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Absence status epilepticus: The first documented case?

2007; Wiley; Volume: 48; Issue: s8 Linguagem: Inglês

10.1111/j.1528-1167.2007.01334.x

1528-1167

P.J. van der Wolf, Eugen Trinka, Gerhard Bauer,

Infant Health and Development

Even the oldest texts on epilepsy indicate awareness of seizures occurring in series. In the story of the epileptic boy in the Gospel of St. Mark, Jesus returns to his disciples from a short period away and finds them dealing with an epileptic boy whom they could not help. This implies the occurrence of repetitive seizures. Two more seizures at short interval follow in the presence of Jesus who then successfully expels the disease demon. It is unknown, however, to what extent this description of a status epilepticus (SE) is based upon real events. The first historical case of SE seems to be documented on an ex-voto table of 1501 in the parish church of Gmünd in Austria (Fig. 1). The neatly written text says: “Oswalt … ain purg–zu gmund ist vor vı jar–n zu–alt–nöting gbes–n mit seine–sun der hinfalt–n siechtu–het ain wenich gepessert & hat ersup im für gnome–nymer gen alt–n öting 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 und vil volk zu im gangen. Niemant erkent noch gsehe–und seines leben gar verzigen hab–n also hat in sein hausfrw her versprochen mit aine–priester und mit aine–waxen hawbt und ist gesund worden, sag er lob und danck der Junckfraw mari–in ebikait. Und ist hie gebest am suntag vor dem auffertag Im 1501 Jar. & [signature]” (“Oswalt …, a citizen of Gmünd, went 6 years ago to the old Ötting with his son who has the falling sickness, was little improved and he resolved never again to take to old Ötting. Now, in the week after Oculi in the year 1501, fell into great illness that he lay with open eyes speechless until the third day and many people came to him, did not recognize or see anybody, and they lost all hope for his life. So his wife engaged him here with a priest and a waxen head, and he recovered. Say honour, praise and thanks to the Virgin Mary in eternity. And this happened here on the Sunday before Ascension Day in the year 1501”. – Altötting or “the old Ötting” is to this day a very popular pilgrimage in Bavaria). The painting appears to be the work of a local artist who may well have seen the patient in the acute phase. It shows a young man prostrate on a bed with his eyes open in a blank stare, a young woman at his side with her hands folded in prayer, and a well-clad older couple (the parents?) at the foot end of his bed, the man seen from the front looking very concerned, the woman from behind talking agitatedly to him. In the air above the bed appears the image of a statue of the Holy Virgin with the Child Jesus (probably the 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. Grammatically, the latter seems to be indicated, and the woman in prayer 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. A similar ambiguity concerns the acute medical condition and its possible diagnosis. If the description in the text is meant to describe the “great illness,” the probable diagnosis would be one of nonconvulsive SE. But it could also be that the term “great illness” was considered to be self-explaining as signifying SE, and the description would then be one of a stupor following convulsive SE and lasting three days. Models of the diseased body part in valuable materials like wax or silver were typical votive gifts. The present of a waxen head to the church shows that the family, a probably well-educated middle-class family with the status of citizens, was fully aware that epilepsy is a condition originating in the brain. The hope and belief in miraculous cures that is visible both in the previous pilgrimage and the emergency action, does not indicate an assumption of supernatural causes but expresses the persons' piety. A special feature in this case—which makes it of such interest—is that there is reason to assume that this is an example of absence (nonconvulsive) SE or a postconvulsive stupor in a person with a diagnosis of idiopathic generalized epilepsy. The ex-voto was brought to our attention by a descendant of Oswalt (the family name is obliterated to protect privacy) whose daughter was seen by one of us (GB). Her diagnosis is childhood absence epilepsy with several absences per day since age 10 and a first generalized tonic–clonic seizure (GTCS) at age 14, normal neurological findings and normal MRI, and an EEG displaying typical 3 Hz generalized spikes and waves, some with clinical absence. The father himself, aged 55, has also sporadic GTCS, and probable absence seizures during childhood and adolescence (although the latter were not diagnosed nor treated by antiepileptic drugs). The epilepsy of both the father and the daughter have followed a very mild, but persistent course even despite proper treatment. The family possesses a family tree and pedigree that identifies Oswalt as one of their ancestors. In this case, therefore, a genetic epilepsy has been documented since the beginning of the 16th century, with seizures in the case 500 years ago which are similar to those observed today. Aside from the historical dimension, this description of the family provides fascinating evidence of a strong genetic preservation of the syndromic category over the generations without any deterioration.