[Partial motor epilepsy in nonketotic hyperglycemia. 2 cases].
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Epilepsies, Partial↗
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[Partial motor epilepsy, intracranial hematoma and protein S defiency in a 49-year-old woman].
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Carotid Artery Diseases↗
Partial motor epilepsy with "negative myoclonus".
Three children had both nocturnal unilateral motor seizures and daytime ipsilateral "negative myoclonus" which occurred so frequently that it resembled asterixis. Neurophysiologic studies demonstrated lateralized spike discharges that were time-locked to postural lapse in the contralateral outstretched arm. The clinical course was characterized by good seizure control with benzodiazepines.
Adolescent↗
[Partial motor epilepsy, immediate postoperative complication in frontal sinusitis. Physiopathologic considerations].
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Adult↗
[Individualization of so-called benign and functional epilepsy at different ages. Appraisal of variations corresponding the predisposition for epilepsy at these ages].
The different types of epilepsy encountered during the various phases of life are presented and the distinction is made between benign and, essentially if not exclusively, functional epilepsy related to a genetically-transmitted predisposition to epilepsy: (1) the primary generalized epilepsies and (2) the benign partial epilepsies with functional foci. In the case of primary generalized epilepsy, seizures are the result of a discharge, generalized from the onset, of the entire cortex in individuals genetically predisposed to epilepsy. The seizures present (1) in older children, adolescents and young adults, the well-known characteristics of Petit Mal and/or Grand Mal; (2) in neonates, infants and young children, a different type of generalized (or hemigeneralized) seizures, the semeiology of which remains unclear. In the case of benign partial epilepsy, the seizures result from an epileptic discharge in a cortical area having a low convulsant threshold in patients genetically predisposed to epilepsy. The semeiology of the seizures varies as a function of the patient's age and the site of the epileptic focus: -in neonates: benign partial epilepsy wih an erratic EEG focus, e.g. 'Fifth day seizures'; -in infants: 'cryptogenic' benign partial epilepsy, which remains poorly defined; -in children: (a) benign partial epilepsy with nocturnal motor seizures and a central EEG focus; (b) benign partial epilepsy with affective seizures and a temporal focus; (c) benign partial epilepsy with visual seizures (often coupled with signs of basilar migraine) and an occipital focus; -in adolescents: benign partial motor epilepsy (often versive) often without an interictal EEG focus or with generalized spike-waves; benign partial epilepsies do not occur after adolescence.
Adolescent↗
[Partial benign crises in adolescence].
The aim of this paper is to present the case of teenage patient with partial seizures fulfilling the criteria of benign partial seizures of adolescence. A 16-year-old male patient had two seizures with a sensory-motor "march" that evolved into a secondarily generalized tonic-clonic seizure on the same day. Several weeks before this event he had had several simple partial sensory seizures. The patient had no previous history of seizures and there was no family history of epilepsy. The neurological examination, EEG and magnetic resonance imaging were normal. The patient was treated with antiepileptic monotherapy during two years. The treatment was gradually tapered and withdrawn over the following six months. He has had no recurrences during the five years of follow-up. The early diagnosis of this entity has the significant prognostic and therapeutic repercussions.
Adolescent↗
[Supplementary sensory-motor seizures--symptomatology, etiology, and surgical management with illustrative case reports].
In the past decade, owing to the advance of epilepsy surgery, growing knowledge has accumulated on the role of the supplementary motor area, described by Penfield and coworkers in the early fifties, in movement regulation and on the characteristics of seizures involving this area. In the Hungarian neurological literature this topic--despite its neurophysiological and practical clinical importance--has been hardly touched. The authors, based on their own experience obtained from surgeries performed within the framework of the "Co-operative Epilepsy Surgery Program", describe the electrophysiological features of this area, its role in movement regulation and the symptoms of epileptic seizures stemmed from or spread onto this area. Using cases as illustrations, they demonstrate the reasoning and various algorithms of the multidisciplinary examination necessary to explore the seizure onset zone and the pathways of seizure spread. Details of the surgical solution are also described.
Adolescent↗
The predictive localizing value of tonic limb posturing in supplementary sensorimotor seizures.
OBJECTIVE: To determine whether early tonic limb posturing is reliable in lateralizing or localizing of the seizure generator in 14 patients with pharmacoresistent supplementary sensorimotor area (SSMA) seizures. METHODS: All patients underwent high-quality MRI scans and stereo-EEG recordings. RESULTS: The SSMA seizure semiology predicted focal or regional ictal onset in the SSMA in six (43%) patients: Three had a focal SSMA seizure onset, and three had a regional seizure onset with involvement of one SSMA plus adjacent neocortex. The eight remaining patients had diffuse uni- or bilateral seizure onset. Eight of 14 patients underwent a frontal or central cortical resection, but a good outcome was seen in only 3: 2 with no SSMA resection and 1 with an extensive central removal. CONCLUSIONS: SSMA semiology is suggestive of early involvement of this region but is by no means a reliable indicator that the primary SSMA contains the seizure focus.
Adolescent↗
[Negative myoclonus].
Negative myoclonus is an involuntary movement produced by a short interruption of muscle contraction. In this paper, recent concepts, pathomechanisms and treatment in negative myoclonus are reviewed. Two patients with negative myoclonus are also presented. One is an 18-year-old girl with Gaucher disease, and the other is a 14-year-old boy with simple partial motor epilepsy. Electrical silent period was demonstrated when negative myoclonus was clinically observed. In both patients, somatosensory evoked potentials showed giant responses, which may indicate that mechanisms of negative myoclonus have a relationship with cortical abnormal excitability.
Electroencephalography↗
Epilepsy and perisylvian lipoma/cortical dysplasia complex.
Intracranial lipomas are congenital malformations composed of mature adipocytes. They are usually located in the midline, particularly in the pericallosal region, a hemispheric location accounting for only 3 to 7% of cases. Review of the literature found 21 previous cases of hemispheric lipoma. Although hemispheric cerebral lipomas are rare, association with epilepsy appears to be frequent. We have recently studied two patients in whom epilepsy was the first clinical manifestation of hemispheric cerebral lipoma in the sylvian region. The patients presented with simple motor partial seizures as the first manifestation of the lesion. Neurological examination was normal. MRI disclosed in both cases a lesion involving the sylvian fissure with characteristics of the lipid signal. MRI also demonstrated abnormalities involving the cerebral cortex in the vicinity of the lesion (pachygyria-like aspect). Partial excision of the lesion was achieved in one patient but was followed by a worsening of seizures and neurological condition (hemiparesis). According to the literature, the prognosis for epilepsy in patients with hemispheric lipoma appears good. Several other arguments support non-surgical management: the lesion is benign and can be identified with a high degree of certainty by imaging; surgery is technically difficult due to adherence to adjacent vascular and cerebral structures and hypervascularity; location near functional brain tissue increases the risk of postoperative sequelae. In addition, mechanisms of epilepsy probably involve vascular and cortical dysplasic abnormalities. In consideration of the complexity of the lesion, hemispheric lipomas are more appropriately classified with localized cortical malformations rather than as simple extracerebral malformations.
Adult↗
Neurocysticercosis and epilepsy in Peru.
This study was carried out to determine the prevailing type of epilepsy in neurocysticercosis. We also evaluated if it could account for the predominance of generalized epilepsy in Peru. We studied the records and EEGs of 49 cases of neurocysticercosis, representing 5.9% of all hospital admissions. Cysticercosis was diagnosed using clinical, blood, CSF (complement fixation, ELISA, immunoblot) and CT scan criteria. We found 49 epileptic patients. Their type of epilepsy was as follows: 35 subjects had generalized epilepsy and 14 had partial epilepsy (5 motor partial, 5 complex partial, both with secondary generalization and 4 motor partial). All but 3 patients were over 25 years old (mean 33 years). We conclude that generalized epilepsy is frequent among cysticercosis patients and that it may account, partially, for the predominance of generalized seizures in Peru and in other areas of South America where cysticercosis is endemic.
Adolescent↗
[Diagnostic focus on the child with epilepsy and neuropsychological deterioration].
Symptomatic epilepsy secondary to hereditary metabolic or degenerative disorders, is usually associated to neurological deterioration. Though epilepsy by itself does not induce neurological deterioration, we should remind that some epileptics encephalopathies, such as the West or Lennox-Gastaut syndromes, do actually induce limited neurological deterioration. Furthermore, in some forms of complex partial epilepsy, motor problems and behavior disorders can be observed, specially in adolescents with temporary lobe epilepsy. Other forms of epilepsy, such as the atypical benign partial epilepsy or the Landau-Kleffner syndrome, can present a certain degree of cognitive deterioration in the evolution, although they can recover later lost functions, totally or partially. The evolution of some refractory epilepsy, as patients are submitted to a multiple treatments, can make us suspect a degenerative disease. In some cases, the diagnosis of the hereditary metabolic and heredodegeneratives diseases can be made by the characteristics of the seizures but in most cases the diagnosis will be established by the symptoms of the basic disease and the lab data.
Adolescent↗
Attention deficit hyperactivity disorder associated with orbitofrontal epilepsy in a father and a son.
The authors report on a father and son with frontal lobe epilepsy and symptoms of attention deficit hyperactivity disorder (ADHD). Attention deficit hyperactivity is a syndrome defined by criteria that include inattention, impulsive behavior, impaired concentration and motor restlessness. It does not require medical or neurobehavioral evaluation to determine an underlying etiology. The father is a 45-year-old man evaluated for possible ADHD. His referral came after the diagnosis of ADHD in his 6-year-old son who responded well to treatment with methylphenidate HCL. Neurobehavioral evaluation of the father suggested frontal lobe dysfunction. Magnetic resonance imaging and electroencephalography (EEG) were normal. Brain 99mTc HMPAO single-photon emission computed tomography (SPECT) revealed left orbitofrontal hypoperfusion. Additional history from his wife revealed episodic symptoms suggestive of nonconvulsive epilepsy that included nonresponsive staring, complex automatic behavior, and amnesic lacunas. Treatment of the father with carbmazepine produced dramatic improvement. Subsequent evaluation of his son, currently on maintenance treatment with methylphenidate HCL for ADHD, elicited a history consistent with atonic and simple motor partial epilepsy. The son's brain SPECT revealed bilateral orbitofrontal hypoperfusion defects. Attention deficit hyperactivity disorder is a syndrome that may be caused by frontal lobe lesions or epilepsy. In the setting of possible ADHD, neurological evaluation is warranted. Although overreliance on structural imaging or EEG in such an evaluation must be discouraged, brain SPECT may be useful to evaluate patients with symptoms of attention disorders for frontal epilepsy.
Attention Deficit Disorder with Hyperactivity↗
Double-blind, randomized controlled pilot study of bilateral cerebellar stimulation for treatment of intractable motor seizures.
PURPOSE: The efficacy and safety of cerebellar stimulation (CS) was reevaluated in a double-blind, randomized controlled pilot study on five patients with medically refractory motor seizures, and especially generalized tonic-clonic seizures. METHODS: Bilateral modified four-contact plate electrodes were placed on the cerebellar superomedial surface through two suboccipital burr holes. The implanted programmable, battery-operated stimulator was adjusted to 2.0 microC/cm(2)/phase with the stimulator case as the anode; at this level, no patient experienced the stimulation. Patients served as their own controls, comparing their seizure frequency in preimplant basal phase (BL) of 3 months with the postimplant phases from 10 months to 4 years (average, eight epochs of 3 months each). During the month after implantation, the stimulators were not activated. The patient and the evaluator were blinded as to the next 3-month epoch, as to whether stimulation was used. The patients were randomized into two groups: three with the stimulator ON and two with the stimulator OFF. After a 4-month postimplantation period, all patients had their stimulator ON until the end of the study and beyond. Medication was maintained unchanged throughout the study. EEG paroxysmal discharges also were measured. RESULTS: Generalized tonic-clonic seizures: in the initial 3-month double-blind phase, two patients were monitored with the stimulation OFF; no change was found in the mean seizure rate (patient 1, 100%, and patient 5, 85%; mean, 93%), whereas the three patients with the stimulation initially ON had a reduction of seizures to 33% (patient 2, 21%; patient 3, 46%; patient 4, 32%) with a statistically significant difference between OFF and ON phase of p = 0.023. All five patients then were stimulated and monitored. At the end of the next 6 months of stimulation, the five patients had a mean seizure rate of 41% (14-75%) of the BL. The second patient developed an infection in the implanted system, which had to be removed after 11 months of stimulation; the seizures were being reduced with stimulation to a mean of one per month from a mean of 4.7 per month (BL level) before stimulation. At the end of 24 months, three patients were monitored with stimulation, resulting in a further reduction of seizures to 24% (11-38%). Tonic seizures: four patients had these seizures, which at 24 months were reduced to 43% (10-76%). Follow-up surgery was necessary in four patients because of infection in one patient and lead/electrode displacement needing repositioning in three patients. The statistical analysis showed a significant reduction in tonic-clonic seizures (p < 0.001) and tonic seizures (p < 0.05). CONCLUSIONS: The superomedial cerebellar cortex appears to be a significantly effective and safe target for electrical stimulation for decreasing motor seizures over the long term. The effect shows generalized tonic-clonic seizure reduction after 1-2 months and continues to decrease over the first 6 months and then maintains this effectiveness over the study period of 2 years and beyond.
Adolescent↗
Rasmussen syndrome: multifocal spread of inflammation suggested from MRI and PET findings.
BACKGROUND: A 6-year-old girl with Rasmussen syndrome (RS) showed multiple small high-signal-intensity areas independently in the right hemisphere by fluid-attenuated inversion recovery (FLAIR) imaging on magnetic resonance imaging (MRI) 1 year after the onset of epilepsy. METHODS: MRI performed 4 months later demonstrated a further increase in the number of these foci and enlargement in the size of the previous FLAIR lesions. RESULTS: An [18F]-fluorodeoxyglucose-positron emission tomography (FDG-PET) study showed a strong, spotty uptake in the right temporooccipital regions, corresponding to the sites of continuous EEG seizure discharges. In contrast, [11C]methionine PET demonstrated multifocal uptake regions, which corresponded anatomically to the FLAIR lesions, suggesting sites of underlying chronic inflammation. CONCLUSIONS: These neuroimaging findings suggested that the inflammatory process in RS spreads either multifocally at the same time, as seen in this case, or from one discrete area to the adjacent region, as reported previously.
Autonomic Nervous System Diseases↗
Periodic lateralized epileptiform discharges in influenza B-associated encephalopathy.
An 18-year-old woman presented with coma, hemicomvulsions, and transient periodic lateralized epileptiform discharges (PLEDs). Serological tests were positive for influenza B, and cerebrospinal fluid PCR for herpes simplex virus DNA was negative. Magnetic resonance imaging later showed abnormal signal intensity in the temporal lobe ipsilateral to the PLEDs. Influenza-associated encephalopathy may cause hemiconvulsions and PLEDs, and can mimic herpes simplex encephalitis.
Adolescent↗
Alternating hemiplegia of childhood: presentation of two cases regarding the extent of variability.
Alternating hemiplegia of childhood is an episodic neurological disorder, the diagnosis of which is solely clinical. In this report, two patients with alternating hemiplegia, one as a representative of the classical picture and the other with unusual features, are presented by video display. Some clinical manifestations and the variability of symptoms are discussed with regard to their place in the diagnosis of the disease. (Published with videosequences).
Anticonvulsants↗
Efficacy of sulthiame (Ospolot) on motor partial seizure status during sleep in a patient with motor cortex epilepsy.
An 18-year-old female patient with a particular form of intractable motor cortex epilepsy, in which motor partial seizure status occurred only during sleep every night, was reported focusing on the drastic efficacy of sulthiame on the seizure status. Moreover, the present study demonstrated that single photon emission computed tomography (SPECT) and pulse oximetry examinations, both of which were performed in the ictal state, were useful for the regional diagnosis of the epileptic focus and observations of the seizure frequency, respectively. The therapeutic effect, epileptic picture and clinical examinations described here appear to be relatively rare in the literature, and therefore, this case report may be of clinical significance.
Adult↗