We will present a clinical case of a boy diagnosed with seizures manifested by atypical symptoms, with ictal vomiting initially, and then unilaterally blinking. The epileptogenic zone was highlighted by video-EEG recording in the right temporal lobe, secondary to a pilocitic astrocytoma. The tumor was surgical excised completely and the symptoms disappeared. This is the first case published with the association of ictal vomiting and unilaterally blinking.
Epileptic seizures represent an excessive neuronal discharge secondary to a genetic predisposition or to a morphological structural abnormality of the neural cortex. Depending on the area within the epileptogenic focus, patients diagnosed with epilepsy have different ictal events, from motor to sensory, autonomous or just mental. Ictal vomiting is a symptom which appears rarely either in a cryptogenic or symptomatic focal epilepsy (most frequently in the non-dominant temporal lobe), or in an idiopathic epilepsy as the occipital lobe (Panyiotopoulos’s type). Thus, neither in terms of etiology nor in location of epilepsy, ictal vomiting isn’t very helpful in classification of epilepsy with these symptoms (1,2).
Also, unilateral blinking on the same side as focus is a rare ictal phenomenon (only 1.5% of all patients diagnosed with epilepsy). Pathogenic mechanism is unknown but is regarded as secondary to brain activation or cerebella connections on the same side. We reported a case of non-dominant temporal focal epilepsy manifested with both rarely ictal symptoms, initially
with vomiting, then with unilaterally blinking (3).
Mario is a four years and six months old boy with normal intellect, who was hospitalized for the first time in the pediatric neurology clinic at the age of 4 years and 2 months for the appearance of paroxysmal events – blinking with the duration between seconds and minutes, and if the symptoms are longer, he would have had vomiting.
Psychomotor development was normal, personal physiological and family history was insignificant.
Somatic examination showed a low weight and height for age, otherwise normal cardio-respiratory and digestive, neurological and psychological examination.
Mario’s mother tells us that from the age of two months, the child had repeated vomiting periods, apparently without any cause, not very common, one episode/several months. In the last two months these symptoms had a higher frequency, he was vomiting daily, 10-15 times per day, both in awaking and in sleep period.
Affirmative, the child lately dropped weight (about 5 pounds in two months).
Mario came to our clinic not for the evaluation of vomiting, but for blinking episodes, in awaking and in sleep period which started two weeks ago.
If blinking episodes had a longer duration they were accompanied by vomiting.
At this stage, after anamnesis and clinical assessment, vomiting was considered secondary to a digestive impaired either structural as in hypertrophy pyloric stenosis, or infectious. The parents didn’t give other information related to blinking so at the moment of admission in the clinic the diagnosis was simple motor tics (blinking).
Long time video-EEG monitoring was recommended to record the events and their details.
There was a period of 8 hours of awaking and sleep which captured four paroxysmal episodes, two from sleep and two from wakefulness: one with vomiting and blin-king, two only with blinking and only one vomiting.
Video recording showed that Mario wasn’t blinking bilateral but only with the right eye, apparently without affecting the consciousness, without any other motor signs, the first event lasted less than two minutes, and the child also vomited (picture 1).
Postictal vomiting suspicion arose, but because the episode with the isolated vomiting was together with other similar epileptiform EEG’s discharges, it was diagnosed as ictal vomiting.
Interictal EEG showed an inter-hemispheric asymmetry, with slower rhythm intricate with epileptiform dischanges, sharp-wave’s type in the parietooccipital derivations both left and right (picture 2).
The EEG from the presentation is during sleep. Ictal EEG showed complex epileptiform discharges peakwave type parieto-temporal right (picture 3).The conclusion of EEG’s recording was abnormal trace with elements of the right lesion intricate with posterior right epileptiform focus.
After registration, there were issues about lateralizing phenomena versus epileptogenic focus localization.
Interictal electroencephalographic recording showed bilateral changes, but ictal EEG showed epileptiform focus in the right posterior derivations. Considering the symptoms, blinking was on the same side as epileptiform focus and the ictal vomiting as clinical element doesn’t help us to recognize the onset of epileptiform changes. Antiepileptic treatment with carbamazepina 20 mg/ kg/day was introduced, with favorable evolution, with the remission of the symptoms.
To fit this epilepsy in a syndrome, brain imaging investigation was recommended, initially cerebral computer tomography (brain CT). It showed a right temporal lesion, which didn’t enhance, but with calcifications in the periphery (picture 4). To further clarify the type of lesion, cerebral MRI was performed which showed a right mesial temporal tumor with hypersignal in T2 and Flair, without enhancement (brain MRI) (picture 5, picture 6).
Fig. 1. Intercritic EEG
Fig. 2. Intercritic EEG
Imagistic aspect of brain tumor lesion, without enhancement, without edema, with calcification in the periphery, suggested a benign structure with slowly progressive evolution.
Therefore, at this point, raise the question if the lesion is responsible for epileptic seizures or there is an epileptic focus in another brain area, and the tumor excision of this patient with epilepsy controlled by medication and without any other symptoms, will bring any benefit.
Initially, for two weeks, the seizures were completely controlled, but then they reappeared, at which time the child has undergone surgery with the excision of the lesion and complete excision. Pathological diagnosis was pilocitic astrocytoma (picture 7). We mention that Mario is right handed, right temporal lobe was non-dominant and there were not performed other presurgical investigations as for cognitive evaluation or for language areas.
He didn’ had apparent deficiency, but there were no post surgical neuro-cognitive tests performed. He never repeated any seizure, but antiepileptic treatment with carbamazepine was still recommended for two years.
Our patient had symptoms that began at the age of infants with vomiting. Vomiting in children is a common symptom, secondary to digestive diseases (as gastro esophageal reflux, esophageal foreign body, rumination, gastrointestinal obstruction, foreign bodies, hypertrophy of the pyloric stenosis, intestinal malrotation, +/- volvulus, Meckel diverticulum, invagination, ascariasis, intestinal hernia, Hirschprung disease, diaphragmatic hernia, allergies to cow’s milk protein, soy, gastroenteritis, celiac disease, peritonitis, paralytic ileus), respiratory diseases (reactive airways disease, respiratory infections, foreign body) or metabolic (metabolic acidosis, galactosemia, fructose intolerance, lactose intolerance, adrenal insufficiency, hypercalcemia), drug intoxication (aspirin, theophylline, digoxin, D vitamin), infectious (hepatitis), or kidney disease (obstructive uropathy, urinary infections, kidney failure) or ENT diseases. Neurological disorders can manifest with vomiting, most often as part of the syndrome of intracranial hypertension secondary to brain tumors, cerebral edema, hydrocephaly or infections.
Fig. 4. Brain CT
Fig. 5. Brain MRI
Vomiting associated with seizures is frequently postictal secondary to intracranial hypertension syndrome caused by edema appeared after seizures (4).
When it appears ictal as singular phenomenon is extremely rare, so the video-EEG recording of crisis can help for the location of epileptiform focus and lateralization. Most authors have described as a starting point the non-dominant mesial temporal lobe, but cases have been described with onset in dominant temporal lobe (5,6).
The etiology of epilepsy was generally as a cerebral lesion, secondary to benign tumors. Penfield and Jasper showed that intestinal peristaltic activity appeared secondary to insular cortex stimulation, suggesting that this region is associated with viscero-motor function accompanied by vomiting (7,8).
Autonomous epileptic seizures are the main events in Panayiotopoulos syndrome, in which the main symptom is vomiting, but it is accompanied by others symptoms as pallor, flushing, mydriasis, cardio respiratory abnormalities or modifications of thermoregulation, sphincter incontinence. Seizures in this syndrome tend to be prolonged as status epilepticus in small children and are accompanied by unanswered and unconsciousness (9). Epilepsy syndrome is benign both in terms of seizure frequency and in the evolution in time, being framed in idiopathic epilepsies, and is not secondary to brain lesions.
Fig. 6. MRI with contrast
Fig. 7. Pilocitic Astrocytoma
Mario, having a lesion observed at imagistic investigation and being compatible with the interictal and ictal electroencephalographic changes, and with ane EEG trace with mixed modifications both lesion and right posterior epileptiform discharges, has common elements with the data described in the literature so far, namely ictal vomiting secondary to a non-dominant temporal focus.
We mention that the onset of symptoms was early, around the age of 2 months and probably also for epileptic seizures, which were not diagnosed at that time.
Because the lesion was benign, it evolved very slowly and the diagnosis was possible only when critical aspect changed with unilaterally blinking (10). Ictal unilateral blinking was differentiated from tics in that it was unilateral, either in waking, and sleeps or from Marcus- Gunn syndrome because it was not associated with other movements during mastication for example.
Ictal unilateral blinking was described in literature for the first time by Benbadis on 14 cases of patients with epilepsy manifested only with ictal sign. Crisis onset was ipsilateral in 10 cases, nine were from left hemisphere (3). The author assessed as positive predictive value for ipsilateral lateralization of the focus at a rate of 83%, but could not determine the exact location of the focus, saying it would more probably to be frontal. Lesser and collaborators reported that they managed to trigger unilateral blinking by structures subdural cortical stimulation and raised the hypothesis of basal hemispheric activation of the trigeminal fibers in the intracranial portion of the trigeminal or facial nerve secondary to local ictal vascular changes (11).
Particularity of the case
This case is the first one described in literature with the association of two atypical ictal elements, initially only vomiting, then unilateral blinking secondary to a non-dominant temporal benign tumor. Although, positive diagnosis in child neurology pathology and especially in epilepsy is based only on historical data, in this case the registration of paroxysmal events guided us to a clear diagnosis in a small child to which tics were very likely. However, the fact that occurred in sleep must raise the suspicion of other causes of these paroxysmal events and when we saw the clinical data with only one side blinking the suspicion of epileptic seizures was very high.
This presentation is another example showing that diseases from pediatric neurology specialty may contain all the signs and symptoms of general pediatrics for the fact that all body functions are controlled or get the information to the central nervous system.
- Benbadis SR, Kotagal P, Klem GH. Unilateral blinking: a lateralizing sign in partial seizures. Neurology 1996; 46: 45-8.
- Devinsky O, Frasca J, Pacia SV, Luciano DJ, Paraiso J, Doyle W: Ictus emeticus: Further evidence of nondominant temporal involvement. Neurology 1995;45:1158-1160.
- Fiol ME, Leppik IE, Mireles R, Maxwell R: Ictus emeticus and the insular cortex. Epilepsy Res 1988;2:127-131.
- Howell SJL: Ictus emeticus (letter). Neurology 1989; 39:457-458.
- Jacome DE, FitzGerald R: Ictus emeticus. Neurology 1982;32:209-212.
- Kramer RE, Luders H, Goldstick LP, Dinner DS, Morris HH, Lesser RP, Wyllie E: Ictus emeticus: An electroclinical analysis. Neurology 1988;38:1048-1052.
- Lesser R.P, Luders H., Klem G.: Ipsilateral trigeminal sensory responses to cortical stimulation by subdural electrodes; Neurology 1985; 35: 1760-3;
- Mitchell WG, Greenwood RS, Messenheimer JA: Abdominal epilepsy: Cyclic vomiting as the major symptom of simple partial seizures. Arch Neurol 1983;40:251-252.
- Panayiotopoulos CP: Vomiting as an ictal manifestation of epileptic seizures and syndromes. J Neurol Neurosurg Psychiatry 1988;51:1448-1451.
- Penfield W, Faulk ME Jr: The insula: Further observations on its function. Brain 1955;78:445-470.
- Wada JA. Unilateral blinking as a lateralizing sign of partial complex seizure of temporal lobe origin. In: Wada JA and Penry JK, eds. Advances in epileptology, Xth Epilepsy International Symposium. New York: Raven Press, 1980: p. 533.