Michelle Bureau, Ozlem Cokar, Bruno Maton, Pierre Genton, Charlotte Dravet
significance and the prognosis concerning both epilepsy and neuropsychological development must be guarded. No reliable clinical or electroencephalographic (EEG) markers that can predict the outcome have been described. In a retrospective series of 10 patients, we found a peculiar EEG pattern seen across sleep stages, but not in the waking state in infants whose first seizures appeared before the age of 1 year, and which were mostly complex focal. In all cases, follow-up showed a favourable outcome with complete seizure remission and no cognitive impairment. The specificity of these EEG changes was 100%, but the sensitivity was lower, since they were not seen in some of the infants with the same favourable outcome. We discuss the clinical similarities between our patients and those cases reported earlier by other authors as benign, non-familial or familial focal epilepsies, in whom, however, no interictal abnormalities had been seen on the EEG. Such EEG changes are probably specific to benign, self-limited, early onset focal epilepsies.
Andrés García-Pastor, Pilar López-Esteban, Rosa Peraita-Adrados
in a patient with occipital seizures that appeared clinically as EN.
The subject, male, 70 years-old, was examined because of generalized tonic-clonic seizures, preceded by left cephalic version. These were controlled using i.v. PHT, but partial seizures persisted, which we recorded using video-EEG.
Clinically, we observed episodes of left conjugate deviation of the eyes, accompanied by horizontal nystagmoid movements, with a rapid leftward component and visual hallucinations. The patient did not lose consciousness. Ictal EEG: spike rhythm in the posterior right occipito-temporal region extending to adjacent and contralateral regions, followed by post-discharge of slow waves. The video-EEG was interpreted as partial oculo-clonic status epilepticus of right temporo-occipital origin. Cranial MRI: old, bilateral hemorrhaging occipital contusions associated with previous cranial injury. The picture persisted for two days, and disappeared with administration of CBZ 600 mg/d.
Our patient's nystagmus seemed to be related to the critical activity recorded in the right occipito-temporal region. The co-existence of visual hallucinations and the video-EEG correlation support this possibility. This phenomenon is probably due to epileptic activation of the cortical center of saccadic movements, with a rapid phase of nystagmus, contralateral to the focus, and a slow ipsilateral phase in association with a defect in the gaze-fixing system ("leaky neural integrator") [published with videosequences].
Piet H.A. Voskuil
as myoclonic jerks predominantly in the upper part of the body and the arms.
During video/EEG monitoring at the age of fifteen months, seizures provoked by the same tactile stimuli were recorded. For clinical and electroencephalographic reasons, these are best described as absence seizures (persistence of minimal myoclonic jerking). To our knowledge, such seizures at this age have not been documented before.
Treatment with antiepileptic agents was not initiated. Six months later the seizures stopped spontaneously and have not recurred. Two years later, psychomotor and behavioural development is normal [published with video- sequences].
Colin D. Ferrie, Michael Koutroumanidis, Shaun Rowlinson, Sue Sanders, C.P. Panayiotopoulos
It predominantly affects children of 3-6 years of age (13% of those with one or more non-febrile seizures). EEG shows great variability, with occipital, extra-occipital spikes or brief generalised discharges alone or in combination; it may also be consistently normal. Occipital spikes do not occur in one third of children. Despite the high prevalence of autonomic status epilepticus, the prognosis of Panayiotopoulos syndrome is usually excellent. Remission usually occurs within 1-2 years from onset, one third have a single seizure but 5-10% may have more than 10 seizures or a more prolonged course. Atypical evolutions with absences, atonic seizures and intellectual deterioration are exceptional; only two cases have been previously reported. We present a girl who initially had a prolonged autonomic status epilepticus typical of Panayiotopoulos syndrome, followed by seizures, with concurrent symptoms of Rolandic epilepsy. She then had an atypical evolution with atypical absences, absence status epilepticus, atonic seizures and mild impairment of scholastic performance. The case emphasises the close links between Panayiotopoulos syndrome and Rolandic epilepsy, both of which probably represent different clinical phenotypes of a maturational-related benign childhood seizure susceptibility syndrome [published with videosequences].
Fritz Leutmezer, Christoph Baumgartner
postictal hemiparesis in 1827, this critical period of time after seizure offset has received little attention in the literature.
In this article, we report some of the most important postictal signs, such as postictal hemiparesis, postictal nose wiping, postictal language dysfunction, postictal headache and postictal cognitive impairment. Summarizing our own experience of the postictal state in more than 160 patients with focal epilepsy, we conclude that postictal signs can provide reliable information for the localization of the seizure onset zone in patients with focal epilepsy. Since the lateralization and localization of the seizure onset zone has become a major issue for the presurgical evaluation of those patients, we argue that more attention should be paid to the postictal state during video EEG monitoring. Moreover, in an ambulatory setting, the postictal period and its related signs is usually the only part of an epileptic seizure that is accessible to the physician's examination and might therefore add some information to the differential diagnosis of paroxysmal events, such as epileptic or psychogenic seizures [published with videosequences].
Walter Fröscher, Ferdinand Keller, Heinrich Vogt, Günter Krämer