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mise à jour du
25 juillet 2002
J Child Neurol
Unusual variants of infantile spasms
Donat JF, Wright FS
Department of Pediatrics, Children's Hospital, Ohio State University School of Medicine, Columbus 43205
Yawning and seizures

The usual clinical symptoms of infantile spasms have been well characterized in earlier studies by analysis of video-electroencephalographic (EEG) data. Typical infantile spasms involve abrupt flexion or extension movements of the extremities, trunk, and neck. Contractions of the facial muscles, eye opening or closure, upward eye rolling, eyelid fluttering, or nystagmoid eye movements may be associated.Large series and review articles conceming infantile spasin patients have mentioned that fragmentary variants of these seizures may occur in some infantile spasin patients. Previous authors have emphasized that these seizures may be extremely subtle and may be missed during history taking and even during video-EEG evaluation, unless specifically looked for. Infantile spasm fragments that have been documented include head nodding, shoulder shrugging, and abdominal contractions. Infantile spasms characteristically occur in a series that crescendos and then decrescendos, and these fragmentary forms may occur at the onset and offset of the series.

Points that are important for the clinical diagnosis of infantile spasms but that have not been clarified in earlier reports include whether fragmentary forms occur in a series without typical infantile spasms and whether fragmentary forms occur as the only form of infantile spasms in some patients. Nodding spasms have been reported to occur in series. Holmes stated that eye movements could occur independently from infantile spasms and precede the development of infantile spasms by weeks. Whether the occurrence of fragmentary forms has any usefulness in predicting prognosis or outcome of treatment has not been commented on previously, except by Lombroso, who said that their significance was uncertain.

In this report, we describe fragmentary variants of infantile spasms that have not previously been reported, we describe their occurrence as series of seizures that did not include typical infantile spasms, and we describe their occurrence both in patients who did and who did not also have typical infantile spasms. We also discuss the physical similarities and differences between fragmentary and typical infantile spasms in patients who had both, recount when during the history of infantile spasms the variants were recorded, and describe how the variants in some patients appear to be modifications that result froin the effects of time or treatment on typical infantile spasms. [...]

Infantile Spasm Variant Found on Later EEG

Four patients had typical infantile spasms present on the first video-EEG evaluation and the variants present on video-EEGs performed 1 day (patient 9) or 1 to 3 months later (patients 8, 10, and 11). Patient 8 had both typical and variant infantile spasms present on the later EEG. In this patient, the variants, which consisted of a mouth opening or yawning movement, did not appear to be fragments of the patient's typical flexor infantfle spasms. The ictal EEG changes also differed: the typical infantile spasms were each associated with a generalized slow-wave transient, but the variants were each associated with a generalized decrement. Both types of infantile spasms responded to an increase in dose of ACTH. Patient 9 had the variants (facial grimace) but no longer had typical infantile spasms on a video-EEG performed for the purpose of a pyridoxine injection 1 day after the first EEG and after two doses of ACTH; the parents of this patient noted the grimace to be part of the generalized infantile spasms, which had disappeared. These episodes subsequently stopped. Patient 10, who had a neonatal cerebral infarct and asymmetrical hypsarrhythmia, had asymmetrical extensor infantile spasms that included a lateral head jerk and facial grimace on the first video-EEG. Variants that consisted of a lateral head jerk and grimace were recorded on video-EEG 1 month later, after treatment with valproic acid, and variants that consisted only of the grimace were recorded on video-EEG 2 weeks after that (Figure 5). At this time, clonazeparn was added and the seizures stopped. 13 Patient 11 had resolution'-of hypsarrhythmia and typical infantile spasms documented on a video-EEG performed 1 month after beginning ACTH treatment. However, ACTH was stopped 6 weeks later because of side effects. Two weeks later, variants (eye opening) were recorded on videoEEG at a tirne when the EEG still showed no interictal epileptiform activity and the parents had noticed no seizures. Treatment with valproic acid was begun, but typical infantile spasms and hypsarrhythmia were again recorded on an EEG 2 months later. Clonazeparn was added and the seizures resolved.


In most of our patients, the infantile spasm variants appeared to be fragments or limited expressions of more typical infantile spasms. In some of these patients, the variants coexisted with typical infantile spasms and probably represented natural variability in physical expression of infantile spasms. However, in some patients, the variants may have resulted from a change in level of consciousness, time, or medication that modified the clinical expression of the infantile spasms. The effect of level of consciousness was seen in patient 6, who had typical infantile spasms in the waking state but had variants during sleep that consisted of only part of the typical infantile spasm. Time appeared to modify typical infantile spasms in patient 4, who, by history, had generalized infantile spasrns that resolved spontaneously 2 weeks before our video-EEG demonstrated the variants, and by patient 5, who developed typical infantile spasms 3 weeks after the variants were recorded. These spontaneous changes suggest that the variants were a stage in the natural evolution of the infantile spasms.

Of particular interest are the two patients whose variants appeared to be modifications that resulted from treatment of typical infantile spasms. Increasingly restricted variant forms were recorded in patient 10 on two occasions during treatment before the seizures ceased. In patient 11, the variants were recorded on a video-EEG that was obtained after ACTH given for typical infantile spasms was stopped because of side effects. This was at a time when the parents thought the seizures were gone and before the interictal EEG showed a recurrence of the hypsarrhythmia pattern. These variants may have been an undetected residual type of infantile spasms that persisted after treatment with ACTH, or they may have heralded the recurrence of infantile spasms after ACTH was stopped. Patient 7 demonstrated a possible difference in therapeutic response between variants and typical infantile spasms: both types of infantile spasms initially coexisted, but onlv the variants persisted after treatment with clonazepam. The variants then resolved after treatment with ACTH.

In other patients, the infantile spasm variants appeared to be separate types of infantile spasms that were not fragments of typical infantile spasms. In patient 8, the infantile spasm variants appeared to be a type of infantile spasms that was different both clinically and electrographically from the saine patient's typical infantile spasms, although both types responded to the saine treatment. In patients 1, 2, and 3, the infantile spasm variants occurred with no previous or subsequent history of typical infantile spasms; unfortunately, due to death or lack of follow-up, video-EEG documentation of the subsequent course was not possible. Also, these patients were treated for infantile spasms after the videc>EEGs demonstrated the seizures to be infantile spasm variants, and therefore the natural historv was altered. The seizures in these patients were diagnostic dilemmas because the clinical descriptions of the seizures did not fit recognized seizure types.

This series of patients demonstrates how extremely subtle and clinically atypical infantile spasm variants can be. It is reasonable to assume that the lcinical significance of the variants is as great as that of typical infantile spasms. Failure to recognize or detect fragmentary variants may account for some of the patients reported to have hypsarrhythmia and electrodecremental episodes without clinical infantile spasms. It can also result in underreporting of the number of infantile spasms in patiéhts undergoing treatment, as cautioned by Kellaway et al. In addition, our experience suggests that medications may modify typical infantile spasms into fragmentary variants and that failure to detect these seizures can result in the erroneous conclusion that seizures have stopped when they have only been modified by medication. Infantile spasm. variants themselves do not appear to affect prognosis. However, their presence could result in a poorer outcome for infantile spasm patients because of failure to recognize and treat them. In our patients, the variants appeared to respond to the same group of medications that we use to treat typical infantile spasms, including ACTH, valproic acid, and clonazepam, although in several patients, a different medication or increase in dose was necessary.
We suggest that clinicians who diagnose and treat patients who have infantile spasms or who have EEGs that show hypsarrhythmia need to be aware of the wide variety and subtlety of variant infantile spasms. Parents should be educated to watch for residual fragments during treatment of infantile spasms. As a result of our experience with these patients, we now obtain video-EEG evaluations in all infants with undiagnosed serial or clustering episodes, however atypical the descriptions may be for infantile spasms. We also routinely perform follow-Up video-EEGs during treatment of infantile spasm patient.
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