Paediatric pain management--the next step?

Congenital bilateral perisylvian syndrome (CBPS) is a recently described, neuronal migration disorder, characterized by pseudobulbar palsy, epilepsy and mental retardation and bilateral perisylvian dysplasia. A 15-year-old boy was diagnosed with CBPS according to the typical clinical, and magnetic resonance imaging (MRI) features. The patient was suffering from atypical absence seizures, repeating daily in spite of antiepileptic drug therapy, since age 7 years. He had also experienced rare generalized tonic-clonic seizures and complex partial seizures. Neurological examination showed severe restriction of tongue movements, severe dysarthria, dysphagia, facial diplegia, mild pyramidal signs and moderate mental retardation. A computed tomographic (CT) scan demonstrated bilateral perisylvian enlargement. The diagnosis was corrected with MRI after six years. Frequent irregular generalized spike and wave abnormalities and focal sharp and slow waves over the posterior regions of both hemispheres were shown by electroencephalograms (EEG). The patient was treated with Na-Valproate, carbamazepine and lamotrigine but did now show any significant change in seizure frequency in the eight-year follow-up period. Intractable seizures, mental retardation and particularly congenital pseudobulbar palsy suggest this congenital entity. Those patients who exhibit these typically clinical features, must have MRI.     

Structural cerebral pathology in schizophrenia: regional or diffuse?

Is brain pathology in schizophrenia topographically distinct? If so, are the putative regional changes unique to the disorder? To address these questions, 56 chronic schizophrenic Ss were compared with 16 psychiatric control Ss with mood disorders and with 31 healthy volunteers on multiple-volume measures of regional cerebral atrophy obtained with computed tomography. Generalized cortical and subcortical enlargement of spaces filled with cerebrospinal fluid sparing only the occipitoparietal cortex was found in the schizophrenic Ss compared with normal control Ss. Statistically significant differences in the extent of perisylvian atrophy were noted between schizophrenic Ss and patients with mood disorders: Schizophrenic Ss evidenced greater dilation of perisylvian fissures and sulci. The implications of the results for future research and for recent theories on the etiology of schizophrenia are discussed.     

Locus of lesion in defective color association.

The relationship between locus of lesion and color association among aphasics was investigated in 2 separate and distinct studies. Data were obtained at different medical centers, with different examiners, different means of screening for color blindness, different procedures for assessment of color amnesia, and with different computerized tomography (CT) imaging equipment and interpreters. Nevertheless, each showed that color association defects were associated with posterior perisylvian lesions and basal ganglia lesions in the left hemisphere. At the same time, both studies also showed that extensive posterior perisylvian lesions and basal ganglia lesions did not inevitably cause defects in color association. Implications of the findings for impaired color association and hemispheric cerebral dominance are discussed. (PsycINFO Database Record (c) 2010 APA, all rights reserved)     

Impact of chromosome 14q loss on survival in primary head and neck squamous cell carcinoma

OBJECTIVE: To investigate somesthetic functions of the perisylvian cortex. METHODS: Somatosensory evoked magnetic fields (SEFs) and somatosensory evoked potentials (SEPs) of the perisylvian cortex were recorded directly from subdural electrodes in a patient with a left frontal brain tumour. RESULTS: The most prominent SEP components after electrical stimulation of the right and left hands and the right foot were double peaked negativity recorded just above the sylvian fissure (latency 80 to 150 ms), respectively (N1a and N1b). Generator sources for the magnetoencephalographic counterparts of those peaks (N1a(m) and N1b(m)) were both localised at the upper bank of the sylvian fissure, and those of N1a(m) were more anteromedially located than those of N1b(m). CONCLUSIONS: These findings suggest the existence of at least two separate somatosensory areas within the human perisylvian cortex.     

Progressive loss of speech output with festinating speech--a case report]

Primary progressive aphasia is a rare disorder of unknown cause. We report a patient with progressive loss of speech output, a clinical variant of PPA, characterized by festinating speech. A 60-year-old right handed woman was admitted to our hospital, because of progressive deterioration of her speech. On admission, she was alert and orientated without dementia. A severe impairment of her articulation was observed: her speech rate was so fast that her speech became almost intelligible. The orofacial apraxia and difficulty in tapping were also present. The other neurological findings were normal. Neuroradiological studies showed the left perisylvian atrophy. Festinating speech has not been previously reported in patients with PPA; patient with PPA usually show a slow speech rate with effortful expression. Since festinating speech is occasionally present in the extrapyramidal disorders, such as Parkinson's disease, progressive supranuclear palsy, or pure akinesia, it appears likely that the combined lesions of the perisylvian region and the basal ganglia are responsible for her characteristic speech disorder with festinating speech.     

Idiopathic epilepsy with generalized seizures in early childhood]

Idiopathic epilepsies with generalized seizures of early childhood are based on a genetic predisposition. The onset takes place between the first and fifth years of age, boys are affected more often than girls. Dependent on the clinical symptomatology you have to distinguish: myoclonic seizures; atonic-astatic seizures; myoclonic-astatic seizures; absences; tonic-clonic seizures. In more than half of the cases a combination of these seizures can be observed. The differentiation of epilepsies with generalized seizures of multifocal origin (infantile spasms, Lennox-Gastaut syndrome and Pseudo-Lennox syndrome [atypical benign epilepsy]) may be difficult but is essential. Therapy of choice is valproate, often in combination with ethosuximide (in children with minor seizures) or with kaliumbromide or phenobarbital (in children with tonic-clonic seizures). Generally the prognosis is more unfavourable if epilepsy starts in the first year of life with afebrile and febrile generalized tonic-clonic or clonic seizures, if children are suffering from longlasting states of seizures and if development is disturbed before beginning of epilepsy.     

Semiological seizure classification

We propose an epileptic seizure classification based exclusively on ictal semiology. In this semiological seizure classification (SSC), seizures are classified as follows: a. Auras are ictal manifestations having sensory, psychosensory, and experiential symptoms. b. Autonomic seizures are seizures in which the main ictal manifestations are objectively documented autonomic alterations. c. "Dialeptic" seizures have as their main ictal manifestations an alteration of consciousness that is independent of ictal EEG manifestations. The new term "dialeptic" seizure has been coined to differentiate this concept from absence seizures (dialeptic seizures with a generalized ictal EEG) and complex partial seizures (dialeptic seizures with a focal ictal EEG). d. Motor seizures are characterized mainly by motor symptoms and are subclassified as simple or complex. Simple motor seizures are characterized by simple, unnatural movements that can be elicited by electrical stimulation of the primary and supplementary motor area (myoclonic, tonic, clonic and tonic-clonic, versive). Complex motor seizures are characterized by complex motor movements that resemble natural movements but that occur in an inappropriate setting ("automatisms"). e. Special seizures include seizures characterized by "negative" features (atonic, astatic, hypomotor, akinetic, and aphasic seizures). The SSC identifies in detail the somatotopic distribution of the ictal semiology as well as the seizure evolution. The advantages of a pure SSC, as opposed to the current classification of the International League Against Epilepsy (ILAE), which is actually a classification of electroclinical syndromes, are discussed.     

Munchausen syndrome by proxy: report of one case with epilepsy

INTRODUCTION: Munchausen syndrome by proxy (MSBP) is a rare form of child abuse in which a parent, usually the mother, fabricates or produces illness in a child, so causing them unnecessary medical investigations, treatments and hospitalizations. One of the commonest false presenting symptom is 'seizures'. CLINICAL CASE: An eight years old boy with Munchausen syndrome by proxy is reported. This child had had genuine seizures well controlled by standard anticonvulsant treatment at the start of the false illness. At the age of seven years, the patient showed very frequent seizures. The child was treated with antiepileptic drugs, but treatments were ineffective and seizures continued. Results of multiple tests, including an extensive blood chemistry analyses, CT, MRI, SPECT, were normal. Electroencephalogram showed posterior slow waves. Acute neurological deterioration was observed six weeks after hospitalization and it was finally proved that seizures were caused or triggered by clomipramine poisoning given by her mother. CONCLUSIONS: MSBP frequently presents as epileptic seizures in these abused children. MSBP diagnosis is more difficult to be made if true seizures exists with multiple fictitious seizures. Pediatrician should be alerted to the possibility of MSBP when seizures are poorly controlled, treatments are ineffective and there is no neurophysiologic dysfunction. Early diagnosis and intervention are essential because high mortality and psychologic morbidity are associated with this syndrome.     

Radiation sensitivity of lymphocytes from human blood and from the thoracic duct

Rats were cerebellectomized 72-96 hr prior to evaluation (1) during maximum electroshock seizures and (2) for their capacity to respond to pentylenetetrazol-induced clonic seizures. Cerebellectomized rats failed to exhibit tonic hindlimb extension, an endpoint characteristic of maximal electroshock seizures. The dose of pentylenetetrazol required to produce clonic seizures or death was not different in cerebellectomized and sham-operated controls. The anticonvulsant efficacy of diazepam, when assessed as a pentylenetetrazol antagonist, was not influenced by removal of the cerebellum. These data indicate that whereas cerebellar influences may suppress seizure activity which is largely focal, seizures of more diffuse origin are not markedly influenced by cerebellar activity. It is, therefore, essential that the role of the cerebellum in suppressing seizures be characterized for each kind of experimentally induced seizure process.     

Epilepsy and the heart

The relations between epilepsy and heart are complex and expressed in two opposite sides. (1) Cardiac arrhythmias may provoke epileptic seizures but these seizures are, in this case, syncopal attacks. Nevertheless, in the past, these clinical features have been individualized as "cardiac epilepsy" or epilepsy in cardiacs. However, true epileptic seizures could be observed in the course of a syncopal attack and a syncope may complicate the issue of an epileptic seizure. (2) On the other hand, epileptic seizures may provoke severe cardiac arrhythmias. The incidence rate of sudden death in patients with epilepsy is estimated to be 1/1000 patients. The exact neural mechanisms in cardiac arrhythmias seizures could explain only some of the sudden unexpected deaths observed in epileptic patients. The role of antiepileptic drugs on cardiac conduction as well as the effects of seizures or status epilepticus on the myocardium are other enigmatic aspects of the relations between epilepsy and heart.     

Otoneurologic abnormalities in insulin-dependent diabetes

The Authors discuss the role of plasma prolactin concentration as a marker to discriminate epileptic and non epileptic seizures. In 40 children was determined prolactin during the first seizure (30 feverish and 10 non feverish seizures). The analysis of the results shows that during non feverish seizures prolactin concentration in higher, but we don't know if the same seizures are the beginning of epilepsy. So plasma prolactin concentration is a parameter of moderate interest in diagnosis and prognosis in childhood paroxismal disorders.     

Dynorphin and epilepsy

Studies on dynorphin involvement in epilepsy are summarised in this review. Electrophysiological, biochemical and pharmacological data support the hypothesis that dynorphin is implicated in specific types of seizures. There is clear evidence that this is true for complex partial (limbic) seizures, i.e. those characteristic of temporal lobe epilepsy, because; (1) dynorphin is highly expressed in various parts of the limbic system, and particularly in the granule cells of the hippocampus; (2) dynorphin appears to be released in the hippocampus (and in other brain areas) during complex partial seizures; (3) released dynorphin inhibits excitatory neurotransmission at multiple synapses in the hippocampus via activation of kappa opioid receptors; (4) kappa opioid receptor agonists are highly effective against limbic seizures. Data on generalised tonic-clonic seizures are less straightforward. Dynorphin release appears to occur after ECS seizures and kappa agonists exert a clear anticonvulsant effect in this model. However, more uncertain biochemical data and lack of efficacy of kappa agonists in other generalised tonic-clonic seizure models argue that the involvement of dynorphin in this seizure type may not be paramount. Finally, an involvement of dynorphin in generalised absence seizures appears unlikely on the basis of available data. This may not be surprising, given the presumed origin of absence seizures in alterations of the thalamo-cortical circuit and the low representation of dynorphin in the thalamus. In conclusion, it may be suggested that dynorphin plays a role as an endogenous anticonvulsant in complex partial seizures and in some cases of tonic-clonic seizures, but most likely not in generalised absence. This pattern of effects may coincide with the antiseizure spectrum of selective kappa agonists.     

Mass ingestion of Jimson Weed by eleven teenagers

Most human seizures occur early in life,consistent with established excitability-promoting features of the developing brain. Surprisingly, the majority of developmental seizures are not spontaneous but are provoked by injurious or stressful stimuli. What mechanisms mediate'triggering' of seizures and limit such reactive seizures to early postnatal life? Recent evidence implicates the excitatory neuropeptide, corticotropin-releasing hormone (CRH). Stress activates expression of the CRH gene in several limbic regions, and CRH-expressing neurons are strategically localized in the immature rat hippocampus, in which this neuropeptide increases the excitability of pyramidal cells in vitro. Indeed, in vivo, activation of CRH receptors--maximally expressed in hippocampus and amygdala during the developmental period which is characterized by peak susceptibility to 'provoked' convulsions--induces severe, age-dependent seizures. Thus, converging data indicate that activation of expression of CRH constitutes an important mechanism for generating developmentally regulated, triggered seizures, with considerable clinical relevance.     

Rhabdomyolysis in transplant patients

In a retrospective analysis of all our patients with seizure onset prior to age 16 years, 25 patients with primary generalized tonic (n = 10) or tonic-clonic (n = 15) seizures were identified. These patients constituted 5.7% of the total seizure patient population in our institute between the ages of 1 month and 16 years. The natural history of generalized tonic-clonic seizures is known to be benign; however, that of isolated primary generalized tonic seizures is not clear. Therefore, an attempt was made to characterize the patients suffering from primary generalized tonic seizures and determine their outcome. Analysis of our patient population shows that both seizure types are characterized by early onset of generalized seizures that appear in normally developed children with a normal electroencephalographic background. The children usually respond quickly to antiepileptic drugs. A long-term follow-up (mean period of 7.6 years) was possible in 84% of the patients, and showed that 95% of them were seizure free at the end of the follow-up period. There was no significant difference between the two groups in regard to age of onset, family history, and seizures at follow-up. In conclusion, the natural history of patients with generalized tonic seizures is similar to the benign course of those with generalized tonic-clonic seizures.     

The potentials for using cocultivation systems in assisted reproductive technologies

A cross-sectional study covering four adjoining villages in Haryana during 1993 was carried out among 8595 subjects to find out cases having seizures or paralysis. The prevalence of seizures (n = 69) was found to be 8.03 per 1000 population. Out of 69 seizures, 48(69%) were true seizures and 21(31%) were febrile seizures. The prevalence of seizures was highest (14.05 per 1000) in the age group of > 1-4 years. Of 40 males with seizures, 22(55%) had epileptic seizures and 18(45%) had febrile seizures. Of the 29 females, 26(89%) had epileptic seizures and 3(11%) had febrile seizures. There were 25 cases of pseudoseizures. The prevalence of paralysis (n = 60) was 6.98 per 1000. Of these, 38 cases (63%) were due to poliomyelitis which is the highest so far the paralysis is concerned. Of 60 cases, 34 occurred among males and 26 among females. The prevalence of paralysis was highest (12.42 per 1000) among 0-1 year age group. As the prevalence of seizures is sufficiently high in rural areas, the primary health care doctors should be trained in counselling and therapy of seizures. As regarding paralysis due to poliomyelitis the recent success of pulse polio immunisations is commendable and which in near future is expected to eradicate poliomyelitis.     

Prenatal ultrasound diagnosis of seizures

We present a case of fetal diagnosed by ultrasound. Clinical aspects of this patient and the differential diagnosis of early neonatal seizures are discussed. A diagnosis of fetal seizures confirmed by ultrasonography is a rare event. Review of the literature shows only four cases of documented fetal seizures. This report describes a case of fetal seizures diagnosed in utero, its management, and the neonatal outcome. We also discuss the differential diagnosis.     

Treatment of malignant gliomas: a new approach

The etiology of seizures associated with cocaine use is unclear. Because cocaine seizures are relatively uncommon, they should be diagnosed by exclusion and a neurological workup to rule out central nervous system (CNS) catastrophe should be made. This report describes the clinical findings, treatment, and blood cocaine and metabolite concentrations in a patient who, on two separate occasions, had seizures associated with crack cocaine ingestion. Approximately 1 hour after the ingestion incidents, the patient had multiple, generalized seizures that abated spontaneously. His workup for CNS bleeding, infection, and trauma was negative. Cocaine concentrations on the first incident peaked at 2.48 mg/L and on the second incident peaked at 3.9 mg/L. Other clinical findings included tachycardia, hypertension, diaphoresis, and disorientation. Blood cocaine and metabolite analysis revealed extremely high concentrations. Other than the incident of seizures and transient cardiovascular aberrations, these high concentrations were tolerated by the patient without further sequelae. A review of cocaine-induced seizures and treatment is included.     

Seizures: classification, etiologies, and pathophysiology

Epilepsy is a disorder of recurrent seizures that are neural in origin. Partial seizures are usually due to a structural cerebrocortical lesion and may be simple or complex. Brain injuries may alter inherent neuronal properties and neuronal circuits and lead to recurrent excitatory activity. Potentiation of excitatory synapses and depression of inhibitory synapses are probable critical events in epileptogenesis. The pathogenic factors underlying primary or idiopathic generalized seizures are not as well understood. A more diffuse or multifocal state of neuronal excitability may be the result of early congenital events that are magnified over time. The progression of subclinical neuronal excitatory activity to a clinical seizure may relate directly to the phenomenon of cortical plasticity.     

Medical causes of seizures

Seizures are commonly encountered in patients who do not have epilepsy. Factors that may provoke such seizures include organ failure, electrolyte imbalance, medication and medication withdrawal, and hypersensitive encephalopathy. There is usually one underlying cause, which may be reversible in some patients. A full assessment should be done to rule out primary neurological disease. Treatment of seizures in medically ill patients is aimed at correction of the underlying cause with appropriate short-term anticonvulsant medication. Phenytoin is ineffective in the management of seizures secondary to alcohol withdrawal, and in those due to theophylline or isoniazid toxicity. Control of blood pressure is important in patients with renal failure and seizures. Non-convulsive status epilepticus should be considered in any patient with confusion or coma of unclear cause, and electroencephalography should be done at the earliest opportunity. Most ill patients with secondary seizures do not have epilepsy, and this should be explained to patients and their families. Only those patients with recurrent seizures and uncorrectable predisposing factors need long-term treatment with anticonvulsant medication.     

Prolonged and monosymptomatic dysphasic status epilepticus

Dysphasic seizures are an infrequent form of epilepsy, and their serial appearance as a partial status epilepticus is quite exceptional. The young patient reported here had a partial dysphasic status epilepticus of 3 weeks' duration without other temporal lobe seizures. Simultaneous serial electroencephalograms, tape recordings of the seizures, and repeated neuropsychologic ictal examinations permitted studies of increased impairment of neuropsychologic function on testing and the appearance of new irritative discharges on encephalography.