Benign myoclonus of early infancy or Fejerman syndrome
Article first published online: 7 MAY 2009
© 2009 International League Against Epilepsy
Volume 50, Issue 5, pages 1290–1292, May 2009
How to Cite
Bernardina, B. D. (2009), Benign myoclonus of early infancy or Fejerman syndrome. Epilepsia, 50: 1290–1292. doi: 10.1111/j.1528-1167.2009.02154.x
- Issue published online: 7 MAY 2009
- Article first published online: 7 MAY 2009
Benign myoclonus of early infancy (BMEI) was first described in 1976 by Natalio Fejerman and presented as a preliminary communication of 10 patients in a southern Latin American meeting. The paper was published in 1977 in the Proceedings of the above-mentioned child neurology meeting (Fejerman, 1977a), in a local journal (Fejerman, 1977b), and the cases were described in the first edition of the book Convulsiones en la Infancia (Fejerman & Medina, 1977). A report including six more cases collected by Cesare T. Lombroso also appeared in 1977 (Lombroso & Fejerman, 1977). Several years later, I took part in a collaborative study reporting four new cases registered with polygraphic ictal recordings, and we interpreted the motor paroxysmal phenomena as spasms. We proposed to name the syndrome benign nonepileptic infantile spasms (BMEI; Dravet et al., 1986). We observed that the motor manifestations included myoclonic jerks, spasms, brief tonic contractions, and shuddering. Fejerman also described one case with atonic head-drops in his first series (Fejerman, 1977a, 1977b).
Pachatz et al. (1999) reported five cases, all monitored with video-EEG (electroencephalography) and polygraphic recordings in three, and defined the motor events as “a shudder type, paroxysmal motor manifestation involving mainly the trunk and sometimes the head, associated with tonic limb contractions of variable intensity….” Obviously, in these cases the ictal EEG was also normal. Shuddering was also considered a main feature of BMEI by Kanazawa (2000).
The present contribution of Caraballo et al. (2009) documents very well how wide the spectrum of the clinical and neurophysiologic motor manifestations characterizing BMEI can be, and how variable these manifestations can be even in the same subject depending on vigilance and posture and the stage of development of the child. In our experience, for example, the coexistence in the same subject of quite different types of motor manifestations is even more frequent than that reported in this paper. Furthermore it is not exceptional to observe the recurrence of motor manifestations in clusters with a pseudo-periodic–type pattern similar to the clusters in epileptic spasms. It has been repeatedly emphasized, on the other hand, that this syndrome is the main differential diagnosis of West syndrome (WS; Maydell et al., 2001; Fejerman, 2008). Idiopathic and cryptogenic West syndrome affects babies of the same age range and is the most frequent epileptic encephalopathy, with onset during the first year of life. In infants with epileptic spasms in clusters without hypsarrhythmia the differential diagnosis is, of course, more difficult (Caraballo et al., 2003).
Considering the variable motor manifestations of BMEI, the use of a term evoking only one of them (benign myoclonus or benign nonepileptic spasms) seems at this point restrictive and misleading. In addition, the use of terms such as benign myoclonus of early infancy or benign nonepileptic spasms creates a risk of ambiguity in considering the differential diagnosis with benign myoclonic epilepsy of infancy and infantile spasms. It, therefore, seems desirable to use a new term to define this condition that must be considered a nonepileptic paroxysmal motor manifestation characterized by variable motor manifestations.
Accordingly, and considering that Dr. Natalio Fejerman has demonstrated a continuous interest in following up these cases for more than 40 years (Fejerman, 1984; Fejerman & Caraballo, 2002; Caraballo et al. (2009) and that Dr. Fejerman’s name has already been attached to this condition in several publications by authors from Argentina, France, and Italy (Gobbi et al., 1982; Beltramino, 1987; Pachatz et al., 2002), I propose that this syndrome is formally given the eponymic title of Fejerman syndrome.
I confirm that I have read the Journal’s position on issues involved in ethical publication and affirm that this report is consistent with those guidelines.
Disclosure: I have no conflicts of interest to disclose.
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