EEG diagnoses of neonatal seizures: clinical correlations and outcome

Electroencephalographic seizures were evaluated in 112 neonates. The first portion of the study involved 80 neonates with clinically identified abnormal movements, 8 of whom (10%) had electroencephalographic evidence of seizures coincident with this activity. Patients with abnormal movements (90%) had no concurrent electrical seizures. In the second part of the study, 40 infants who had electrical seizures were investigated. Eight of these infants had been identified during the first part of the study. Two-thirds of the patients (25) were premature. Sixteen patients (40%) died; 90% had brain lesions documented by computed tomography and/or postmortem study. Cerebral infarction and intraventricular hemorrhage were the most common lesions. One-third of the survivors (8 of 24 patients) were normal at a mean age of 3 years, while two-thirds had significant neurologic and developmental abnormalities. Neonatal seizures often are subtle, not associated with observable clinical expression, and associated with adverse development. Electroencephalographic confirmation is important in the evaluation of neonatal seizures.     

Psychogenic gelastic seizures in a patient with hypothalamic hamartoma

Gelastic seizures are classically associated with hypothalamic hamartoma. The most effective treatment for gelastic epilepsy is surgery, although confirming that a hypothalamic hamartoma is an epileptic lesion prior to surgical intervention is challenging. Here, we report the case of a patient with a hypothalamic hamartoma who was diagnosed with psychogenic non-epileptic gelastic seizures using video-EEG monitoring. [Published with video sequences].     

Complex partial status epilepticus presenting as gelastic seizures: a case report

A middle-aged man, who presented to the emergency room because of bizarre outbursts of laughter, was found to be in partial complex status epilepticus. His seizure disorder had been misdiagnosed, at various times, as a variety of "functional" psychiatric disorders. Despite proper diagnosis and aggressive treatment, management was difficult, being complicated by postictal agitation and confusion, postictal psychosis, and interictal compulsive and paranoid personality features. This case is described, and issues of diagnosis and management in partial complex epilepsy are briefly discussed. The importance of not overlooking organic and especially epileptic factors, despite the presence of prior psychiatric illness, psychologic contributors, and environmental stressors, is emphasized.     

Cognitive deficits in children with gelastic seizures and hypothalamic hamartoma.

OBJECTIVE: To characterize the cognitive deficits in children with gelastic seizures and hypothalamic hamartoma and investigate the relationship of seizure severity to cognitive abilities. METHODS: Eight children with gelastic seizures and hypothalamic hamartoma completed a neuropsychological battery of standardized and age-normed tests, including the Woodcock-Johnson Psycho-Educational Battery-Revised: Tests of Cognitive Ability, Peabody Picture Vocabulary Test-III, and initial-letter word fluency measure. RESULTS: All children displayed cognitive deficits, ranging from mild to severe. Gelastic/complex partial seizure severity was correlated with broad cognitive ability standard scores (r = -0.79; r2 = 0.63; (F[1,6] = 10.28; p = 0.018]. Frequency of gelastic/complex partial seizures was also correlated with broad cognitive ability standard scores (r = -0.72; r2 = 0.52; F[1,6] = 6.44; p = 0.044). Significant intracognitive standard score differences were found, with relative weaknesses in long-term retrieval (mean = 64.1; SD = 13.3) and processing speed (mean = 67.7; SD = 21.6) and a relative strength in visual processing (mean = 97.6; SD = 12.8). Performance in visual processing differed from performance in long-term retrieval (p = 0.009) and processing speed (p = 0.029). CONCLUSION: These findings are consistent with cognitive functions and affective/emotional states associated with conduction pathways of the hypothalamus involving cortical association areas and amygdala and hippocampal formation. These abnormalities can account for the prominent deficit found in integrating information in the processing of memories.     

Ictogenesis and symptomatogenesis of gelastic seizures in hypothalamic hamartomas: an ictal SPECT study

Purpose: To topographically localize the ictogenic zone within hypothalamic hamartomas (HHs) and the symptomatogenic zone for gelastic seizure (GS), we analyzed data from both interictal and ictal single photon emission computed tomography (SPECT). Methods: Ictal SPECT was performed immediately after GS onset in 21 patients with HH (aged 2–36 years, mean 13.8 years) who underwent stereotactic radiofrequency thermocoagulation (SRT). SPECT data were statistically analyzed by means of subtraction ictal SPECT coregistered to magnetic resonance imaging (MRI) (SISCOM) and statistical parametric mapping (SPM). Topographic localization of ictal hyperperfusion areas was evaluated. Results: SISCOM obtained in 27 studies demonstrated ictal hyperperfusion in the HH interface zone in 16 studies of 13 patients (hot HH group). In these patients, HHs were significantly larger than those without hyperperfusion of HH in 11 studies of 8 patients (21.4 ± 10.3 vs. 12.3 ± 7.3 mm in diameter, p < 0.05, t‐test). In all patients and in the hot HH group, SPM group analysis of individual differences between interictal and ictal data revealed significantly (p < 0.001) hyperperfused areas in the ipsilateral hypothalamus, mediodorsal (MD) nucleus of the thalamus and putamen, bilateral pontine tegmentum, and contralateral inferior semilunar lobule of the cerebellum. There was no hyperperfusion in the mammillothalamocingulate pathway. Discussion: The present study confirmed that ictogenesis occurs in the HH interface zone, which should accordingly be the target for SRT. We suggest that a thalamopontocerebellar circuit plays an important role for stereotypical and automatic symptomatogenesis of GS and that the hypothalamus and MD nucleus of the thalamus are potentially involved in epileptic encephalopathy.     

Stereotactic radiofrequency thermocoagulation for hypothalamic hamartoma with intractable gelastic seizures

Management of hypothalamic hamartoma with intractable gelastic epilepsy remains controversial. We have used stereotactic thermocoagulation for treatment of hypothalamic hamartoma with intractable gelastic epilepsy since 1997. Herein, we review our experience in five cases to clarify the usefulness of this treatment. A total of five patients with hypothalamic hamartoma were treated by stereotactic thermocoagulation at our hospital during the period October 1997 through February 2004. In all patients, the hamartoma was less than 10mm in diameter and was located on the floor of the third ventricle with sessile attachment to the wall. To identify ictal onset, chronic intracranial electroencephalography was performed in three patients with the use of a depth electrode implanted in the hamartoma. Attempts were made to induce gelastic seizure by electrical stimulation of the hamartoma in three patients. After magnetic resonance imaging-guided targeting, radiofrequency thermocoagulation of the boundary between the hamartoma and normal hypothalamus was performed to achieve disconnection effects. Marked reductions in seizure frequency were obtained in all cases, with three patients becoming seizure-free after the procedure. No intraoperative complications occurred except in one patient who experienced acute and transient panidrosis with hot flushes during coagulation. Our results suggest that stereotactic thermocoagulation of hypothalamic hamartoma is an acceptable treatment option for patients with intractable gelastic seizures.     

Psychogenic seizures: a clinical study with follow-up data

We evaluated the natural history and prognosis of psychogenic or hysterical seizures in patients discharged from the Johns Hopkins Hospital in the 3 years between 1971 and 1974. Follow-up data were obtained 5 years or more after discharge. Among 41 patients with convincing evidence of psychogenic seizures, there were coexisting organic neurologic disorders in 18 (44%). Mental subnormality or retardation was present in 17% and true epileptic seizures in 37%. EEG abnormalities found in 38% of individuals with psychogenic seizures were attributed to these organic neurologic disorders or anticonvulsant drug toxicity. There was significant long-term morbidity in 56% of these individuals because of psychosocial problems rather than misdiagnosis of psychogenic seizures.     

A case of frontal lobe epilepsy presenting with gelastic seizures]

We described a 9-year-old boy with frontal lobe epilepsy presenting with gelastic seizures. CT-scan showed mild widening of the left sylvian fissure. Abnormal findings in the left frontal operculum were detected by both MRI and SPECT. Attacks mainly consisted of gelastic seizures with comfortable feeling followed by screaming with fear. Administration of anticonvulsants resulted in reducing the frequency and severity of seizures. Finally the patient had brief laughter attacks only. In the present case, the clinical course suggests that the gelastic seizures does not occur by way of the spreading of epileptic discharges to the temporal or hypothalamic region; rather it might occur as a focal symptom of the frontal region.     

Hypothalamic GH Deficiency and gelastic seizures in a 10-year-old girl with MELAS

A case of mitochondrial encephalomyopathy, lactic acidosis and stroke-like episodes, in which a pituitary growth hormone (GH) secretion deficiency of hypothalamic origin was revealed through neuro-endocrinological examinations, was described. The case was a 10-year-old girl, who had been suffering from generalized tonic seizures since age 5, four episodes of alternating hemiplegia since age 6, stunted growth since age 7, and simple partial motor seizures as well as gelastic seizures since age 8. Marked elevation of lactate and pyruvate in both serum and CSF, abundant ragged red fibers in biopsied muscle, and low density areas in the left occipital lobe and bilateral globus pallidus in addition to diffuse brain atrophy on CT scan and MRI of the head were demonstrated, although the activities of muscle enzymes complex I-IV were within normal ranges. Pituitary GH secretion was deficient under the loadings with insulin, L-DOPA, sleep, and a single growth hormone releasing factor (GRF) administration, but normal GH response was registered under the repetitive stimulation with GRF. Activities of other hormonal axes were normal. It is likely that short stature commonly observed in MELAS patients is due to hypothalamic dysfunction, which might be brought out by chronic ischemia and energy deficiency of the diencephalon based upon mitochondrial abnormality of that region. It is likely that gelastic seizure in this case is due to hypothalamic dysfunction.     

Surgical treatment of extra‐hypothalamic epilepsies presenting with gelastic seizures

We provide an overview of the surgical outcome of extra‐hypothalamic epilepsies with gelastic seizures based on an original case report and a summary of the literature. Twenty‐two articles providing information on the outcome of resective surgery in 39 patients with extra‐hypothalamic gelastic seizures from the temporal (19 patients) or frontal lobe (20 patients) were selected. We add another case of temporal lobe gelastic seizures to the literature with a video demonstrating the mirthful component of this patient's laughing seizures. Drug‐refractory cases of gelastic seizures from the temporal or frontal lobes are amenable to surgical treatment following thorough investigation with imaging, as well as scalp and intracranial EEG.     

Natural history and outcome of psychogenic seizures: a clinical study in 50 patients

Summary The natural history and the outcome of psychogenic seizures was studied in 50 patients by retrospective analysis and follow-up after a mean of 2 years. Concomitant epilepsy was definite in only 8% and possible in 14%, while 50% took anticonvulsants. Overall, 66% of patients showed heterogeneous psychiatric abnormalities, most commonly a depressive syndrome (24%), whereas hysterical personality features were rare (8%). Forty-two percent of patients were unemployed. Follow-up of 41 patients (82%) showed that 34% had become seizure free. Outcome was poor in those with a long history of psychogenic seizures and pathological psychiatric findings. In contrast, almost all patients with recent onset of psychogenic seizures and normal psychological status had become seizure free. At follow-up, 56% of patients were found to be in a poor or very poor state, which resulted from a combination of physical, psychic and social problems in most cases.     

Magnetoencephalography and diffusion tensor imaging in gelastic seizures secondary to a cingulate gyrus lesion

Gelastic seizures are relatively uncommon and rarely observed secondary to frontal lobe lesions. This report presents magnetoencephalography (MEG) and diffusion tensor imaging (DTI) findings in an adolescent with gelastic seizures secondary to a left anterior cingulate gyrus lesion. Ictal scalp video EEG showed bilateral frontal 4 Hz theta discharges. Interictal EEG showed left fronto-temporal spikes or sharp waves. Interictal MEG showed spike sources over bilateral temporal regions. DTI and tractography delineated slightly shifted corpus callosum posterior to the lesion, unaffected uncinate and inferior longitudinal fasciculi. The patient became seizure free for 12 months after surgical excision of a pleomorphic xanthoastrocytoma in the left anterior cingulate region. In our patient, MEG and EEG did not localize the deep-seated epileptogenic zone. The combination of DTI and neurophysiologic studies, however, possibly disclosed neuronal connections within the epileptic network and indicated that epileptic discharges propagated via the uncinate fibers from the primary epileptogenic zone in the anterior cingulate region to the mesial temporal region in this case with gelastic seizures secondary to a cingulate lesion.     

A case of intractable epilepsy showing frequent gelastic seizures by administration of clobazam

A 13-year-old boy patient had severe mental retardation and spastic quadriplegia due to fetal distress and hypoxic-ischemic brain damage in the perinatal period. He suffered from West syndrome at the age of 7 months, and subsequently was diagnosed as having symptomatic localization-related epilepsy. His intractable epileptic seizures were not controlled by combination of various antiepileptic drugs. After prescribing nitrazepam and zonisamide for more than 1 year, we added clobazam (CLB), which has been marketed in Japan since 2000, to this combination therapy. After the introduction of CLB, tonic seizures disappeared. However, gelastic seizures laughing with a stiff face and a wry mouth appeared frequently before falling asleep, and sleep disturbance worsened subsequently. It has not been reported previously that gelastic seizures are a side effect of CLB, although irritability and sleep disturbance have been described.     

Retrospective analysis of surgical treatment outcomes for gelastic seizures: a review of the literature.

Gelastic seizures are known to be refractory to medical treatment and to date surgical therapy has yet to pinpoint the best treatment for these refractory seizures. There has been a multitude of case reports published on gelastic seizures and different surgical treatments, thus we performed a review of the literature on gelastic seizures and surgical treatments to elucidate the best surgical approaches for medically refractory gelastic seizures.     

Spontaneous primary intraventricular hemorrhage: clinical data, etiology and outcome

The clinical features, etiology, and neurological outcome in patients with primary intraventricular hemorrhage (PIVH) have rarely been reported. We retrospectively reviewed the clinical data, complementary examinations, outcome, computed tomography (CT) blood amount, and ventricle size of 13 patients (mean age 60 years, five men). We defined PIVH as hemorrhage detected by CT in the ventricular system only. The major symptoms included headache (n = 13), decreased level of consciousness (n = 9), and nausea/vomiting (n = 7). The cause was unknown in five patients; and was associated with arterial hypertension in five, vascular malformations in two, and tumor in one, although arteriography was performed in only five patients. Outcomes were death in three, asymptomatic in six, mild disability in three, and moderate disability in one. Prognosis was not related to clinical or CT data. Clinical features can suggest the diagnosis of PIVH, but cerebral CT is required for confirmation. Received: 2 March 1998 Received in revised form: 16 June 1998 Accepted: 23 July 1998     

Long-term clinical outcome of primary progressive MS: predictive value of clinical and MRI data

The authors sought to identify clinical and MRI predictors of outcome in primary progressive multiple sclerosis (PPMS). Clinical and MRI assessments were performed at baseline and 2 and 5 years (clinical only). At baseline, disease duration, expanded disability status scale (EDSS) and brain volume predicted outcome. Adding short-term change variables, baseline EDSS, changes in T2* lesion load and cord area, and number of new lesions were predictive. Clinical and MRI variables predict long-term outcome in PPMS.