AERRPS, DESC, NORSE, FIRES: multi-labeling or distinct epileptic entities?

In this review, we report a case of an adolescent girl presenting with epileptic encephalopathy preceded by febrile illness, demarcate the clinical phenotypic homogeneity among previously reported cases, and hypothesize on potential mechanisms based on current experimental evidence. Our literature review revealed >249 cases that share several main features: febrile illness with no preceding condition, negative laboratory studies including cerebrospinal fluid (CSF) analysis, status epilepticus refractory to conventional pharmacotherapy, and long-term developmental delays. This condition appears to have many names, the most recent of which is "FIRES" (fever-induced refractory epileptic encephalopathy). It seems likely that the described cases are representing the same entity. The possibility of a genetic or acquired channelopathy can be raised in light of negative infectious, autoimmune, microscopic, and gross pathology findings.

Medicolegal assessment of the ability to drive a motor vehicle in persons with epilepsy.

BACKGROUND: Determining whether a patient with epilepsy ought to be allowed to drive a motor vehicle is a challenging matter for the physician. This situation is fraught with medicolegal uncertainties, not just for general practitioners, but also for specialists and occupational health physicians. METHODS: A case is reported and discussed in the light of a selective review of the literature and the German medicolegal assessment guidelines for the determination of the ability to drive a motor vehicle. RESULTS: The risk associated with driving can be estimated as a function of the type of epilepsy from which the patient suffers and of the frequency of seizures. The expert can then express an opinion on the basis of this assessment, within the framework of the German medicolegal assessment guidelines and the German Driver Licensing Act ("Fahrerlaubnisverordnung"). There is no general duty of physicians to report a patient's unsuitability for driving a motor vehicle, but physicians have a right to report to the authorities that a patient should not be allowed to drive when the patient fails to comply with a recommendation not to drive and when a higher legally protected interest is at risk. CONCLUSIONS: The drawbacks of secondary prevention, as illustrated by the case presented here, could be minimized if the treating physician took the patient's occupational history and gave the relevant advice early on in the course of the patient's illness. Likewise, early counseling about driving would lessen the need for physicians to tell the authorities that patients should not drive in cases where the public interest overrides the patient's right to confidentiality.

[Non convulsive status epilepticus]. / Etats de mal épileptiques non convulsifs.

Non convulsive confusional status epilepticus is classically divided on the basis of the ictal EEG into absence status (AS) and complex partial status epilepticus (CPSE). The clinical presentation is often insufficient to establish diagnosis and emergency EEG is required. AS is a polymorphic condition that can complicate many epileptic syndromes. In half of cases, confusion of varying intensity is associated with bilateral periocular myoclonias. Clinical and EEG normalization is obtained after intravenous benzodiazepine injection. From a nosographic point of view, four types of AS may be recognized. Typical AS occurs as part of an idiopathic generalized epilepsy. Atypical AS occurs in patients with symptomatic or cryptogenic generalized epilepsies. "De novo" AS of late onset is characterized by toxic or metabolic precipitating factors in middle-aged subjects with no previous history of epilepsy. AS with focal characteristics occurs in subjects with a preexisting or newly developing partial epilepsy, most often of extratemporal origin. Most cases are transitional forms between these four entities. CPSE is characterized by continuous or rapidly recurring complex partial seizures which may involve temporal and/or extratemporal regions. Cyclic disturbance of consciousness is characteristic of CPSE of temporal lobe origin, which requires vigorous treatment to prevent recurrence or cognitive sequelae. CPSE of frontal lobe origin is a diagnostic challenge: it is rare, the symptoms are unusual, and extensive documentation is required. A focal, frontal lesion is discovered in one third of cases.

Benign focal seizures of adolescence: a prospective study.

PURPOSE: To characterize the clinical and EEG findings and evolution of the syndrome of benign focal seizures of adolescence (BFSA), as described by Loiseau et al. METHODS: A prospective study was performed in adolescents with normal clinical and neurologic examinations and normal neuroradiologic studies who had focal seizures that occurred isolated or in a cluster, with or without secondary generalization in the first 24 to 48 h after onset. None of the patients was treated with antiepileptic drugs (AEDs). RESULTS: Between January 1996 and January 2002, 15 patients with BFSA were enrolled in the study. Median age at onset of BFSA was 14 years. Thirteen patients had focal sensory or motor seizures. In two patients, the ictal manifestation was motion arrest associated with oral automatisms. Eight of them evolved to generalized tonic-clonic seizures. Thirteen patients had seizures only when awake, and the other two, both when awake and during sleep. Repeated interictal EEGs were normal, but in four of the patients who had seizures in a cluster, we were able to record an EEG within 8 h after seizure onset. Two of these four patients had focal seizures, and their waking EEG showed focal centroparietal theta activities. The other two patients had secondarily generalized seizures, and their waking EEG showed bilateral theta activities instead. Prognosis was excellent. CONCLUSIONS: BFSA is a well-defined seizure syndrome, recognizable by clinical and EEG features, as described by Loiseau et al. In teenagers with these electroclinical features with a normal neurologic examination and normal neuroradiologic findings, AEDs should be avoided.

Psychoses and epilepsy: are interictal and postictal psychoses distinct clinical entities?

PURPOSE: To evaluate further the relevance of designating psychotic episodes as either postictal or interictal, we compared several biologic variables between epilepsy patients with and without psychosis. METHODS: The study subjects comprised 282 patients with psychosis (36 with postictal psychosis, 224 with interictal psychosis, and 22 with both postictal and interictal psychoses, i.e., bimodal psychosis), and 658 epilepsy patients without psychosis. The clinical characteristics of these patients were reviewed retrospectively by experienced neuropsychiatrists. Factors predicting the development of each type of psychosis were determined by serial multivariate logistic regression analyses. RESULTS: Factors that were comparable between postictal and interictal psychoses were intellectual function, family history of psychosis, epilepsy type, and the presence of complex partial seizures. In contrast, age at the onset of epilepsy and at the onset of psychosis and the presence of generalized tonic-clonic seizures differed for the three types of psychosis. Patients with bimodal psychosis showed characteristics associated with both postictal and interictal psychoses. CONCLUSIONS: This study documented conditions, including both general factors and epilepsy-related factors, common to epilepsy patients with psychosis, regardless of chronologic distinctions. Certain epileptic processes appear to have equal influence on postictal and interictal psychoses. However, some differences between postictal and interictal psychoses suggest that these chronologic descriptors are valid. Our findings confirmed that psychosis associated with epilepsy should not be defined as a single, simple condition but rather as a complex condition with several possible subcategories.

[Complicated benign partial epilepsy]. / Epilepsia parcial benigna complicada.

OBJECTIVE: We review the complicated forms of different types of idiopathic benign partial epilepsy pointing out their relation to continuous point and wave tracings in slow sleep (POCS), the epileptic opercular syndrome and the Landau Kleffner syndrome. We insist on the role of some anti epileptic drugs in triggering the problem and withdrawal of these drugs as the most important aspect of the treatment of these complications replacing them by CLB in all cases. DEVELOPMENT: We describe various clinical cases which illustrate the problem

Utility of the scalp-recorded ictal EEG in childhood epilepsy.

PURPOSE: To evaluate the usefulness of the scalp-recorded ictal EEGs in diagnosing childhood epilepsy. METHODS: We analyzed the ictal EEGs of 259 seizures in 183 patients who visited the department of child neurology, Okayama University Medical School, during the past 6 years. RESULTS: We divided all seizures into the following four categories, according to the diagnostic usefulness of ictal EEGs in determining the seizure type: 1. (a) Ictal EEGs confirmed the diagnosis of the seizure type based on seizure symptoms (101 seizures); (b) Ictal EEGs aided in the classification of the seizure type based on the seizure symptoms (101 seizures); (c) Ictal EEGs corrected errors in the classification (37 seizures); and (d) Ictal EEGs revealed previously unreported/undocumented seizure type (20 seizures). 2. Of the 37 misdiagnosed seizures (group C), 11 were nonepileptic seizures misdiagnosed as epileptic seizures, eight were complex partial seizures (CPS) misdiagnosed as the other seizure types, and 10 were other seizure types misdiagnosed as CPSs. 3. Of the 20 previously unreported/undocumented seizures (group D), nine were myoclonic seizures, five were absence seizures, five were CPS, and one was tonic spasms. 4. Seventy-two patients had CPS. Among them, 11 patients showed no epileptic spikes in their interictal EEG recordings. Therefore, ictal recordings confirmed the diagnosis of epilepsy. CONCLUSIONS: Ictal EEG recording is a very useful diagnostic tool not only for determining seizure types, but also for uncovering the existence of the unsuspected seizure types. It supplies the physician with useful information for the classification and the treatment of epilepsy. In particular, ictal EEGs are useful in diagnosing patients with CPS.

Benign partial epilepsy in infancy with complex partial seizures (Watanabe's syndrome): 12 non-Japanese new cases.

International epilepsy classification includes different epileptic syndromes with favourable outcomes in pediatric age. In addition to these, other forms probably exist and in various papers in international literature they are proposed as new entities. This article presents a survey of benign complex partial epilepsy in infancy, a new epileptic syndrome first proposed by Watanabe, in 1987. Our work represents the only description of non-Japanese cases although similar but familial cases had been referred by Vigevano in 1992. We present data for 12 children (aged up to 9 years) followed over 2 years who had all the typical clinical features characterizing Watanabe's cases. For all of them we obtained EEG seizure recordings demonstrating the partial nature of their fits, arising from occipital or temporal regions. Interictal EEG were completely normal, both in waking and sleep. Evolution demonstrated benign outcome and all the children are seizure-free (eight of them have already stopped all medication) and all have normal psychomotor development.

Recurrent seizures in metachromatic leukodystrophy.

The unusual presentation of juvenile onset metachromatic leukodystrophy (MLD) and frequent complex partial seizures in a patient led us to perform a retrospective study of 18 patients with MLD to identify the prevalence and type of recurrent seizures during the first 2 years of the disease. Five of 17 patients (29%) had developed recurrent seizures within 12 months of the onset of symptoms, and one patient was lost to follow-up. By 24 months after onset of symptoms, 5 patients were lost to follow-up, and 6 of the remaining 13 patients (46%) had developed recurrent seizures. In all, 7 patients, 4 with late infantile-onset and 3 with juvenile-onset disease, developed recurrent seizures. Four patients, including 3 with juvenile-onset disease had complex partial seizures. We conclude that recurrent seizures are common in MLD and may occur at any stage of the disease, particularly in patients with juvenile onset. Generalized seizures are more frequent in patients with late infantile-onset, whereas partial seizures are more common in those with juvenile-onset disease.

Electroclinical study of localization-related epilepsies in early infancy.

To clarify the characteristics of localization-related epilepsies in early infancy, we performed an electroclinical study of 28 epileptic patients whose first seizures occurred before 6 months of age. The patients were retrospectively divided into two groups: the seizure-controlled group (8 patients) and the refractory group (20 patients). The seizure-controlled group included the patients whose seizures were suppressed within 1 year after onset; the refractory group included all other patients. The characteristics of the refractory group were as follows: (1) most patients had serious underlying pathologies; (2) the seizure type in most cases was simple partial seizure or complex partial seizure without secondary generalization; (3) the interictal EEG showed focal abnormalities and severe dysrhythmia on the basic pattern associated with multifocal spikes in most cases; and (4) in some patients, West syndrome developed after localization-related epilepsies and generalized seizures appeared later in some cases.

Phenomenology of simple partial seizures.

Based on a sample of 325 inpatients we present the subjective experiences during simple partial seizures. In a majority of cases, auras comprised composed forms of different symptomatic qualities. We describe rules which seem to govern sequences of aura phenomena. Autonomous and vestibular sensations were shown to have preceding positions related to others, olfactory and gustatory sensations preferred a following position. The tentative explanation of the findings favours the idea of heterogeneity rather than the concept of a focal discharge in a simple partial seizures.

Basic mechanisms of seizure expression.

While there are many types of seizures, our understanding of their pathophysiology is limited to a few types. On the basis of the behavior of neurons during a seizure, two fundamental types of paroxysms are recognized--spike-wave electrographic seizures and tonic-clonic electrographic seizures. When the former type of paroxysm takes place throughout the forebrain, an absence seizure ensues. When the latter type of paroxysm takes place within a limited set of neurons, a simple partial or complex partial seizure takes place, depending on the functional anatomy. When the latter type of paroxysm takes place throughout the forebrain, a generalized convulsion takes place. The basic cellular and synaptic processes that underlie electrographic spike-wave and tonic-clonic paroxysms are complex and dissimilar between the two types of discharges. This paper reviews these multiple processes. The diverse pathophysiological mechanisms presented provide a theoretical substrate for rational polypharmacy directed to seizure suppression.

Classifications and epidemiologic considerations of epileptic seizures and epilepsy.

The manifestations of epileptic disorders are widely known to be extremely protean. Classifying the disorders is a way of achieving a common understanding of the terminology used in identifying seizure disorders in the clinical or research settings. Two International Classifications have been developed, namely the International Classification of Epileptic Seizures and the International Classification of Epilepsy and Epileptic Syndromes. The first divides epileptic seizures into two major categories: partial and generalized. Partial epileptic seizures are further classified according to the impairment or the preservation of consciousness into simple partial and complex partial seizures. Either condition may secondarily generalize into tonic-clonic seizures. Under the International Classification, epilepsy and epileptic syndromes are initially classified according to their corresponding types of seizures into localization-related and generalized disorders. Each disorder is further classified according to the relationship to etiologic or predisposing factors into symptomatic, cryptogenic, or idiopathic types. The classifications undoubtedly will be further revised as more is learned about epileptic disorders with advances in electrophysiology, neuroimaging techniques, and molecular genetics. An overview of the epidemiology of epileptic disorders shows that, contrary to the popular belief, they are primarily disorders of childhood; age-specific incidence rates of first unprovoked seizure and of epilepsy are highest in the elderly. An appreciation of the epidemiology of seizure disorders is essential in their clinical and laboratory evaluation.

The concept of an organic dissociative syndrome: what is the evidence?

The question of whether organic conditions, such as complex partial seizures, can cause dissociative symptoms is controversial. Although a diagnostic category for organic dissociation is included in the tenth edition of International Classification of Disease, it has never been identified in the Diagnostic and Statistical Manual. Its inclusion in the upcoming DSM-IV is currently under debate. This article surveys representative literature regarding the role of organic factors in the causation of dissociative symptoms and considers the differential diagnosis of organic dissociation from current and historic perspectives. Dissociative symptoms and disorders (including amnesia, fugue, depersonalization, multiple personality, automatisms, and certain furors) can be induced by a variety of medications, drugs of abuse, and medical illnesses or conditions affecting cerebral function. Organic dissociation can be distinguished from intoxication, amnestic disorder, and delirium. Psychiatric nosology and our conceptualization of altered mental states and functions would benefit from use of the concept of an organic dissociative syndrome, which has clinical, neurophysiologic, and medicolegal significance. Such a category should be included in DSM-IV.

Occurrence of epileptiform activity in the routine EEG of epileptic patients.

In a retrospective study, the occurrence of epileptiform activity (EA) in routine EEG records of epileptic patients was investigated. Data were obtained from 1078 EEGs of 373 patients (199 men and 174 women; aged 17-87 (mean 34.2 +/-14.7)). The percentage of 38% of patients with EA in a single EEG could be increased to 77% by repeated records. After the 5th record however, the gain in new information decreased remarkably. No differences between seizure types could be detected. There was a relationship between EA and nonspecific EEG abnormalities. High EA rates were found for short time intervals since last seizure, young patients, long durations of the seizure disorder and for high seizure frequencies.