These Authors contributed equally.
A Case with Atypical Childhood Occipital Epilepsy “Gastaut Type”: An Ictal Migraine Manifestation with a Good Response to Intravenous Diazepam
Article first published online: 17 AUG 2007
Volume 48, Issue 11, pages 2181–2186, November 2007
How to Cite
Parisi, P., Kasteleijn-Nolst Trenité, D. G., Piccioli, M., Pelliccia, A., Luchetti, A., Buttinelli, C. and Villa, M. P. (2007), A Case with Atypical Childhood Occipital Epilepsy “Gastaut Type”: An Ictal Migraine Manifestation with a Good Response to Intravenous Diazepam. Epilepsia, 48: 2181–2186. doi: 10.1111/j.1528-1167.2007.01265.x
This work was supported by the Marie Curie Grant MEXC-CT-2005-24224: Visual Sensitivity.
- Issue published online: 17 AUG 2007
- Article first published online: 17 AUG 2007
- Accepted July 7, 2007; Online Early publication August 17, 2007.
- Occipital epilepsy;
- Autonomic status epilepticus;
- Intermittent photic stimulation;
- Visual sensitivity
We report the history of a 14-year-old girl with atypical childhood occipital epilepsy “Gastaut type” whose first generalized tonic–clonic seizure was preceded by migraine without aura and followed by a status migrainosus. This status lasted for 3 days despite standard analgesic therapy. An EEG recording revealed an occipital status epilepticus during her migraine complaints. Seven minutes after intravenous administration of 10 mg diazepam under continuous EEG recording, a suppression of the epileptiform discharges over the right occipital was seen, while the headache subsided 3 min later. After precise questioning about the circumstances that possibly could have led to these events, it appeared that she had played for hours with a play station on the new color TV and she had visited an exhibition of Matisse and Bonnard with bright colors and contrast-rich text. Standardized extensive intermittent photic stimulation (IPS), 2 days after the status migrainosus, evoked besides asymmetrical right-sided driving, green spots in her left visual field, while in the EEG sharp waves were recorded over the right parietotemporal region. After further IPS with 20 Hz (eye closure), she started complaining of a light pulsating headache right occipitally and in the EEG right parietotemporal sharp-waves were seen. This lasted for about 10 min. Later, an interictal routine EEG was normal except for some theta over the right temporooccipital area. The most likely diagnosis is an atypical form of occipital epilepsy “Gastaut type.” We would therefore advocate recording EEGs with photic stimulation in patients with atypical migraneous features.
A 14-year-old girl was admitted to the first aid because of a generalized tonic–clonic seizure in the school bus after having visited the exhibition of Bonnard and Matisse in Rome. Before this excursion she had video gamed for hours on the new home TV (flat screen with bright colors and contrast). This had given her headache on the right backside of the head without any other symptoms or signs lasting about 3 h before the seizure onset. Her clinical history revealed only sporadic (2–4/year) migraine attacks without aura. Moreover, she has never had any visual disturbance or vertigo. The tonic–clonic seizure started with elevation of the left arm and head deviation to the right, followed by loss of consciousness with cyanosis and massive jerking. Examination in the hospital (post-ictally) revealed no abnormalities except anisocoria (left > right) and persistence of her headache complaints. MRI of the brain, with IR and FLAIR sequences, was normal. The headache did not resolve spontaneously and continued even for 3 days with nausea, repeated vomiting, phonophobia and photophobia. Paracetamol (15 mg/kg) and ibuprofen (10 mg/kg) consecutively administered per os, nor oxygen 10 L per minute dissolved the headache. It was then decided to record an EEG, which showed, especially with eyes closed, subcontinuous theta activity intermingled with sharp waves of high amplitude over the right occipital region (Fig. 1). During hyperventilation (HV) these anomalies tended to become more generalized. After informed consent was given by both parents and the patient, 10 mg slow (administration lasted about 5 min) intravenous diazepam was subsequently administered. After 7 min the right occipital anomalies disappeared completely and symmetrical alpha rhythm could now be seen (Fig. 2); 3 min later she had no more migraine complaints.
IPS with a Grass PS 33 photic stimulator with eyes open and eyes closed, 12 min after the diazepam injection, evoked a clear driving response on the right side. The girl became more and more drowsy and slept for the rest of the afternoon. A control EEG including HV and IPS 5 h later while still being without headache, was normal except for the again strong asymmetrical driving response on the right side. She was then dismissed from the hospital without further medical treatment.
Two days later, a long-term video-EEG registration was made with HV and extensive visual stimulation, in accordance to the international protocol (Kasteleijn-Nolst Trenité et al., 1999): symmetrical alpha- rhythm of 8–9 c/s could be seen with some spike-and-waves, and sharp waves over the right temporoparietal area and propagation to the left side (delta waves). HV enhanced the anomalies. The anomalies were however lower of voltage and far less frequent than in the period with the headache complaints. With eyes closed, and open in darkness, these epileptiform phenomena were more evident than with eyes open in normal room lighting.
A driving response at the right occipital area was seen during IPS between 5 and 18 Hz. IPS between 2 and 12 Hz, and between 30 and 60 Hz suppressed the epileptiform discharges, while those between 18 and 25 Hz did increase them. During stimulation with 40 Hz in the eye closure condition she saw green spots in her left visual field, while in the EEG sharp waves were recorded over the right parietotemporal region. No neurological abnormalities were found and the spots disappeared rather soon. After further IPS with 20 Hz (eye closure), she started complaining of a light pulsating headache right occipitally and in the EEG right parietotemporal sharp-waves were seen (Fig. 3). This lasted for about 10 min.
The interictal EEG, recorded 4 months later, showed a normal background with sporadic bursts of high voltage theta waves over the temporooccipital area (Fig. 4).
This adolescent girl was send to the child neurologist because of long-lasting migraine complaints. Surprisingly she did not react to current antimigrainous therapies. Although no clinical signs or symptoms like loss of vision or visual hallucinations could be discovered that could point towards an epileptic origin of her long-lasting headache, we decided to record an EEG and found continuous epileptiform abnormalities over the occipital area, that could however also be considered as an migrainous EEG manifestation (Beaumanoir, 1993; De Romanis et al., 1993).
In order to be sure it was an epileptic event, we decided to give diazepam intravenously with complete suppression of the EEG abnormalities (and headache complaints) as a result.
To our knowledge this case report proves, for the first time, that migrainous complaints can be suppressed by intravenous benzodiazepines and can thus in some cases be considered as an ictal epileptic phenomenon.
This is in line with the hypothesis of transient cortical excitability as a possible mechanism underlying both epilepsy and headache (Chronicle et al., 2006; Calabresi et al., 2007). Especially the Na+–K+ ATPase pump may play a role in regulation of both seizure susceptibility by acting on membrane depolarization and influencing Cortical Spreading Depression (CSD) (Haglund and Schwartzkroin, 1990) which is considered as primum movens for the trigemino-vascular system activation in the headache physiopathogenesis (Ayata et al., 2006). Moreover, a Na+–K+ ATPase pump novel mutation associated with both Familial Hemiplegic Migraine (FHM) and Benign Familial Infantile Convulsions (BFIC) has been described (Vanmolkot et al., 2003).
Migraine as “autonomic epilepsy” was already suggested as such by Lennox WF (Lennox, 1960). Later publications with ictal-EEG recordings in patients with migraine or basilar migraine have shown rhythms like those commonly observed during an epileptic attack (Beaumanoir, 1993; De Romanis et al., 1993). In our case the clinical picture and history do not fit completely into those for basilar-type migraine (Camfield et al., 1978). The criteria for discrimination of migraine and epilepsy rely heavily on the duration of the complaint as recognized by the official classification schemes by both the International Headache Society (IHS) and the International League against Epilepsy (ILAE). This however does not take into account the well-known long duration of an autonomic status epilepticus, with possible migrainous headache as sole expression. From this point of view, this condition could be termed as “pure autonomic status epilepticus” (Ferrie et al., 2007).
Interestingly, in the present case, both the generalized tonic clonic seizure and the headache complaints started after excessive visual stimulation (video gaming on a new TV screen for hours and a colorful exhibition of Matisse). Also in the laboratory IPS evoked headache and for the first time visual elementary hallucinations.
A diagnosis of idiopathic photosensitive occipital lobe epilepsy must be taken into account in this and similar type of cases (Guerrini et al., 1995). Occipital epilepsies are known to have mainly autonomic symptoms, are relatively long lasting and can often be precipitated by strong visual stimuli (Ricci and Vigevano, 1993; Guerrini and Genton, 2004). Indeed, in our case the seizure did not begin with a visual elementary symptom as described in all cases both in the idiopathic photosensitive occipital lobe epilepsies and the typical late onset childhood occipital epilepsies “Gastaut type.”
More than to stimulate debate and interest, even controversy about the overlap between epilepsy and migraine, this article demonstrates the ictal epileptic origin of migraine-like manifestations.
We would therefore advocate recording EEGs in patients with migrainous features, unresponsive to antimigraine drugs.
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