A 49-year-old man with right temporal lobe epilepsy developed the chronic delusion that his body emitted a foul odor (olfactory reference syndrome). Before the delusional symptoms, he had partial seizures with unpleasant olfactory hallucinations. Subsequently ictal olfactory symptoms abated but a disabling delusional syndrome developed. The only prior case of ...

The relationship between febrile seizures and epilepsy has long been debated. We argue that there is some specificity to the types of epilepsy that follow febrile seizures, rather than febrile seizures being a nonspecific marker of a lowered seizure threshold. The relationship between febrile seizures and later epilepsy is frequently ...

We studied twins to examine the genetics of epilepsy syndromes. We ascertained 358 twin pairs in whom one or both reported seizures. After evaluation, 253 of 358 (71%) had seizure disorders and 105 pairs were false positives. Among the monozygous (MZ) pairs, more were concordant for seizures (48 of 108; ...

The electroencephalogram (EEG) plays an important role in the evaluation of a child with developmental delay. An EEG is often required to classify seizures in children with developmental delay. Equally important is the role of the EEG in the identification of specific electroclinical syndromes in children who may or may ...

Fourteen cases of shaken baby syndrome seen between 1993 to 1997 at the Kaohsiung Medical College Hospital were reviewed. The common clinical presentations were seizure, respiratory problem and disturbed consciousness. The signs and symptoms of this form of head trauma were nonspecific. The findings may mimic infection, intoxication, seizure disorder ...

West syndrome is a multi-etiological condition. Recent progress in perinatal medicine and the recent development of new neuroimaging techniques may have changed the etiological panorama of West syndrome. Our recent study has disclosed an increasing percentage of the perinatal group and a decreasing percentage of the doubtful group. The increase ...

Benign occipital epilepsy of childhood is an idiopathic partial epilepsy syndrome with elementary visual symptomatology, frequently associated with other ictal phenomena. Seizures are usually followed by postictal headache and are often associated with interictal occipital rhythmic paroxysmal EEG activity that appears only after eye closure. In some children the ictal ...

This study analyzes the relative frequency and age of onset of the different seizure types in a 20-year cohort of a pediatric neurology outpatient clinic of an urban hospital that serves the majority of the city's population (Tel Aviv Medical Center). Only patients with two or more unprovoked seizures were ...

PURPOSE: To determine the point prevalence of active childhood epilepsy in a defined area and evaluate the usefulness of ILAE classification of seizures, and epilepsies/syndromes with special interest in severe epilepsies. METHODS: By using the latest ILAE International Classification of Epileptic Seizures (ICES, 1981) and Epilepsies and Epileptic Syndromes (ICE, ...

PURPOSE: In adult studies, MRI volumetrics is a proven technique in presurgical assessment of epilepsy. Hippocampal volume loss is maximal in the syndrome of mesial temporal lobe epilepsy. We aimed (a) to validate this methodology in a pediatric outpatient epilepsy population (b) to determine the relationship of hippocampal asymmetry (HA) ...

Lennox-Gastaut syndrome a combination of various generalized seizures including atypical absences and tonic seizures with generalized slow spike waves and mental deterioration, is often difficult to distinguish from a subgroup of myoclonic-astatic epilepsy, other generalized epilepsy syndromes, and various symptomatic generalized epilepsies. Conventional antiepileptic medication is poorly effective in this ...

We report the case of a 20-year-old woman with Noonan syndrome. She had severe mental retardation and intractable epilepsy. Magnetic resonance imaging revealed dilated perivascular spaces and a dysplastic lesion in the left temporal lobe, which is thought to have caused her neurologic symptoms. These findings suggest that neuronal in ...

The possible associations of myoclonic phenomenae, progressive or non-progressive encephalopathies and epileptic features are reviewed, with special emphasis on pediatric age. This leads to recognize the following five groups of conditions: (1) Myoclonus without encephalopathy and without epilepsy; (2) Encephalopathies with non-epileptic myoclonus; (3) Progressive encephalopathies presenting myoclonus seizures of ...

Of the epileptic syndromes beginning in early infancy, we described the clinical and electrical characteristics of early myoclonic encephalopathy (EME) and Ohtahara syndrome (OS), and the differential diagnosis between the two syndromes, reviewing previous articles and our own study of 6 cases with EME and 15 cases with OS. With ...

A high prevalence of epilepsy (up to 1.3%) was observed in an area hyperendemic for onchocerciasis in the province of Bururi, Burundi. Following this observation, epileptics and controls were examined in 2 communes (administrative units) with different endemicity for onchocerciasis. Altogether, onchocerciasis was more frequent in epileptics (81.8%) than in ...

Hallucinations are uncommonly encountered in the young child. The differential diagnosis of such behavior includes a number of potentially serious syndromes such as central nervous system malignancy, encephalitis, temporal lobe epilepsy, closed head trauma with frontal lobe confusion, hypoglycemia, drug intoxications, and childhood psychiatric syndromes. Organic explanations are most often ...

Heinrich Klüver and Paul Bucy described a constellation of symptoms in monkeys following large resections of the temporal lobe that they termed the "temporal lobe syndrome"; now commonly referred to as the Klüver-Bucy syndrome. The aim of this paper is threefold: (1) to review Heinrich Kluver's behavioral studies on monkeys ...

PURPOSE: To describe periictal Capgras syndrome as a result of rapid, successive bouts of ictal fear. METHODS: This periictal psychotic state was documented by depth EEG recordings. RESULTS: Episodes of paroxysmal fear, experienced as a vivid sensation of someone being nearby, corresponded with unilateral left limbic ictal discharge. CONCLUSIONS: Depersonalization, ...

Idiopathic generalized epilepsy (IGE) comprises several subsyndromes. These are principally: benign neonatal familial convulsions, benign neonatal convulsions, benign myoclonic epilepsy in infancy, childhood absence epilepsy, juvenile absence epilepsy, juvenile myoclonic epilepsy, epilepsy with generalised tonic-clonic seizures on awakening. In addition, there are less well-recognized syndromes, such as eyelid myoclonia with ...

A Japanese boy with Kabuki make-up syndrome associated with West syndrome is reported. He developed periodic tonic spasms at 6 months of age while his electro-encephalogram also revealed hypsarrhythmia. Although only a few previously reported cases of Kabuki make-up syndrome have been associated with epilepsy, it is likely that epileptic ...

Landau-Kleffner is currently considered a rare syndrome that combines acquired aphasia and epilepsy in children. We present two cases and discuss evidence in the literature that suggest how this syndrome is actually only part of a more generalized situation in which epilepsy (a paroxysmal disorder) produces or is accompanied by ...

OBJECTIVE: To identify the neuropsychological features of the syndrome of mesial temporal lobe epilepsy (MTLE), a surgically remediable epileptic syndrome defined by the presence of hippocampal sclerosis, using a broad and comprehensive neuropsychological test battery. SETTING: Epilepsy surgery center. PATIENTS: After scalp adn invasive electroencephalographic monitoring, a consecutive series of ...

Angelman's syndrome is a rare genetic disorder characterized by developmental delay, craniofacial abnormalities, ataxia, paroxysmal laughter, and seizures. The diagnosis is suspected in infants who have the characteristic clinical features and electroencephalographic (EEG) abnormalities and is confirmed by the genetic identification of a maternally derived 15q11-13 deletion. We report on ...

Secondary epileptogenesis as it applies to humans remains a controversial topic despite 40 years of investigation. Part of the controversy stems from disagreement about the definition of secondary epileptogenesis, and part of the controversy stems from the imperfect fit of animal models to the human epileptic syndromes. It may be ...

PURPOSE: Large numbers of families with many members having seizures have been used to understand the role of hereditary factors in the pathogenesis of human epileptic syndromes. We aimed to establish a genetic database to form a hypothesis on the possible genetic contributions in different epileptic syndromes. METHODS: The occurrence ...

West syndrome is an age-specific epileptic syndrome with onset in infancy. It comprises infantile spasms (usually flexion convulsions), EEG pattern of hypsarrhythmia and mental retardation. Current therapy involves ACTH, corticosteroids, valproic acid, pyridoxine and vigabatrine. The treatment is difficult and more effective antiepileptic drugs are required. Unfortunately, there is no ...

Malignant epileptic encephalopathies in children comprise a series of age-related generalized epilepsy syndromes: neonatal myoclonic encephalopathy and early epileptic encephalopathy in the neonatal period, migrating focal seizures in early infancy, infantile spasms and West syndrome, severe myoclonic epilepsy and non-progressive myoclonic encephalopathies in infancy, and Lennox-Gastaut syndrome, myoclonic astatic epilepsy, ...

Six girls (five Saudis and one Sudanese) aged between 3.5 and 12 years demonstrated the classic features of Rett syndrome (RS), including developmental regression with dementia, loss of acquired speech and hand function, and stereotypic hand movements. Myoclonic seizures were observed in two of them. Electroencephalography showed diffuse slowing in ...

We describe eight patients with slowly progressive speech production deficit combining speech apraxia, dysarthria, dysprosody and orofacial apraxia, and initially no other deficit in other language and non-language neuropsychological domains. Long-term follow-up (6-10 years) in 4 cases showed an evolution to muteness, bilateral suprabulbar paresis with automatic-voluntary dissociation and frontal ...

The current International Classification of Epilepsies and Epileptic Syndromes has classified a number of age-related epileptic syndromes in children, but some of these entities have not been well delineated and many syndromes not included in the International Classification have been proposed. This article is intended to supplement some of the ...

The I.L.A.E. classification of the epilepsies and epileptic syndromes was applied retrospectively to the epileptic seizure disorders of 1902 consecutive patients collected from a neurological consultant practice over a 30 year period. There were 265 patients with only a solitary seizure when they presented. These 265 included fewer instances of ...

A 45-year-old female migraineur with a long-standing history of drug-induced headache is described. She had been abusing caffeine (250 mg/day) and aspirin (5 gr/day). On the third day after discontinuation a withdrawal syndrome characterized by headache and a generalized tonic-clonic seizure occurred. The temporal association makes it likely that the ...

We described a profoundly intellectually disabled 24-year-old man with Wolf-Hirschhorn syndrome, left hemiplegia, epilepsy, atrophy of the right cerebral hemisphere, and dilatation of the right ventricle. The patient had a small ventricular septal defect, was wheelchair bound, and totally dependent. He had no speech, but vocalised to show his feelings. ...

PURPOSE: Episodic loss of consciousness presents a diagnostic challenge to the neurologist. A perhaps underrecognized cause of episodic loss of consciousness, which we call the ictal bradycardia syndrome, occurs when epileptic discharges profoundly disrupt normal cardiac rhythm, resulting in cardiogenic syncope during the ictal event. We attempt to determine whether ...

A prospective study of 32 children with epileptic encephalopathies 12 years or younger revealed a high incidence of focal cortical metabolic defects on 18-fluorodeoxyglucose positron emission tomography (PET) not suspected from clinical, EEG, or magnetic resonance imaging findings. PET scans were normal in all five children with typical de novo ...

A unique infarction limited to the posterior insula and intrasylvian parietal opercular cortex produced a subtype of conduction aphasia, characterized by a predominance of semantic paraphasias. Temporal lobe hypoperfusion seen on hexamethylpropyleneamineoxime single-photon emission computed tomography in the absence of any signs of ischemia suggested that cortical diaschisis played a ...

We attempted to classify, according to the 1989 International Classification of Epilepsies and Epileptic Syndromes, 1076 patients examined during a 4-year period (1 January 1988 to 31 December 1991) in the Child Neurology Unit. We aimed to determine the proportion of the clearly defined epileptic syndromes or the non-specific categories ...

Epilepsia partialis continua (EPC) has previously been reported in only two patients with Leigh syndrome (LS). We report here a subject in whom LS and partial deficit of cytochrome c oxidase (COX) were associated with EPC. Epilepsy in this subject appeared when neurologic impairment was clearly evident and MRI showed ...

We describe a male infant with findings typical of Schinzel-Giedion syndrome. Characteristic features include: midface retraction, widely patent fontanelles, hirsutism, choanal stenosis, hypospadias with chordae, club feet and broad ribs. The patient suffered from seizures and died at 14 months of age of fulminant bronchopneumonia. Pathological examination revealed steatosis in ...

We describe 2 patients with chronic alcoholism who over a period of several years developed epilepsy associated with a cerebellar syndrome and action myoclonus. The clinical picture suggested progressive myoclonic epilepsy, but subsequent investigations did not confirm this diagnosis. Myoclonus worsened during abstinence and was relieved during indulgence. We believe ...

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 ...

In this paper I review the recently introduced syndrome of focal retrograde amnesia-a disproportionate impairment of remote memory in the presence of normal or near normal anterograde memory. It is concluded that the modal form of the syndrome has been reliably demonstrated and that the underlying pathology involves damage to ...

OBJECTIVE: To describe the association between the use of the fluoroquinolone ofloxacin in an elderly man and an unusual acute encephalopathy with characteristics suggestive of Tourette's syndrome. CASE SUMMARY: An unusual syndrome was observed in a 71-year-old man temporally related to the initiation of ofloxacin therapy that resolved completely after ...

We present a case of uncontrolled temporal lobe epilepsy due to a focal left temporal neuronal heterotopia associated with the nevoid-basal-cell carcinoma syndrome. This is the first pathologically described lesion associated with temporal lobe epilepsy in this syndrome. Because the patient's seizures resolved after a modified left anterior temporal lobectomy, ...

Three patients who presented with grand mal seizures and an associated behavioral disorder were recognized as suffering from a severe butalbital withdrawal syndrome. All were migraineurs who had become dependent on barbiturates. We propose that the occurrence of seizures, psychotic behavior, or a recent personality change should be considered clues ...

Epilepsy syndromes occupy an important position in the current nosology of the epilepsies, describing and classifying seizure disorders with shared clinical and EEG features. Increasingly, this schema is being refined as new information becomes available and our understanding of etiology and presentation of each syndrome widens. Advances in neuroimaging and ...

Paroxysmal choreoathetosis is the essential symptomatology in the following 4 syndromes: (a) paroxysmal kinesogenic choreoathetosis; (b) paroxysmal nonkinesogenic choreoathetosis; (c) supplementary sensorimotor seizures, and (d) paroxysmal nocturnal dystonia. All these syndromes are characterized by similar clinical symptomatology of the paroxysmal events. However, the pathophysiology of the choreoathetotic episodes differs considerably. ...

We report 15 children with clinical and electroencephalographic evidence of Ohtahara syndrome. The onset of seizures in most of them was within the first week of life. The seizures were predominantly polymorphic and refractory to treatment with ACTH and anticonvulsants. Suppression-burst patterns were recorded on the EEG in all cases. ...

The authors studied the clinical course and electroencephalograms (EEGs) of 27 patients with periventricular leukomalacia (PVL), to investigate the relation between PVL and West syndrome. Seven of the 27 patients with PVL developed WS; in all seven the PVL was severe. Bilateral parieto-occipital dominant irregular polyspike-and-wave (PO-polySpW) bursts were seen ...

The acute effects of two GABAB receptor antagonists (phaclofen and CGP-35348) were studied in two types of epileptogenic activity: that produced by intracortical injections of baclofen and that appearing after withdrawal of chronic intracerebral GABA infusion (the GABA-withdrawal syndrome, GWS). Intracortical baclofen induced two types of electrographic paroxysmal discharges: one ...