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ties and hypotheses. The careful study of autobio- extreme myopia with concomitant insufficiency of
graphical and biographical data, using original the internal rectal muscles (18). Signs of central
German sources, allowed us to reconstruct chorioretinitis (not further detailed) in both eyes,
Nietzsche’s medical biography quite accurately. most pronounced on the right side, are mentioned
We are aware of the fact that a historical medical once (18). Eye pressure measurements and ophtal-
analysis may be an anachronistic historical exer- moscopic evaluations were always normal (2).
cise, but the method of analyzing historical accurate Already at young age (before age 13) anisocoria
data using modern theoretical concepts is a com- was noted by Nietzsche’s mother and by professor
monly used and necessary perspective and method. Schellbach, ophthalmologist in Jena (19). On
Hence a well described and documented historical September 28, 1876 the same anisocoria (right >
clinical picture can be used to propose a retrospec- left) was noted by Dr. Heinrich Schiess-
tive diagnosis, using a current concept of disease Gemuseus (20). Forty years after the first descrip-
with proposed diagnostic criteria. tion, the same anisocoria was observed at the asy-
lum in Basel and was assumed to be a new symp-
Results tom and a key sign for the diagnosis of neu-
rosyphilis (4).
NIETZSCHE’S MEDICAL HISTORY At older age (4th decade), Nietzsche clearly suf-
fered from fluctuating visual disturbances of the
Nietzsche’s medical history consisted of several right eye. Eventually, visual problems occurred in
major problems including severe headache, visual both eyes. In 1873, Nietzsche described for the first
difficulties, psychiatric disturbances, cognitive time a sudden “weakness of his eyes” with strongly
decline and stroke. We will summarize each prob- diminished vision and accommodation cramps of
lem in chronological order. the eye muscles (21). Ophtalmological evaluation at
1. Headache. Nietzsche’s headaches began when that time confirmed blindness of the right eye. In
he was 9 years old (6). These headaches were usu- 1878, Nietzsche’s vision suddenly worsened and he
ally very severe and had a major impact on his daily became almost completely blind. Apparently,
life and later on his professional activities. They besides his well known myopia and eye muscle
were almost always located on the right side, most- insufficiency, Nietzsche suffered also from fluctuat-
ly frontal and above the right eye, but also at the ing visual disturbances with probable transient near
right hemicranium, and were typically associated blindness. Signs of chorioretinitis centralis or chori-
with gastrointestinal symptoms like nausea and oditis were found by 2 ophtalmologists, Dr. Kruger
vomiting (7-9). Because of these headaches, he and Dr. W. Vulpius, but were never mentioned by
sometimes also kept his eyes closed to lessen the other ophtalmologists (22).
discomfort experienced from external light, sug- 3. Mental illness : mood disorder and delusions.
gesting photophobia, and he avoided physical activ- In 1882, Nietzsche began to show depressive symp-
ities and went to bed (8-10). The headaches usually toms with suicidal ideas (23-25). These symptoms
persisted for several hours or even days. We found recurred intermittently and in 1887 Nietzsche
numerous descriptions of a duration of these described his mood as a persistent depression (26).
headaches ranging from 4 to 44 hours (9). Rarely, This depressive mood had a clear impact on his
the headache was not lateralized and lasted for four social and professional life. On several occasions
to six days (11). We found no clear descriptions of Nietzsche expressed bizarre ideas that reflected
possible premonitory symptoms, preceding aura or delusions (27). In 1883, he labelled his own mental
frequency and nature of the headaches. Nietzsche state for the first time as madness and in several let-
described his headaches several times as migraine ters he expressed his worries about suffering from
and this diagnosis was also mentioned in several madness (28-30). In 1884 he even described a visu-
original medical notes on his complaints (12, 13). al hallucination, consisting of a profusion of fantas-
Summarizing, we can state that Nietzsche’s tic flowers, twining round each other and constant-
headaches fulfil the criteria of migraine without ly growing, changing in shape and colour with exot-
aura as proposed by the International Headache ic opulence (28). His mental state evolved within
Society (IHS) (14). the following years and at the end of 1888, the final
2. Visual problems. Nietzsche’s visual problems mental breakdown appeared in Turin with manifest
also started at young age. He mentioned them for delusions and inability to take care of himself (31).
the first time in 1856, when he was 12 years At that time he was admitted to a psychiatric asy-
old (15). As a child Nietzsche often complained lum in Basel, Switzerland (4).
about “bad light”, “tiredness of the eyes” and 4. Dementia. Shortly after his mental breakdown
“episodes of eye weakness with altered vision” (16, in 1888, a progressive cognitive decline developed
17). He never mentioned complaints that could be and Nietzsche succumbed to dementia in 1889. In
suggestive for visual symptoms typical for migrain- January 1889, Nietzsche arrived at the psychiatric
ous aura. Nietzsche underwent repeated examina- asylum in Basel, where dementia paralytica was
tions by different ophtalmologists. They found an diagnosed, also known as general paresis of the
THE NEUROLOGICAL ILLNESS OF FRIEDRICH NIETZSCHE 11
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- FIGS. 1 and 2. — Two rare pictures showing Friedrich
ing pyramidal involvement (see figures 1 and 2). Nietzsche in 1899 with the clinical signs of a left hemiparesis,
This left hemiplegia is most likely to have been with adduction of the left arm and a bedridden state.
caused by a vascular lesion or stroke. Diagnosis of Klassik Stiftung Weimar ; Hans Olde, may 1899.
stroke at the end of his life was already reported by
Podach in 1931 (8).
6. Pneumonia & death. In the last years of his age of 36 years after a two years of mental illness
life, Nietzsche was bedridden and totally dependent (“Nervenabspannung” (tiredness of the nerves) and
from his relatives for his activities of daily living. “Gehirnaffektion” (brain disorder)), followed by
On August 25 1900, at the age of 56, he finally died increasing “Abzehrung” (wasting), speech prob-
from pneumonia, probably secondary to a final lems ending in aphasia, which prevented him from
stroke (4, 8). There are no post mortem data avail- doing his job as a priest, and visual loss (1, 36). At
able and an autopsy has never been performed (4). that time he was already completely bedridden.
Eventually, Nietzsche’s father died on July 30,
MEDICAL FAMILY HISTORY 1849. An autopsy was performed and revealed
that a quarter of the brain was affected by
On several occasions Nietzsche compared his ill- “softening” (“Gehirnerweichung”), probably of
ness to his father’s (35). During his life, Carl vasculo-ischemic origin (37).
Ludwig Nietzsche (1813-1849), a Lutheran priest, Nietzsche’s mother, Franziska Oehler, did not
suffered from several episodes of depression. suffer from major health problems, except some
Starting in 1846, epileptic seizures occurred (with abdominal troubles, and she died from abdominal
staring, inability to communicate, and postictal cancer in 1897 at the age of 70. Franziska’s relatives
amnesia). At that time he also had severe attacks of did not suffer from major health problems. She had
headache, mostly at the left frontal side of his head, one brother, Theobald, who committed suicide (1).
together with nausea and vomiting, and lasting Nietzsche’s little brother Joseph died at the age of
more or less one day. When an attack of headache almost 2 years (1848-1850) after an acute illness
appeared, most of the time he went to bed and when with general malaise and seizures. Friedrich
he woke up, the headache had almost disappeared. Nietzsche’s sister, Elisabeth, died at the age of
These characteristics point at migraine as probable 89 years and didn’t suffer from serious health prob-
cause of the headache (15). When Friedrich lems during her life. Carl Ludwig Nietzsche had
Nietzsche was 41/2 years old, his father died at the 2 sisters, Rosalia and Auguste. Rosalia was
12 D. HEMELSOET ET AL.
described as a nervous person, but none of them development. However, Nietzsche’s mother had
suffered from psychiatric illness (38). Friedrich already noticed that his right pupil was larger than
Nietzsche did not have children. his left when he was a child (4). This finding was
confirmed by a professional eye examination.
Discussion Several explanations can be given for the slow reac-
tion of the right pupil to light. A pre-existing eye
1) THE CASE OF SYPHILIS (PRO & CONTRA) condition (e.g. Adie’s pupil), a secondary phenom-
enon caused by migraine, or a tumour pressing to
For a long time Nietzsche’s illness has been con- the third nerve, are alternative possibilities. The
sidered to be a case of syphilis, and general paraly- appearance of grandiosity and bizarre ideas were
sis (or paretic neurosyphilis) in particular. supposed to be a sudden phenomenon when
Neurosyphilis is often referred to as a tertiary or Nietzsche was brought to the asylum in Basel, but
late effect of syphilis. However, the central nervous this assumption was incorrect. In fact, these mental
system involvement spans the entire course of the disturbances were the culmination of a trend of
syphilitic infection. Different stages of syphilis can many years (4). The occasional description of
be complicated by several neurological syndromes chorioretinitis could have been an additional ele-
like acute syphilitic meningitis, cerebrovascular or ment in favour of the diagnosis of syphilis, as chori-
meningovascular syphilis, paretic neurosyphilis and oretinitis can be caused by syphilis. However, there
tabes dorsalis (39). Meningovascular syphilis is are many other possible causes of chorioretinitis,
often preceded by a clinical course of weeks to like herpesviruses, Lyme disease, and systemic dis-
months before the onset of a clear stroke syn- eases (e.g. lupus). The description of signs of chori-
drome (39). Dr. Houston Merrit, a leading twenti- oretinitis remains unclear and has only been con-
eth-century expert on syphilis, showed that the firmed once afterwards. At the end of the 19th cen-
onset of neurological symptoms had an average tury, the commonest aetiology for a subacute onset
latency of seven years. Prodromal symptoms con- of dementia in a middle-aged man was syphilis, but
sisted of headache, vertigo, insomnia and various Nietzsche’s clinical presentation was not typical for
psychiatric disturbances (emotional lability or per- paretic syphilis. Moreover, Nietzsche showed none
sonality changes) (40). The interval between of the five cardinal signs of neurosyphilis identified
syphilitic infection and symptoms of paretic neu- by Merritt. From medical descriptions made upon
rosyphilis (general paresis, dementia paralytica) is his arrival at the asylum in Basel, we can read that
10 to 20 years (range 3 to 30 years) (41). his facial expression remained vivid, his reflexes
Early symptoms consist of memory problems, were normal, he showed no tremor, his handwriting
cognitive disturbances, irritability and decline in remained stable and his speech was not slurred, but
personal appearance. This stage is followed by remained fluent (4). Upon his arrival in Basel,
intellectual decline ending in progressive dementia. Nietzsche was supposed to be another case of neu-
A great diversity of psychiatric symptoms may rosyphilis. Since his mother was not financially
occur, including psychotic signs with delusional able to afford a first-class treatment with specific
symptoms (39). Merritt identified five typical clini- medical attention during his stay in the asylum in
cal signs of paretic syphilis : an expressionless face, Jena, and since Nietzsche was not a famous person
hyperactive tendon reflexes, tremor of facial mus- at that time (which e.g. was also confirmed by S.
cles and tongue, problems with handwriting due to Simchowitz, one of his contempories who was
intention tremor and dysarthria with slurred among Binswanger’s pupils in Jena when Nietzsche
speech (42). The full clinical picture includes was admitted) (43), no specific attention was given
dementia, dysarthria, myoclonic jerks, action to his clinical picture and it appears that the diagno-
tremor, seizures, hyperreflexia, Babinski signs and sis of neurosyphilis in Nietzsche’s case was paid in
Argyll-Robertson pupils. Eventually, a bedridden spite of, and not because of, the clinical evi-
state and diverse, focal neurological symptoms may dence (4). Moreover, during Nietzsche’s life, some
develop. Without treatment there is a progressive doctors already doubted the diagnosis of neu-
mental breakdown and death occurs within 3 to rosyphilis. Dr. Muthmann, a psychiatrist at the
4 years (39). Basel asylum, concluded that the content of
In his paper on Nietzsche’s dementia, Leonard Nietzsche’s notebooks were sufficient evidence to
Sax gave a good overview of the arguments pro and reject the diagnosis of progressive paralysis due to
contra syphilis as the cause of Nietzsche’s ill- syphilis (2). Sax describes four important features
ness (4). The diagnosis of paretic syphilis in of Nietzsche’s clinical presentation that are not
Nietzsche’s case was based on his asymmetrical accounted for, or even contradict, the diagnosis of
pupils with a slow reaction of the right pupil to neurosyphilis. Nietzsche’s migraine was not typical
light, the appearance of bizarre ideas and grandios- for the headaches caused by neurosyphilis, which
ity, and the development of dementia (4). When normally precede the general collapse by a few days
Nietzsche was admitted to the asylum in Basel, the to a few months. In Nietzsche’s case there is a peri-
asymmetrical pupils were assumed to be a new od of 35 years between the onset of migraine at the
THE NEUROLOGICAL ILLNESS OF FRIEDRICH NIETZSCHE 13
age of nine and the general collapse at the age of 44. tion of migraine, mood disorders, ischaemic strokes
Nietzsche’s length of life after his collapse was also and dementia. It starts in early adulthood and on
unusually long (12 years) for patients with neu- average leads to death in 10 to 20 years (47).
rosyphilis. The laterality of Nietzsche’s symptoms Rarely, the first stroke appears before the age of 30.
with right-sided headaches, speech problems and The reported peak of stroke incidence is in the
hemiparesis of his left side are also atypical for neu- fourth and fifth decade (47). Diagnostic criteria for
rosyphilis, which generally affects both cerebral CADASIL were proposed by Davous (49). To
hemispheres with generalized and bilateral signs accept the diagnosis of probable CADASIL five cri-
and symptoms. It has been suggested that perhaps teria are necessary : a young age at onset of symp-
the most important elements that make the neu- toms (< 50 years), presence of at least two of the
rosyphilis-hypothesis questionable are the lack of four major neurological features (migraine, stroke-
evidence that Nietzsche has been in a situation like episodes, major mood disturbances and subcor-
where he could have been infected with Treponema tical dementia), the absence of any vascular risk
pallidum, and the lack of diagnostic evidence that factors aetiologically related to the deficit, the evi-
Nietzsche actually suffered from syphilis (4). dence of an inherited autosomal dominant transmis-
sion, and the presence of abnormal MRI imaging
2) ALTERNATIVE DIAGNOSES findings of the white matter without cortical
infarcts (49). Exclusion criteria are : a late age at
Alternative diagnoses explaining Nietzsche’s onset (> 70 years), severe hypertension or compli-
medical health problems have been proposed in the cated heart or systemic vascular disease, absence of
past. Sax proposed a retrobulbar meningioma of the any other case in a documented pedigree and nor-
right optic nerve, underlying the right frontal lobe mal MRI imaging after age 35 in symptomatic sub-
of the brain as most likely diagnosis (4). This jects (49). Nietzsche’s medical history fulfils 4 out
hypothesis was based upon the slow progression of of 5 criteria, since there are no imaging data on his
the symptoms, the association of visual and psychi- cerebral white matter available. In several reviews
atric symptoms (including visual phosphenes), and of major symptoms and signs of CADASIL
the co-occurrence of migrainous headaches and the observed at onset with related age, stroke and
retinal disease. However, the occurrence of focal stroke-like episodes were the most frequent symp-
neurological symptoms like dysarthria and com- toms, affecting 36.5% to 67.6% of the patients.
plete hemiplegia are very unlikely to be caused by a Migraine was the second mode of onset in the
right frontal meningioma. CADASIL population (34.6%) (49, 50). When
Cybulska and Schain, two other opponents of the migraine was present, it was usually the earliest
hypothesis of neurosyphilis, proposed the diagnosis symptom, frequently beginning in the second
of manic depression or manic psychosis as most decade (51). Migraine may begin even in the first
likely explanation for Nietzsche’s mental illness (3, decade, but more commonly during the third
5). Nietzsche’s other medical problems (headaches, decade, with a peak around the fifth decade and the
visual problems, stroke) were not incorporated in oldest age in the eighth decade (47, 51). Migraine is
this diagnosis and were considered as being sepa- present in 22-38% of the mutation carriers (47).
rate, unconnected health problems. Recently, it was Migraine corresponding to the IHS criteria can be
suggested that Nietzsche’s mental illness was either with or without aura and may predominate in
caused by frontotemporal dementia (44). These some families. The aura is often atypical, long last-
alternative hypotheses do not consider Nietzsche’s ing or exceptionally severe (46). However, some
medical personal and family history. reports describe the occurrence of migraine without
aura in CADASIL (50, 52, 53). Davous even men-
3) A NEW HYPOTHESIS tioned a prevalence of common migraine (or
migraine without aura) of 20% in CADASIL
Reviewing Nietzsche’s different health problems, patients (49). Mood disorders are the most frequent
we think that they all could have been part of one psychiatric disturbances and include major depres-
neurological syndrome. We hypothesize that sion, manic depressive disorder, panic disorder, but,
Nietzsche suffered from cerebral autosomal domi- although not typical for the most common psychi-
nant arteriopathy with subcortical infarcts and atric disturbances in CADASIL, also hallucinatory
leukoencephalopathy or CADASIL, an inherited, syndromes, delusions and even psychosis may
generalized small-artery disease caused by muta- appear (51, 54).
tions in the Notch 3 gene on chromosome The natural course of CADASIL is variable. It
19q12 (45). CADASIL is characterized by a was shown that in CADASIL an insidious cognitive
nonatherosclerotic, nonamyloid systemic angiopa- decline may appear and may start in the pre-stroke
thy with a purely neurological clinical expression phase, before the first onset of symptomatic
due to involvement of the small arteries penetrating ischemic episodes, due to cumulative brain
the cerebral white matter (46-48). Clinically, lesions (48). Cognitive decline predominantly
CADASIL is mainly characterized by the associa- involves frontal lobe functions with mental slowing,
14 D. HEMELSOET ET AL.
53. VAHEDI K., CHABRIAT H., LEVY C., JOUTEL A., FEDERICO A. Acute unilateral visual loss as the first
TOURNIER-LASSERVE E., BOUSSER M. G. Migraine symptom of cerebral autosomal dominant arterio-
with aura and brain magnetic resonance imaging pathy with subcortical infarcts and leuko-
abnormalities in patients with CADASIL. Arch. encephalopathy. Arch. Neurol., 2004, 61 : 577-580.
Neurol., 2004, 61 : 1237-1240. 59. MALANDRINI A., CARRERA P., CIACCI G., GONNELLI S.,
54. DICHGANS M. Cerebral autosomal dominant arteri- VILLANOVA M., PALMERI S., VISMARA L.,
opathy with subcortical infarcts and leukoen- BRANCOLINI V., SIGNORINI E., FERRARI M.,
cephalopathy : Phenotypic and mutational spectrum. GUAZZI G. C. Unusual clinical features and early
J. Neurol. Sci., 2002, 203-204 : 77-80. brain MRI lesions in a family with cerebral autoso-
55. FILLEY C., THOMPSON L., SZE C. I. et al. White matter mal dominant arteriopathy. Neurology, 1997, 48 :
dementia in CADASIL. J. Neurol. Sci. 1999, 163 : 1200-1203.
163-167.
56. MELLIES J., BÄUMER T., MÜLLER J. SPECT study of a
German CADASIL family. A phenotype with
migraine and progressive dementia only. Neurology,
1998, 50 : 1715-1721. Dr. Dimitri HEMELSOET, M.D.,
57. OPHERK C., PETERS N., HERZOG J., LUEDTKE R.,
DICHGANS M. Long-term prognosis and causes of
Ghent University Hospital,
death in CADASIL : a retrospective study in Department of Neurology,
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58. RUFA A., DE STEFANO N., DOTTI M. T., BIANCHI S., B-9000 Ghent (Belgium).
SICURELLI F., STROMILLO M. L., D’ANIELLO B., E-mail : dimitri.hemelsoet@ugent.be