Instances of MS When the Disease Did Not Have a Name
http://mscare.com/a0106/page_05.htm
International Journal of MS Care
Jock Murray, MD
Dr. Murray is Professor of Medical
Humanities at Dalhousie University and Director of the Dalhousie MS Research
Unit, Halifax, Nova Scotia, Canada. This paper is an abbreviated version
of a Presidential Address to the Consortium of MS Centers’ Annual Meeting
1999 and will appear in an expanded version in a forthcoming book on the
history of MS, to be published by Demos Publications, New York.
Several historical figures experienced
many of the manifestations of what we now call multiple sclerosis (MS).
Descriptions of their symptoms and the medical treatments of the day are
discussed. While modern clinicians may argue whether these people had MS,
presumed MS, or another condition entirely, the case histories of Lidwina
of Schiedam, Margaret Davis of Myddle, William Brown, Sir Augustus D’Esté,
Heinrich Heine, Allan Stephenson, and Margaret Gatty provide an intriguing
glimpse into the history of MS, medical treatment, and the social context
of neurologic illness in previous centuries.
Suggested citation: The Saint, the
King’s Grandson, the Poet, and the Victorian Writer: Instances of MS When
the Disease Did Not Have a Name. Murray, J. Int J MS Care [Serial on-line].
June 2001; 3(2).
"A disease does not exist until it
has a name."1
Clearly, people were experiencing
the biological illness now called multiple sclerosis (MS) centuries before
Jean-Martin Charcot gave it a name—experiencing it as a condition recognizable
in that era. In those times, the people were regarded as having a chronic
illness—palsy or paraplegia.
Multiple sclerosis was "framed" as
a disease in the mid-19th century by physicians and pathologists in France,
Germany, and Austria by defining the clinical and pathological features.
They knew that the disease existed, but they probably considered it to
be a rare neurologic condition.
It is instructive to examine concepts
of disease in general, and diseases of the nervous system in particular,
and observe how people with such illness were treated before the illness
had a name. The stories of their lives, their diseases, and the therapies
they received from physicians are found in medical records, biographies,
diaries, and personal accounts.
The Virgin Lidwina of Schiedam
"Believe me, there is no cure for
this illness, it comes directly from God."
Lidwina was born on April 18, 1380
in Schiedam, Holland. She was the daughter of a laborer, and one of nine
children. She was healthy and active as a child and teenager, but in the
winter of 1395-1396 she developed an acute illness, of which we have few
details, but from which she gradually recovered. On February 2, 1396 she
was feeling well and her friends encouraged her to go skating on the frozen
canal. She fell while skating and was thought to have broken ribs on the
right side. Healing was slow and it was concluded that she had an internal
abscess in the area of the fracture. She had difficulty walking and used
furniture for support. She was described as having violent lancinating
pain in her teeth, which we would now suspect was trigeminal neuralgia,
suggestive of MS when it occurs in a young adult.
Her sympathetic parish priest, Father
John Pot, visited her regularly and suggested she consider her pain as
similar to that of the Lord. She came to believe that she was called to
be a victim for the sins of others. Her suffering became a source of joy
and she took steps to increase her discomfort, sleeping on planks instead
of on her feather bed. Such was her joy in suffering for others that she
said she would reject recovery even if it could be achieved by praying
one Hail Mary.
One eye became blind, the other sensitive
to light. She spent much of the time in bed, able to move only her left
arm. She had an "unbearable neuritis" in her right shoulder. By the time
she was 19, walking was more difficult. She developed paralysis in her
right arm and her sporadic pains increased. There is mention of a split
face and hanging lip, which may refer to facial weakness, but some of the
descriptions suggest a deep cut down the bridge of her nose. Soon she was
unable to walk and had to be carried. She also had some loss of sensation
and eventually developed sores, which may have been pressure sores or decubitus
ulcers.
Her suffering gained public attention.
Many physicians were consulted, including the prominent Andrew of Delft.
Word of her plight reached William VI, Count of Holland, and his wife Margaret
of Burgundy, who sent their own physician, Godfried de la Haye. He is sometimes
called Godfreid Zonderdank, or Sonderdank, but "zonderdank" is a nickname
that means "don’t mention it," which he said repeatedly to the many poor
who thanked him in the streets for curing them without charge.3 Godfried
somberly reported that Lidwina’s illness was incurable because it came
directly from God. Any attempts to cure her would just impoverish the father
and would do no good, as even Hippocrates and Galen could do little in
this situation.
Lidwina’s illness progressed, and
she became increasingly weak. She had difficulty swallowing—first solid
food and later liquids—and experienced more pain. The disease progressed
slowly, with occasional periods of improvement.
Beginning in 1407, she experienced
supernatural gifts, ecstasies, and visions in which she participated in
the Passion of Christ, saw purgatory and heaven, and visited with saints.
During these "extasies," she had improvement in her sight and was more
mobile.
Although a cult was growing around
her when she was alive, not everyone was so impressed. When Father Pot
was replaced by a new priest, the newcomer felt she was a hypocrite, refused
her communion, and asked the parishioners to pray that she might be delivered
from her diabolical hallucinations. Only intervention by the local magistrates
prevented him from being run out of the parish. An ecclesiastical inquiry
declared Lidwina to be of good faith; she was again permitted to receive
communion.
In later years many members of her
family died while she was lingering in her quiet, dimly lit room. She was
going blind in the last years of her life. The room, always dark, emitted
such a glow that some raised the alarm of a fire. It was said that she
had only communion as sustenance for the last 19 years of her life and
did not sleep for the last seven years. Townspeople reported that her putrefying
body gave off a fragrant perfume. These stories are difficult to credit
in their extremes. Such enthusiastic, exaggerated reports and myth-building
by those who revered her saintliness make interpretations of her condition
difficult for the historian. For instance, the official document prepared
by the municipality of Schiedam in 1421 (12 years before she died) declared
that at that time she had not had anything to drink for seven years.
Her condition worsened further on
the Tuesday after Easter, 1433, and she died on April 14, after 37 years
of suffering. As early as 1434, a chapel was built over her grave.4 Through
the efforts of Godfried de la Haye’s son, a hospital was built on the site
of Lidwina’s humble home.3 A cult developed around her memory and led eventually
to her beatification by the church. An ecclesiastical commission declared
her experiences to be valid.5 She was said to be a "prodigy of human suffering
and heroic patience."6
The first document commenting on
Lidwina van Schiedam (1380-1433) was an official document, dated August
4, 1421, in which Jan van Beieren, Count of Holland, acknowledged a letter
from the Schiedam local authorities about Lidwina’s disease; it further
stated that he personally had seen the young woman.2 Details of Lidwina’s
illness came from her biographer, the Franciscan priest Johannes Brugman
(1400-1473), who acquired information from relatives, her priest and confessor,
local clerics, and other "reliable persons." Fifteen years after Lidwina’s
death, another biographer, Thomas à Kempis, wrote Vita Lidewigis
Virginis, which was based on the information published by Brugman.6 The
1497 incunabulum T’Leven van Liedwy die Maghet van Scyedam was written
by a relative of Lidwina, who had lived with her for two years. There is
also a biography of Sainte Lydwine in Hubert Muffel’s 1925 volume Les Saints.3
Although some claim that the plight
of Lidwina is the first known case of MS, I am more convinced that the
evidence suggests elements of marked religiosity, mysticism, histrionic
behavior, and even self-mutilation. Despite the possibility of some underlying
neurological disease, the diagnosis must be left open.
Margaret Davis of Myddle
The delightful Antiquityes and Memoyres
of the Parish of Myddle was written by Richard Gough (1634-1723) over the
years 1700 and 1706.7 The author, an elderly man of the parish of Myddle,
describes the parish and the objects of interest in Myddle. In a unique
approach to local history, he then proceeds to take each pew in the church
and discusses the persons and families who sat there. Pew 7, which adjoined
the south wall of the church, was used by the family Davis, who lived in
the tenement owned by Vicar Gittens. Thomas Davis, a weaver, had a wife
named Margaret, who had a lameness that progressed over 20 years. I think
you will agree it is a story best told in Gough’s own words.
"When hee removed to Myddle, Thomas
Davis, a weaver who now lives att the Wood Lesows by Myddle Wood, came
to bee tenant to it. Of him I have spoken before, but somewhat I must say
of his wife; Margaret the wife of Thomas Davis dyed on the 17th of this
instant, January, 1701. Shee tooke cold in childe-bearing, above twenty
yeares before her death; shee was seized therby with paine and lameness
in her limbs, and made use of severall remedyes for curing therof, butt
all proved ineffectual. At last, as shee was in an Apothecary’s shop buying
ointments and ingredients for fomentations, my uncle, Mr. Richard Baddley,
an able chirurgeon, saw her and asked her how shee gott her lamenesse:
shee sayd by taking cold in child-birth. Then says hee spare this charges
and labour, for all the Doctors and Surgeons in England cannot cure it.
Thou mayest live long, butt thy strength will still decay. After this shee
went to lytle more charges, only when King James II, came his progresse
to Shrewsbury, shee was admitted by the King’s Doctors to goe to His Majesty
for the Touch, which did her noe good. Shee was forced to use crootches
almost 20 yeares agoe, and I thinke it is now 10 yeares since shee grew
so weake that shee was faine to bee carryed in persons’ armes. About two
years-and-an-halfe before her death, shee kept her bedde continually; shee
was bowed soe togeather, that her knees lay cloase to her brest; ther was
nothing but the skin and bones upon her thighs and legges. About a yeare-and-a-halfe
past, her thigh bones broake as shee lay in bedde, and one of them burst
through the skin and stood out about an inch, like a dry hollow sticke,
but there was noe flesh to bleed or corrupt; shee could stir noe part of
her body save her head and one of her hands a lytle. When shee was dead
they did not endeavor to draw her body straite, butt made a wide coffin
and putt her in as shee was. I heard one say that was present att laying
her in her coffin, that as they layd her downe one of her legge bones broke
and gave a cracke, like a rotten sticke; and it is not to bee forgotten
that the Vicar Gittens, seeing that Thomas Davis had a great charge of
children, and his wife lame upon his hands, did give him his house and
garden rent free while hee lived in itt."7
Gough doesn’t relate whether two
pigeons came to roost on the house two weeks before death and then left,
which is a superstition he describes about deaths in the parish, which
he "had knowe them doe three severall times."
The unfortunate Margaret Davis was
a young woman who developed a progressive neurologic disease with onset
related to childbirth. It is not clear if this was in the six months after
childbirth, a time when onset or exacerbation of the disease is a recognized
phenomenon. She had a progressive lameness that was recognized by the doctor
as one that would last a long time (but was incurable) and would progress
to paralysis of her legs and then her arms. Later developments included
contractures and pressure sores, and death after two decades, which certainly
suggests the diagnosis of MS.
William Brown, Hudson’s Bay Trader
A case for MS can be made for William
Brown, a 19th-century Hudson’s Bay trader in the Canadian West. Brown was
born in Ayrshire, Scotland in 1790 and was sent by the Hudson’s Bay Company
to be the company’s trader at their post at York Factory. In 1811, at age
21, he began to experience weakness in his legs and visual problems. He
found it increasingly difficult to carry out some of his duties. When he
felt he was too weak to withstand the rigors of a long survey assignment,
he sent a replacement in his stead. For that action, he was censured by
Governor Simpson. He had an intermittent and progressive disorder with
visual symptoms, weakness, and gait difficulty. His symptoms progressed,
he was relieved of his post, and he returned to Scotland to be cared for
by his family. He died a few years later. It would be possible to develop
a differential diagnosis for the condition in this young man, but certainly
MS would lead the list.
Sir Augustus D’Esté
An early case of possible MS, for
which there is no doubt about the diagnosis, is that of Augustus d’Esté
(1794-1848), the grandson of George III and cousin of Queen Victoria. He
documented his relapsing and remitting neurologic symptoms and progressive
disease in his diary from 1822 to 1848 and in an almanac dated 1847-1848.
His diary is a remarkable saga of medicine and therapy as practiced on
the upper classes in the early 19th century and a moving story of a young
man trying to understand and cope with a relentless disease.
Both the diary and almanac of Augustus
d’Esté were found at the Letherhead School for the Blind during
a 1940 wartime drive for paper in England. How the diaries came to be at
the school is uncertain. Dr. Douglas Firth rescued the diary, which was
partially destroyed by time, pilfering, and rats, and published an outline
of d’Esté’s life in a paper in 19418 and in a monograph in 1948,9
using available account books, letters, and the manuscripts of d’Esté
and his mother, Augusta d’Ameland. The diary is now in the collection of
the Royal College of Physicians in London, which also has a file on d’Esté
compiled by R. R. Hughes of Liverpool.10
Augustus d’Esté’s diary begins
in 1822. On the first page he describes blurring of vision after leaving
the funeral of a beloved relation. His vision deteriorated to the point
that others had to read for him, but it gradually cleared without treatment.
Visual blurring would recur twice over the next few years, and he consulted
Dr. Henry Alexander, surgeon oculist to the Queen. The next year he had
another episode of visual loss similar to the initial onset, and Dr. Spanganberg
sent him to a spa at Driburg, where he "drank steel-water, bathed in it,
and douched my eyes with it. My eyes again recovered."
In 1827 he complained that the heat
of the country was intolerable, and he developed visual loss and double
vision. In the next few years he developed numbness in his legs and difficulty
walking. He consulted Dr. Kent who did not agree with the previous therapies
and recommended that he "eat beef steaks twice a day, drink London porter
and sherry and madeira wines." His legs were to be rubbed twice a day with
brushes and his back with a liniment made of camphorated alcohol, opium,
and Florence oil. Augustus was pleased to write in his diary, "This new
system succeeded completely. Every day I found strength returning." This
would be the hopeful observation of so many patients taking so many different
treatments for MS—a disease that waxes and wanes. He continued to have
his legs rubbed, but discontinued the back rubs, substituting them with
slaps on the back by the open hand of a servant. He later took up horseback
riding, a common therapy in the 17th and 18th century that would be revived
in the late 20th century.
A Milan physician treated the pain
in the area of d’Esté’s kidneys with a counterirritant plaster that
was supposed to produce an eruption of the skin over the area. This was
of no help, so he switched to flannel bandages as hot as he could tolerate.
He took daily shower baths and a wash of zinc sulfate and aqua plantaginis.
He took 20 to 30 drops of valerian twice a day mixed in the herbs and flowers.
On the recommendation of his father, he saw a number of prominent physicians,
including Sir Astley Cooper; Dr. W.C. Mattin, physician to the Westminster
Hospital; Dr. Kent; and Mr. Pettigrew.
He followed their prescriptions and
also took up sea bathing. At the seaside he developed a liaison with a
young woman, but noticed that his "acts of connection" lacked a "wholesome
vigor." He consulted Dr. Courtenay, who passed bougies and a metal catheter
into his penis and gave him some medicines and pills that proved of some
benefit. He later underwent a course of electricity, tepid douches to the
loins and sacrum, and a course of Galvanism, all with disappointing results.
He derived more benefit from a trip to Scotland where he was "much braced
and invigorated by the Highland air." He found horseback riding and walking
to be helpful. He continued the warm baths and douches and the visits to
various spas.
By 1840, 18 years after the onset
of his disease, he noted in his diary that he no longer used any measures
for the improvement of his health or for the restoration of his vigor and
strength, presumably because of disappointment with all of the previous
treatments. At this point he read a book on hydropathy and decided to visit
the celebrated Vincenz Priessnitz, often thought of as the leader of the
hydrotherapy movement, who thought his infirmity originated in the nerves.
He was treated five times over two days with the application of wet sheets
and friction and walked around wearing a wet cincture around his waist.
He next consulted Sir Benjamin Brodie, and a surgeon, Mr. John Scott, who
prescribed tincture of Lyttae or Spanish fly, which seemed to have little
effect. His prescriptions over the next few years included zinc sulfate,
Spanish fly, strychnine, quinine, silver nitrate, and stramonium. Later
he developed vertigo, but other than a little brandy with water, he took
no medicines. Scott consulted with his colleague, John Farre, and they
agreed the diagnosis was "paraplegia," a condition that can be active or
passive, functional or organic, and can be "very imperceptible." They agreed
that d’Esté’s paraplegia was of the passive phase that could continue
for a long time in the functional form, but the transition had not yet
been confirmed. Healing would require improvement in the circulation, but
because the illness is in the patient (and not a result of an external
influence), it can frustrate the best medical therapy. They suggested that
d’Esté control his diet, keep his emotions calm, and his heart quiet.
The therapy would be iron, mercury, and a period of rest at the Brighton
shore.
John Scott recommended that d’Esté
ride a horse every day for as long as he could, and walk as long as he
could. d’Esté underwent another course of electricity, which he
felt was making him worse. In February 1844, he was seen by Sir Richard
Bright, who agreed with increasing the amount of iron in d’Esté’s
medicine. A review of the casebooks of Richard Bright reveals two cases
that might have been MS but neither of these patients was d’Esté.
By this time he was walking poorly—some
days buoyed by how well he was doing, other days anxious and alarmed at
how weak he was. His handwriting became shakier; many of the earlier diary
sections are by an amanuensis. In 1847 he began to write in a small volume
of Simpson’s Gentleman’s Alamanak and Pocket Journal for 1847. He recorded
his daily visits and commented on the weather ("magnificent day out of
heaven!"). On the left of the pages he evaluated church sermons, and on
the right he kept a compulsive recording of the time he walked around his
rooms—times to the fraction of a minute by a chronometer that he took pains
to keep in good repair.
Sept. 27— "I rejoice to write that
I have walked in my rooms 50 min."
"Having received a Present of Indian
Moccassins I put them on—and I walk in them without my Left-Foot, which
some time ago always turned outwards at the Ankle joint unless supported
by a Steele-Upright, showing any disposition so to do—surely this is a
decided Improvement! Thanks to The Almighty!"
There is adequate detail in the diary
of Augustus d’Esté to make a conclusive diagnosis of MS. This was
a young man who developed a recurrent and remitting neurologic disease
characterized by repeated visual loss, diplopia, sensory change, intermittent
and progressive paralysis in his legs, bladder difficulty, and impotence.
He had a remitting progressive course to death in 26 years—a characteristic
picture of MS.
Heinrich Heine, Poet
Although many may not recognize the
works of the early 19th-century German lyrical poet Heinrich Heine (1797-1856),
his poems and songs inspired still familiar music by Schubert, Schumann,
Mendelssohn, Liszt, Berlioz, and Gounod. His poems inspired two of Wagner’s
operas, Flying Dutchman and Tannhäuser; Richard Strauss’ Salome; and
Adams’ ballet Giselle. His poetry is admired in every language, and perhaps
more in France than in his native Germany. Even England, a country he did
not like very much, ranks him second only to Goethe among the great German
poets.11 His reputation has provoked embattled discussion in Germany in
recent times, and Sammons says his reputation was "whipsawed" in the discussion
of the German-Jewish question (ie, Heine being used by both sides for their
own purposes). Interest in Heine is increasing again, and although he died
nearly a century and a half ago, his grave in Montmartre, outside Paris,
is covered with flowers from admirers each year on his birthday.
Heinrich (originally Harry) Heine,
the oldest of four children, was born of Jewish parents in Düsseldorf
on December 13, 1797. Heine’s parents were not able to receive permission
to marry, so he was born illegitimate. Like his mother, he had frequent
migraine headaches. Despite the assistance of a wealthy uncle, his entree
into business as Harry Heine and Co. went bankrupt after one year. He then
changed careers and studied law. Because there was persecution of Jews
in Germany at that time, and he was not a practicing Jew, he accepted baptism
in 1825 and took the name Heinrich. He was politically active, with a manner
that was abrasive and outspoken.12
During his 20s he suffered from severe
migraines and recurrent depressions. He went to spas for relief. The neurologic
illness that interests us began much later, with a transient palsy of two
fingers on his left hand in 1832, when he was 35 years old. Dr. Ferdinand
Koreff (1783-1851) was consulted. Two years later Heine had a bout of depression
and had some visual loss from which he recovered.13 In 1837, his left arm
was weaker. He had sudden bilateral loss of vision, beginning first in
his right eye and then in his left, which nearly blinded him in a few hours.
This improved over the next two weeks, worsened again in three months,
then improved again. He had an episode of double vision six months later,
followed by some worsening of his vision. He then described having giddy
turns, which caused objects in his vision to vacillate, and to impart a
greyish, partly silver color to them. The prominent ophthalmologist, Dr.
J. Sichel, applied leeches, which improved Heine’s vision. Jellinek speculated
that a fundus picture in Sichel’s successful Traité d’Ophthalmie
showing optic atrophy in a 45-year-old patient may have been that of Heine.14
In 1841 Heine’s vision worsened and
then improved, but he became depressed and developed more neurologic symptoms,
including right facial weakness, diplopia, pain in the eye, and numbness
all down the left side of his body. He complained, "Moreover the left eye
is feeble and hurts, does not often agree with the right, and this causes
a confusion of sight which is much more intolerable than the darkness of
full blindness."15 It was said that, despite his youthful promiscuity,
he became impotent soon after marriage that year to his wife, an illegitimate
girl, age 19, whom he called Mathilde.
Much of the information about Heine’s
illness comes from correspondence with his brother Max, a physician. During
the course of his illness, Heine was treated by numerous physicians who
used a number of therapies, including spas, purges, sulfur baths, bloodletting,
morphine, leeches, iodine mixtures, laxatives, diets, enemas, and cutaneous
ointments applied over an incision that was kept open on the nape of his
neck. Although he complied with their advice, he was not very confident
of his physicians or the quacks he consulted. His wife was even less happy
with their recommendations. The prominent physician, Dr. Leopold Wertheim,
was given a black eye and dismissed by Mathilde for his criticism of her
care of Heine. In fact, she chased away all the physicians except the Hungarian
Dr. David Gruby, who had arrived in Paris three years earlier from Vienna,
where he had studied anatomical pathology under Rokitansky and had received
his MD degree. Dr. Gruby was one of the prominent microscopists in Paris
at that time, but gave up research for private practice.
In 1843 Heine developed ptosis of
the left eyelid, hyperesthesia of the left side of his face, and left facial
weakness. In 1844 he had three episodes of deterioration of his vision,
and he was blind for a period of four weeks. The next year he became paraparetic,
which kept him in bed for a period. He tried spa therapy, but his condition
worsened. He described the spa bathtubs as a kind of coffin, and the baths
a period of preparation for death.
In 1845 he developed bilateral ptosis,
worsening gait, and further visual symptoms. In 1846 he developed numbness
around his mouth, some loss of taste, and difficulty with swallowing and
speech. His gait continued to worsen, and he referred to his paralysis
as "like an iron band pressed into the chest"—a description of the band-like
sensory level of a spinal cord lesion. The next year he had right facial
paralysis, cramps, and incontinence. On September 20, 1847, he wrote from
Montmorency to Princess Beliojoso that his feet, legs, and lower torso
were paralyzed, and that he was unable to walk. He then made a moderate
recovery, but in 1848 he described his ultimate physical collapse on his
last walk, looking at the Venus de Milo in the Louvre. He felt she was
looking down on him with wistful sympathy, saying, "Can’t you see I have
no arms and can’t help?"
He felt he was like a fading, drooping
flower, not yet completely withered. He now had paralysis of his right
arm, sensory loss, neuralgic pains, dysarthria, right-sided ptosis, facial
weakness, altered taste, and swallowing difficulties. In addition, he was
losing weight, was constipated, and had respiratory complications. Visitors
noted his muscle loss, deformed feet, and contractures. From then on he
lived in a hospital, in what he called his matratzengruft (mattress grave).
He said that he read every medical treatise he could find that related
to his condition so that when he reached Heaven he could lecture upon the
futility of physicians who tried to deal with spinal disease. In 1849 Dr.
Gruby again took over Heine’s treatment, with some relief of symptoms.
Periodically, a black woman would cauterize his spine to relieve his distress
and pain. Remarkably, he was still writing. He described his state as:
"This living death, this unlife...
my lips are paralyzed like my feet, the tools of eating are also paralyzed
as well as the channels of excretion. I can neither chew nor shit, and
have to be fed like a bird..."
By this time he was using morphine
and opium often and in large doses for his pain and spasms, but tried to
keep his head clear. He called one of his poems "Morphine." Although his
writing and his poems were of a high standard to the end, he was often
under a "desolate narcosis" and in a "doped wilderness." On one occasion
he wrote, "I have at this moment so much opiate in my body that I hardly
know what I am dictating."
A friend asked if he had made his
peace with God. Heine answered, "Don’t bother yourself. God will forgive
me—that’s what He’s there for."15 He wrote that he had two rooms: "the
one I die in, and the grave." He died in 1856 of respiratory complications
at age 59, 24 years after the onset of his illness.
His poetry lives on, and his popularity
and importance are increasing. There were four new biographies of Heine
in 1997, mostly exploring his political influence. Although his lasting
fame is as a poet, he said he cared little about this, and wished to be
buried with a sword and remembered as one who fought for liberty.
Heine’s disabling illness baffled
historical diagnosticians for the next century and a half, and the arguments
have not ended. Putnam16 and others felt he had multiple sclerosis, but
others have suggested he might have had neurosyphilis, amyotrophic lateral
sclerosis (ALS), sarcoidosis, encephalomyelitis, porphyria, spinal muscular
atrophy, chronic polyneuropathy or spinal tuberculosis.17 Heine himself
thought his illness was syphilitic due to his promiscuity in youth, as
did his friends, and Macdonald Critchley15 felt this was as about as definite
as it could be without serologic confirmation. The psychiatrist Nathan
Roth reviewed the differential diagnosis, and provided details and psychoanalytical
musings about Heine’s complex love life, but concluded he had porphyria.
Stenager discussed the many differential diagnoses but leaned towards neurosyphilis,
as did many authors in the 19th and early 20th century. Certainly, Heine’s
expulsion from school for breaking the rule of chastity, and his frequent
couplings with prostitutes in Paris and London added to the suspicion.
Despite all of these arguments, the pattern of his recurrent symptoms over
24 years suggested to Putnam,16 Jellinek,14 and many other recent authors14
that multiple sclerosis should be strongly considered.
Did Heine have MS? The recurrent
nature of the visual loss and the paraplegia, his impotence and incontinence,
the recurrent brain stem symptoms, Lhermitte’s sign, Uhthoff’s sign, heat
sensitivity, and the relentless progression due to a constellation of symptoms
common in MS over 24 years makes it a plausible diagnosis. One would only
hesitate because ptosis is rare in MS; it has, however, been mentioned
in the reports of Charcot, Jellinex, Sparks, Zenker, Grauck, Edwards, and
Hammond. Heine’s clarity of mind to the end is unusual, but, again, not
out of the question in someone who had symptoms that primarily related
to spinal, brain stem, and optic nerve involvement. His life and letters
have been sifted repeatedly in support of one diagnosis or another, and
it is unlikely that other helpful medical details will come to light. Given
the evidence, I would conclude that, in modern parlance, the diagnosis
is probable MS.
"Lighthouse" Stephenson
The Stephensons were a remarkable
Scottish clan who were lighthouse keepers for many generations. They tended
many of the great lighthouses of Scotland and the Hebrides. Allan Stephenson
(1807-1865) was a third generation lighthouse keeper and an uncle of the
author Robert Louis Stephenson. He was supervising 35 lighthouses in Scotland,
many of them difficult to reach, and he complained of marked fatigue. "I
am still grievously afflicted by drowsiness," he complained in 1844, when
he was age 37. Soon after, he noted that his suffering was extreme, and
he described his symptoms as rheumatism. He then recovered but a month
later was complaining that he was again tired and ill. He described his
symptoms in his letters as rheumatism, paraplegia, and lumbago. Bella Bathurst,
biographer of the Lighthouse Stephensons, felt he was suffering from MS.
He was getting worse, so he returned to Edinburgh to seek treatment from
his brother-in-law, Dr. Adam Warden. He reduced his workload and began
to frequent spas. His disease continued to worsen, and he was able to do
less and less. He had to retire in 1853 and submitted letters from his
doctors that indicated that he suffered from an unusually severe form of
paraplegia. He continued to worsen, and felt his disease was due to his
past sins. Allan Stephenson died 21 years after the onset of his relapsing
and progressive illness.
Margaret Gatty, Victorian Writer
"Then you see I wage daily warfare
with myself when I feel sad at my disablement from even the simplest action."
Mrs. Gatty was well known as a writer,
founder of the very popular children’s publication Aunt Judy’s Magazine,
and author of the five-volume Parables from Nature and the beautiful and
authoritative guide British Sea-Weeds (1863).18 Her interest in natural
sciences began in Hastings in 1848 when she took ill and her sympathetic
doctor lent her books that excited her interest in seaweeds. The nature
of that illness is not known, but she experienced her first neurologic
symptoms that affected her general system. At that rather late time in
her life (age 41), she began her writing career.
Because of continuing symptoms over
the years, she consulted a physician in London in 1860 but was declared
"sound organically," as she apparently had no outward manifestations of
disease. She was clearly uncertain about this conclusion and wrote in her
diary, "But still one must believe the Drs. know." In the next breath she
wondered about the possible benefits of homeopathic therapies. Nine months
later she wrote that her hand was "losing its cunning" and had a tremor.
Her illness was at first intermittent, causing her to remain in bed for
days due to attacks of "muscular or nervous rheumatism." She repeatedly
visited the prominent Dr. Thomas King Chambers and was relieved to find
the cause of her problem, as he was quite definite about his diagnosis.
"He calls it ‘atrophic degeneration of the muscular fibers from overuse,’
so my troubles have at any rate got a fine name!"
She began to write with her left
hand and complained of pain in the paralyzed arm and shoulder. The fourth
volume of her Parables from Nature was written with her left hand. She
was heartened that the doctors said that she would ultimately recover from
her condition. Despite the reassurance, she continued to deteriorate and
was referred to a London surgeon, Mr. Paget, who recommended a leather
sling and a splint for her arm. She noted that her left arm was now developing
symptoms and assumed, as many MS patients do, it was due to the overuse
brought about by the weakness of the other hand.
She seemed unaware at the time that
Dr. Chambers had described her condition in a presentation to medical colleagues
and later published her case in Lancet as muscular atrophy due to excessive
physical effort.19 He concluded that the weakness in her arms was due to
overwork, which does not allow renewal of the nerve-forces needed by muscles.
He explained that when this is exhausted, no new store of muscular substance
can be laid in, the muscle degenerates into elastic fiber, and finally
into pale fatty tissue of low vitality.
At this point, Mrs. Gatty developed
a "tic" pain in her face that she thought was due to dental problems and
wished to have her teeth removed. Dr. Chambers suggested, however, that
she would be worse rather than better with their removal. She persisted
and wanted to know if there was not someone who could remove them to relieve
the tic pain using the new chloroform, which she had when she delivered
two of her eight children. The tic pain in her face was probably trigeminal
neuralgia, often thought to be dental when it appears in MS patients, but
trigeminal neuralgia in a person under the age of 55 is almost always due
to MS.
Dr. Chambers recommended rest for
her muscle degeneration and suggested that she spend the winter at the
renowned Bath mineral waters in southwestern England. At this point she
was unable to write and was having trouble walking due to weakness and
curling of the foot muscles in her right leg. However, she continued to
be reassured by the doctors that she might recover. In fact, her left arm
did show some recovery, and the surgeon, Mr. Hawthorn, still felt her condition
was temporary and due to "time of life." She was spending much of her time
on a couch, able to walk a little, but going outside of the house in a
chair.
In 1868 she consulted "Dr. Radcliffe,
a paralytic & nervous Dr. who told me there was no doubt whatever about
my case: that it was spinal: a want of blood in the spinal cord. And he
ridiculed the idea that it was ‘degeneration’ of the muscles!" In return,
her doctors, Chambers and Paget, ridiculed Radcliffe, and still spoke hopefully
to her of recovery.
Her speech became affected and she
began falling. Cold weather was causing her leg muscles to tighten, Mr.
Hawthorn said. She became very ill with an attack of "liver illness," her
condition deteriorated further, her legs began to jerk, and her feet curled.
Her illness had a variable course
that moved from one arm to the other, and then to her legs. She had a recurrence
of the painful tic in her face and some speech difficulty. After many attacks,
"which perfectly disabled me without making me ill," she became progressively
worse and confessed she was willing to try anything, even quackery and
mesmerism.
Mr. Hawthorn informed her that Dr.
Chambers had mentioned her case in his lectures some six years earlier.20-21
She notes in a letter that "Dr. C. seems to consider it quite a peculiar
case" but was buoyed by his confidence in her diagnosis. In August 1870
she wrote, "You must prepare to see me unable to hold up my head—I tie
it up sometimes." Her legs were weaker, although her left arm was somewhat
improved. A few months later she noted she was failing, despite the massage
of her spine night and morning with a lotion suggested by another doctor.
By January, 1871, she noted regretfully
that "there was nothing definite except the undeniable fact that I am getting
lamer gradually." Her arms felt a little stronger but her hands were weaker.
She saw a notice about the benefits of oxygenating the blood and wrote
to Mr. Hawthorn, who prescribed a medicine he felt would have the same
effect. Her leg and foot spasms were getting worse. She noticed that all
movements were slower, such as combing her hair, patting the dog, and brushing
her nails, but she also noted that there was a woman at St. Leonards who
was worse afflicted, and could not even swallow. She could go out in a
bath chair and noted, "I am a complete cripple & feel very weak, but
not in bad spirits."
She was attended constantly by attendants
who had to turn her repeatedly during the night—a situation that distressed
her—but she added that although she was sad to have these services, she
certainly would be in dire straits if she were a poor person. She continued
to weaken, although "Enry" (Dr. Henry Hawthorn) kept insisting she was
better and quite rightly felt she would improve after her deterioration
during a bout of pneumonia.
After an additional year of complete
disability, she developed a respiratory infection and died peacefully on
October 4,1873. Because she had begun the idea of financially supporting
a cot for sick children at the Great Ormond Street Hospital, and over the
years her readers contributed to many cots in that and other hospitals,
it was appropriate that there was an outpouring of support from her readers
in all parts of the world to support more cots in her memory. This idea
of endowing cots in hospitals was then taken up by many institutions, and
these are a lasting memorial to a magnificent Victorian lady.
Conclusion
In every era, patients suffer with
conditions that medicine has yet to fully understand or to treat effectively.
Nonetheless, with the concepts and views of their day, they proceeded as
best they could and were as confident as we are today dealing with conditions
we understand incompletely. Just as we now understand illnesses like MS
in our current paradigms and struggle to find ways to manage our patients
better, they did their best in their age. MS has many mysteries and unanswered
questions, and only time will show just how similar our struggles are to
those of the early physicians in helping their patients with a disease
they only barely understood.
Acknowledgments
I would like to thank my publisher
and friend, Diana Schneider of Demos Publications, for allowing me to provide
this brief outline of a chapter in my forthcoming book on the history of
MS. Dr. Susan Drain introduced me to Margaret Gatty, Dr. Nick LaRocca to
the Lighthouse Stevensons, Anna Aguayo to William Brown, and Heather Spears
to Margaret Davies. Much of the research depended on the resources and
the professional kindness and assistance of staff of the Wellcome Library,
London, the Royal College of Physicians of London, and the British Library.
Full acknowledgment of all who helped will be in the forthcoming book on
the history of MS by Demos Publications.
References
page 1, Volume 3, Issue 2
June 2001
Abstract
—Charles Rosenberg
The observation quoted above by
the prominent medical historian Charles Rosenberg makes a very important
point, because we experience a disease in many ways other than biological
changes. Think of AIDS, cancer, or fibromyalgia: Those diseases are experienced
as much more than the biological effects. Once they are named and there
is a new way of thinking about them, attitudes and responses are changed—the
disease and its experiences are new and quite different from when there
was only a biological effect. The disease impacts those afflicted socially,
emotionally, and very personally. It impacts as well on the response of
communities, institutions, and government agencies that determine how the
person will experience the illness.
—Godfried de la Haye, court physician,
13962
Sept. 29— "Alas! I only walk in
my room for 28 3/4 min."
Despite Dr. Firth’s biographical
comments on d’Esté’s arrogance and immaturity in his youth, Augustus
d’Esté’s diaries reveal that he is a sensitive man, dealing courageously
with his failing strength, and delighting in the visits of friends and
family. He worries about the health of a friend with dropsy who is so kind
as to come to the door to see him, recognizing that he is unable to negotiate
the stairs. He is so concerned with the plight of the Irish famine that
he sends them funds, the same amount he was offered for his phaeton, in
which he loved to travel, but was advised to sell. He then got around in
a bath chair, but found his leg spasms and his fatigue were so marked that
he was comfortable only in bed. On the last page of his diary—dated Monday,
December 17—is the following entry:
Augustus d’Esté died in 1848
and was buried in the d’Esté mausoleum in Ramsgate, which he had
spent a great deal of time designing with the architects. In the mausoleum
there is a tablet with the details of his parents’ marriage, and the sad
consequences of the Royal Marriage Act that would not allow his legitimacy.
The likely reason the marriage was not legitimized was that the Prince
Regent also had a clandestine marriage to Mrs. Fitzherbert, which produced
two children. He subsequently married Princess Caroline. If d’Esté’s
parents’ marriage was declared legitimate, then so would be the marriage
of the heir to the throne, and thereby give George IV a Catholic wife,
a charge of bigamy, and the children of the first marriage more claim to
the throne than Queen Victoria.10
He had pain, shock-like sensations,
and was developing pressure sores. He wrote to his mother in July 1851
saying that during hot weather his eyes were worse, and he had often commented
that he was better in cold weather, a pattern common in MS.
—Mrs. Margaret Gatty, 9 January
1870
Mrs. Margaret Gatty (1809-1873),
a naturalist and author of very popular Victorian-era children’s books,
probably had MS. Although she lived past the time of Charcot’s lectures
and was herself published in the medical literature some years earlier,
her life ended just as the first publications on MS as an identifiable
entity were appearing in the English literature.
© 2001 International Journal of
MS Care