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Biomedical subjects

A D Rothner

Publications and source records attributed to A D Rothner.

At least 55 records · Page 3Linked to original sources
PubMed1989

Dystonic posturing in complex partial seizures of temporal lobe onset: a new lateralizing sign.

We observed unilateral dystonic posturing of an arm or leg in 41 complex partial seizures (CPS) from 18 patients. In all cases this was contralateral to the ictal discharge. Unilateral automatisms occurred in 39 of 41 seizures on the side opposite the dystonic limb. Version occurred in 11 of the 41 CPS to the same side as the dystonic posturing and always followed the posturing. Subdural recordings of seven seizures showed ictal onset from the mesial basal temporal lobe. At the onset of dystonic posturing, maximum ictal activity was in the basal temporal lobe with minimal involvement of the cerebral convexity. Unilateral dystonic posturing occurs frequently in CPS of temporal lobe onset and is a lateralizing sign with a high degree of specificity. It probably reflects spread of the ictal discharge to basal ganglia structures.

Dystonia↗
PubMed1989

'Not everything that shakes is epilepsy'. The differential diagnosis of paroxysmal nonepileptiform disorders.

Many infants, children, and adolescents exhibit unusual mannerisms, behaviors, and spells. These events cause concern to parents and are frequently brought to the attention of physicians. If the spells are infrequent and do not interfere with function, no intervention may be necessary. If they are severe and recurrent, however, diagnostic evaluation is necessary. This article has reviewed the most common spells that can be misdiagnosed as epilepsy by pediatricians. Other spells such as head banging, head rolling, body rocking, enuresis, nightmares, bruxism, obsessions, compulsions, self-injurious behavior, self-stimulating behavior, and stereotypies are reviewed elsewhere. Emphasis is placed on the importance of a thorough history, a complete general physical and neurologic examination, and the judicious use of laboratory testing. The diagnostic methodologies currently available, including cardiac evaluation, video-EEG monitoring, and polysomnography, will frequently result in a specific diagnosis, and allow appropriate treatment. In many cases, however, even after the above diagnostic tests have been utilized, no diagnosis is forthcoming, but reassurance and follow-up can be of help to the young patient and the family.

Cerebrovascular Disorders↗
PubMed1989

Incidence, types, and management of cancer in patients with neurofibromatosis.

Neurofibromatosis Type 1 (NF1) is a common genetic disease with an incidence of 1 in 3,000 to 4,000. Clinical manifestations may include cafe-au-lait macules, axillary freckling, neurofibromas, Lisch nodules, skeletal dysplasias, learning disabilities, and a variety of neoplasms. In persons with NF1, some malignancies occur at an increased incidence compared to the general population. These include central nervous system astrocytomas, especially involving the visual pathways; ependymomas; meningiomas; neurofibrosarcomas; rhabdomyosarcomas; nonlymphocytic leukemias; and pheochromocytomas. The authors discuss the dysplastic and malignant features associated with NF and the concept of NF as a "neurocristopathy."

Adult↗
PubMed1988

The association of neuroblastoma with myoclonic encephalopathy of infants: the use of magnetic resonance as an imaging modality.

The association of neural crest tumors with myoclonic encephalopathy of infants has been well described. Since these tumors often are clinically occult the diagnostic modalities used to identify these lesions need to be sensitive. We describe a child with neuroblastoma associated with infantile myoclonic encephalopathy in whom magnetic resonance imaging identified the lesion while other techniques were unable to demonstrate a mass clearly. Magnetic resonance imaging should be considered as a diagnostic modality when adrenal lesions are suspected but not apparent with other imaging techniques.

Adrenal Gland Neoplasms↗
PubMed1988

Subdural electrodes in the evaluation for epilepsy surgery in children and adults.

To evaluate the utility and safety of chronic extraoperative subdural EEG and functional localization studies in children compared to adults, we studied 61 patients each of whom had similar evaluations for epilepsy surgery, regardless of age. The 23 children and adolescents (3 to 18 years old) and the 38 adults (20 to 41 years old) each had several days and nights of extraoperative EEG from scalp and chronically-implanted subdural electrodes, and the same percentage of children and adults also had functional localization studies including cortical electrical stimulation. The methods and results of these studies and of the subsequent resections did not differ between the two groups, and they both had similar rate of complications and similar incidence of good outcome. The subdural technique was as effective and well-tolerated in children and adolescents as in adults. Other invasive EEG techniques have not yet been systematically compared between children and adults, but the subdural technique, at least, appears to be suitable for patients over a wide age range.

Adolescent↗
PubMed1987

Complex partial seizures of childhood onset. A five-year follow-up study.

Few detailed studies have examined the long-term outcome of complex partial seizures (CPS) in children and adolescents. Previous studies have selected patients on clinical criteria only or have included those with benign focal epileptiform discharges of childhood, nonepileptiform sharp transients, and generalized epileptiform discharges. We have followed up 29 patients with clinically and electroencephalographically defined CPS of childhood onset for five years or more. Twelve patients are seizure free, eight of them after having epilepsy surgery. Of the remaining, eight patients have intractable seizures. Only one patient was able to discontinue anticonvulsant therapy after epilepsy surgery. No patient had spontaneous remission of seizures without anticonvulsant medications. School difficulties, behavior problems, and unemployment were seen in half the group. We believe CPS in this age group are difficult to control and need aggressive management. In selected patients, epilepsy surgery offers a good chance of seizure control and improvement of educational, social, and vocational potential.

Adolescent↗
PubMed1987

The natural history of Tourette syndrome: a follow-up study.

Initial reports described Tourette syndrome as a lifelong disorder. Since then, others have noted that some patients experience remissions during late adolescence. To examine this issue, we sent questionnaires to 99 patients with Tourette syndrome who were 15 to 25 years old. The majority of the 58 respondents indicated that they had fewer tics as they reached late adolescence or young adulthood. Although most reported associated behavior or learning problems, the majority felt they were coping well. The long-term outcome in many patients with Tourette syndrome may be more optimistic than previously reported.

Adaptation, Psychological↗
PubMed1987

Headaches in children and adolescents. Classification and recommendations.

Headaches in children and adolescents are more common than generally appreciated, and even an experienced clinician may have difficulty determining whether the pain is organic or functional. As an aid to proper diagnosis, headaches can be classified into four groups, each with a distinctive time-related pattern that provides a starting point for recognition of a specific syndrome. In most cases, thorough history taking and physical examination plus selected laboratory tests guide the physician to the correct diagnosis.

Adolescent↗
PubMed1987

Congenital granuloprival hypoplasia of cerebellar and hippocampal cortex.

Two siblings with congenital granule cell hypoplasia of the cerebellum and hippocampus are reported. The patients, both male, showed severe psychomotor retardation, microcephaly, hypotonia, athetosis, and seizures; they died at the ages of 3 7/12 years and 5 10/12 years, respectively. Postmortem examinations in both cases revealed nearly complete absence of the granule cells of the cerebellum with relative preservation of the Purkinje cells. Also absent were the granule cells of the fascia dentata of the Ammon's horns of the hippocampus, without any detectable gliosis; this has not previously been reported. Twenty-three autopsy cases of granuloprival cerebellar hypoplasia are reviewed. The present cases illustrate a singularly unique disease process, comparable to the classical cerebellar hypoplasia experimentally induced by parvoviruses, and suggest a granule cell specific insult to the brain during the late second trimester.

Atrophy↗
PubMed1987

Clinical outcome after complete or partial cortical resection for intractable epilepsy.

This is the first epilepsy surgery series to analyze the definition of "completeness" of resection, based solely on results of chronic scalp and subdural EEG recording. When patients had complete removal of all cortical areas with ictal and interictal epileptiform discharges, the clinical outcome was usually good. When areas with epileptiform discharges were left behind, good outcome was significantly less frequent. This correlation between complete resection and good outcome was independent of the presence or absence of CT-detected structural lesions or sharp waves on post-resection electrocorticography. These results support completeness of resection, defined by prolonged extraoperative EEG, as an important factor in seizure surgery.

Adolescent↗
PubMed1986

Infantile spasms. Comparative trial of nitrazepam and corticotropin.

Fifty-two patients were enrolled in a four-week randomized multicenter study comparing nitrazepam and corticotropin in the treatment of infantile spasms. The drugs' efficacy was evaluated in 48 patients, all less than 2 years of age. Both treatments resulted in a statistically significant reduction in spasm frequency from that at baseline, but the difference between treatments was not significant. The number of patients who experienced side effects was similar in the two treatment groups, but the adverse effects encountered among the patients treated with corticotropin were qualitatively more severe and required the discontinuation of treatment in six patients.

Adrenocorticotropic Hormone↗
PubMed1986

Four dichotic speech tests before and after temporal lobectomy.

Thirty patients were tested with four dichotic speech tests before and after temporal lobectomy for control of intractable seizures. Ipsilateral ear scores improved on all tests postoperatively; these improved scores reached statistical significance for the Staggered Spondaic Word Test and for consonant-vowel syllables. This result, combined with a nonsignificant decrease for contralateral ear scores, produced postoperative increase in the ipsilateral minus contralateral ear difference scores, similar to previous literature. Preoperative tests for a larger group of patients showed significantly poorer performance than for normal subjects, for all four tests. Total correct scores, used as a measure of overall auditory processing capacity, were impaired for these patients, but unchanged or slightly improved after surgery.

Adolescent↗
PubMed1985

Gilles de la Tourette's syndrome: effects of stimulant drugs.

We reviewed the medication histories in 200 children with Gilles de la Tourette's syndrome to investigate how frequently CNS stimulants may unmask a latent syndrome or worsen existing tics. Forty-eight patients had received stimulant drugs. Nine were treated before the onset of tics, but only four were still receiving stimulants when tics began. In 39 patients with preexisting tics, stimulants increased tics in 11, caused no change in 26, and decreased tics in 2. Behavior improved in 22 patients. Thirteen of these 22 had no increase in tics when stimulants were used. Cautious trials of stimulant therapy may benefit some patients with this syndrome.

Central Nervous System Stimulants↗
PubMed1985

Developmental anomalies of the optic disc and carotid circulation. A new association.

Three patients with developmental abnormalities of the optic disc (two morning glory anomalies and one retinochoroidal-optic disc coloboma) had angiographically documented aberrancies of the carotid circulation including large trunk occlusions, moya-moya-like collaterals, dolichoectasia, and absent ophthalmic artery. Although developmental optic disc abnormalities have been linked with other problems such as basal encephalocele, congenital heart defects, and eyelid hemangiomas, these three patients are the first to our knowledge to have related malformations of the intracranial circulation. We suggest, therefore, that the presence of a congenital optic disc anomaly may herald a similar defect in the cerebral circulation.

Abnormalities, Multiple↗