ROMBERG'S SIGN IN THE 19TH CENTURY:

Supplementary material

for posting on

NEUROLOGY WEB SITE

DOUGLAS J. LANSKA, M.D., M.S, M.S.P.H.

CHRISTOPHER G. GOETZ, M.D.

From the Veterans Affairs Medical Center, Great Lakes Health Care System,

Tomah, WI (DJL); the Department of Neurology, University of Wisconsin,

Madison, WI (DJL); and the Department of Neurological Sciences, Rush

University, Chicago, IL (CGG).

Correspondence to Douglas J. Lanska, M.D., Chief of Staff (11), Veterans

Affairs Medical Center, Tomah, WI 54660

Phone (608)372-1778 FAX (608)372-1654

Email Douglas.Lanska@med.va.gov

Running title: Romberg sign

Key words: history of medicine ? Romberg sign; neurological examination

history; 19th century; neurology; neurosyphilis; tabes dorsalis

Duchenne de Boulogne: In one of several case histories, French

neurologist Duchenne de Boulogne described the importance of vision in

maintaining equilibrium in patients with tabes dorsalis:

"When the eyes are closed ... she cannot stand upright or walk, and she drops objects out of

her hands. On the other hand, with the aid of eyesight, she accomplishes these tasks

relatively well without any uncoordinated movement [pg. 177] [1]."

Duchenne also recognized that vision could be compromised and

even lost with progression of tabes dorsalis, and that when vision declined,

the patient's ataxia worsened.

"In a relatively short period of time, the patient will probably lose her muscular sensibility in

the lower extremities with the result that, already deprived of her vision, she will be

condemned to being completely confined well before the actual coordination difficulty would

have reduced her to this point if instead she retained her eyesight [pg. 169] [1]."

Just as the Romberg sign demonstrates that the patient falls with

the eyes shut, the existence of the patient with combined loss of

proprioception and blindness precluded safe standing:

"All too often, however, amaurosis is bilateral. Nothing is more pathetic than the life of these

unfortunates. Deprived already of the ability to coordinate their lower limbs due to difficulty in

feeling the ground, and no longer aided by their vision, which as we know has a large impact

on locomotion, these patients? ability to ambulate and stand becomes more difficult and

extremely problematic in the presence of their uncoordinated movements [pg. 60] [1]."

Charcot: Jean-Martin Charcot's informal classroom presentations were

transcribed by his students and published as the Leçons du Mardi (Tuesday

Lessons) [2,3]. His international audience and wide reputation made his

classroom a ready vehicle for dissemination of neurological information and

solidification of neurological terminology. These texts demonstrate Charcot's

method of neurological examination and the manner in which he used the

Romberg sign in differential diagnosis.

In presenting a patient with Thomsen?s disease and

superimposed neuropathy, Charcot demonstrated first hand his neurological

technique for eliciting the Romberg sign:

"I will now show one of these new phenomena that I just discussed. [turning and addressing

the seated patient] Please stand up my friend. Now, there is the muscle spasm that occurs in

Thomsen?s disease ? Let?s wait for that to pass. Good, now, the muscles are again relaxed.

[addressing the patient who is standing] Now, close your eyes. [The patient wavers and

nearly falls as soon as he closes the eyes] You see, that is what is called the Romberg Sign and

it is quite pronounced." [July 10, 1888, p. 538] [2].

Charcot considered the Romberg sign as typical of tabes dorsalis,

but found it present also in Friedreich's disease, alcoholic neuropathy, and

sometimes hysteria. He recognized the underpinnings of decreased sensory

function for Romberg's sign, and, in a time when the distinctions between

spinal cord, nerve, and muscle diseases were still being delineated, he

recognized that this sign was useful for excluding conditions without sensory

abnormalities (e.g., primary muscle disease). In tabes dorsalis, Charcot

linked the Romberg sign directly to the development of ataxia, gait

dysfunction, and nighttime falling:

Patient: "I cannot walk at all in the dark now. If I do not hold on to something, I waver

for a moment, then fall to the right or left."

Charcot: "That is the typical picture: Several day periods of pain followed by pain-free

intervals, all lasting about ten years, then, motor incoordination appears one

day when the patient enters a dark place. That is what we call the Romberg

sign and it often marks the beginning point of the ataxic period of the disease

[March 20, 1888, p. 268] [2]."

In association with the Romberg sign, Charcot assessed gait and

used other neurological signs to arrive at a proper clinical diagnosis.

Presenting two patients with Romberg signs, one with alcoholic neuropathy

and one with tabes dorsalis, he asked both to walk in front of his classroom

audience:

"So here we have a patient [with alcoholic peripheral neuropathy and a "stepper" gait], and

next to him I have placed an ataxic who came this morning for an outpatient consultation ...

The foot [of the "stepper"] is floppy; it is impossible for the patient to lift it, with both the

flexors and the extensors affected to approximately the same extent. In order to walk, the

patient needs to over-flex at the knees and the thighs move upwards more so than is normal.

When the foot hits the floor, the toes hit before the heel, so that you can hear quite distinctly

these two successive sounds ... Now, look at how the gait of our ataxic patient contrasts with

that of the stepper ... The ataxic walks by throwing his legs forward in extension, barely or only

mildly flexing at the knee. The foot hits the ground with the heel, making a single sound. Our

second patient is a true tabetic with ataxia, whereas the first one I showed you, the alcoholic,

is a pseudo-tabetic, with only some tabetic features. And, you know that the lesion in the

alcoholic is not spinal, but rather appears to be within the peripheral nerves. True tabes, on

the other hand, always involves the central nervous system and affects the posterior columns

even at the beginning [March 27, 1888, pg. 288]. [2]."

The Lecons du mardi, though extensive and varied, never reveal

a full neurological examination, so modern readers cannot know the method

Charcot used in screening neurological patients. Rather, individual signs

were demonstrated in selected patients with expected abnormalities , and

not tested in others. Charcot rarely touched his patients, and in most of his

teaching sessions, interns or assistants were called on to elicit signs.

Without photographs or drawings, it remains conjectural in most instances

how exactly these signs were elicited. In the case of the Romberg sign, that

required no tools, but only verbal instructions on the part of Charcot, these

lessons provide first hand documentation of Charcot's method, his ready and

frequent reliance on the Romberg sign, and his interpretation of its

significance in a number of neurological disorders.

Hammond: American neurologist William Alexander Hammond presented

his observations on the Romberg phenomenon (without attribution) in 1871

in his A treatise on diseases of the nervous system, the first American

neurologic text [4]"

"Very often the first evidence of any motor difficulty is experienced in regard to [the] faculty of

standing. This difficulty is, however, not one of paralysis, for, if the patient looks at his feet, he

has no more trouble in standing alone than a perfectly sound man. A gentleman connected

with the city government of Brooklyn consulted me a short time since for an affection which

was very evidently posterior spinal sclerosis [i.e., tabes dorsalis]. The first indication of

disease, as he informed me, was that it had been his habit, while at his morning ablutions, to

shut his eyes, and he had noticed, about two months previously, that when he did so he could

not maintain his equilibrium. When he visited me he was unable to stand with his eyes shut...

That there is little paralysis of motion to account for these abnormalities, can readily be shown

by a few inquiries and experiments. Thus it will ordinarily be found that the patient who is

unable to stand with his eyes shut or take a step in the dark, can push strongly with his legs, or

walk a short distance with a good deal of vigor [pgs. 487-489] [4]."

Observations at the Infirmary for Nervous Diseases: In 1887,

Philadelphia neurologist Silas Weir Mitchell (1829-1914) and his assistant

Morris J. Lewis (1852-1928) reported a clinical study of postural sway and

muscle stretch reflexes in patients with tabes dorsalis seen at the

Philadelphia Orthopedic Hospital and Infirmary for Nervous Diseases [5].

They employed a simple sway meter designed by Mitchell [6], consisting of a

set of two rulers graduated in inches and placed perpendicular to each other

upon a stand. Impairment of station progressed with duration of disease and

was associated with both progressive loss of muscle stretch reflexes and loss

of the ability to augment these with reinforcement. Surprisingly, Mitchell and

Lewis made no distinction between performance with eyes open or closed,

despite their indication that these were encompassed by the concept of

"station."

From 1887 to 1890, Guy Hinsdale (1858-1948) -- assistant to

both Mitchell and William Osler (1849-1919) at the Infirmary for Nervous

Diseases [7] -- published two studies of postural sway using Mitchell's sway

meter and more refined graphical methods [8,9]. One graphical method

employed a simple recording apparatus used in Mitchell's clinic

contemporaneously with the sway meter: a piece of cardboard covered with

smoked paper was attached to the patient's head, and the patient was then

placed under an index, which traced curves on the smoked paper as the

subject swayed beneath it [8]. Hinsdale demonstrated markedly increased

postural sway in tabetics compared to normal individuals, but did not

apparently attempt to record a difference in sway with eyes open and closed.

References:

1. Duchenne de Boulogne [GBA]. De l'ataxie locomotrice

progressive: recherches sur une maladie caractérisée

spécialement par des troubles généreaux de la coordination des

mouvements. Arch gén Méd 1859;13:36-62,158-181,417-451.

2. Charcot J-M. Leçons du Mardi: Policlinique 1887-1888. Paris:

Bureaux du Progrès Médical, 1888.

3. Goetz CG. Charcot the clinician: the tuesday lessons. New York:

Raven Press, 1987.

4. Hammond WA. Sclerosis of the posterior columns of the spinal

cord (locomotor ataxia). In: A treatise on diseases of the nervous

system. New York: D. Appleton & Co., 1871:484-516.

5. Mitchell SW, Lewis MJ. The tendon-jerk and muscle-jerk in

disease, and especially in posterior sclerosis. Am J Med Sci

1886;92:363-372.

6. Mitchell SW, Dercum FX. Nervous diseases and their treatment:

general consideration. In: Dercum FX, ed. A text-book on nervous

diseases by American authors. Philadelphia: Lea Brothers & Co.,

1895:2-50.

7. Dr. Guy Hinsdale. In: Tilney F, Jellife SE, eds. Semi-centennial

anniversary volume of the American Neurological Association:

1875-1924. Albany, NY: American Neurological Association,

1924:185-186.

8. Hinsdale G. The station of man, considered physiologically and

clinically. Am J Med Sci 1887;93:478-485.

9. Hinsdale G. Observations on station with reference to

respiration. N Y Med J 1890;51:292-294.