Absence status epilepticus: The first documented case?

Absence status epilepticus: The first documented case?
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   Epilepsia,  48 (Suppl. 8):4–5, 2007doi: 10.1111/j.1528-1167.2007.01334.x HISTORICAL VIGNETTE Absence status epilepticus: The first documented case? ∗ Peter Wolf,  † Eugen Trinka, and  † Gerhard Bauer  ∗ Danish Epilepsy Centre, Dianalund, Denmark;  † University of Innsbruck, Innsbruck, Austria Even the oldest texts on epilepsy indicate awareness of seizures occurring in series. In the story of the epilepticboy in the Gospel of St. Mark, Jesus returns to his disci-ples from a short period away and finds them dealing withan epileptic boy whom they could not help. This impliesthe occurrence of repetitive seizures. Two more seizuresat short interval follow in the presence of Jesus who thensuccessfully expels the disease demon. It is unknown, how-ever, to what extent this description of a status epilepticus(SE) is based upon real events.The first historical case of SE seems to be documentedon an ex-voto table of 1501 in the parish church of Gm¨und in Austria (Fig. 1). The neatly written text says:“Oswalt  . . .  ain purg – zu gmund ist vor vı jar  – nzu – alt – n¨oting gbes – n mit seine – sun der hinfalt – nsiechtu – het ain wenich gepessert & hat ersup im f¨ur gnome – nymer gen alt – n ¨oting kume – . Nun in der woche – nach oculi im 1501 Jar in grosse kranckhait gfalle – das er gleg – n ist mit offen augen unrednt pis an de – dritte – tag undvil volk zu im gangen. Niemant erkent noch gsehe – undseines leben gar verzigen hab – n also hat in sein hausfrwher versprochen mit aine – priester und mit aine – waxenhawbt und ist gesund worden, sag er lob und danck der Junckfraw mari – in ebikait. Und ist hie gebest am suntagvor dem auffertag Im 1501 Jar. & [signature]” (“Oswalt  . . .  ,acitizenofGm¨ und,went6yearsagototheold  ¨ Otting with his son who has the falling sickness, waslittle improved and he resolved never again to take to old ¨ Otting. Now, in the week after Oculi in the year 1501, fellinto great illness that he lay with open eyes speechless untilthe third day and many people came to him, did not recog-nize or see anybody, and they lost all hope for his life. Sohis wife engaged him here with a priest and a waxen head,and he recovered. Say honour, praise and thanks to the Vir-gin Mary in eternity. And this happened here on the Sun-day before Ascension Day in the year 1501”. – Alt¨ otting or “the old  ¨ Otting” is to this day a very popular pilgrimagein Bavaria). The painting appears to be the work of a local artist whomay well have seen the patient in the acute phase. It shows Address correspondence and reprint requests to Professor Peter Wolf,Danish Epilepsy Centre, Kolonivej 1, DK—4293 Dianalund, Denmark.E-mail: peterw@vestamt.dkBlackwell Publishing, Inc. C  International League Against Epilepsy Figure 1. Epilepsia  C  ILAE a young man prostrate on a bed with his eyes open in ablank stare, a young woman at his side with her handsfoldedinprayer,andawell-cladoldercouple(theparents?)at the foot end of his bed, the man seen from the front look-ing very concerned, the woman from behind talking agitat-edly to him. In the air above the bed appears the image of a statue of the Holy Virgin with the Child Jesus (probablythe statue in the parish church).The text is detailed, but somewhat condensed. It is, thus,not clear if the wife who takes action is the wife of Oswalt(i.e., the father) or of the young man himself. Grammati-cally, the latter seems to be indicated, and the woman inprayer at his bedside could very well be his wife. However,his dependence and the fact that his own name is never mentioned, make this perhaps less likely.4  5  Absence Status Epilepticus A similar ambiguity concerns the acute medical condi-tion and its possible diagnosis. If the description in thetext is meant to describe the “great illness,” the probablediagnosis would be one of nonconvulsive SE. But it couldalso be that the term “great illness” was considered to beself-explaining as signifying SE, and the description wouldthen be one of a stupor   following  convulsive SE and lastingthree days.Models of the diseased body part in valuable materialslike wax or silver were typical votive gifts. The present of a waxen head to the church shows that the family, a prob-ably well-educated middle-class family with the status of citizens, was fully aware that epilepsy is a condition srci-nating in the brain. The hope and belief in miraculous curesthat is visible both in the previous pilgrimage and the emer-gency action, does not indicate an assumption of supernat-ural causes but expresses the persons’ piety.A special feature in this case—which makes it of suchinterest—is that there is reason to assume that this is an ex-ample of absence (nonconvulsive) SE or a postconvulsivestupor in a person with a diagnosis of idiopathic general-ized epilepsy.The ex-voto was brought to our attention by a descen-dant of Oswalt (the family name is obliterated to protectprivacy) whose daughter was seen by one of us (GB). Her diagnosis is childhood absence epilepsy with several ab-sences per day since age 10 and a first generalized tonic– clonic seizure (GTCS) at age 14, normal neurological find-ings and normal MRI, and an EEG displaying typical 3 Hzgeneralized spikes and waves, some with clinical absence.The father himself, aged 55, has also sporadic GTCS, andprobable absence seizures during childhood and adoles-cence (although the latter were not diagnosed nor treatedby antiepileptic drugs). The epilepsy of both the father and the daughter have followed a very mild, but persistentcourse even despite proper treatment. The family possessesa family tree and pedigree that identifies Oswalt as one of their ancestors. In this case, therefore, a genetic epilepsyhas been documented since the beginning of the 16th cen-tury, with seizures in the case 500 years ago which are sim-ilar to those observed today. Aside from the historical di-mension, this description of the family provides fascinatingevidence of a strong genetic preservation of the syndromiccategory over the generations without any deterioration.  Epilepsia ,  48 (Suppl. 8):4–5, 2007doi: 10.1111/j.1528-1167.2007.01334.x
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