The present review describes advances in Rasmussen's encephalitis (also known as Rasmussen's syndrome), an unihemispheric intractable epileptic disease with persistent inflammation. Specific attention is given to the recent recognition of cytotoxicity by CD8+/granzyme-B-positive T lymphocytes as a new pathogenic mechanism of neuronal damage.

Blue rubber bleb nevus syndrome is an uncommon neurocutaneous disorder characterized by distinctive vascular malformations on the body surface. Vascular malformations of internal organs (typically the gastrointestinal tract) are also frequently present. However, malformations of the central nervous system have only rarely been described. We report a case of blue ...

Panayiotopoulos syndrome is a relatively common condition with susceptibility to early onset benign childhood seizures, which manifests primarily with autonomic and mainly emetic symptoms. It predominantly affects children of 3-6 years of age (13% of those with one or more non-febrile seizures). EEG shows great variability, with occipital, extra-occipital spikes ...

Epilepsy is one of the commonest disorders presenting the neurological clinics in developing countries. There are attendant complications especially in untreated or poorly treated cases. In this article, we report an uncommon complication known as Landau-Kleffner syndrome in a Nigerian girl. The syndrome, typically seen in children consists of an ...

Infantile spasms and Lennox-Gastaut syndrome are rare but are important to child neurologists because of the intractable nature of the seizures and the serious neurologic comorbidities. New antiepileptic drugs offer more alternatives for treating both infantile spasms and Lennox-Gastaut syndrome. Selected children with infantile spasms are candidates for epilepsy surgery. ...

Efforts to duplicate the genetic and molecular breakthroughs of autosomal dominant frontal lobe epilepsy have lead to increased interest in familial temporal lobe epilepsy. In this report we describe three kindreds. The epilepsy syndrome described manifests after the teenage years and was generally mild and treatment responsive. The predominant seizure ...

Lennox-Gastaut syndrome is a type of childhood epilepsy that has enormous detrimental effects on the patient's physical and developmental health and can also take a dramatic toll on the well-being of the patient's family. Lennox-Gastaut syndrome is characterized by variable etiology, multiple types of intractable seizures, and cognitive impairment in ...

Characteristic appearances of epileptiform EEG activity in children aged between 2 and 10 years are described. Emphasis is placed on the value of careful analysis of background EEG and how the epileptiform activity itself may overlap between benign and malignant epileptic syndromes. Illustrations are used to underscore this emphasis. Irrespective ...

The Lennox-Gastaut syndrome (LGS) is widely known as one of the most severe and prognostically unfavourable epileptic conditions. Its entity as a syndrome is based on the occurrence of certain characteristic types of seizure (particularly typical drop attacks and axial tonic seizures) and on EEG patterns such as the slow ...

We report three patients with hemiconvulsion-hemiplegia-epilepsy syndrome who presented acutely and were shown to have striking neuroimaging findings suggestive of diffuse cytotoxic edema confined to one hemisphere, including extensive diffusion-weighted imaging abnormalities in two cases. Two patients subsequently developed progressive and extensive atrophy of the involved hemisphere. These findings are ...

Ohtahara syndrome (OS) is characterized by frequent tonic spasms, with or without clustering, of early onset within a few months of life, and a suppression-burst (S-B) pattern in electroencephalography (EEG). Tonic spasms occur in not only waking but also sleeping state in most cases. Partial seizures are observed in about ...

Neonatal seizures frequently accompany neonatal encephalopathies. Seizures occur in approximately 1.8-5/1,000 live births in this country and are caused by virtually any condition that affects neonatal brain function. This review provides a simple classification of seizures and emphasizes that many abnormal intermittent behaviors in this age group are not accompanied ...

In recent years, a great number of very different and in part contradictory terminologies and classifications of delusional misidentification syndromes (DMS) have been published. In various case reports, several authors have also made contradictory comments on questions concerning classification, etiology and assignment to a possible entity of DMS. Further development ...

Brucellosis is an infectious disease with multisystemic involvement caused by the genus Brucella. Neurological complications, including meningitis, meningoencephalitis, myelitis-radiculoneuritis, brain abscess, epidural abscess and meningovascular syndromes, are rarely encountered. We present a patient with epileptic seizures and aggressive mood due to chronic neurobrucellosis of 2.5 y duration, which was misdiagnosed ...

In infancy, partial epilepsies have been considered with suspicion for their probable association with brain lesions. Japanese authors first described partial epilepsies in infancy with a favorable outcome and called them benign partial epilepsy in infancy with complex partial seizures. Similar, but familial, cases with onset during the first year ...

Landau-Kleffner syndrome (LKS), or acquired epileptiform aphasia, is an epilepsy syndrome involving progressive neuropsychological impairment related to the appearance of paroxysmal electroencephalograph (EEG) activity. LKS appears to share a common pathophysiologic mechanism with continuous spike-wave of sleep (CSWS), acquired epileptic opercular syndrome (AEOS), and even benign childhood epilepsy with centrotemporal ...

To clarify the pathophysiology of tonic spasms, 21 patients with West syndrome were analyzed using ictal and interictal single photon emission computed tomography (SPECT). We focused on whether ictal perfusion changes were observed in the focal cortical region. Eight of the patients studied showed definite focal cortical ictal hyperperfusion, indicating ...

It is difficult to give a country report from Malaysia. A study done in 1999 reported the incidence of West Syndrome to be 3% among newly diagnosed cases of epilepsy. In this 3 year retrospective hospital-based study (1997-1999), the prevalence of early epileptic encephalopathy (EEE) and West Syndrome were 4.1 ...

In 1978, Dravet proposed a clinical entity called severe myoclonic epilepsy in infancy (SMEI). In the same year, a patient group, which was later called high voltage slow wave-grand mal syndrome (HVSW-GM), is reported in Japan. Both syndromes are very similar, except for seizure manifestation: generalized tonic-clonic convulsions (GTC) with ...

For the critical lesions and pathomechanism of early-infantile epileptic encephalopathy (EIEE) with suppression-bursts, we investigated the brains of EIEE, early myoclonic encephalopathy (EME), and West syndrome (WS) patients using immunohistochemical technique and neuropathological examination. We could compare with the results of these diseases. The EIEE patients had the most severe ...

The progressive encephalopathy with edema, hypsarrhythmia, and optic atrophy (PEHO) syndrome is a pediatric disorder of unknown origin, characterized by a combination of postnatally progressive encephalopathy, hypsarrhythmia, and optic atrophy. The pathological findings are early progressive atrophy of the cerebellum, brainstem, and optic nerves. Nitric acid (NO) has recently been ...

Thyrotropin-releasing hormone (TRH) has been successfully used for treating children with neurologic disorders including epilepsy. The effectiveness of TRH and a TRH analog has been reported in West syndrome, Lennox-Gastaut syndrome, and early infantile epileptic encephalopathy that were intractable to anticonvulsants and adrenocorticotrophic hormone (ACTH). However, the peptide has not ...

The case of a 66-year-old patient is reported in view of the rarity of his condition: a case of subacute encephalopathy with seizures in alcoholics (SESA syndrome), described first in 1981 by Niedermeyer, et al. Wernicke-type aphasia, epileptic seizures (generalized tonic-clonic) and PLEDs EEG pattern dominated the neurological picture, in ...

The spectrum of clinico-electrical characteristics of epileptic spasms associated with cortical malformation was studied in detail. The subjects were 15 patients suffering from spasms and cortical malformation demonstrated by MRI. The types of cortical malformation causing spasms were various, including hemimegalencephaly, diffuse pachygyria, focal cortical dysplasia, and polymicrogyria. Ohtahara syndrome ...

We describe two children with the velocardiofacial syndrome and benign partial-onset seizures. Both presented with slight dysmorphic traits, mild to moderate mental delay, and high-arched palate. A cardiac defect was present in only one of them. In each patient, sporadic rolandic or occipital partial-onset seizures with the clinical and electroencephalographic ...

Early-onset benign childhood occipital seizure susceptibility syndrome (EBOSS) recently described by Panayiotopoulos, is an early-onset variant of benign childhood epilepsy with occipital paroxysms. EBOSS is characterized by partial seizures that are predominantly manifested at night and associated with deviation of the eyes, vomiting and impairment of consciousness, but without ictal ...

Report of a case of SESA syndrome: a rare CNS complication of chronic alcoholism, known since 1981 and characterized by epileptic seizures, multiple and reversible neurological deficits, as well as PLEDs in the EEG. The MRI showed enhanced occipital signals in the T2-weighted sequence, which resolved together with the clinical ...

A 1.5-month-old boy with Sandifer's syndrome is described. After an uneventful delivery, he presented torticollis, seizure-like dystonic neck movements usually associated with feeding, episodic vomiting, inspiratory stridor and hand tremor in the first month of life. Barium esophagogram demonstrated gastroesophageal reflux, for which medical therapy was started. Children with torticollis ...

Seizures have not historically been considered a major component of Down syndrome. We examined the prevalence of epileptic seizures in 350 children and adolescents with Down syndrome evaluated at a regional center between 1985 and 1997. Results showed that 28 patients (8%) had epileptic seizures: 13 (47%) partial seizures; 9 ...

We report a case of congenital varicella syndrome after maternal infection during the 10th week of pregnancy. None of the typical abnormalities were manifested at birth; however, the child experienced neonatal tonic convulsions that evolved to refractory focal epilepsy with adversive, complex partial, and secondarily generalized seizures. At the age ...

We report a case study of a frontal and parietal lobe syndrome with memory loss after unilateral left-sided cerebellar damage caused by a stroke in a patient with right cerebellar unusual developmental agenesis. The syndrome consisted of severe deficits in planning an organized sequence of events, in visuo-constructive abilities and ...

We report here a girl aged 5 years 3 months with cryptogenic localization-related epilepsy who showed a prolonged episode characterized by dysarthria, dysphagia, drooling and paresis of the right arm associated with almost continuous diffuse sharp-slow wave complexes during sleep. These symptoms were not directly related to seizures or to ...

PURPOSE: Severe myoclonic epilepsy of infancy (SMEI) is an intractable epilepsy of early childhood of unknown etiology. It is often associated with a family history of seizure disorders, but epilepsy phenotypes have not been well described. We sought to characterize the seizure phenotypes of relatives to better understand to the ...

A syndrome of idiopathic generalised epilepsy with phantom absences of undetermined onset has been recently described. This syndrome clinically becomes apparent in adulthood with generalised tonic clonic seizures and frequently absence status epilepticus. We report an 11 year-old normal girl with frequent episodes of absence status and no other overt ...

Infantile spasm is a catastrophic form of epilepsy found only in infants and young toddlers. Onset is before one year of age, with a peak incidence occurring between 4 to 7 months of age. The prevalence is difficult to calculate, but previous reviews have estimated between 1 per 2000 to ...

Rasmussen's syndrome (chronic encephalitis with epilepsy) is a rare neurological disorder of unknown cause characterized by severe epilepsy, hemiplegia, dementia, and inflammation of the brain, and progressive functional and structural destruction of a single cerebral hemisphere. While one mechanism underlying the pathogenesis of Rasmussen's encephalitis has been hypothesized to be ...

Infantile spasms constitute both a distinctive seizure type and an age-specific epilepsy syndrome that have been extensively described for over a century. Standardization of the classification of infantile spasms has evolved, culminating in recent recommendations for separately recognizing and distinguishing the seizure type (spasms or epileptic spasms) and the epilepsy ...

The alien hand syndrome is rare, occurring with lesions in the mesial frontal lobe area and corpus callosum. We report an unusual presentation of seizures in a 65-year-old woman with a presumed low-grade glioma involving the mesial frontal lobe region, with the "alien hand" seen only during a seizure. Neuropsychological ...

"Generalized epilepsy with febrile seizures plus" (GEFS+) syndrome has been recently described. This term defines a heterogeneous group of generalized epilepsies observed in several members of large pedigree studies. The syndrome spectrum has been widened by including others forms of generalized epilepsy. We report two Italian families in which the ...

Epileptic encephalopathies are conditions in which neurologic deterioration results mainly from epileptic activity. It can be due to very frequent or severe seizures, or to subcontinuous paroxysmal interictal activity. The former consists mainly of severe myoclonic epilepsy in infancy (SMEN), in which patients exhibit seizures from the middle of the ...

Typical absences are brief (seconds) generalised seizures of sudden onset and termination. They have 2 essential components: clinically, the impairment of consciousness (absence) and, generalised 3 to 4Hz spike/polyspike and slow wave discharges on electroencephalogram (EEG). They differ fundamentally from other seizures and are pharmacologically unique. Their clinical and EEG ...

Motor cortex hyperexcitability in Rett Syndrome (RS) has been ascribed to the failure of an inhibiting system arising from the frontal lobes, a system needed in childhood and early adolescence. It is also presumed that, on a virtually dysfunctional (nonstructural) basis, motor cortex hyperexcitability in ADHD is also due to ...

Encephalocraniocutaneous lipomatosis (ECCL) is a rare congenital neurocutaneous syndrome comprising unilateral cranial lipomas, lipodermoids of the eye and brain abnormalities. A 3-year-old boy who presented at birth with a scalp lipoma and an ipsilateral epibulbar lipodermoid is described. Infantile spasms developed at 9 months of age and cerebral imaging showed ...

The authors present 11 cases of idiopathic generalized epilepsy that began in adulthood at a mean age of 39 years. All patients had myoclonic jerks, five had absence seizures, and nine had infrequent generalized tonic-clonic seizures. A majority had a family history of seizures. EEG in all patients showed generalized ...

PURPOSE: Epilepsy syndromes can be identified very early in the course of a seizure disorder. It is unclear how accurate and resilient such early classifications are. We compared the classification of epilepsy syndromes made previously on the basis of information available at diagnosis with those made 2 years later in ...

Multifocal independent spike syndrome (MISS) is an identifiable electroclinical syndrome, which combines intractable motor seizures, mental retardation and multifocal independent spike discharges. Similarities to the Lennox Gastaut syndrome cause frequent misdiagnosis; however, MISS is a distinct electroclinical syndrome in children with a better prognosis and a different EEG pattern. We ...

We reported a 15-year-old boy with idiopathic hypoparathyroidism who presented with paroxysmal kinesigenic choreoathetosis at age 10. Calcium levels were low and intact parathyroid hormones were undetectable in serum. Computed tomography showed calcifications in the basal ganglia, thalamus, and cerebral white matter. He had a history of infantile convulsions with ...

West syndrome, although classified as a generalized epilepsy, is associated with localized cerebral lesions in some cases. However, similar localized cortical abnormalities usually can result in partial epilepsy, instead of West syndrome. We performed this study to determine the additional factors that result in West syndrome instead of partial epilepsy ...

Early onset benign occipital susceptibility syndrome (EBOSS) is a recently delineated form of idiopathic childhood partial epilepsy, which is still not recognized by the International League Against Epilepsy as a distinct epileptic syndrome. It occurs with generally nocturnal, prolonged partial seizures, which can become a status epilepticus. The seizures are ...

Negative phenomena can occur with seizures, but some ictal negative manifestations are rare and may lead to misdiagnosis. A patient series is presented with unusual ictal negative phenomena: neglect syndrome, catastrophic depression, apraxia, aphasia, amnesia, homonomous hemianopsia, and hemiparesis. One had repeated episodes with PLEDs but no parenchymal lesions. Clinicians ...