculation:
The size of [Rachmaninov’s] hands may have been a manifestation of Marfan's syndrome, their size and slenderness typical of arachnodactyly. However, Rachmaninov did not clearly exhibit any of the other clinical characteristics typical of Marfan's, such as scoliosis, pectus excavatum, and eye or cardiac complications. Nor did he express any of the clinical effects of a Marfan-related syndrome, such as Beal's syndrome (congenital contractural arachnodactyly), Ehlers-Danlos syndrome, homocystinuria, Stickler syndrome, or Sphrintzen-Goldberg syndrome. There is no indication that his immediate family had similar hand spans, ruling out familial arachnodactyly. Rachmaninov did not display any signs of digital clubbing or any obvious hypertrophic skin changes associated with pachydermoperiostitis.Acromegaly is an alternative diagnosis. From photographs of Rachmaninov in the 1920s and his portrait by Konstantin Somov in 1925 (Figure 1), at a time when he was recording his four piano concerti, the coarse facial features of acromegaly are not immediately apparent. However, a case can be made from later photographs...During a heavy concert schedule in Russia in 1912, he interrupted his schedule because of stiffness in his hands. This may have been due to overuse, although carpal tunnel syndrome or simply swelling and puffiness of the hands associated with acromegaly may have been the cause. In 1942, Rachmaninov made a final revision of his troublesome Fourth Concerto but composed no more new music. A rapidly progressing melanoma forced him to break off his 1942–1943 concert tour after a recital in Knoxville, Tennessee. A little over five weeks later he died in the house he had bought the year before on Elm Drive in Beverly Hills. Melanoma is associated with acromegaly and may have been a final clue to Rachmaninov's diagnosis.

But then again, perhaps he just had big hands.

Ramachandran, Manoj; Aronson, Jeffrey K. (2006). "The diagnosis of art: Rachmaninov's hand span". Journal of the Royal Society of Medicine. 99 (10): 529–530. doi:10.1258/jrsm.99.10.529. PMC 1592053free to read. PMID 17066567

So evidence does not appear to support any Marfan syndrome or physical deformity or anomaly.

Zane


Return to “The Life of Sergei Vasilyevich Rachmaninoff”

Who is online

Users browsing this forum: No registered users and 3 guests