Abstract
In 1945, Lennox was the first to describe the epileptic states mainly expressed by various degrees of consciousness disturbance, which have their onset in children who present epileptic absences correlated with ictal EEG patterns of spike-wave complex discharges at about 3 Hz. As the clinical picture seemed to be similar to an uninterrupted series of absences, this led to the definition “Petit Mal Status” (PMS). Many authors have subsequently reported that PMS can occur in epileptic subjects who have never presented absences (and even in subjects without a previous history of epilepsy) and that the related EEG pictures were characterised by paroxysmal generalized activity of various morphology, but hardly ever consisted of the continuous rhythmic spike-wave or polyspike-wave complexes at 3 Hz found in petit mal absences. Finally, in reporting the onset and recurrence of this condition typically in adults and the elderly, some authors have proposed the existence of a particular form of PMS (dependent on different types of pathologic factors and characterising a specific syndrome of this age) that is different from that of the “real PMS” typical of childhood and related to petit mal absences. This paper describes fifteen patients in whom the onset of the condition occurred at different ages, and who seem to exemplify the various possible clinical expressions of PMS, with the aim of making a contribution towards the better nosographic definition of this epileptic condition.
On the basis of our study, we sustain that the so-called PMS is a seizure type of Idiopathic Generalized Epilepsy which may appear at nearly all ages, and may occur in isolation or in association with other epileptic manifestations, but cannot itself be considered as characterising one or more age-dependent syndromes.
Sommario
Lennox per primo (1945) descrisse degli Stati Epilettici esprimentisi principalmente con un disturbo della coscienza di grado variabile, insorgenti in bambini che presentavano Assenze epilettiche, correlati con pattern EEG ictale di scariche di complessi PO a circa 3 Hz. Il quadro clinico sembrava pertanto poter essere assimilato al susseguirsi ininterrotto di Assenze, donde la definizione di “Petit Mal Status” (PMS). Successivamente molti Autori hanno segnalato che il PMS poteva ricorrere in soggetti epilettici che non avevano mai presentato Assenze o, addirittura, in soggetti senza precedenti di Epilessia, e che i quadri EEG correlati erano caratterizzati da attività pasossistiche generalizzate di varia morfologia, ma quasi mai costituiti da complessi PO e PPO ritmici continui a 3 Hz, come nelle Assenze del Piccolo Male. Alcuni Autori, infine, nel segnalare l'esordio e la ricorrenza di tale condizione tipicamente in età adulta e avanzata, hanno proposto la individuazione di una particolare forma di PMS, dipendente da fattori patologici di vario genere, caratterizzanti una sindrome specifica di questa età, diversa da quella del “vero PMS” tipico della infanzia e correlato con le Assenze del Piccolo Male.
In questo lavoro viene proposta la descrizione di quindici casi esorditi alle varie età, che sembrano paradigmatici delle varie possibili espressioni cliniche del PMS, allo scopo di contribuire ad una migliore definizione nosografica di tale condizione epilettica. Sulla base di questa esperienza gli Autori sostengono che il cosiddetto PMS si configura come un tipo di crisi dell'Epilessia Generalizzata Idiopatica, che può insorgere in quasi tutte le età della vita e presentarsi isolato ovvero associato ad altre manifestazioni epilettiche, senza che di per sè possa caratterizzare una o più Sindromi età-dipendenti.
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Tiacci, C., D'Alessandro, P., Cantisani, T.A. et al. So-called “Petit Mal Status”: Epileptic syndrome or seizure type?. Ital J Neuro Sci 16, 279–294 (1995). https://doi.org/10.1007/BF02249103
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DOI: https://doi.org/10.1007/BF02249103