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Biomedical subjects

C T Lombroso

Publications and source records attributed to C T Lombroso.

At least 37 records · Page 2Linked to original sources

Early myoclonic encephalopathy, early infantile epileptic encephalopathy, and benign and severe infantile myoclonic epilepsies: a critical review and personal contributions.

Nosological confusion within the epilepsies with myoclonic manifestations occurring in early life has led several epileptologists to separate more rigorously true myoclonic seizures from pseudomyoclonic ones and to identify clusters of homogeneous parameters that may lead to the formulation of syndromatic groupings. In recent years, four neonatal, infantile, and early myoclonic syndromes have been proposed: early myoclonic encephalopathy (EME), early infantile epileptic encephalopathy (EIEE), benign infantile myoclonic epilepsy (BIME), and severe infantile myoclonic epilepsy (SIME). These are reviewed critically, historically, and in the context of personal observations. The author's conclusions are that there is some justification to support, provisionally, a nosological place for the EME syndrome, that a nosologically separate position for the EIEE syndrome appears less firm, and that it seems safer to consider it at this time as an early variant of the West syndrome. From personal observations it appears that BIME and SIME, while justifiably constituting recognizable entities, may best be combined into a single syndrome of "infantile myoclonic epilepsy following febrile convulsions," with variable clinical outcomes.

Cerebral Cortex↗

Neonatal herpes simplex meningoencephalitis: EEG investigations and clinical correlates.

We studied the sequential EEGs of 15 neonatal herpes simplex virus meningoencephalitis (NHSV-ME) patients and correlated them with corresponding clinical and laboratory findings. During days 1 to 4 of the illness, 8 had EEGs. All but 1 had abnormal tracings and 3 (38%) showed the multifocal periodic pattern (MPP). Three had an early abnormal EEG at a time when their cranial CT/ultrasound studies were normal. During days 5 to 11, 13 had EEGs: all were abnormal and 3 showed the MPP. After day 11, EEGs (available on 10) showed a very low voltage background in 9, and only 1 had normal EEG and development. During 1 year in which 1 patient with NHSV-ME was observed, we noted that 9/324 (2.8%) of neonates with other CNS conditions manifested the MPP. All, however, had CSF findings that distinguished them from herpes cases. We conclude that: (1) In patients with suspected NHSV-ME, EEG is a sensitive test that is superior to radiologic procedures in detecting early cerebral involvement. Most of the early EEGs show nonspecific background and paroxysmal abnormalities. (2) In the presence of inflammatory CSF, the MPP, an otherwise nonspecific finding, is highly suggestive of NHSV-ME. (3) Sequential EEGs may be important in the follow-up of neonates with NHSV-ME.

Electroencephalography↗

Intermittent home treatment of status and clusters of seizures.

Clinical trials in children and adults indicate that the intermittent oral or rectal administration of diazepam is an effective and safe alternative for the acute management of recurrent paroxysmal disorders. Prompt home treatment of dangerous or upsetting relapses can prevent the morbid effects of prolonged seizures and offer the patients and their families a sense of control over the illness.

Administration, Oral↗

William G. Lennox: a remembrance.

William G. Lennox, author of Epilepsy and Related Disorders, had a lasting effect on our understanding of this illness. He postulated that epilepsy was not a unitary condition and that neuronal chemistries differed from one form of the disease to another. A leader in the use of electroencephalography in epilepsy, he described the first nearly pathognomonic EEG pattern and demonstrated specific features for each of the three most common types of seizure. His pioneering investigations into the biochemical basis of epilepsy helped to identify pathological mechanisms in epileptic attacks. Lennox stood alone in his belief, now generally accepted, that the genetics of epilepsy could be understood only through a multifactorial mode of inheritance. The author presents an affectionate portrait of the physician, the teacher and the man, the founder of the Seizure Unit and the unifying force in the study of epilepsy by both professionals and lay persons.

Epilepsy↗

Etiologic and preventive aspects of epilepsy in the child--bridging the gap between laboratory and clinic.

Four broad categories of basic phenomena are pertinent to developing ways to prevent epilepsy. These include mechanisms of epileptogenesis, ictal initiation and temporary entrainment by the seizure discharge of normally functioning brain, seizure propagation, and control mechanisms that function both to restrain the cascade of epileptic events culminating in a seizure and to arrest the epileptic event and restore the interictal state. In newborns and children, hypoxia-ischemia is a major factor leading to epileptogenesis, and several schemes are proposed to classify, quantify, and prevent hypoxic-ischemic encephalopathy. Control mechanisms must be better understood in order to develop prophylactic recommendations for epilepsy, and an experimental model of "kindling antagonism" may increase our understanding of these. Programs of prevention of seizures in children will evolve only if basic researchers and clinicians work productively together to develop an adequate understanding of factors important in epileptogenesis and antiepileptogenic control mechanisms.

Barbiturates↗

Stability in waking-sleep states in neonates as a predictor of long-term neurologic outcome.

Thirty-three full-term neonates were ranked blindly on a scale ranging from the least to the highest "risk" for future neurologic complications on the basis of available perinatal biographies, tests, and examinations performed during the newborn period. Four prolonged polygraphic-behavioral recordings were obtained one week apart beginning at ten days after birth. Five waking and sleep states were scored in each session as percentages of total observation time, giving a total of 20 scores for each baby to be subjected to analysis of variance. These measures also provided individual profile consistency or variability in maintaining waking-sleep states over the selected period of postnatal time. The whole cohort, except three infants who could not be followed adequately, was then reexamined periodically over a period ranging from 3 to 4 years (+/- 6 months) for neurologic and developmental assessments. Except for two scores that produced a low level of statistical significance (P less than .05), the other 18 scores were found to be not associated with long-term outcomes. Even the first two scores were not satisfactory discriminators for the outcome of the individual babies. However, when coefficients of concordance (W) were computed from each individual baby profile, significant statistics (P less than .001) emerged to indicate good correlations between high or low W values in the newborn period and long-term outcomes. All 17 newborns who had W scores greater than 0.9 were found to be normal at follow-up regardless of the poor ranking given several of them during the newborn period. Among the 13 newborns who had W scores less than 0.9, 11 had a poor clinical outcome at follow-up, though several had been ranked initially as falling within the least "risk" group.

Analysis of Variance↗

Extraction of clinical information from electroencephalographic background activity: the combined use of brain electrical activity mapping and intravenous sodium thiopental.

Traditional visual inspection of electroencephalographic (EEG) tracings and computer-assisted topographic mapping were compared in their abilities to detect and locate supratentorial lesions following intravenous sodium thiopental administration. Of the 13 subjects, 8 had atrophic and 5 had mass lesions, all defined by computed tomographic scan and 11 confirmed at operation. EEGs made before and after thiopental administration were evaluated separately from topographic maps of statistical difference between EEGs at multiple frequency ranges made before and after thiopental injection. Topographic mapping of statistical difference accurately detected all thirteen lesions, whereas EEG detected eight. In addition to demonstrating reduced beta production overlying structural abnormalities, topographic mapping revealed regionally augmented beta, especially over irritative lesions. Moreover, localization was possible with the topographic method after the first thiopental injection, whereas a second injection was required for EEG localization by visual inspection. Changes in slow (delta) activity were also useful in delineation of atrophic lesions, in which delta was usually augmented but occasionally regionally reduced. Multielectrode studies with topographic mapping appear essential in delineating cerebral abnormalities, because both slow and fast activities may be increased or reduced over such areas. The relative response of EEG background activity to thiopental at different frequencies may assist lesion characterization as well as localization.

Adolescent↗

External ophthalmoplegia, alpha and spindle coma in imipramine overdose: case report and review of the literature.

A 13-year-old boy with imipramine overdose developed seizures, respiratory arrest, and coma. Abnormalities of oculovestibular reflexes, electroencephalograms, and brainstem auditory evoked potentials were monitored in relation to measurements of drug levels. An alpha-coma electroencephalographic pattern evolved into one evidencing spindle coma and eventually into a normal pattern. Prolonged brainstem auditory evoked potentials also normalized as coma and oculocephalic reflex abnormalities resolved. In spite of the history that suggested hypoxic damage, the absence of reflex eye movements in a comatose patient and the presence of alpha- and spindle-coma electroencephalographic patterns, even with prolonged brainstem auditory evoked potentials, are not reliable prognostic indicators in tricyclic drug overdose.

Adolescent↗

Paroxysmal spike and wave activity in drowsiness in young children: its relationship to febrile convulsions.

Paroxysmal rhythmic theta waves appearing during early stages of sleep have long been recognized in the EEGs of normal children. A similar pattern with intermixed spikes appears to have a different correlation. We called this pattern 'hypnagogic paroxysmal spike wave activity' or 'hypnagogic PSW.' This investigation was designed to test whether there exists a relationship between hypnagogic PSW, febrile convulsions (FC) and age. We selected 3 cohorts of children: G-1 or normal control consisted of 94 children selected with the following criteria: complete normal neurodevelopmental examination and no personal or family history of FC, epilepsy, or other neurological disorder. They all had one EEG obtained without drug induction showing a clear sequence of awake-drowsy-sleep-arousal-awake states. G-2 or pathological control group consisted of 126 children selected at random, referred to the Division of Neurophysiology at the Children's Hospital Medical Center for any problem excluding FC. At least one EEG met the criteria mentioned above. G-3 or FC group consisted of 375 children in whom the very first seizure was associated with fever without evidence of other causes for the convulsion. All met the same EEG criteria of G-1 and G-2. The medical record of 169 children of this group (40 with hypnagogic PSW and 129 without hypnagogic PSW) were reviewed.(ABSTRACT TRUNCATED AT 250 WORDS)

Brain↗

Somatosensory evoked potentials: variability analysis in unilateral hemispheric disease.

A new method to study SEP using signal-to-noise estimate was applied to 35 patients (age 3 days-81 years, mean 12.1 years) with unilateral supratentorial cerebral lesions. The patients were grouped according to severity of deficits: minimal, mild, moderate and severe. Based on the observation that the SEPs were much more variable over the diseased hemisphere, we proposed the following hypothesis: normal neurons when stimulated generate a series of wave forms which are stable and reproducible. These are seen as stable and 'clean' scalp EPs. Diseased neurons on the other hand are malfunctioning and may tend to depolarize erratically. This can be seen as variable, 'noisy' and poorly reproducible EPs. To test this hypothesis, we measured the degree of variability of the SEPs on both the normal and affected sides, using the technique of Wong and Bickford (1980). Overall, 30 of the 36 SEPs (83%) had a greater variability on the diseased side. The prediction accuracy was greatest with the severely affected cases and least in the minimally affected cases. There are minor suggestions that the variability parameter may be useful for long-term follow-up. Clearly, if used with care, the amount of 'noise' in an EP can be a useful parameter.

Adolescent↗

Psychological complications of temporal lobe epilepsy.

A series of young adults with temporal lobe epilepsy included 36% with psychological complications. The incidence of overt psychosis was 11%. Psychopathology was more common in subjects with left temporal lobe spike foci (43%) and in males (42%), but these trends did not attain statistical significance. Psychological complications became manifest in adolescence in 85% of the affected cases, and were more likely (p less than 0.01) to occur when seizures began in the second 5 years of life. Although seizures nearly always antedated psychological problems, the onsets of epilepsy and psychological complications did not directly correlated (p = 0.43).

Adolescent↗

Brain electrical activity mapping (BEAM): a method for extending the clinical utility of EEG and evoked potential data.

The difficulties inherent in extracting clinically useful information by visual inspection alone from the massive amounts of data contained in multichannel polygraphic recordings have placed limits on the accuracy and range of utility of electroencephalography and evoked potentials. A method for condensing and summarizing the spatiotemporal information contained in recordings from multiple scalp electrodes is described. Data dimensionality is reduced and visibility increased by computer-controlled topographic mapping and display of data as color television images. Examples are given in which such brain electrical activity mapping (BEAM) (1) localizes tumors in patients with normal or nondiagnostic EEGs, (2) adds additional information to that visible on computerized axial tomography, and (3) demonstrates electrophysiological abnormalities in patients with functional lesions but normal CT scans. A sensitivity to the functional component of a neurological lesion suggests that BEAM may provide complementary information to the anatomical definition provided by the CT scan.

Adolescent↗

Quantified electrographic scales on 10 pre-term healthy newborns followed up to 40-43 weeks of conceptional age by serial polygraphic recordings.

Full-term newborns with seizures exhibit EEG patterns that appear reliable in predicting neurological outcome in almost 75% of the cases in a prospective investigation. These patterns are unreliable for over 25% of infants with seizures and even more so for term and pre-term babies with other complications. Observations that such newborns often show maturational delay in various physiological and/or behavioral parameters, raises the issue of whether indices other than specific EEG patterns might prove reliable in predicting prognosis. As a prerequisite for testing such an hypothesis in prospective investigations of term and pre-term babies with 'risk' factors other than seizures, it appears necessary to obtain quantification of these parameters in normals. Hence a longitudinal study was designed to obtain simultaneously 3 parameters in a control group of newborns born after 30-32 weeks of gestational age, followed with serial polygraphic recordings at weekly intervals until a conceptional age of 43 weeks. The selected parameters were purposefully restricted to three that can be obtained and measured easily in routine recordings without need for more complex instrumentation and analysis. These were: the percentage of interhemispheric synchrony between bursts of 'trace alternant'; the number of spindle-delta complexes ('brushes') during 5 min of REM and NREM sleep; and the concordance between various parameters during the same 5 min epochs. The range of these indices was thus established in a group of normal prematures followed prospectively. Establishing such normative data will allow greater inter-study reliability, and form the basis for other prospective studies of infants 'at risk' to investigate whether those that lag behind the norm in these indices when followed serially during the newborn period differ prognostically from those who recover and reach expected norms for conceptional age.

Brain↗