Alessandra Morano

Ictal epileptic headache as “subtle” symptom in generalized idiopathic epilepsy

Epilepsy and migraine are common neurologic chronic disorders with episodic manifestations characterized by recurrent attacks and a return to baseline conditions between attacks. Epilepsy and migraine are frequently observed in comorbidity, with the occurrence of one disorder increasing the probability of the other: Migraine occurs in about one‐fourth of patients with epilepsy, whereas epilepsy is present in 8–15% of patients with migraine. The link between headache and seizures is controversial and multifactorial. In epilepsy, headache can be seen as a preictal, ictal, or postictal phenomenon. In this report, we describe a case of a 37‐year‐old patient, affected by both drug‐resistant generalized idiopathic epilepsy and headache, who displayed the sudden onset of a headache attack referred during a 24‐h electroencephalography (EEG). The EEG tracing during this event revealed the activation of subcontinuous epileptic activity consisting of generalized spike‐wave discharges (GSWDs) and generalized polyspike and wave discharges (GPSWDs) that persisted for 60 min, that is, until the disappearance of the headache. The case we describe appears to be original in that it represents one of the few EEG‐documented ictal epileptic headaches in generalized idiopathic epilepsy.

Oxcarbazepine-induced myoclonic status epilepticus in juvenile myoclonic epilepsy.

Juvenile myoclonic epilepsy (JME) is a frequent idiopathic generalised epilepsy syndrome with typical clinical and EEG features that can usually be controlled by valproate monotherapy. JME may be underdiagnosed or misdiagnosed; in the latter case, it may be mistaken for partial epilepsy. The incorrect diagnosis of JME is likely to result in inappropriate therapy, which may, in turn, worsen the seizures. While a number of studies have documented that carbamazepine aggravates idiopathic generalised epilepsy, few have shown a worsening of symptoms following the administration of oxcarbazepine (OXC).We report the case of a 44-year-old male affected by JME in which the inappropriate use of OXC precipitated a dramatic worsening of myoclonic seizures. In this case, video-EEG monitoring documented myoclonic status epilepticus with positive and negative myoclonus, correlating with repetitive, continuous, rhythmic, generalised polyspike-and-wave discharges. This is the first case of myoclonic status epilepticus induced by OXC in a patient with JME which is clearly documented by video-EEG. A review of the literature with regards to OXC-induced worsening of seizures is also presented.

The spectrum of epileptic syndromes with fixation off sensitivity persisting in adult life.

The term “fixation off sensitivity” (FOS) was proposed by Panayiotopoulos to describe epilepsy/electroencephalography (EEG) changes evoked by the suppression of central vision and fixation. The EEG pattern usually consists of spike/polyspike and waves localized in occipital regions. FOS occurs mainly in children with idiopathic occipital partial epilepsies and rarely in adults. In this retrospective study we evaluated the clinical data, EEG, and magnetic resonance imaging (MRI) findings of patients with epilepsy and FOS persisting in adult life to better define the spectrum of syndromes.

Natural evolution from idiopathic photosensitive occipital lobe epilepsy to idiopathic generalized epilepsy in an untreated young patient

Idiopathic photosensitive occipital lobe epilepsy (IPOE) is an idiopathic localization-related epilepsy characterized by age-related onset, specific mode of precipitation, occipital photic-induced seizures--frequently consisting of visual symptoms--and good prognosis. This uncommon epilepsy, which usually starts in childhood or adolescence, has rarely been observed in families in which idiopathic generalized epilepsy also affects other members. We describe a nuclear family in which the proband showed electro-clinical features of idiopathic photosensitive occipital lobe epilepsy in childhood, which subsequently evolved into absences and a single generalized tonico-clonic seizure in early adolescence. His mother had features suggestive of juvenile myoclonic epilepsy. This case illustrates a continuum between focal and generalized entities in the spectrum of the so-called idiopathic (genetically determined) epileptic syndromes.

Ictal Epileptic Headache in Occipital Symptomatic Epilepsy: Not Only a Matter of Cortex

Epileptic seizures and headache attacks are two common neurological episodic events characterized by transitory modifications of homeostatic mechanisms regulating the balance between excitatory and inhibitory neurotransmission. The two phenomena can have similar clinical features and evolution, suggesting the involvement of common cortical and subcortical networks. Although the link between these two attacks is not completely clear, they are often observed in comorbidity, and can be sometimes related in time, a condition known as “headache attributed to epileptic seizure.” In this debated and expanding chapter of International Classification of Headache Disorders, the inclusion of some variants of inter-ictal and peri-ictal (pre-ictal, ictal, post-ictal) headache, the most interesting and rare condition being ictal epileptic headache, has been proposed. We report the case of a 23-year-old man affected by structural focal epilepsy with a history of atypical headache attacks. VideoEEG recordings performed during such episodes showed sub-continuous epileptic activity over the left occipital region, clinically associated with migrainous headache, bilateral eyelid flutter and strabismus. Our paper represents one of the few well EEG-documented cases of ictal epileptic headache and helps to speculate about the cortical-subcortical networks involving occipital regions during epileptic discharges and the pathophysiological link between migraine and epilepsy. Among neurological events, headache attacks and epileptic seizures represent two common paroxysmal phenomena that temporarily disturb brain functions. These two attacks are often comorbid, occurring independently in the same individual but, sometimes, they can be related in time, a condition known as “headache attributed to epileptic seizure.” In the current literature different authors, who also refer to this specific context as seizurerelated headache (SRH), have proposed to encompass some entities, not all formally included in International Classification of Headache Disorders ICHD-IIIb: interictal and peri-ictal (pre-ictal, ictal, post-ictal) headache. Occipital seizures are often associated with postictal headache (the most common form of SRH), that may have migraine-like features and occasionally respond to sumatriptan. The most interesting and rare condition in the context of SRH is represented by ictal epileptic headache (IEH), which is defined as headache/migraine occurring as the sole ictal epileptic manifestation and represents 3-5% of all SRH cases. In the borderland between headache and epilepsy, another rare phenomenon is represented by migralepsy, a condition defined as a seizure triggered by migraine aura. However, according to the better knowledge of IEH and the related critical revision, migralepsy could be revisited as an IEH followed by other symptoms or signs. These clinical entities suggest the existence of a pathophysiological link between migraine and epilepsy, underlying their complex reciprocal relations. A 23-year-old man was admitted to our Epilepsy Unit for a history of seizures started at the age of 10 and characterized by confusion and impairment of consciousness, followed by long-lasting headache (at that time interpreted as post-ictal headache). Family history was positive in the maternal line for headache suggestive of migraine and in paternal line for structural focal epilepsy related to multiple cavernomatosis. A brain MRI showed multiple cavernomas without evidence of previous bleeding: one of them, located in the left temporo-occipital

Cryptogenic focal epilepsy and "hidden" celiac disease in adulthood: a causal or accidental link?

Purpose. Celiac disease (CD) is an immuno-mediated small bowel disease characterized by chronic inflammation due to a permanent intolerance to gliadin. Several neurological complications have been described, including epilepsy, whose evolution might often improve by adopting gluten-free diet (GFD). We studied a population of adult patients affected by posterior drug-resistant epilepsy of unknown cause by performing an accurate screening for CD. In the selected patients presenting the association of epilepsy and CD, we characterized the related electro-clinical features. Materials and Methods. We consecutively identified 211 adult subjects affected by drug-resistant cryptogenic focal epilepsy with posterior seizures. All these patients underwent serological screening for CD. In 10 subjects positive serological tests allowed to perform a CD diagnosis (confirmed by duodenal biopsy). For each patient clinical and EEG data, neuroimaging studies, serological and histological findings were revised, as well as response to GFD, defined as an improvement in seizure outcome. Results. A significant delay between diagnosis of epilepsy and CD was documented. Visual ictal manifestations were reported in half of subjects. In all cases, interictal EEG showed slow and epileptiform abnormalities over parietal-occipital and temporal regions; in three cases, FOS phenomenon was observed. Four patients had familiar history of CD and six cases showed clinical signs/symptoms of malabsorption. GFD led to a reduction of seizure frequency in half of patients. Conclusions. “Posterior” ictal semiology, peculiar EEG patterns and drug-resistance emerge as the most interesting characteristics. CD screening should be performed in epilepsy patients presenting such features.

Ictal pain in focal non-convulsive status epilepticus

We report an adult with acute unilateral pain as isolated manifestation of acute symptomatic focal non-convulsive status epilepticus. Pain is rarely a manifestation of epileptic seizures. Traditionally, painful seizures have been thought to originate in either the parietal or temporal lobes, but their localising value is debatable. Recent functional neuroimaging studies and electrophysiological findings obtained by using intracerebral recordings have shown the involvement of the insular cortex along with several other brain structures in the processing of painful inputs, comprising a more widespread anatomo-functional network. Despite their rarity as a distinct clinical entity, especially in adults, painful somatosensory seizures can be disabling and misdiagnosis or delayed diagnosis is common; it is therefore essential to consider epilepsy as a possible cause of paroxysmal pain to ensure proper assessment and appropriate treatment.

Cannabis in epilepsy: From clinical practice to basic research focusing on the possible role of cannabidivarin

Cannabidivarin (CBDV) and cannabidiol (CBD) have recently emerged among cannabinoids for their potential antiepileptic properties, as shown in several animal models. We report the case of a patient affected by symptomatic partial epilepsy who used cannabis as self‐medication after the failure of countless pharmacological/surgical treatments. Clinical and video electroencephalogram (EEG) evaluations were periodically performed, and the serum levels of CBDV, CBD, and Δ9‐tetrahydrocannabinol were repeatedly measured. After cannabis administration, a dramatic clinical improvement, in terms of both decrease in seizure frequency and recovery of cognitive functions, was observed, which might parallel high CBDV plasma concentrations. To widen the spectrum of CBDV possible mechanisms of action, electrophysiological methods were applied to investigate whether it could exert some effects on γ‐aminobutyric acid (GABA)A receptors. Our experiments showed that, in human hippocampal tissues of four patients affected by drug‐resistant temporal lobe epilepsy (TLE) transplanted in Xenopus oocytes, there is decrease of current rundown (i.e., reduction of use‐dependent GABAA current) after prolonged exposure to CBDV. This result has been confirmed using a single case of Rasmussen encephalitis (RE). Our patients electroclinical improvement supports the hypothesis that cannabis could actually represent an effective, well‐tolerated antiepileptic drug. Moreover, the experimental data suggest that CBDV may greatly contribute to cannabis anticonvulsant effect through its possible GABAergic action.

Effectiveness of Rufinamide in the Treatment of Idiopathic Generalized Epilepsy With Atypical Evolution Case Report and Review of the Literature

Rufinamide (RFD) is a novel drug that was recently approved as an adjunctive treatment for Lennox-Gastaut syndrome. Despite its reported effectiveness in generalized seizures (tonic, atonic, or tonic-clonic) in this syndrome, few data on its use in idiopathic generalized epilepsy are available. Indeed, the scientific evidence to date is limited to anecdotal cases or isolated clinical experiences. We report an uncommon, though paradigmatic, case of a woman affected by juvenile absence epilepsy (JAE) who, following a prolonged seizure-freedom period and the consequent withdrawal of valproate, presented a seizure relapse accompanied by a worsening in her electroclinical pattern. In view of this atypical evolution of JAE, characterized by drug-resistant seizures (absence and generalized tonic-clonic) and the progressive increase in electroencephalographic (EEG) abnormalities, several antiepileptic drugs were used, though to no benefit. The use of RFD instead led to a gradual control of the seizures and normalization of the EEG findings. In addition to this clinical experience, we briefly review the literature on the use of RFD in refractory generalized epilepsy.

Clinical experience with intravenous valproate as first-line treatment of status epilepticus and seizure clusters in selected populations

Objective: The aim of this study was to evaluate the efficacy and safety of intravenous valproate (i.v. VPA) as first-line treatment of status epilepticus (SE) and seizure clusters in selected patient populations. Methods: We enrolled 23 patients (11 females and 12 males; mean age: 61 years) with SE who received i.v. VPA as first-line therapy (25 mg/kg in 100 mL saline infused over 15 min). ECG tracing was monitored before, during, and after infusion. Liver function and serum ammonia tests were conducted after 24 and 72 h of treatment. We evaluate the response of SE to i.v. therapy and short-term outcome. Results: In 15 out of 23 patients (65%), i.v. VPA was effective. In our population, we retrospectively identified three different subgroups: patients with cardiorespiratory comorbidities discouraging the use of traditional SE first-line drugs, patients with specific epileptic subsyndromes (such as idiopathic generalized epilepsy), and patients affected by psycho-organic syndromes. No significant adverse effects were detected. Discussion: Our study shows the clinical relevance of i.v. VPA as first-line therapy of SE in patients with medical conditions contraindicating the use of traditional first-line antiepileptic drugs for SE, and in those presenting with specific forms of SE.