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The inside experience of epilepsy: An essay about the importance of subjectivity

  • Peter Wolf
    Correspondence
    Correspondence to: Dag Hammarskjölds Allé 5, 1.tv, DK, 2100, Copenhagen, Denmark.
    Affiliations
    Danish Epilepsy Centre Filadelfia, Dianalund, Denmark

    Programa de Pós-Graduação em Ciências Médicas, Universidad Federal de Santa Catarina, Florianópolis, SC, Brazil
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Open ArchivePublished:January 24, 2021DOI:https://doi.org/10.1016/j.seizure.2021.01.006

      Highlights

      • Subjective seizure symptoms are equally important as objective signs.
      • They have high localizing value.
      • Patients’ reactions to subjective symptoms can have therapeutic consequences.
      • Patients should be recognized as inside experts of seizures.

      Abstract

      This essay addresses three aspects of the inside experience of epilepsy, i) the high semiological significance of subjective seizure symptoms, ii) the therapeutic consequences, both positive and negative, of subjective seizure experiences, and iii) the importance of recognizing the patient as the ‘inside expert’ of epilepsy. Subjective symptoms are often not spontaneously reported but ignoring them may be associated with serious risks. They can be experienced as neutral, negative or positive, and this can have important consequences for therapy. Only patients have full and first-hand knowledge of subjective symptoms but an understanding of these symptoms and an adequate response to them requires expert assistance. The inside and outside views of seizures are different but of equal importance. To get the full picture, both are needed to supplement each other.

      Keywords

      “The patient’s half, that he can contribute to knowledge, is ignored” (Evans, The Nightingale Silenced).
      The differential diagnosis of seizures is not always easy. The exact sequence of signs and symptoms, especially of movements, is of the highest importance but much of this is not registered by patients, and witnesses – if they exist – may not be good observers or describers. Therefore, video registrations of seizures have acquired a central role in epileptology, and we regularly ask patients and families to bring home video recordings of seizures to the consultation. The ease with which seizure videos can be recorded now has greatly improved the reliability of seizure diagnostics. However, there is also a downside to this development, as it has directed attention towards what is visible, towards the objective, and put subjective seizure symptoms at risk of being neglected. This has several negative consequences.

      1. The semiology of subjective symptoms

      Focal seizures can be generated anywhere in the cerebral gray matter. Their first signs or symptoms depend upon their site of origin and typically reflect its physiological function. Leaving aside for the moment the (not uncommon) situation that the seizure origin is in a “silent” zone of the brain and that the first signs and symptoms express the first step of seizure spread, seizures may originate either in a zone with motor or with subjective expression. Amazingly, the relative frequency of these two classes of initial seizure manifestations seems not to have been investigated, but there is no doubt that large parts of the cerebral cortex subserve sensory or psychical functions, and that these are reflected by subjective experiences if seizures originate there. Therefore, the semiological significance of subjective seizure symptoms is not one bit less important than that of objective signs, neither qualitatively nor quantitatively.
      How are these relations reflected in the official documents of the International League against Epilepsy (ILAE), i.e. the international classifications of seizures? The classification of 1981 (which is a revision of the classification of 1970) is the first document built upon the analysis, by the Commission on Classification and Terminology, of video-documented seizures [
      Commission on Classification and Terminology of the ILAE. Proposal for revised clinical and electroencephalographic classification of epileptic seizures.
      ]. In spite of this predominantly objective approach, subjective seizures were not forgotten. Focal motor seizures represent one class juxtaposed with three classes of subjective seizures (somatosensory/special sensory; autonomic; psychic) two of which are further subdivided into 6 sub-classes each (Table 1).
      Table 1Focal (partial) seizures in the 1981 ILAE seizure classification.
      A) Simple focal (partial) seizures (consciousness not impaired)
       1. With motor signs
        a. Focal motor without march
        b. Focal motor with march (Jacksonian)
        c. Versive
        d. Postural
        e. Phonatory
       2. With somatosensory or special sensory symptoms
        a. Somatosensory
        b. Visual
        c. Auditory
        d. Olfactory
        e. Gustatory
        f. Vertiginous
       3.With autonomic symptoms or signs
       4. With psychic symptoms
        a. Dysphasic
        b. Dysmnesic
        c. Cognitive
        d. Affective
        e. Illusions
        f. Structured hallucinations
      B) Complex partial seizures
      C) Focal seizures evolving to secondarily generalized seizures
      This list appears as a fairly adequate representation of the categorical diversity of subjective seizure symptoms, and it was expected that the understanding of their anatomical significance, which in 1981 was rather insufficient in many instances, would improve over subsequent years [
      Commission on Classification and Terminology of the ILAE. Proposal for revised clinical and electroencephalographic classification of epileptic seizures.
      ]. Some work on the localizing value of auras was performed, e.g. by Palmini and Gloor [
      • Palmini A.
      • Gloor P.
      The localizing value of auras in partial seizures: a prospective and retrospective study.
      ], and more recently, the anatomical understanding of subjective symptoms has increased particularly through the meticulous semiological work of research groups involved in presurgical investigation, where the highest anatomical precision is mandatory. This, however, has hardly been noticed by the ILAE classification commission. Whereas, in the 2017 seizure classification [
      • Fisher R.S.
      • Cross J.H.
      • French J.A.
      • Higurashi N.
      • Hirsch E.
      • Jansen F.E.
      • et al.
      Operational classification of seizure types by the International League Against Epilepsy: position paper of the ILAE commission for classification and terminology.
      ] the classification of motor seizures has become more refined, the classification of subjective seizures was reduced to a more rudimentary level (Table 2), revealing an attitude towards the subjective half of epilepsy that is the opposite of what is promoted in this article.
      Table 2Focal seizures in the 2017 ILAE seizure classification.
      Focal onset
      Aware - Impaired awareness
      Motor onset
       Automatisms
       Atonic
       Clonic
       Epileptic spasms
       Hyperkinetic
       Myoclonic
       Tonic
      Nonmotor onset
       Autonomic
       Behaviour arrest
       Cognitive
       Emotional
       Sensory
      Focal to bilateral tonic-clonic
      This official document of the ILAE is unlikely to help the next generation of epileptologists realize the importance of patients’ subjective seizure experiences. This is alarming as the neglect of non-motor seizure symptoms has been shown to result in a 10-fold delay in diagnosis and a strongly increased risk of motor vehicle accidents [
      • Pellinen J.
      • Tafuro E.
      • Yang A.
      • Price D.
      • Friedman D.
      • Holmes M.
      • et al.
      Focal nonmotor versus motor seizures: the impact on diagnostic delay in focal epilepsy.
      ].

      2. Subjective symptoms as a therapeutic entry

      How do patients react to subjective symptoms and to the conscious perception of being in a seizure? Both are not the same. Patients who exclusively have subjective seizures (isolated auras) are often not aware that they are pathological at all. Many never talk about them with their doctor and, if they do, the doctor mostly does not recognize them for what they are. Isolated auras are probably most often diagnosed in retrospect when a first “complete” seizure has clarified the diagnosis [
      • Pellinen J.
      • Tafuro E.
      • Yang A.
      • Price D.
      • Friedman D.
      • Holmes M.
      • et al.
      Focal nonmotor versus motor seizures: the impact on diagnostic delay in focal epilepsy.
      ]. This is not new knowledge as it was already noted by Herpin in his seminal work “On Incomplete Seizures” [
      • Herpin Th
      Des accès incomplets d’épilepsie.
      ], and the character Kirillov, Dostoyevsky’s alter ego in his novel “Devils” of 1871/72, is an example of a person with frequent isolated auras who has no notion of their being anything pathological [
      • Wolf P.
      • Yacubian E.M.
      The epileptic experience in the works of Dostoyevsky and Machado de Assis.
      ].
      When the diagnosis has become clear, the subjective symptoms are recognized as the onset of an event that can evolve more seriously e.g. into loss of awareness, automatic behaviour, or convulsive seizures. At this point, the patient may already have the experience that he/she can to some extent influence the seizure development. In particular, many patients spontaneously detect and develop countermeasures to arrest seizures at the aura stage and report that these can be successful. It may often be objectively difficult to decide if a seizure stops spontaneously or in response to an effort to interrupt it, but when we talk about subjective experiences this is not the point.
      Self-invented countermeasures usually belong to one of three categories: general relaxation, concentration, and specific sensory or motor interventions related to the individual seizure symptoms [
      • Wolf P.
      Aura interruption: how does it become curative?.
      ]. Thus, since the era of Galen, patients and doctors knew that a focal motor seizure commencing in the periphery of a limb could be arrested by a proximal ligature, and a patient of Efron [
      • Efron R.
      The effect of olfactory stimuli in arresting uncinate fits.
      ] in a particularly well-documented case could arrest seizures with an olfactory aura by the application of a well-selected olfactory stimulus. One of the clearest distinguishing features in conversation analytic comparisons of seizure accounts of patients with epilepsy or dissociative seizures was that typically, patients with epilepsy spontaneously report attempts of counteracting seizures whereas patients with dissociative seizures do not [
      • Schwabe M.
      • Reuber M.
      • Schöndienst M.
      • Gülich E.
      Listening to people with seizures: how can linguistic analysis help in the differential diagnosis of seizure disorders?.
      ]. In most cases that have come to our knowledge, countermeasures succeed sometimes but not always. Occasionally, however, systematic application of seizure interruption methods may lead to complete seizure control without the need for drugs [
      • Wolf P.
      Aura interruption: how does it become curative?.
      ,
      • Efron R.
      The effect of olfactory stimuli in arresting uncinate fits.
      ] and probably, there are patients who completely control their seizures in this way, never see a doctor and are perhaps completely unaware that they have epilepsy. To reach the full possible benefit of seizure arrest, however, most patients need professional advice to optimize their intervention [
      • Wolf P.
      Aura interruption: how does it become curative?.
      ].
      However, not all patients try to counteract their seizures. Some will let them happen or even induce them. According to Fenwick [
      • Fenwick P.
      Psychogenic epileptic seizures and behavioural therapy of epilepsy.
      ], one of the few authors who have studied this aspect, 25 % of 76 patients attending the epilepsy clinic at Maudsley Hospital, London, “could generate their own seizures at will or knew what they had to do to bring a seizure on”, “allowing oneself the luxury of a mental state that you know is likely to induce a seizure”. Aura experiences may be fascinating - as very well described by the English writer Margiad Evans who integrated her unrecognized aura experiences in her literary work [
      • Wolf P.
      Margiad Evans (1909–1958): A writer’s epileptic experiences and their reflections in her work.
      ]. People who may have grown an innocent habit of enjoying and perhaps provoking isolated auras without knowing what they are may find it difficult to drop it once the diagnosis is revealed. They may be ashamed of this behaviour or consider it as too intimate to be spontaneously reported, but for the doctor it is highly important to know about this phenomenon as it may be useful or even necessary to include psychoeducative approaches in the therapeutic process. A good initiating question is whether the patients experience their symptoms as something negative, positive or neutral. The positive ones are the attractive ones that may ‘seduce’ a patient to induce and play with them.
      For other patients, subjective seizure symptom are so irritating and disabling that they even may become the target of epilepsy surgery if they are pharmacoresistant. The prejudice that isolated auras do not constitute a potential indication for surgery because they are “only” subjective events is ill-founded. It is up to the patient to decide if he can live with these symptoms or is willing to accept the potential risk of invasive procedures in exchange for the chance of becoming seizure free.

      3. The patient with epilepsy as the ‘insider’

      If epilepsy patients have any subjective seizure symptoms at all, they are the ones, and the only ones with any first-hand knowledge of these symptoms. What they know is not directly accessible to anybody else. This does not mean, however, that they are the best experts of their condition. To have first-hand experience is one thing, to understand its meaning is quite another. To reach an optimal understanding of epilepsy, the patient’s immediate experience and the doctor’s expert interpretation need to be combined. This often fails to happen either. Whereas it is a priori expected that the patient accepts and recognizes the doctor’s expertise, doctors are not always prepared to recognize and appreciate the patient’s insider status. Nor are they always aware of the necessity to create the kind of confidential space where the patient feels at ease and invited to report their experiences, especially the problematic ones and those that make them uneasy. Patients who have symptoms like visual illusions, episodes of depersonalization or derealization, out-of-body experiences or forced thinking often hesitate to talk about them for fear of being considered crazy; some are afraid that they are developing a psychiatric condition. One of them was the above-mentioned writer, Margiad Evans, in whose case, however, even her “craziest” experiences can be traced back to her seizure symptoms [
      • Wolf P.
      Margiad Evans (1909–1958): A writer’s epileptic experiences and their reflections in her work.
      ,
      • Evans M.
      The nightingale silenced and other late unpublished writings.
      ].
      Professional writers with epilepsy are particularly worth listening to since they are trained to express themselves in ways that are as differentiated and precise as possible. Of these writers, Evans is probably the one who wrote and reflected most about her seizures. She keenly felt and expressed that her inside experiences were unique and she had a lot to “describe for the doctors and then they will have something to work on”. She had a “desire to put into physicians' hands a book of clues to the sensations of such an epileptic as myself” and felt disappointed, misunderstood and despised when she was not met with the interest she felt was her due [
      • Wolf P.
      Margiad Evans (1909–1958): A writer’s epileptic experiences and their reflections in her work.
      ,
      • Evans M.
      The nightingale silenced and other late unpublished writings.
      ].
      The inside of epilepsy does not stop with the experience of seizures. It also includes all the consequences that epilepsy has for the patients’ lives, possibilities and more or less well-founded restrictions, discrimination and stigmatization but also understanding and help to cope with the condition, to surmount its challenges and achieve a full and rewarding life. These aspects of the patients’ experience, however, are more easily accessible for others. The exclusive inside experience is with the seizures.
      To conclude, in this age of video-diagnostics of seizures there is a disquieting risk that the entire subjective half of our patients’ seizure semiology is ignored, with potentially serious consequences. To understand subjective symptoms and adequately respond to them, patients need expert assistance. ‘Inside’ and ‘outside’ aspects of seizures are different but equally important. To get the full picture, both need to supplement each other.

      Declaration of Competing Interest

      The author has no conflict of interest to reveal.

      Acknowledgements

      This work received no funding.
      This essay is dedicated to my dear friend Esper Cavalheiro, initiator and infinitely inspiring director of the Latin American Summer School on Epilepsy (LASSE).

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