A case of frontal lobe epilepsy presenting with recurrent speech arrest]

We report a 55-year-old right-handed man with frontal lobe epilepsy manifesting recurrent speech arrest. He was known to have hypertension, hypertriglyceridemia, and gout. In the three days prior to admission, he had episodes of sudden inability to talk. These episodes lasted 10 to 30 seconds and recurred ten to twenty times a day. On admission, speech comprehension and other mental functions were normal, as were findings on neurologic examination. During the period of speech arrest, he understood spoken commands, and there was no abnormal motor activity or paresis. The episodes of speech arrest were thought to be short aphasic periods due to transient ischemic attacks in the left carotid territory. Computed tomography and magnetic resonance imaging demonstrated a small calcified lesion in the upper medial portion of the left frontal lobe. Left internal carotid angiography demonstrated no abnormal findings. After neuroradiological examination finished, he suddenly raised his right hand and followed it with his gaze and a right head turn. The EEG seizure pattern in which 20-25 Hz activity began in the left fronto-central region and spread rapidly to the right fronto-central region, which after about 8 seconds was replaced by 12-14 Hz flattening rhythmic polyspikes was detected 9 times within 60 minutes. It is most unusual for supplementary motor area seizure to present pure paroxysmal speech arrest without accompanying paroxysmal motor activity. As in our case, epileptic arrest of speech may be confused with a transient ischemic attack of the dominant hemisphere.     

A case with frontal lobe epilepsy presenting with absence seizures as cardinal manifestation: ictal EEG findings]

We report here a 9-year-old boy presenting with absence and complex partial seizures. Absence seizures occurred several times a day, with sudden arrest of speech and gesture, alteration of consciousness, myoclonus of unilateral or bilateral angles of the mouth, occasional simple automatism and brisk recovery of consciousness. Complex partial seizures occurred once to three times a month with loss of consciousness, salivation, deviation of the head and eyes toward the left, elevation of upper limbs and tonic convulsion of the left upper and lower limbs. Interictal EEG showed right frontal pole-dominant high-voltage slow waves or spike-and-waves. Ictal simultaneous video-EEG recordings of absence seizures revealed a frontal dominant 3-3.5 Hz spike-wave burst lasting several seconds. A partial seizure never preceded the absence seizure. Transverse topographical analysis revealed that the first spike component of the spike-wave burst of absence seizure always showed phase reversal on the right anterior temporal electrode. The following ones, however, showed phase reversal on the left anterior temporal electrode. Ictal EEG of the complex partial seizure could not be detected because it rarely occurred. There was no abnormal finding on brain MRI. Interictal single photon emission tomography (SPECT) indicated hypoperfusion of the dorsal and medial cortex of the right middle frontal lobe. Interictal positron emission tomography (PET) also indicated hypometabolic areas in the dorsal and medial cortex of the right frontal lobe, together with those in the right temporal and parietal cortex. EEG evolution and neuroimaging studies suggested that the epileptic focus of the absence seizure might have originated at the dorsal cortex of the right middle frontal lobe and immediately spread to the medial cortex. Both the seizures were well controlled by the combination of phenytoin and high dose sodium valproate.     

Cryptogenic gelastic epilepsy of frontal lobe origin: a paediatric case report.

Gelastic (laughing) seizures are an uncommon seizure type which in most cases has an organic cerebral pathology and specifically a hypothalamic hamartoma. The interictal EEG frequently shows focal activity. This report describes a 3 1/2-year-old boy who presented with episodes of unmotivated laughter associated with other epileptic symptomatology before the age of 3 years. Prolonged ambulatory EEG monitoring recorded electroclinical seizures starting in the right frontal area and spreading to the adjacent frontotemporal region. Neurological examination and brain magnetic resonance imaging were normal. Vigabatrin resulted in immediate remission of the seizures and normalization of the EEG.     

A case of temporal lobe epilepsy with recurrent dysphasic seizures]

We reported a 40-year-old right-handed female with temporal lobe epilepsy manifesting recurrent dysphasic seizures. At age 25, the patient developed a complex partial seizure, who subsequently showed frequent auditory seizures that often evolved to complex partial or secondarily generalized seizures at age 25-27, and dysphasic seizures (DSs) at age 27 -40. DSs were characterized by total aphasia without impairment of consciousness, which were often accompanied by functional hallucination. Brain CT, cerebral angiography, and brain MRI demonstrated no abnormal findings. At age 39 (May 20, 1989), recurrent aphasic state was unexpectedly observed during medical examinations. An EEG was performed immediately, and the EEG seizure pattern (duration: ca. 8-17 sec) in which 15 -16 Hz spikes began in the left posterior temporal region and spread rapidly to the left midtemporal, inferior frontal, and central regions was detected 14 times within 30 minutes. During the seizure patterns, the patient was aphasic.     

Pilomotor seizures in frontal lobe epilepsy: case report.

We report a 27-year-old man with pilomotor seizures originating in the right frontal lobe. Subtracted ictal SPECT coregistered with MRI showed multifocal hyperperfused areas in the anterior medial frontal area, mainly involving the cingulate gyrus. Chronic electrocorticography with subdural electrode arrays during the piloerection demonstrated that the pilomotor seizures were originating in the anterior medial frontal region. After resection of the focus, the patient became seizure free.     

Differentiating frontal lobe epilepsy from psychogenic nonepileptic seizures

Patients with psychogenic nonepileptic seizures (PNES) remain one of the most challenging patient populations. The misdiagnosis of PNES is costly to patients, the health care system, and to society. The first step in treatment is proper diagnosis. Video electroencephalography remains the gold standard for PNES diagnosis. Differentiating frontal lobe epilepsy (FLE) from PNES can be difficult; however, clinical findings and laboratory advances are emerging that more clearly establish the diagnosis of PNES. This article provides clues to differentiating FLE from PNES so that neurologists and mental health providers are better equipped to offer treatments for PNES.     

Successful epilepsy surgery in frontal lobe epilepsy with startle seizures: a SEEG studyFrontal epilepsy with startle seizures

Pre‐surgical assessment and surgical management of frontal epilepsy with normal MRI is often challenging. We present a case of a 33‐year‐old, right‐handed, educated male. During childhood, his seizures presented with mandibular myoclonus and no particular trigger. As a young adult, he developed seizures with a startle component, triggered by unexpected noises. During his ictal episodes, he felt fear and grimaced with sudden head flexion and tonic axial posturing. Similar seizures also occurred without startle. Neuropsychological assessment showed executive dysfunction and verbal memory deficit. The cerebral MRI was normal. Electro‐clinical reasoning, investigations performed, the results obtained and follow‐up are discussed in detail. [Published with video sequence]     

Nocturnal frontal lobe epilepsy presenting with restless leg syndrome-like symptoms

We describe the case of a 22-year-old male affected by NFLE reporting paroxysmal RLS-like symptoms. The patient was referred to our Sleep Center due to nocturnal paresthesias and cramps involving the left leg and leading to sleep fragmentation. At age 4, the patient presented with secondary generalized seizures preceded by left leg discomfort, controlled on CBZ. After successive therapy discontinuation, leg symptoms built up in frequency and duration until a secondary generalized seizure re-occurred. On CBZ prompt resumption no further GM seizures occurred albeit persistence of night-time frequent cramps and paraesthesia. Sleep EEG demonstrated asymmetric interictal sharp theta on the right posterior frontal areas, whereas brain MRI results were consistent with a Taylor type right frontal cortical dysplasia. CBZ augmentation and add on therapy with LEV led to further frequency reduction of sensory symptoms.     

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.     

The seizures of frontal lobe epilepsy. A study of clinical manifestations

We describe ictal clinical manifestations of frontal lobe epileptic seizures in 22 patients. After examination of all ictal clinical data, 14 catergories of signs and symptoms were established. The validity of the ictal clinical data used was confirmed on the basis of 99 frontal lobe seizures recorded by tele-electroencephalogram or tele-stereo-electroencephalogram. The main conclusion is that the frontal lobe appears to be partially connected with motor acitivity.     

Repeated seizures induce prefrontal growth disturbance in frontal lobe epilepsy

Background

The possible consequences of seizures in the immature brain have been the subject of much conjecture. We prospectively measured frontal and prefrontal lobe volumes using three-dimensional (3D) magnetic resonance imaging (MRI)-based volumetry in patients with frontal lobe epilepsy (FLE) presenting with the same seizure semiology. The pathogenesis of repeated seizure-induced brain damage is discussed herein.

Methods

Serial changes in regional cerebral volumes were measured in two patients with FLE presenting with intractable clinical courses and cognitive impairments/behavioral problems (FLE(+)) and four FLE patients without cognitive impairments/behavioral problems (FLE(−)). Eleven normal subjects (4–13 years old) served as controls. Volumes of the frontal and prefrontal lobes were determined using a workstation, and the prefrontal-to-frontal lobe volume ratio was calculated.

Results

Frontal and prefrontal lobe volumes revealed growth disturbance in FLE(+) compared with those of FLE(−) and control subjects. In addition, prefrontal-to-frontal lobe volume ratio increased serially in FLE(−) similarly to controls, but was stagnant or decreased in FLE(+). Prefrontal growth also revealed more rapid recovery in a FLE(+) patient with shorter active seizure period.

Conclusion

These findings suggest that repeated seizures may lead to prefrontal growth disturbance. The occurrence of frequent seizures in patients with FLE may be associated with prefrontal lobe growth retardation, which relates to neuropsychological problems and ultimate neuropsychological outcome.     

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.     

A young adult female case of Wilson's disease presenting with mental disorder and frontal lobe signs]

We report a young adult female case of Wilson's disease presenting with mental disorder and frontal lobe signs. The patient was admitted to our neurological unit on October 4, 1999 because of schizophrenia-like symptom, dysphagia, dysarthria and gait disturbance. She showed slowly progressive rigidity and dystonia. Her parents were the second cousins. Neurological examination revealed bilateral pyramidal and extrapyramidal signs, frontal lobe signs (include the imitation behavior). Tendon reflexes were slightly exaggerated in all extremities. Bilateral Babinski, Chaddock and Hoffmann signs were positive. Her verbal IQ on the Wechsler Adult Intelligence Scale-revised was 49. Biochemical examination revealed low plasma copper and ceruloplasmin concentration. Cerebrospinal fluid was normal. Cranial MRI demonstrated diffuse brain atrophy and enlargement of the lateral ventricles. T2-weighted images of the MRI demonstrated hyperintense signal in both thalamus and basal ganglia. SPECT showed hypoperfusion in the left frontal lobe, both thalamus and basal ganglia. EEG revealed diffuse theta wave. The diagnosis of Wilson's disease was made and the treatment of D-penicillamine 900 mg per day was started. This hypoperfusion of SPECT and EEG findings improved after 2 months under D-penicillamine therapy. Neurological findings showed slight improvement. A few Wilson's disease patients presenting with mental disorder have been reported. Wilson's disease should always be considered in differential diagnosis of mental disorders. We emphasize the importance of early diagnosis and treatment of Wilson's disease.     

Clinical and neurophysiologic markers of epilepsy with frontal lobe seizures in a series of patients diagnosed by video-EEG-telemetry]

The general and ictal clinical features, including EEG findings, of 20 consecutive patients with frontal lobe epilepsy are presented. Diagnosis in all cases was based on video-EEG telemetry using strict criteria. Among the ictal motor phenomena, turning movements, dystonic, choreic and ballistic features prevailed. Ten patients vocalised during their seizures but loss of consciousness was rare, even if there was pronounced motor involvement. The duration of attacks generally was short, not exceeding 60 seconds in 18 patients, in whom sudden onset and cessation of seizures was noted. Ten patients showed inter-ictal epileptiform EEG activity and 19 had ictal abnormalities. The short duration and ictal clinical course may be helpful in differentiating frontal seizures from pseudoseizures.     

Acute postoperative seizures after frontal lobe cortical resection for intractable partial epilepsy

PURPOSE: To evaluate the incidence and prognostic importance of acute postoperative seizures (APOSs) occurring in the first week after a focal corticectomy in patients with partial epilepsy of frontal lobe origin. METHODS: We retrospectively evaluated 65 patients who underwent a frontal lobe cortical resection for intractable partial epilepsy between April 1987 and December 2000. All patients were followed up for a minimum of 1 year after surgery. RESULTS: APOSs occurred in 17 (26%) patients. None of the following factors was shown to be significantly associated with the occurrence of APOSs: gender, duration of epilepsy, etiology for seizure disorder, use of subdural or depth electrodes, surgical pathology, or postoperative risk factor for seizures. Patients with APOSs were older at seizure onset and at the time of surgery (p = 0.003 and p = 0.05, respectively). At last follow-up, patients who had APOSs had a seizure-free outcome similar to that of individuals without APOSs (47.1% vs. 50.0%; p = 0.77). Patients with APOSs appeared less likely to have a favorable outcome [i.e., fewer than three seizures per year and >95% decrease in seizure activity (58.8 vs. 70.8%; p = 0.35)]. This result may not have reached statistical significance because of the sample size. No evidence suggested that precipitating factors or the timing of APOSs was an important prognostic factor. CONCLUSIONS: The presence of APOSs after frontal lobe surgery for intractable epilepsy does not preclude a significant reduction in seizure tendency. These findings may be useful in counseling patients who undergo surgical treatment for frontal lobe epilepsy.     

A clinical study on frontal lobe epilepsy

Of 30 patients whose epileptogenic focus was presumed to be located in the frontal lobe on the basis of a clinico-electrographic correlation, the principal seizure manifestation consisted of peculiar automatisms in about 40% of the cases. However, the nature of the automatisms was of a short duration lasting about one minute but in most cases it was accompanied by extremely violent movements, but this was not true in all cases. Of the 30 cases, only 5 cases showed marked improvement one year after being discharged from the hospital so that this type of seizure event is extremely intractable.     

Effects of antiepileptic treatment on sleep and seizures in nocturnal frontal lobe epilepsy

ObjectiveTo study the effects of antiepileptic treatment on sleep parameters and video-polysomnography (VPSG) seizures in nocturnal frontal lobe epilepsy (NFLE).MethodsTwenty patients with a clinical and VPSG diagnosis of NFLE (baseline polysomnography [PSG]) underwent a clinical follow-up and performed a second VPSG after effective antiepileptic treatment lasting for at least 6 months. Conventional sleep measures, cyclic alternating pattern (CAP) parameters, and objective VPSG seizures were assessed in NFLE patients before and after treatment and were compared with the results of 20 age- and gender-matched control subjects.ResultsAntiepileptic treatment determined a partial reduction of objective VPSG seizures of approximately 25% compared to baseline condition. Alterations of most conventional sleep measures recovered normal values, but nonrapid eye movement (NREM) sleep instability remained pathologically enhanced (CAP rate, +26% compared to controls) and was associated with persistence of daytime sleepiness.ConclusionsResidual epileptic events and high levels of unstable NREM sleep can define a sort of objective resistance of both seizures and disturbed arousal system to the therapeutic purpose of the antiepileptic drugs in NFLE. This finding could determine the need for new therapeutic options in this particular form of epilepsy.     

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.