The term “mania” was used to denote madness for a long period of time. However, it
was only in the first half of the nineteenth century that scholars started to advocate
that mania should be regarded as an affective disorder[1 ].
In 1899, Emil Kraepelin, a highly prominent German psychiatrist, outlined in the chapter
“Manic-depressive Illness”, from the 6th edition of his treatise, a concept of a mental disease that encompassed a variety
of clinical pictures, such as the periodic and circular insanity, simple melancholia
and mania. He stated that, contrary to what was observed with dementia praecox , after a mood disorder episode the symptoms could totally remit. Nevertheless, patients
with severe and multiple attacks of manic-depressive illness, or during aging, might
have lower odds of full recovery and end up with chronic symptoms[2 ].
Chronic mania has long been observed and described by many clinicians, such as Pinel
(1802), Esquirol (1838), Griesinger (1865), Schott (1904), Kraepelin (1913) and Wertham
(1929). Many authors of that time described the evolution of chronic mania into moria , within a generally-accepted idea that affective states could evolve into a secondary
chronic mental weakness and into dementia[3 ].
Schott, who was cited by Kraepelin in his 8th edition[4 ], reviewed chronic mania, described four cases, and concluded that the clinical picture
was similar to a mild presentation of acute mania, with minor loss of attention, memory,
knowledge and mental activity, but with marked ethical loss, poor judgment and insight[5 ]. Acknowledging those protracted impairments, Schott considered chronic mania as
a “special form of dementia” and emphasized the extreme heritability in most cases.
In 1978, Krauthammer and Klerman expanded the concept of mania and proposed the nomenclature
“secondary mania” to those cases supposedly associated with organic dysfunction, either
medical or pharmacological, particularly in patients without a history of affective
disorder[6 ]. In this context, the behavioral variant of frontotemporal dementia (bvFTD) has
repeatedly been reported as a possible cause of manic presentations[7 ]. Indeed, diagnostic criteria for FTD were first proposed in 1994, and updated in
1998, and from the outset, both sets of criteria highlighted symptoms commonly observed
in bipolar patients during a manic episode.
Recently, diagnostic criteria for bvFTD have been revised, bringing substantial gains
in sensitivity. Accordingly, core behavioral features include: 1) social disinhibition,
2) loss of sympathy or empathy, 3) apathy, 4) hyperorality and dietary changes, 5)
perseverative, stereotyped or compulsive behavior, and 6) a neuropsychological profile
characterized by executive dysfunction. According to current recommendations, a bvFTD
phenotype can be confirmed if progressive deterioration of behavior and/or cognition
is established, along with the presence of at least three of the six main clinical
symptoms[8 ].
In the following paragraphs we dissect Kraepelin’s authoritative description of chronic
mania[9 ], correlating current criteria for bvFTD with his report.
Patients with bvFTD typically manifest behavioral disinhibition, with socially inappropriate
conduct, including loss of manners, careless actions and even legal infractions. Kraepelin
stated: “They like to
interfere in everyt
hing, act as guardia
ns to the feebler pa
tients, snarl at the
m, take from them wh
at they want.” (…)“T
hey can meantime sca
rcely, or not at all
, be employed for pr
ofitable work on acc
ount of their unstea
diness and indiffere
nce as well as their
inclination to all
sorts of mischief.”(
…) “... talk more th
an their share, swag
ger, try to gain for
themselves all poss
ible little advantag
e.”
The lack of empathy, loss of sympathy and social isolation are a hallmark of bvFTD.
Those symptoms were described by Kraepelin as: “The finer emo
tions are considerab
ly injured. The pati
ents show little int
erest in their relat
ives, do not shrink
from making coarse j
okes about them,…”
Apathy is frequently observed in bvFTD and may be the main behavioral manifestation
of the disorder. These symptoms are also highlighted in Kraepelin’s description of
chronic mania: “…do not trouble themselves about their
affairs, do not wor
ry at all about thei
r position and their
future, at most onc
e in a day they beg
without energy for d
ischarge.”(…)“…every
thing else has becom
e to them more or le
ss indifferent.”
The presence of dietary changes and hyperorality are also included as a diagnostic
behavioral symptom of bvFTD. Patients may have increased cigarette or alcohol consumption,
and gluttonous overeating. Interestingly, Kraepelin also hints about these changes:
“On
ly the coarser enjoy
ments, eating, drink
ing, smoking, snuffi
ng, still arouse in
them vivid feelings,
further the satisfa
ction of their perso
nal wishes and wants
.”
Compulsive and ritualistic behaviors, including collectionism and hoarding, are observed
in patients with bvFTD. Patients are typically prone to pick up objects in the surroundings
and store them, collecting useless, old or damaged items. Again, as Kraepelin: “They collect al
l possible rubbish i
n their pockets, mak
e a mess with it all
round about, rub an
d wipe things, adorn
themselves with rag
s and scraps of ribb
on,…” ([Figure ]).
Figure Self-decorated manic patient.(Kraepelin 1904/translated in 1912, p. 397)
In summary, the description of chronic mania provided by Kraepelin in 1913 depicts
a cluster of symptoms that closely resembles the bvFTD phenotype. We should keep in
mind that Kraepelin was especially concerned with the description and classification
of mental diseases based upon symptoms, course and outcome, since he considered etiologic
and neuroanatomical findings a step ahead of the clinical identification of diseases[10 ],[11 ]. We must also recognize, that after the last twenty years of accumulated research
on FTD, we stand on more solid footing, which was unavailable to Kraepelin or to past
clinicians. Accordingly, we did not expected to find the neuropathologic descriptions
that we know today in his texts. Indeed, in his reports on the “physical causes” of
manic-depressive illness, we found nothing but a review of several unspecific affections[9 ]. Nevertheless, we may conceive that some chronic mania patients of that time, as
described by Kraepelin, could have fit within current diagnostic criteria for possible
bvFTD.
Though being aware of the limitations imposed by the restricted bibliography consulted,
the present work may be read as an historical illustration of the complex relationships
between primary severe psychiatric disorders and neuropsychiatric symptoms secondary
to neurodegenerative conditions. Recently, neuroimaging and genetic studies have demonstrated
that there may be a common neurobiological foundation between those conditions, at
least in a small subset of patients[12 ]. It is known that selected mutation carriers display a slowly progressive disease
course, sometimes with a long lasting psychiatric prodrome. Further research may elucidate
to what extent the clinical descriptions of bvFTD and chronic mania refer to distinct
clinical entities or to a spectrum with common pathophysiological processes.
