Gelastic epilepsy: symptomatic and cryptogenic cases.

PURPOSE: To describe the etiology, characteristics, and clinical evolution of epilepsy in patients with gelastic seizures (GSs). METHODS: Nine patients whose seizures were characterized by typical laughing attacks were observed between 1986 and 1997. Patients were selected based on electroencephalogram (EEG) or video-EEG recordings of at least one GS and on magnetic resonance imaging (MRI) study. RESULTS: Five patients were affected by symptomatic localization-related epilepsy (LRE), with four of the patients' disorders related to a hypothalamic hamartoma (HH) and one to tuberous sclerosis (TS) without evident hypothalamic lesions. In four patients (the cryptogenic cases) MRI was negative also in these cases, clinical and EEG data suggested a focal origin of the seizures. The epileptic syndrome in the HH cases was usually drug-resistant, and was surgically treated in two of the patients. The patient with TS became seizure free with vigabatrin. In the cryptogenic cases, the ictal, clinical, and EEG semiology were similar to the symptomatic cases: the clinical evolution was variable, with patients having transient drug resistance or partial response to treatment. No cognitive defects were observed in the cryptogenic patients. None of the nine patients had precocious puberty. CONCLUSIONS: We confirm the frequent finding of HHs in GSs and further underline how GSs may also be observed in patients without MRI lesions and with normal neurologic status. In these patients, clinical and EEG seizure semiology is similar to symptomatic cases, but the clinical evolution is usually more benign.

Cutis verticis gyrata--mental deficiency syndrome: a patient with drug-resistant epilepsy and polymicrogyria.

We report a case of cutis verticis gyrata-mental deficiency syndrome (CVG-MD) which was associated with drug-resistant epilepsy and bilateral occipital polymicrogyria. Genetic analysis showed an increased number of breaks at the 3p14 and 16q23 sites. We hypothesize that a deleterious factor acting at a critical period of intrauterine development could result in the cerebral malformation and in the development of CVG. Neuroradiological investigation is warranted in cases of CVG-MD.

Transient global amnesia of epileptic origin accompanied by fever.

The case of a previously healthy 69-year-old female patient is described who presented, in a period of 6 months, 3 long-lasting (from 2 hour- to 10 hour-duration) episodes of transient global amnesia accompanied by a temperature rise. During one of these episodes an EEG was obtained, showing a diffuse alteration, focal slowing, and bitemporal asynchronous paroxysmal activity giving rise to electrical ictal discharges. Interictal EEGs were normal. Cerebral computed tomography was normal. Carbamazepine was given with complete control of the attacks. These episodes may be interpreted as complex partial status with unusual semeiology.

Unusual seizures with a benign course in a case of acute lymphoblastic leukemia of childhood.

We report the unusual seizures in a patient with acute lymphoblastic leukemia. The convulsive disorder began acutely as partial somatomotor status epilepticus and with diffuse EEG slowing. The seizures then became myoclonic-atonic (drop attacks) and an EEG focus appeared on the left paramedian centro-parietal areas, activated by proprioceptive stimuli. Despite the severity of the clinical picture at onset, the seizures showed a benign course, and disappeared on carbamazepine therapy. We think that this epileptic syndrome may have been caused by diffuse iatrogenic encephalopathy, probably related to intrathecal methotrexate therapy.

Epilepsia arithmetices: study of four cases.

In a 12-year period, in a total of about 2000 new patients referred to our Epilepsy Centre, we observed four patients with seizures exclusively or predominantly triggered by calculation or by card and board games (epilepsia arithmetices, EA). In agreement with observations reported in the literature, all the patients suffered from idiopathic generalized epilepsy (IGE), and probably from juvenile myoclonic epilepsy of Janz. In only one patient was it possible, during arithmetic tasks of increasing difficulty and stress, to evoke electroencephalographic (EEG) paroxysmal discharges, progressively increasing to clinical seizures. In the remaining patients the diagnosis of EA was exclusively clinical, as it was not possible to record EEG interictal or ictal paroxysmal activity specifically triggered by arithmetic tasks. Consequently, it is emphasized that in some cases the diagnosis of EA in a patient with IGE may be based exclusively on clinical criteria. As reported in the literature, it is possible to observe during mathematical calculation or during games both clinical (myoclonic jerks of the right hand) and EEG (localized small spikes) focal signs, which suggest a localized activation of specific areas in a patient with IGE.

The use of EEG activating procedures in epileptology.

Epileptic seizures and EEG interictal paroxysmal activity (PA) usually occur in an apparently unpredictable fashion, and a small number of patients with truly epileptic seizures may not present any PA on repeated EEGs. With the aim of increasing the possibility of recording interictal or ictal PA, several activation procedures are routinely carried out. In rarer instances, seizures seem to be more or less specifically evoked by unusual triggering procedures, or are chronologically related to biological rhythms. These different activating procedures may have different effectiveness depending on the type of epileptic syndrome. In the present paper the Authors describe the different activating procedures, both routinely employed and unusual ones. Their possible differential use with regard to the different epileptic syndromes and to the specific epileptologic history of the patient are examined. A selective use of these procedures is suggested, as an accurate choice of such techniques may increase the diagnostic usefulness of the EEG, in particular in patients whose basal recording is not contributory.

Recurrent frontal status epilepticus with polymorphic clinical features. A case report.

A patient who, two years after the surgical excision of a right frontal astrocytoma, presented with recurrent episodes of Partial Status Epilepticus of long duration is described. Ictal manifestations are initially inconspicuous from a clinical standpoint, but are accompanied by a marked interictal impairment of consciousness. The EEGs show a persistent depression of the background activity and very frequent subclinical right frontal discharges. Successively, seizures become more evident with polymorphic features (versive, oculoclonic, somatomotor, autonomic, automatic, laughing manifestations). The difficulties of an early definition of the clinical picture and the diagnostic importance of the EEG study are stressed, together with other peculiar features (polymorphism of seizures, difficulties of management, progressive mental impairment occurring after every episode of Status Epilepticus).