Keywords bladder pain syndrome - interstitial cystitis - pudendal nerve - compression - Georg
Zülzer
Introduction
Peripheral nerve compression, above all of the pudendal nerve, is still frequently
overlooked in the differential diagnosis of pelvic pain even, in recent reviews.[1 ]
Objectives
The objective of this article is, on the occasion of the centenary of an article entitled
“Irritation of the Pudendal Nerve (Neuralgia). A Frequent Clinical Picture during
War Feigning Bladder Catarrh,” to make this very early report on pudendal nerve compression
accessible for English-speaking physicians and therapists caring for individuals affected
by pain related to the pudendal nerve and to discuss its author Georg Zülzer (1870–1949).
Methods
An English translation of the original German report is provided together with a summary
on the biography and work of its author. His clinical observations are discussed regarding
modern diagnosis and therapy of pudendal nerve compression.
Results
Translation of the Original Report
In 1915, Zülzer, then head physician of a military hospital during World War (WW)
I, published the following observations in a German medical journal[2 ]:
“A clinical picture, which I have experienced only twice in practice during peace,
but which is occurring frequently (also confirmed to me from other side) during war
and whose diagnosis is not always made correctly, is the irritation of the pudendal
nerve. The patients report, in a consistent manner, an extraordinarily frequent urge
to urinate, combined with a painful pressure in the bladder area and pain during urinating.
The urine is without exception clear, sour, free from protein, without sediment. The
singularly voided portions of urine are small. They vary between about 20 and 100 ccm.
That only single drops are voided, as in acute bladder catarrh, does not occur. The
mentioned clinical symptoms distinguished the addressed ailment from a bladder catarrh.
If one tries to define the area of pain with the needle, as I had recommended in my
paper on spinalgia for all diseases accompanied with pain, it was possible in all
cases observed by me, about a number of 10 to 12, to document that a skin area which
delimits the perineum in a rhomboid shape, shows an extraordinary hypersensibility.
The front lace of the rhomboid is located 2 to 3 finger widths above the symphysis.
The rear lace is located a little bit below the sacrum, approximately in the middle
of the nates (clunium), about at the level of the after. The lateral laces are located
lateral to the perineum, about the width of a hand, at the inner side of the thigh.
This skin area corresponds to the skin area of the pudendal nerve with his two branches
deriving from the pudendal plexus. The subjectively experienced, paroxysmally occurring
(and always imitating urgency to urinate) complaints and the proof, that the skin
hypersensibility is belonging to a defined nerve leave no doubt about the diagnosis,
irritation of the pudendal nerve. If this irritation can be labelled as neuralgic,
as I have done in the mentioned communication about the intercostal and other nerves
appears of minor importance. The therapy of the ailment is, however, that of neuralgia,
antineuralgica, as pyramidon, aspirin or alike, and local heat (hot air apparatus).
Under this therapy, I could heal the ailment in a few cases in 6 to 10 days; not only
the urge to urinate disappeared, but also the needle hypersensibility.
A short medical history shall be presented as a paradigma; Joseph J., 30 years old.
Formerly always been healthy, he was suffering since 14 days from urge to urinate
and pain during urinating, pressure in the bladder. He has to leave urine 40 to 50
times per 24 hours. During the last 24 hours, he has lost urine spontaneously. He
has no explanation for the cause of the disease. Status: strong man, inner organs
without peculiarities, urine clear, sour, free of protein and sugar, without sediment.
The bladder does not seem to be filled by percussion. the needle examination reveals
the above sketched rhomboid. Treatment: local hot air treatment, pyramidon thrice
daily. After 5 days, significant improvement observed. During the last 24 hours only
approximately 10 times of urinating. After 10 days, no hypersensibility anymore in
the area of the pudendal nerve. Without complaints released to the troupe as healed.
The pudendal nerve irritation, which is very rare, as I have mentioned in my former
observation, can be regarded as disease of the trench. Its diagnosis is possible in
the field without problems, even under the simplest conditions, if you only take into
account the macroscopically clear urine on the one side and on the other side if you
document the bladder sensibility through the characteristically limited hypersensibility
of the skin area of the pudendal nerve by simple needle examination. Recently, I also
saw this neuralgia occurring in a case of typhus by the way.”
Short Biography and Work of Georg Zülzer (1870–1949)
Georg Ludwig Zülzer ([Fig. 1 ]) was a German physician practicing in Berlin who emigrated to New York City in 1934,
because he was discriminated as a Jew. He pioneered diabetes mellitus research treating
diabetic dogs with extracts of calf pancreas and, in 1906, treating a patient dying
from diabetic coma with an extract called “Acomatrol.” The patient first showed improvement,
but later suffered from side effects, and died when the supply of Zülzer's pancreatic
extract was exhausted. Zülzer continued to seek a suitable remedy for diabetes mellitus,
but his laboratory was closed by the German military during WW I. A breakthrough occurred
in the early 1920s when Canadian physicians Frederick Banting (1891–1941) and Charles
Best (1899–1978) developed an extract that saved the life of a 14-year-old diabetic
patient. They received the Nobel prize in 1923, although they acknowledged that their
initial clinical trials were as “not so encouraging as those obtained by Zuelzer in
1908.”[3 ]
[4 ]
Fig. 1 Georg Ludwig Zülzer (1870–1949).
Discussion
As summarized in a recent review, interstitial cystitis or bladder pain syndrome (IC/BPS)
is a chronic, severely painful, and disabling disease of unknown origin and probably
the most misdiagnosed urologic condition affecting millions of patients.[1 ]
[5 ] Treatment is often frustrating and pain may be unresponsive even to removal of pelvic
organs.[6 ] Classic symptoms include perineal pain, urinary urgency, and frequency despite sterile
urine cultures, exactly the same as described by Zülzer in his soldier patients during
WW I in whom he diagnosed pudendal nerve compression due to a constant cutaneous hypersensitivity
in its anatomical area of innervation. Interestingly, research pioneered by Antolak
and others newly supports a link between interstitial cystitis and pudendal nerve
compression with overlapping symptoms including pelvic pain, typically in the perineal,
rectal, and genital area, sexual and voiding dysfunction, difficulty with defecation
and a feeling that a foreign object may be in the body.[7 ] Newly described as “cyclist's syndrome,” “pudendal canal syndrome,” or “Alcock's
syndrome,” pudendal nerve compression can be triggered by chronic or acute pressure
to the sitting area, repeated vaginal infections and chronic constipation, as well
as secondary trauma due to childbirth, surgery, and biomechanical abnormalities (e.g.,
sacroiliac joint dysfunction or pelvic floor dysfunction).[8 ]
[9 ] As in IC, diagnosis is made by ruling out other causes, for example, from urology
or gynecology and identification of sensory abnormality of the pudendal nerve, in
a rhomboid, from above the symphysis pubis to the middle of sacrum and to the lateral
borders of the perineum as already defined by Zülzer in 1915. Testing may be done
by pinprick, neurophysiologic, or neurosensory testing with the Pressure-Specified
Sensory Device as first applied by Hruby et al who diagnosed compression of the dorsal
branch of the pudendal nerve in men in 2009.[10 ] X-ray-guided perineural injections may relieve or eliminate symptoms,[11 ]
[12 ] carefully selected patients can benefit from surgical decompression of the pudendal
nerve, and its branches or neurectomy, if the nerve has been directly injured and
a neuroma is present with high success rates.[10 ]
[13 ]
[14 ]
[15 ]
[16 ]
[17 ]
Although Zülzer used the term “pudendal neuralgia,” in this interpretation of his
writings, the preferred peripheral nerve terminology of either pudendal nerve “compression”
or “neuroma” of a particular branch of the pudendal nerve is used to clarify pathophysiology
and surgical treatment options.[18 ]
For conjecture, one can ask why trench warfare might predispose to chronic pudendal
nerve compression. A hypothesis is that the pudendal nerve crosses from posterior
to anterior within the fascia of the obturator internus, making it susceptible to
increased intrapelvic pressure. Increased intrapelvic pressure would result in decreased
pudendal nerve blood flow, leading acutely to ischemic symptoms, and, in the long
term, chronic scarring along the course of the pudendal nerve. Increased standing,
straining to have a bowel movement, changes in pelvic pressure related to bladder
pain, and perhaps sitting for prolonged periods with wet clothing about the ischial
tuberosity may each contribute to some degree to increase the intrapelvic pressure
upon the pudendal nerve. This hypothesis lends itself to clinical investigation by
measuring and the correlating either intravaginal or intrarectal pressures as a proxy
for intrapelvic pressure and correlating that with symptoms related to pudendal nerve
compression.[16 ]
[17 ]
As a take-home message taken from the inspiring historical description by Georg Zülzer
dating from exactly 100 years ago, pudendal nerve compression should always be taken
into account when examining and treating patients with symptoms of IC/BPS.
