Keywords
Hysteria - Conversion Disorder - Dissociative Disorders - Epilepsy - Epilepsy, Tonic-Clonic
Palavras-chave
Histeria - Transtorno Conversivo - Transtornos Dissociativos - Epilepsia - Epilepsia
Tônico-Clônica
INTRODUCTION
Hysteria, or the suffocation of the mother,[1]
[2] as described by Edward Jorden (1569–1632), has metamorphosed from a disease specific
to women and their wombs (from the Greek hysterikós, “relative to the womb”) to the concept of functional neurologic disorders.[3] In fact, Jorden's considerations on hysteria figure among the firsts attempts to
demystify its medieval misconception, as a work of witchcraft and manifestation of
demonic possession in the female body, heading back to the Hippocratic argument of
a genuine disease whose pathology relies on the connections of the womb to many body
systems and whose symptoms were “monstrous and terrible to behold.”[2]
[4] After Jorden, many eminent physicians, such as Thomas Sydenham (1624–1689) and,
later, Philippe Pinel (1745–1826), also defined hysteria as an illness, either organic
or mental.[4]
[5] In addition to recognizing it as an emulator of almost all organic ailments, Sydenham
also stated for the first time that this malady is not restricted to women, also affecting
men of “sedentary or studious lives,” removing the uterus from the main stage and
presenting the brain as candidate for its origin.[4]
[5] This last theory was also shared by his contemporaries Charles Lepois (1563–1633)
and Thomas Willis (1621–1675), as well as by Pierre Briquet (1796–1881), over a century
later.[6]
[7]
Jean-Martin Charcot (1825–1893) stands out in the history of hysteria both scientifically
and by means of spectacle and art, due to his “Leçons du Mardi à la Salpêtrière,”
brilliantly documented by his disciples Bourneville (1840–1909) and Regnard (1850–1927)
in the repository Iconographie Photographique de la Salpêtrière, and his work with Paul Richer (1849–1933), Les Démoniaques dans l'art
[6]
[8]. Of interest to this assay is the first part of the latter opus, which describes
les démoniaques convulsionnaires (the convulsive demons) or La Grande Attaque Hystérique (the great hysterical attack), with Richer's personal drawings to represent the so-called
hysteroepileptic phenomena resembling grand mal seizures and their four periods, as previously proposed by Charcot: période épileptoide (epileptoide period), grands mouvements/clownisme (great movements/clownism), attitudes passionnelles (emotional gestures), and Période terminale (final delirium).[8]
[9] In the book's second part, Charcot recognizes a variation of the third phase of
the attack, which he calls les extatiques (the ecstatic), which could include quietness, feelings of ecstasy, and negative
sensory phenomena such as blindness, delusional speech, and hallucinations, often
with religious or even erotic connotations, referencing many masterpieces of religious
art, which has been reviewed elsewhere.[10]
Charcot's saints are a beautiful and visionary attempt to describe psychogenic nonepileptic
seizures (PNES) or functional seizures and other stereotyped neurological events in
relation to the “sacred disease” (that is, epilepsy) and its uttermost presentation:
grand mal seizures. The aim of the present work is to make a brief reference to PNES
as represented by Charcot in his use of the iconography of saints and how those same
signs are perceived under the lenses of modern epilepsy monitoring units (EMUs), as
a puzzle piece on the often difficult diagnosis of PNES.
METHODS
A comparison between Charcot and Richer's iconography in Les Démoniaques dans l'arts and our institution's repository of video-electroencephalograms (vEEGs) recorded
between 1996 and 2019 was performed to find typical characteristics of PNES also ascribed
in Charcot's times to the grande attaque hystérique and its semiological phenomena. All patients consented to the use of their data for
academic purposes.
RESULTS
Frames from 7 vEEGs were selected and are shown in [Figures 1],[2], representing the four canonical phases of the grande attaque hystérique, along with the proper remarks.
Figure 1 (A) Période épileptoide of the hysterical attack from Les Démoniaques dans l'art.[8] (B) Similar semiological features are present in a patient in our practice diagnosed
with psychogenic non-epileptic seizure (PNES), who shows both upper limbs in extension,
crossed legs, tilted head, and closed eyes and fists, along with unresponsiveness
during the functional seizure. (C) Période de clownisme and grands mouvements in a male patient in the Richer drawing depicted with opisthotonus − the arc de cercle (D) and in a patient of ours diagnosed with PNES. (E) L'attitude passionnelle, the contemplative attitude found in Les Extatiques (F) and the same facial expression in a patient of ours with the diagnosis of PNES after
vEEG. (G) Période épileptoide, phase de résolution − after the seizure-like phenomena of the Période épileptoide, follows the resolution phase of the hysteric attack as depicted by Richer. (H) A similar asthenic expression was also observed in one of our patients following
a functional seizure. The patients' eyes were blacked out to preserve their identities
for ethical purposes.
Figure 2 (A) Grands mouvements. Pelvic thrusting movements and sustained elevation of the legs present in the clownism
phase of the grande attaque hystérique as depicted in Les Démoniaques dans l'arts.[8] On the frames of the upper panel, a young male patient presents alternating movements
of the lower limbs and trunk over the hips, both arms were crossed in a posture unfit
for an epileptic seizure. The movements could be stopped and be induced by the touch
of the technician. (B) In the lower panel, one can see the generalized contractures in the période terminale of the attack as depicted by Charcot and Richer, and examples of generalized contractures
and bizarre postures at the end of a functional seizure. Both patients tend to opisthotonus,
with the patient on the left lying down with both eyes open and a similar dystonic
posture of the hand as shown in the drawing, while the patient at the botton right
of the figure presents with hand drop, eyes closed and an asymmetrical contracture
of head and neck muscles with jaw deviation to the left. The patients' eyes were blacked
out to preserve their identities for ethical purposes.
DISCUSSION
The International League Against Epilepsy (ILAE) defines PNES as paroxysmal, time-limited,
alterations with motor, sensory, autonomic, and/or cognitive signs, as well as symptoms
not caused by ictal epileptiform activity.[11] They represent approximately 10% of seizures in the emergency room, encompassing
from 5 to 10% of patients under care for epilepsy, and 20 to 40% of the diagnosis
in tertiary epilepsy centers.[12]
[13] More importantly, between 50 and 81% of all PNES seizures will emulate grand mal
seizures[14] and, in spite of their often bizarre presentation, they are commonly mistaken by
epileptic seizures, delaying the correct diagnosis by a mean of 7 years, increasing
treatment cost and morbidity.[15]
Over the past 40 years, based on systematic analysis of vEEGs, several clinical discriminators
between epileptic seizures and PNES have been proposed.[11]
[15] Remarkable examples include the ictal eye closure, opisthotonus, and hand clawing.[15]
[16]
Despite the fact that sensorial symptoms (that is, the Charcot stigmata) may be considered
an archetypical manifestation of hysteria, these phenomena may also be found in epileptic
seizures (such as focal onset non-motor seizures). But, when present in PNES, they
commonly pose anatomical incompatibilities with motor symptoms.[17] On a similar account, the ecstatic crisis can also be related to epilepsy, often
related to the nondominant temporal lobe.[10]
[18]
Regardless their accuracy, the indisputable fact is that these stigmata of PNES were
reported by a set of remarkable clinicians unaided by technology and subjected to
the mystical and religious influence of their time. Nonetheless, their observations
survived through the sieve of time, and remain as solid and inspirational clinical
tools, influencing the diagnostic skills of young physicians armed with home videos
sent to their cell phones. It is worth mentioning the work by Amin et al.,[19] who investigated 44 patients from their epilepsy center and found 94% of agreement
between the interpretations of two blinded physicians for the ictal phenomena in question
– either epileptic or not – when comparing the patients' standard vEEGs to smartphone
homemade videos. There are other reports based on vEEG, highlighting pictorial traits
in drawings as useful clinical hints to teach how to discriminate between epileptic
and nonepileptic phenomena.[17]
Stepping away from the epileptology, it is important to briefly address the social
and anthropological ramifications of Charcot's works. Being an anticlerical thinker,
he spent his life advocating, although less actively than his pupil Bourneville, for
the secularization of medical science. His work in Les Démoniaques dans l'art is a firm expression of this belief, defying the dominant religious ideas of nineteenth
century Parisian society.[6]
[20]
Charcot's privileged clinical mind and Richer's fine tracing combined shed an initial
light on the challenging field currently known as neurological functional disorders.
At the end of the day, the accuracy and detailing of their observations own very little
to the findings on our videos today, even without the freezing, framing, and rewinding
capabilities.
Bibliographical Record
Marlon Wycliff Caeira, Leo Coutinho, Igor Abrahim Nascimento, Luciano de Paola, Hélio
Afonso Ghizoni Teive. An essay on the Charcot and Richer hysteria: from charcoal drawings
to cell phones. Arq Neuropsiquiatr 2024; 82: s00441789229.
DOI: 10.1055/s-0044-1789229
