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R J Wilkus

Publications and source records attributed to R J Wilkus.

At least 19 recordsLinked to original sources

Temporal ictal electroencephalographic frequency correlates with hippocampal atrophy and sclerosis.

We studied 328 complex partial seizures (CPS) in 63 consecutive patients with temporal lobe epilepsy who underwent scalp electroencephalography/video monitoring, magnetic resonance imaging (MRI), and surgery. The initial ictal discharge (IID), defined as the first sustained electrical seizure pattern localized to the surgical site, was determined. If the IID was rhythmic waves, the median frequency was measured. To determine if IID frequency correlates with hippocampal atrophy (HA) or sclerosis (HS), hippocampal volume ratios (HVRs) were measured (n = 52) or assessed visually (n = 11) on MRI, and mesial temporal histopathology specimens (n = 22) were graded for HS. Sixteen patients (25%) had no or mild HA (HVR = 0.78-1.02), and 47 patients (75%) had moderate-to-marked unilateral (HVR = 0.33-0.76), or bilateral, HA. Theta frequency IIDs were significantly more commonly associated with moderate-to-marked HA than were delta IIDs. Theta frequency IIDs occurred in 19% of patients with mild or no HA, and 79% of patients with moderate-to-marked HA; delta IIDs occurred in 63% of patients with little to no HA, and 13% of those with moderate-to-marked HA. In addition, the median IID frequency inversely correlated with HVR and directly correlated with HS severity. In conclusion, faster frequency rhythmic IIDs during temporal lobe CPS correlate with greater degrees of ipsilateral HA on MRI, and higher grades of HS.

Adolescent↗

Is partial epilepsy progressive? Ten-year follow-up of EEG and neuropsychological changes in adults with partial seizures.

PURPOSE: This study was undertaken to determine what changes, if any, occur in the electroencephalogram (EEG) and in neuropsychological test findings of adults with medically intractable complex partial epilepsy over a 10-year period. METHODS: We studied 35 adults, with a mean age of 32 years (range, 16-59 years) at time of initial testing. We compared the distribution of epileptiform patterns documented on the initial pair of waking and sleeping EEGs to those observed on another pair obtained 10 years later. During this same 10-year period, we also examined changes in the Wechsler Adult Intelligence Scale (WAIS) and on the tests from the Neuropsychological Battery for Epilepsy. RESULTS: The EEGs of 28 (80%) of patients at the tenth year were identical to those seen initially. Five (14%) of patients demonstrated EEGs after 10 years with either no discharges or only unilateral discharges, where bilateral discharges were seen a decade earlier. Only two (6%) of patients had EEGs at the tenth year that showed bilateral discharges where only unilateral discharges were seen originally. We found no general change in intelligence or neuropsychological functioning after 10 years, although a few subtle losses were noted on several neuropsychological measures. CONCLUSIONS: For most adults with medically intractable complex partial epilepsy, the EEG and neuropsychological test scores remain reasonably stable over a decade.

Adolescent↗

Cataplexy and monoamine oxidase deficiency in Norrie disease.

Norrie disease (ND) is an X-linked recessive disorder causing ocular atrophy, mental retardation, deafness, and dysmorphic features. Virtually absent monoamine oxidase (MAO) type-A and -B activity has been found in some boys with chromosome deletions. We report the coexistence of cataplexy and abnormal REM sleep organization with ND. Three related boys, referred for treatment of medically refractory atonic spells and apneas, underwent extended EEG-video-polysomnographic monitoring. They demonstrated attacks of cataplexy and inappropriate periods of REM sleep during which they were unarousable. One boy also had generalized tonic-clonic seizures. Previous testing revealed that all three have complete ND gene deletions. In all subjects, platelet MAO-B activity was absent, serum serotonin levels were markedly increased, and plasma catecholamine levels were normal. Data from the canine narcolepsy syndrome model implicate abnormal catecholaminergic and cholinergic activities in the pathogenesis of cataplexy. Our findings suggest that abnormal MAO activity or an imbalance between serotonin and other neurotransmitter levels may be involved in the pathogenesis of human cataplexy.

Adult↗

Multiple subpial transections for partial seizures in sensorimotor cortex.

We report six patients with complex partial seizures arising from the primary sensorimotor cortex who underwent invasive long-term ictal electroencephalogram/video monitoring and brain mapping and then multiple subpial transections. Although four patients demonstrated no abnormalities on magnetic resonance imaging, each patient showed moderate to marked gliosis in cortex biopsied from the site of ictal onset. Extensive preoperative and postoperative neuropsychological tests demonstrated no functional deficits resulting from surgery. Only one patient failed to derive significant postoperative seizure improvement, and he subsequently underwent additional subpial sectioning without further significant improvement. We propose a modification for this surgical technique and hypothesize that these patients may represent a syndrome of central cortical epilepsy.

Adolescent↗

Use of psychological and neuropsychological variables in selection of patients for epilepsy surgery.

Cortical resection surgery for epilepsy is costly, involves risk, and does not result in significant seizure relief in a number of patients. Therefore, it is important to develop methods which will as accurately as possible identify in advance those persons who will or will not be helped by this procedure. From a review of the literature and from a presentation of original data, it is concluded that psychological/neuropsychological variables are valid predictors of cessation of seizures following surgery but that they are only infrequently used. A multidisciplinary, multivariate statistical approach, using EEG findings and psychological/neuropsychological variables in combination, provides the most accurate predictions which are superior to those derived from using either EEG variables or psychological/neuropsychological variables alone. This approach also avoids much of the predictive instability encountered when single variables are evaluated. Since psychological/neuropsychological variables are obtained at low cost and are non-invasive, further evaluation of their role as predictors of seizure relief following surgery is needed.

Adolescent↗

Etiology and prognosis of alpha coma.

We reviewed our experience with alpha coma, the finding of predominantly alpha-frequency rhythms in the EEGs of unconscious patients, and identified 50 patients. Cardiac arrest, either inside (n = 25) or outside (n = 24) the hospital, was the cause of unconsciousness in all except one patient who had hyperglycemic, hyperosmolar coma. After out-of-hospital cardiac arrest, the risk of never regaining consciousness or dying during hospitalization did not differ significantly between unconscious patients with (n = 24) and without (n = 69) alpha frequencies in their EEGs. A review of the literature supports our findings that alpha coma most commonly follows cardiac arrest and does not preclude the possibility of neurologic recovery. We conclude that alpha coma is a descriptive term and lacks prognostic significance in itself.

Alpha Rhythm↗

Multidisciplinary prediction of seizure relief from cortical resection surgery.

Preoperative variables from a full range of medical specialties were used to predict degree of seizure relief from cortical resection surgery as treatment for epilepsy in 100 patients. General, seizure history, electroencephalographic (EEG), radiological, surgical, and psychological/neuropsychological data were considered. The patients were divided into one large predictive group (n = 75) and a smaller independent cross-validation sample (n = 25). Eight predictive variables emerged: single EEG focus; anterior-midtemporal lobe discharges; discharges only from the side of surgery; rate of occurrence of discharges in surgical area; Wechsler Adult Intelligence Scale Digit Symbol subtest; Marching Test, preferred hand, time; Minnesota Multiphasic Personality Inventory (MMPI) Hysteria scale score; and MMPI Paranoia scale score. By use of multivariate procedures, increased predictability of surgical outcome was obtained not only with the predictive group but with the independent cross-validation sample. The results demonstrate that predictions of seizure relief from epilepsy surgery can be made with 80% accuracy using multiple, rather than single, predictors.

Adolescent↗

Intensive EEG monitoring and psychological studies of patients with pseudoepileptic seizures.

EEG/closed-circuit television long-term monitoring was used as a definitive diagnostic tool to identify and characterize 25 patients with pseudoepileptic seizures and a similar group of subjects with epilepsy, confirming the value of the procedure. The groups did not differ with respect to intelligence, neuropsychological impairment, or incidence of potential etiological factors for seizures. Scores on the Minnesota Multiphasic Personality Inventory (MMPI) and the Hypochondriasis, Hysteria, and Schizophrenia Scales were significantly higher for the pseudoepileptic group than for the other subjects. As a whole, the former patients exhibited an MMPI profile pattern frequently seen in the conversion form of hysteria. A set of three rules derived from the MMPI profiles was used to classify the patients correctly in 80-90% of cases. As evaluated by the Washington Psychosocial Seizure Inventory, psychosocial problems of patients with pseudoepileptic seizures were more severe in certain areas, and appeared to reflect early family background problems and inappropriate management of their disorders.

Adult↗

Carbamazepine and the electroencephalogram of epileptics: a double blind study in comparison to phenytoin.

In double blind crossover 4 month trials, carbamazepine was compared to phenytoin as sole treatment for 45 patients with uncontrolled partial and generalized epilepsy. EEGs performed at the end of these trials revealed that while using carbamazepine the patients manifested a significant overall increase in diffuse slow waves and an increase in generalized epileptiform discharges without significant accompanying changes in seizure incidence. Also, during the carbamazepine trial generalized epileptiform discharges activated by hyperventilation were more frequent in patients with a higher seizure incidence compared to subjects with a lower seizure incidence of patients taking phenytoin. No significant focal EEG changes occurred.

Adult↗

Neuropsychological correlates of the electroencephalogram in epileptics: III. Generalized nonepileptiform abnormalities.

A broad battery of neuropsychological tests was given to 111 adult epileptics treated primarily with phenytoin. The patients were grouped according to degree of generalized nonepileptiform EEG abnormalities in waking EEGs. In patients classified as None/Mild, Moderate, or Marked in terms of these EEG abnormalities performances on 15 neuropsychological measures were contrasted. Statistically significant differences across the groups were found on 13 of the 15 neuropsychological variables. An orderly decrease in performance occurred as a correlate of an increase in EEG abnormalities. These results were compared to a similar analysis of the correlates of topographic distribution of epileptiform discharges (Absent, Focal, Generalized). It was discovered that degrees of generalized slow wave EEG abnormalities were more potent in differentiating levels of neuropsychological performance than epileptiform discharges. Nevertheless, when discharges and degree of nonepileptiform abnormalities were simultaneously considered, a maximal level of correlation was reached.

Adolescent↗

Neuropsychological correlates of anticonvulsants and epileptiform discharges in adult epileptics.

Neuropsychological correlates of anticonvulsants and of EEG epileptiform discharges were examined through the administration of neuropsychological measures to groups of adult epileptics. Among the anticonvulsants, performances with high and low serum phenytoin levels were compared and performances with sulthiame vs. phenytoin and carbamazepine vs. phenytoin were evaluated. Various drug effects from these studies were noted and there was some suggestion that the neuropsychological impacts of specific anticonvulsants may be selective rather than general. The neuropsychological correlates of the topographic distribution and average rate of occurrence of discharges were considered. Striking neuropsychological correlates were found with the poorest performances in connection with discharges which were generalized and those which were at a rate greater than 1/min. The importance of using a broad range of neuropsychological measures was stressed.

Adolescent↗

Usher syndrome in four hard-of-hearing siblings.

Retinitis pigmentosa (RP) and congenital deafness comprise the autosomal recessive Usher syndrome. The four affected siblings discussed here have audiometric curves characteristic of the 10% of patients with the syndrome who are not profoundly deaf. The oldest already has RP. Even though the younger three siblings have no visual symptoms, they do have auditory signs of the syndrome; they almost certainly will develop RP and become deaf-blind adults. Electroretinography indicates that the second oldest boy has early evidence of rod dysfunction. Special audiometric tests and electronystagmographic results support previous reports of a peripheral rather than central auditory lesion. The heterozygote parents show unilateral high-frequency hearing losses with normal retinal and vestibular function.

Adolescent↗

Neuropsychological correlates of the electroencephalogram in epileptics: I. Topographic distribution and average rate of epileptiform activity.

In 57 male and 33 female uncontrolled adult epileptics treated solely by diphenylhydantoin, scores on a broad battery of neuropsychological tests were compared with the separate and simultaneous consideration of the topographic distribution and average rate of occurrence of their EEG epileptiform discharges. These studies were given within a median of 1.24 days of each other. In the battery of tests, it was statistically significant that the group with generalized discharges did the poorest, patients with focal epileptiform activity were intermediate, and those without discharges did the best. When the rates of occurrence of discharges were compared to the neuropsychological test scores, results were less often significantly correlated with the EEG findings. In general, however, individuals with discharges occurring at the rate of more than 1/min did worse than those with fewer discharges. When the topographic distribution and average rate variables were considered in combination, their impacts on psychological performance were cumulative. The results suggest that EEG epileptiform discharges serve as an index of brain function having a bearing upon neuropsychological performance of epileptics.

Adult↗

Neuropsychological correlates of the electroencephalogram in epileptics: II. The waking posterior rhythm and its interaction with epileptiform activity.

Relationships between psychological functions and waking dominant posterior rhythm frequency (DPRF) of the EEG were evaluated with and without simultaneous evaluation of epileptiform discharges. Ninety adult epileptics with uncontrolled seizure disorders treated solely by diphenylhydantoin underwent EEGs during wakefulness and administrations of an extensive battery of neuropsychological tests. The DPRF was particularly related to abilities simultaneously requiring attention an complex mental manipulations. Rhythms slower than 7.5-8.0 Hz were accompanied by relatively poorer performance than rhythms which were faster. When epileptiform discharges were considered in combination with the DPRF, larger differences in abilities were demonstrated, and the psycholoigcal correlates tended to be more substantial for the epileptiform discharge variable than for the DPRF variable. The selection of tests employed and the range of rhythms assessed are critical to these findings, and the substantial construction of these variables by many previous investigators is believed to be related to the negative findings often reported.

Alpha Rhythm↗

Electrophysiologic observations in the classical form of Pelizaeus-Merzbacher disease.

In a 20-year-old patient with the classic form of Pelizaeus-Merzbacher disease, electroencephalograms during wakefulness were moderately diffusely abnormal, and an overnight polygraphic sleep recording showed distorted nonrapid eye movement sleep patterns without vertex sharp waves, K-complexes, spindles, or positive occipital sharp transients. Rapid eye movement sleep could be identified. Cerebral responses evoked by light flashes, clicks, and electric stimulation of the median nerves were abnormal.

Adult↗

Relationships between intelligence and electroencephalographic epileptiform activity in adult epileptics.

Intellectual correlates of electroencephalographic epileptiform activity were examined by administering the Wechsler Adult Intelligence Scale to epileptic patients divided into groups on the basis of presence or absence, average rate, and topographic distribution of discharges. The results showed that lower intelligence levels were associated with the presence of discharges, especially when they were generalized rather than focal and when they occurred at an average rate of more than one per minute. An examination of patients having discharges ipsilateral and contralateral to their handedness showed few findings, as did an analysis based on an assessment of strengths and weaknesses within individuals. The results suggest that electroencephalographic epileptiform activity is significantly related to the intellectual functioning of epileptics.

Adult↗