Mariarita Albini

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.

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.

Total recovery from Parkinson syndrome after surgical removal of a meningioma: a clinical case.

Signs of parkinsonism, such as resting tremors, rigidity, bradykinesia, and gait disturbance, typically have a unilateral onset and result from a malfunction in the extrapyramidal system involving the basal ganglia. Parkinson’s disease is mostly idiopathic and is caused by progressive and irreversible loss of dopaminergic neurons in the substantia nigra. However, in some cases, parkinsonism may be secondary to various conditions such as the following:

Epileptic seizures heralding a relapse in high grade gliomas

PURPOSE Seizures are a common clinical symptom in high-grade gliomas (HGG). The aim of the study was to investigate the relationship between seizures and HGG relapse (HGG-R). METHODS We retrospectively evaluated 145 patients who were surgically treated for HGG-R. By analyzing clinical characteristics in these patients (all operated and treated by the same protocol), we identified 37 patients with seizures during follow-up. This cohort was divided into four subgroups according to a) presence or absence of seizures at the time of diagnosis and b) temporal relationship between seizure occurrence and HGG-R during follow-up: subgroup A (25pts) had seizures at follow-up but not at onset, subgroup B (12pts) had seizures both at follow-up and onset, subgroup C (30pts) had seizures before MRI-documented HGG-R, and subgroup D (7pts) had seizures after MRI-documented HGG-R. RESULTS Although the datum was not statistically significant, survival was longer in patients with seizures during follow-up than in those without seizures (59.3% vs 51.4% alive at 2 years). In 30 patients (subgroup C) seizures heralded HGG-R. In a correlation analysis for this last subgroup, the time interval between seizure and the HGG-R was significantly associated with the number of chemotherapy cycles (r=0.470; p=0.009) and follow-up duration (r=0.566; p=0.001). A linear regression model demonstrated a reciprocal association between the above factors and that it may be possible to estimate the timing of HGG-R by combining these data. CONCLUSIONS Seizures may herald HGG-R before MRI detection of relapse, thus suggesting that seizures should always be considered a red flag during follow-up.

Behavioral and Movement Disorders due to Long-Lasting Myoclonic Status Epilepticus Misdiagnosed as ADHD in a Patient With Juvenile Myoclonic Epilepsy Electroclinical Findings and Related Hemodynamic Changes

Epilepsy and attention-deficit/hyperactivity disorder (ADHD) likely share common underlying neural mechanisms, as often suggested by both the evidence of electroencephalography (EEG) abnormalities in ADHD patients without epilepsy and the coexistence of these 2 conditions. The differential diagnosis between epilepsy and ADHD may consequently be challenging. In this report, we describe a patient presenting with a clinical association of “tics” and behavioral disorders that appeared 6 months before our first observation and had previously been interpreted as ADHD. A video-EEG evaluation documented an electroclinical pattern of myoclonic status epilepticus. On the basis of the revised clinical data, the EEG findings, the good response to valproate, the long-lasting myoclonic status epilepticus, and the enduring epileptic abnormalities likely causing behavioral disturbances, the patient’s symptoms were interpreted as being the expression of untreated juvenile myoclonic epilepsy. The EEG–functional magnetic resonance imaging study revealed, during clinical generalized spike-and-wave and polyspike-and-wave discharges, positive blood oxygen level–dependent (BOLD) signal changes bilaterally in the thalamus, the prefrontal cortex (Brodmann area 6, supplementary motor area) and the cerebellum, and negative BOLD signal changes in the regions of the default mode network. Such findings, which are typical of BOLD changes observed in idiopathic generalized epilepsy, may also shed light on the anatomofunctional network underlying ADHD.