The neurological illness of Friedrich Nietzsche
Abstract
Friedrich Nietzsche (1844-1900), one of the most profound and influential modern philosophers, suffered since his very childhood from severe migraine. At 44 he had a mental breakdown ending in a dementia with total physical dependence due to stroke. From the very beginning, Nietzsche's dementia was attributed to a neurosyphilitic infection. Recently, this tentative diagnosis has become controversial. To use historical accounts and original materials including correspondence, biographical data and medical papers to document the clinical characteristics of Nietzsche's illness and, by using this pathography, to discuss formerly proposed diagnoses and to provide and support a new diagnostic hypothesis. Original letters from Friedrich Nietzsche, descriptions by relatives and friends, and medical descriptions. Original German sources were investigated. Biographical papers published in medical journals were also consulted. Nietzsche suffered from migraine without aura which started in his childhood. In the second half of his life he suffered from a psychiatric illness with depression. During his last years, a progressive cognitive decline evolved and ended in a profound dementia with stroke. He died from pneumonia in 1900. The family history includes a possible vascular-related mental illness in his father who died from stroke at 36. Friedrich Nietzsche's disease consisted of migraine, psychiatric disturbances, cognitive decline with dementia, and stroke. Despite the prevalent opinion that neurosyphilis caused Nietzsche's illness, there is lack of evidence to support this diagnosis. Cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL) accounts for all the signs and symptoms of Nietzsche's illness. This study adds new elements to the debate and controversy about Nietzsche's illness. We discuss former diagnoses, comment on the history of a diagnostic mistake, and integrate for the first time Nietzsche's medical problems.
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Abstract
Background : Friedrich Nietzsche (1844-1900), one of
the most profound and influential modern philosophers,
suffered since his very childhood from severe migraine.
At 44 he had a mental breakdown ending in a dementia
with total physical dependence due to stroke. From the
very beginning, Nietzsche's dementia was attributed to a
neurosyphilitic infection. Recently, this tentative diagno-
sis has become controversial.
Objective : To use historical accounts and original
materials including correspondence, biographical data
and medical papers to document the clinical character-
istics of Nietzsche's illness and, by using this pathogra-
phy, to discuss formerly proposed diagnoses and to pro-
vide and support a new diagnostic hypothesis.
Materials : Original letters from Friedrich Nietzsche,
descriptions by relatives and friends, and medical
descriptions. Original German sources were investigat-
ed. Biographical papers published in medical journals
were also consulted.
Results : Nietzsche suffered from migraine without
aura which started in his childhood. In the second half of
his life he suffered from a psychiatric illness with depres-
sion. During his last years, a progressive cognitive
decline evolved and ended in a profound dementia with
stroke. He died from pneumonia in 1900. The family his-
tory includes a possible vascular-related mental illness
in his father, who died from stroke at 36.
Conclusions : Friedrich Nietzsche's disease consisted
of migraine, psychiatric disturbances, cognitive decline
with dementia, and stroke. Despite the prevalent opinion
that neurosyphilis caused Nietzsche's illness, there is
lack of evidence to support this diagnosis. Cerebral auto-
somal dominant arteriopathy with subcortical infarcts
and leukoencephalopathy (CADASIL) accounts for all
the signs and symptoms of Nietzsche's illness. This study
adds new elements to the debate and controversy about
Nietzsche's illness. We discuss former diagnoses, com-
ment on the history of a diagnostic mistake, and integrate
for the first time Nietzsche's medical problems.
Key words : Nietzsche ; history of neurology ; CADASIL
; hypothesis.
Introduction
Friedrich Wilhelm Nietzsche (further called
Nietzsche) was born October 15, 1844 in Röcken,
Saxony, Germany (1). He is considered as one of
the most influential modern thinkers of the last two
centuries. He had and still has a major impact on
many philosophers, artists, novelists and many
intellectual and artistic movements. He is the author
of many books like "Die Fröhliche Wissenschaft"
("The Gay Science"), "Also sprach Zarathustra"
("Thus Spoke Zarathustra"), "Jenseits von Gut und
Böse" ("Beyond Good and Evil"), and "Ecce
Homo". Nietzsche suffered from many health prob-
lems. Ever since his early youth he tried to find
explanations for these health problems and
described them exhaustively in his correspondence.
Eventually, Nietzsche developed a mental illness at
age 44 and died at the age of 56. For a long time
Nietzsche's illness was thought to be neurosyphilis.
However, recently there has been a lot of controver-
sy about this diagnosis, but a clear and definite
diagnosis has not been established yet (2-5). In this
paper, we describe and integrate Nietzsche's differ-
ent health problems, we review formerly proposed
diagnoses, we focus on the history of a diagnostic
mistake, and propose a new diagnosis, explaining
Nietzsche's illness. Both medical and historical ele-
ments of Nietzsche's illness, and the focus on the
historical and current diagnostic approach might be
interesting for neurologists, historians and others
who are interested in Nietzsche's life and work.
Methods and materials
Autobiographical data from letters, first-hand
descriptions from relatives, friends and doctors, and
biographical material were used. Nietzsche wrote a
huge amount of letters and exhaustively described
his medical complaints and symptoms. These
descriptions of his illness are central to our under-
taking. A meticulous overview of Nietzsche's life,
including detailed medical notes by his treating
doctors and some pictures reflecting his physical
state at the end of his life, is available. We studied
several Nietzsche-biographies to look for occasion-
al references to his health. Finally, we studied med-
ical papers on Nietzsche's illness in order to have an
overview over past and current diagnostic difficul-
Acta neurol. belg., 2008, 108, 9-16
The neurological illness of Friedrich Nietzsche
D. HEMELSOET
1
,K. HEMELSOET
2
and D. DEVREESE
2
1
Department of Neurology, Faculty of Medicine and Health Sciences, Ghent University Hospital, Ghent, Belgium ;
2
Department of German, Faculty of Arts and Philosophy, Ghent University, Ghent, Belgium
————
ties and hypotheses. The careful study of autobio-
graphical and biographical data, using original
German sources, allowed us to reconstruct
Nietzsche's medical biography quite accurately.
We are aware of the fact that a historical medical
analysis may be an anachronistic historical exer-
cise, but the method of analyzing historical accurate
data using modern theoretical concepts is a com-
monly used and necessary perspective and method.
Hence a well described and documented historical
clinical picture can be used to propose a retrospec-
tive diagnosis, using a current concept of disease
with proposed diagnostic criteria.
Results
N
IETZSCHE'S MEDICAL HISTORY
Nietzsche's medical history consisted of several
major problems including severe headache, visual
difficulties, psychiatric disturbances, cognitive
decline and stroke. We will summarize each prob-
lem in chronological order.
1. Headache. Nietzsche's headaches began when
he was 9 years old (6). These headaches were usu-
ally very severe and had a major impact on his daily
life and later on his professional activities. They
were almost always located on the right side, most-
ly frontal and above the right eye, but also at the
right hemicranium, and were typically associated
with gastrointestinal symptoms like nausea and
vomiting (7-9). Because of these headaches, he
sometimes also kept his eyes closed to lessen the
discomfort experienced from external light, sug-
gesting photophobia, and he avoided physical activ-
ities and went to bed (8-10). The headaches usually
persisted for several hours or even days. We found
numerous descriptions of a duration of these
headaches ranging from 4 to 44 hours (9). Rarely,
the headache was not lateralized and lasted for four
to six days (11). We found no clear descriptions of
possible premonitory symptoms, preceding aura or
frequency and nature of the headaches. Nietzsche
described his headaches several times as migraine
and this diagnosis was also mentioned in several
original medical notes on his complaints (12, 13).
Summarizing, we can state that Nietzsche's
headaches fulfil the criteria of migraine without
aura as proposed by the International Headache
Society (IHS) (14).
2. Visual problems. Nietzsche's visual problems
also started at young age. He mentioned them for
the first time in 1856, when he was 12 years
old (15). As a child Nietzsche often complained
about "bad light", "tiredness of the eyes" and
"episodes of eye weakness with altered vision" (16,
17). He never mentioned complaints that could be
suggestive for visual symptoms typical for migrain-
ous aura. Nietzsche underwent repeated examina-
tions by different ophtalmologists. They found an
extreme myopia with concomitant insufficiency of
the internal rectal muscles (18). Signs of central
chorioretinitis (not further detailed) in both eyes,
most pronounced on the right side, are mentioned
once (18). Eye pressure measurements and ophtal-
moscopic evaluations were always normal (2).
Already at young age (before age 13) anisocoria
was noted by Nietzsche's mother and by professor
Schellbach, ophthalmologist in Jena (19). On
September 28, 1876 the same anisocoria (right >
left) was noted by Dr. Heinrich Schiess-
Gemuseus (20). Forty years after the first descrip-
tion, the same anisocoria was observed at the asy-
lum in Basel and was assumed to be a new symp-
tom and a key sign for the diagnosis of neu-
rosyphilis (4).
At older age (4
th
decade), Nietzsche clearly suf-
fered from fluctuating visual disturbances of the
right eye. Eventually, visual problems occurred in
both eyes. In 1873, Nietzsche described for the first
time a sudden "weakness of his eyes" with strongly
diminished vision and accommodation cramps of
the eye muscles (21). Ophtalmological evaluation at
that time confirmed blindness of the right eye. In
1878, Nietzsche's vision suddenly worsened and he
became almost completely blind. Apparently,
besides his well known myopia and eye muscle
insufficiency, Nietzsche suffered also from fluctuat-
ing visual disturbances with probable transient near
blindness. Signs of chorioretinitis centralis or chori-
oditis were found by 2 ophtalmologists, Dr. Kruger
and Dr. W. Vulpius, but were never mentioned by
other ophtalmologists (22).
3. Mental illness : mood disorder and delusions.
In 1882, Nietzsche began to show depressive symp-
toms with suicidal ideas (23-25). These symptoms
recurred intermittently and in 1887 Nietzsche
described his mood as a persistent depression (26).
This depressive mood had a clear impact on his
social and professional life. On several occasions
Nietzsche expressed bizarre ideas that reflected
delusions (27). In 1883, he labelled his own mental
state for the first time as madness and in several let-
ters he expressed his worries about suffering from
madness (28-30). In 1884 he even described a visu-
al hallucination, consisting of a profusion of fantas-
tic flowers, twining round each other and constant-
ly growing, changing in shape and colour with exot-
ic opulence (28). His mental state evolved within
the following years and at the end of 1888, the final
mental breakdown appeared in Turin with manifest
delusions and inability to take care of himself (31).
At that time he was admitted to a psychiatric asy-
lum in Basel, Switzerland (4).
4. Dementia. Shortly after his mental breakdown
in 1888, a progressive cognitive decline developed
and Nietzsche succumbed to dementia in 1889. In
January 1889, Nietzsche arrived at the psychiatric
asylum in Basel, where dementia paralytica was
diagnosed, also known as general paresis of the
10 D. HEMELSOET ET AL.
insane, progressive paralysis or paretic syphilis.
Later on, he was transferred to the asylum in Jena,
where professor Otto Ludwig Binswanger (1852-
1929) confirmed the diagnosis of progressive paral-
ysis. In March 1890, Nietzsche's mother decided to
take care of her son and he left the asylum in Jena.
In 1891, severe memory problems evolved, togeth-
er with apathy, irritability, behavioural disorders,
lack of insight, aggression, change of character and
personality, loss of self-control, regression (with
childish interest and thoughts), increasing delusions
and prosopagnosia (4, 32). His mental disorder at
that time fulfilled the diagnostic criteria for demen-
tia (DSM-IV), with severe memory problems,
involvement of other higher cortical functions and a
major impact on his activities in daily life and on
his professional activities (33).
5. Stroke. In the last years of his life Nietzsche
developed several acute neurological symptoms
with speech disturbances, probably evolving to
anarthria, and facial paresis (2, 34). These symp-
toms are very likely to have been caused by stroke
episodes. Several descriptions mentioned the occur-
rence of such acute episodes with focal neurologi-
cal deficits (e.g. facial nerve paresis) (34). At the
end of his life, Nietzsche was bedridden and clear-
ly suffered from a left hemiparesis or hemiplegia.
Several photographs from 1899 clearly show
Nietzsche in a bedridden state, with a paresis of the
left hemisoma with flexion of the left arm, suggest-
ing pyramidal involvement (see figures 1 and 2).
This left hemiplegia is most likely to have been
caused by a vascular lesion or stroke. Diagnosis of
stroke at the end of his life was already reported by
Podach in 1931 (8).
6. Pneumonia & death. In the last years of his
life, Nietzsche was bedridden and totally dependent
from his relatives for his activities of daily living.
On August 25 1900, at the age of 56, he finally died
from pneumonia, probably secondary to a final
stroke (4, 8). There are no post mortem data avail-
able and an autopsy has never been performed (4).
M
EDICAL FAMILY HISTORY
On several occasions Nietzsche compared his ill-
ness to his father's (35). During his life, Carl
Ludwig Nietzsche (1813-1849), a Lutheran priest,
suffered from several episodes of depression.
Starting in 1846, epileptic seizures occurred (with
staring, inability to communicate, and postictal
amnesia). At that time he also had severe attacks of
headache, mostly at the left frontal side of his head,
together with nausea and vomiting, and lasting
more or less one day. When an attack of headache
appeared, most of the time he went to bed and when
he woke up, the headache had almost disappeared.
These characteristics point at migraine as probable
cause of the headache (15). When Friedrich
Nietzsche was 4
1
/
2
years old, his father died at the
age of 36 years after a two years of mental illness
("Nervenabspannung" (tiredness of the nerves) and
"Gehirnaffektion" (brain disorder)), followed by
increasing "Abzehrung" (wasting), speech prob-
lems ending in aphasia, which prevented him from
doing his job as a priest, and visual loss (1, 36). At
that time he was already completely bedridden.
Eventually, Nietzsche's father died on July 30,
1849. An autopsy was performed and revealed
that a quarter of the brain was affected by
"softening" ("Gehirnerweichung"), probably of
vasculo-ischemic origin (37).
Nietzsche's mother, Franziska Oehler, did not
suffer from major health problems, except some
abdominal troubles, and she died from abdominal
cancer in 1897 at the age of 70. Franziska's relatives
did not suffer from major health problems. She had
one brother, Theobald, who committed suicide (1).
Nietzsche's little brother Joseph died at the age of
almost 2 years (1848-1850) after an acute illness
with general malaise and seizures. Friedrich
Nietzsche's sister, Elisabeth, died at the age of
89 years and didn't suffer from serious health prob-
lems during her life. Carl Ludwig Nietzsche had
2 sisters, Rosalia and Auguste. Rosalia was
THE NEUROLOGICAL ILLNESS OF FRIEDRICH NIETZSCHE 11
FIGS. 1 and 2. — Two rare pictures showing Friedrich
Nietzsche in 1899 with the clinical signs of a left hemiparesis,
with adduction of the left arm and a bedridden state.
Klassik Stiftung Weimar ; Hans Olde, may 1899.
described as a nervous person, but none of them
suffered from psychiatric illness (38). Friedrich
Nietzsche did not have children.
Discussion
1) T
HE CASE OF SYPHILIS (PRO & CONTRA)
For a long time Nietzsche's illness has been con-
sidered to be a case of syphilis, and general paraly-
sis (or paretic neurosyphilis) in particular.
Neurosyphilis is often referred to as a tertiary or
late effect of syphilis. However, the central nervous
system involvement spans the entire course of the
syphilitic infection. Different stages of syphilis can
be complicated by several neurological syndromes
like acute syphilitic meningitis, cerebrovascular or
meningovascular syphilis, paretic neurosyphilis and
tabes dorsalis (39). Meningovascular syphilis is
often preceded by a clinical course of weeks to
months before the onset of a clear stroke syn-
drome (39). Dr. Houston Merrit, a leading twenti-
eth-century expert on syphilis, showed that the
onset of neurological symptoms had an average
latency of seven years. Prodromal symptoms con-
sisted of headache, vertigo, insomnia and various
psychiatric disturbances (emotional lability or per-
sonality changes) (40). The interval between
syphilitic infection and symptoms of paretic neu-
rosyphilis (general paresis, dementia paralytica) is
10 to 20 years (range 3 to 30 years) (41).
Early symptoms consist of memory problems,
cognitive disturbances, irritability and decline in
personal appearance. This stage is followed by
intellectual decline ending in progressive dementia.
A great diversity of psychiatric symptoms may
occur, including psychotic signs with delusional
symptoms (39). Merritt identified five typical clini-
cal signs of paretic syphilis : an expressionless face,
hyperactive tendon reflexes, tremor of facial mus-
cles and tongue, problems with handwriting due to
intention tremor and dysarthria with slurred
speech (42). The full clinical picture includes
dementia, dysarthria, myoclonic jerks, action
tremor, seizures, hyperreflexia, Babinski signs and
Argyll-Robertson pupils. Eventually, a bedridden
state and diverse, focal neurological symptoms may
develop. Without treatment there is a progressive
mental breakdown and death occurs within 3 to
4 years (39).
In his paper on Nietzsche's dementia, Leonard
Sax gave a good overview of the arguments pro and
contra syphilis as the cause of Nietzsche's ill-
ness (4). The diagnosis of paretic syphilis in
Nietzsche's case was based on his asymmetrical
pupils with a slow reaction of the right pupil to
light, the appearance of bizarre ideas and grandios-
ity, and the development of dementia (4). When
Nietzsche was admitted to the asylum in Basel, the
asymmetrical pupils were assumed to be a new
development. However, Nietzsche's mother had
already noticed that his right pupil was larger than
his left when he was a child (4). This finding was
confirmed by a professional eye examination.
Several explanations can be given for the slow reac-
tion of the right pupil to light. A pre-existing eye
condition (e.g. Adie's pupil), a secondary phenom-
enon caused by migraine, or a tumour pressing to
the third nerve, are alternative possibilities. The
appearance of grandiosity and bizarre ideas were
supposed to be a sudden phenomenon when
Nietzsche was brought to the asylum in Basel, but
this assumption was incorrect. In fact, these mental
disturbances were the culmination of a trend of
many years (4). The occasional description of
chorioretinitis could have been an additional ele-
ment in favour of the diagnosis of syphilis, as chori-
oretinitis can be caused by syphilis. However, there
are many other possible causes of chorioretinitis,
like herpesviruses, Lyme disease, and systemic dis-
eases (e.g. lupus). The description of signs of chori-
oretinitis remains unclear and has only been con-
firmed once afterwards. At the end of the 19
th
cen-
tury, the commonest aetiology for a subacute onset
of dementia in a middle-aged man was syphilis, but
Nietzsche's clinical presentation was not typical for
paretic syphilis. Moreover, Nietzsche showed none
of the five cardinal signs of neurosyphilis identified
by Merritt. From medical descriptions made upon
his arrival at the asylum in Basel, we can read that
his facial expression remained vivid, his reflexes
were normal, he showed no tremor, his handwriting
remained stable and his speech was not slurred, but
remained fluent (4). Upon his arrival in Basel,
Nietzsche was supposed to be another case of neu-
rosyphilis. Since his mother was not financially
able to afford a first-class treatment with specific
medical attention during his stay in the asylum in
Jena, and since Nietzsche was not a famous person
at that time (which e.g. was also confirmed by S.
Simchowitz, one of his contempories who was
among Binswanger's pupils in Jena when Nietzsche
was admitted) (43), no specific attention was given
to his clinical picture and it appears that the diagno-
sis of neurosyphilis in Nietzsche's case was paid in
spite of, and not because of, the clinical evi-
dence (4). Moreover, during Nietzsche's life, some
doctors already doubted the diagnosis of neu-
rosyphilis. Dr. Muthmann, a psychiatrist at the
Basel asylum, concluded that the content of
Nietzsche's notebooks were sufficient evidence to
reject the diagnosis of progressive paralysis due to
syphilis (2). Sax describes four important features
of Nietzsche's clinical presentation that are not
accounted for, or even contradict, the diagnosis of
neurosyphilis. Nietzsche's migraine was not typical
for the headaches caused by neurosyphilis, which
normally precede the general collapse by a few days
to a few months. In Nietzsche's case there is a peri-
od of 35 years between the onset of migraine at the
12 D. HEMELSOET ET AL.
age of nine and the general collapse at the age of 44.
Nietzsche's length of life after his collapse was also
unusually long (12 years) for patients with neu-
rosyphilis. The laterality of Nietzsche's symptoms
with right-sided headaches, speech problems and
hemiparesis of his left side are also atypical for neu-
rosyphilis, which generally affects both cerebral
hemispheres with generalized and bilateral signs
and symptoms. It has been suggested that perhaps
the most important elements that make the neu-
rosyphilis-hypothesis questionable are the lack of
evidence that Nietzsche has been in a situation
where he could have been infected with Treponema
pallidum, and the lack of diagnostic evidence that
Nietzsche actually suffered from syphilis (4).
2) A
LTERNATIVE DIAGNOSES
Alternative diagnoses explaining Nietzsche's
medical health problems have been proposed in the
past. Sax proposed a retrobulbar meningioma of the
right optic nerve, underlying the right frontal lobe
of the brain as most likely diagnosis (4). This
hypothesis was based upon the slow progression of
the symptoms, the association of visual and psychi-
atric symptoms (including visual phosphenes), and
the co-occurrence of migrainous headaches and the
retinal disease. However, the occurrence of focal
neurological symptoms like dysarthria and com-
plete hemiplegia are very unlikely to be caused by a
right frontal meningioma.
Cybulska and Schain, two other opponents of the
hypothesis of neurosyphilis, proposed the diagnosis
of manic depression or manic psychosis as most
likely explanation for Nietzsche's mental illness (3,
5). Nietzsche's other medical problems (headaches,
visual problems, stroke) were not incorporated in
this diagnosis and were considered as being sepa-
rate, unconnected health problems. Recently, it was
suggested that Nietzsche's mental illness was
caused by frontotemporal dementia (44). These
alternative hypotheses do not consider Nietzsche's
medical personal and family history.
3) A
NEW HYPOTHESIS
Reviewing Nietzsche's different health problems,
we think that they all could have been part of one
neurological syndrome. We hypothesize that
Nietzsche suffered from cerebral autosomal domi-
nant arteriopathy with subcortical infarcts and
leukoencephalopathy or CADASIL, an inherited,
generalized small-artery disease caused by muta-
tions in the Notch 3 gene on chromosome
19q12 (45). CADASIL is characterized by a
nonatherosclerotic, nonamyloid systemic angiopa-
thy with a purely neurological clinical expression
due to involvement of the small arteries penetrating
the cerebral white matter (46-48). Clinically,
CADASIL is mainly characterized by the associa-
tion of migraine, mood disorders, ischaemic strokes
and dementia. It starts in early adulthood and on
average leads to death in 10 to 20 years (47).
Rarely, the first stroke appears before the age of 30.
The reported peak of stroke incidence is in the
fourth and fifth decade (47). Diagnostic criteria for
CADASIL were proposed by Davous (49). To
accept the diagnosis of probable CADASIL five cri-
teria are necessary : a young age at onset of symp-
toms (< 50 years), presence of at least two of the
four major neurological features (migraine, stroke-
like episodes, major mood disturbances and subcor-
tical dementia), the absence of any vascular risk
factors aetiologically related to the deficit, the evi-
dence of an inherited autosomal dominant transmis-
sion, and the presence of abnormal MRI imaging
findings of the white matter without cortical
infarcts (49). Exclusion criteria are : a late age at
onset (> 70 years), severe hypertension or compli-
cated heart or systemic vascular disease, absence of
any other case in a documented pedigree and nor-
mal MRI imaging after age 35 in symptomatic sub-
jects (49). Nietzsche's medical history fulfils 4 out
of 5 criteria, since there are no imaging data on his
cerebral white matter available. In several reviews
of major symptoms and signs of CADASIL
observed at onset with related age, stroke and
stroke-like episodes were the most frequent symp-
toms, affecting 36.5% to 67.6% of the patients.
Migraine was the second mode of onset in the
CADASIL population (34.6%) (49, 50). When
migraine was present, it was usually the earliest
symptom, frequently beginning in the second
decade (51). Migraine may begin even in the first
decade, but more commonly during the third
decade, with a peak around the fifth decade and the
oldest age in the eighth decade (47, 51). Migraine is
present in 22-38% of the mutation carriers (47).
Migraine corresponding to the IHS criteria can be
either with or without aura and may predominate in
some families. The aura is often atypical, long last-
ing or exceptionally severe (46). However, some
reports describe the occurrence of migraine without
aura in CADASIL (50, 52, 53). Davous even men-
tioned a prevalence of common migraine (or
migraine without aura) of 20% in CADASIL
patients (49). Mood disorders are the most frequent
psychiatric disturbances and include major depres-
sion, manic depressive disorder, panic disorder, but,
although not typical for the most common psychi-
atric disturbances in CADASIL, also hallucinatory
syndromes, delusions and even psychosis may
appear (51, 54).
The natural course of CADASIL is variable. It
was shown that in CADASIL an insidious cognitive
decline may appear and may start in the pre-stroke
phase, before the first onset of symptomatic
ischemic episodes, due to cumulative brain
lesions (48). Cognitive decline predominantly
involves frontal lobe functions with mental slowing,
THE NEUROLOGICAL ILLNESS OF FRIEDRICH NIETZSCHE 13
concentration problems, slowing of motor functions,
disinhibition and perseveration (47, 48). Variant
forms of CADASIL have been reported. CADASIL
can appear as an isolated slowly progressive neu-
robehavioral disorder with personality disorder, psy-
chosis, mood disorder and eventually dementia over
an extended period of time (55). In 10-15% of the
patients, dementia even develops without acute
stroke episodes (56). CADASIL eventually leads to
death most often 10 to 20 years after symptom onset
(range, < 1 to 65 years) (47, 50). Davous reported
stroke as the most frequent primary cause of death,
followed by bronchopneumonia and other decubitus
complications (49). In another study on the long-
term prognosis and causes of death in CADASIL
pneumonia was the most frequent (primary and sec-
ondary) cause of death (38%) (57).
Although we are aware of the fact that most of
Nietzsche's visual problems cannot be explained by
CADASIL (e.g. signs of chorioretinitis), it is not
impossible that the episodic loss of vision, which
occurred at various times during Nietzsche's life,
could have been part of CADASIL. Visual loss due
to transient or stable ischemic events involving the
optic nerve and occurring at a young age, without
evidence for other cardiovascular risk factors, is
considered to be part of the CADASIL phenotype in
some patients (58).
Some of Nietzsche's acute episodes with neuro-
logical impairment could be considered as epileptic
seizures (e.g. complex partial seizures). We cannot
exclude that Nietzsche suffered from epileptic fits.
However, this is no argument against our
CADASIL hypothesis, since epileptic seizures
already have been reported as an atypical clinical
presentation in CADASIL (59). Moreover,
Nietzsche's father also suffered from epilepsy,
which could have been part of the inherited disease
and supports our hypothesis.
Besides the clinical aspects of Nietzsche's dis-
ease, an important argument to our hypothesis is the
family history. The similar disease history in
Nietzsche's father may point at a dominant heri-
tance of the disease. Unfortunately, no further med-
ical history of other family members on the father's
side is available. This lack of a complete and
detailed family history makes it difficult to make
definitive conclusions.
The integration of these major medical problems
(migraine, psychiatric disturbances, dementia,
stroke, and possibly the visual problems), together
with a history of mental and cognitive problems in
several family members, and migraine with epilep-
tic seizures and cognitive decline and stroke in
Nietzsche's father, corresponds to the proposed
diagnostic criteria of CADASIL and supports our
hypothesis that Friedrich Nietzsche may have suf-
fered from CADASIL. This disease entity most
closely fits all of Nietzsche's medical data avail-
able.
Conclusion
Friedrich Nietzsche suffered from severe
migraine, psychiatric disease, dementia, visual loss
(possibly from a vascular origin), and stroke or
stroke-like episodes. Based upon these clinical
symptoms, together with the family history, we
think that Friedrich Nietzsche might have suffered
from CADASIL. Considering the proposed diag-
nostic criteria, Nietzsche's symptoms meet the cri-
teria of 'probable CADASIL'. This retrospective,
tentative and speculative diagnosis integrates his
major medical problems and provides a better
explanation than former diagnoses proposed by
other authors (3, 4). We are convinced that current-
ly CADASIL is the best working hypothesis.
Hence, the case of Friedrich Nietzsche might be the
first well-documented historical case of CADASIL.
The final diagnosis remains unclear due to the
absence of any diagnostic instrument (e.g. imaging)
at the end of the 19
th
century. Theoretically, the pro-
posed diagnosis of CADASIL could be confirmed
with DNA analysis, even post mortem, although a
typical Notch 3 mutation may be absent in the pres-
ence of granular osmiophilic material in several tis-
sues. Unfortunately, since Nietzsche did not have
children, it is not possible to trace descendants for
further investigation of our hypothesis.
Acknowledgements
The authors wish to thank Benjamin Biebuyck,
Hendrik Vantilborgh, Bart Leroy and Luc Crevits for crit-
ical reading and making useful suggestions. They also
thank Ms. Duyts for excellent technical assistance and
the Klassik Stiftung Weimar for giving permission to
publish the photographs of Friedrich Nietzsche.
Disclosure : The authors report no conflicts of interest
(financial or other).
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16 D. HEMELSOET ET AL.
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