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Biomedical subjects

B F Westmoreland

Publications and source records attributed to B F Westmoreland.

At least 19 recordsLinked to original sources

Cerebral hemiatrophy with superficial siderosis and PLEDs due to a germ cell tumor of the basal ganglia.

The diagnosis of basal ganglia germ cell tumors may be delayed due to slow progression and minimal early changes on magnetic resonance imaging (MRI). The cystic nature of some tumors may lead to non-diagnostic biopsies. We describe the clinical, imaging, laboratory, and postmortem findings of a basal ganglia germ cell tumor in a 19-year-old man. Clues to an early antemortem diagnosis based on MRI findings and determination of tumor markers are discussed. An early diagnosis and accurate characterization of basal ganglia germ cell tumors is essential for optimal therapy. The presence of cerebral hemiatrophy and hemorrhagic or cystic components is suggestive. Measurement of serum and cerebrospinal fluid markers such as human chorionic gonadotropin may suggest the diagnosis.

Adult↗

Corticobasal degeneration and frontotemporal dementia presentations in a kindred with nonspecific histopathology.

We report the clinical, neuropsychological, electroencephalographic and radiologic findings in a kindred with varying clinical presentations of a neurodegenerative disorder. Postmortem examination of one member with clinically suspected corticobasal degeneration (CBD) revealed nonspecific histopathology maximally involving the frontoparietal cortex with negligible degenerative changes in the basal ganglia and substantia nigra. The findings in this and other kindreds demonstrate that (1) similar findings on ancillary testing can occur in relatives presumably suffering from the same pathophysiologic process despite dissimilar clinical presentations, (2) the 'CBD syndrome' is not specific for CBD, (3) extrapyramidal dysfunction can exist in the absence of appreciable basal ganglia and nigral degeneration, (4) nonspecific histopathology can underlie familial focal/asymmetric cortical degeneration syndromes and (5) many of the findings in CBD are comparable to those reported in frontotemporal dementia.

Basal Ganglia Diseases↗

Periodic lateralized epileptiform discharges after evacuation of subdural hematomas.

Periodic lateralized epileptiform discharges (PLEDs) have been recorded in some patients with subdural hematomas. In most of the patients, PLEDs occurred before the hematoma was evacuated. Five patients (three men and two women) who ranged in age from 57 to 88 years had PLEDs and seizures after evacuation of a subdural hematoma. The PLEDs occurred 1 to 9 days after evacuation and were associated with clinical seizures and altered consciousness. After treatment with anticonvulsant agents, the PLEDs and seizures resolved. The presence of PLEDs should be considered in patients who, after evacuation of a subdural hematoma, have altered consciousness, lateralized neurologic findings, or seizurelike activity (or a combination of these features).

Aged↗

Multifocal periodic lateralized epileptiform discharges (PLEDs): EEG features and clinical correlations.

OBJECTIVE: To analyze the clinical and EEG findings of patients with multifocal periodic lateralized epileptiform discharges (PLEDs). METHODS: EEGs containing multifocal PLEDs (3 or more foci of PLEDs) were reviewed. Thirty-five patients (15 males and 20 females), from 2.5 months to 91 years old, met the criteria for multifocal PLEDs. RESULTS: The disease processes identified in the patients included vascular lesions in 9, central nervous system infections in 7, metabolic/toxic disorders in 6, exacerbation of a chronic seizure disorder in 6, hypoxic ischemic insults in 3, and fat embolism, paraneoplastic encephalitis, cerebral metastasis, and multiple sclerosis in one each. Twenty patients died. Detection of the spatiotemporal distribution of multifocal PLEDs was facilitated by the use of Laplacian montages. CONCLUSIONS: Multifocal PLEDs were recorded in 35 patients and were associated with processes resulting in diffuse or multifocal cerebral dysfunction. Multifocal PLEDs indicate a significant disturbance of cerebral function and are associated with a mortality rate of 57%.

Adolescent↗

Defective alpha reactivity with mental concentration.

We have studied 120 patients with EEGs that show defective reactivity of alpha activity in which one side fails to attenuate during the performance of mental arithmetic. The side of defective reactivity was the right side in 74 patients and the left in 46. A similar asymmetric reactivity of alpha activity to eye opening (Bancaud phenomenon) was seen in 32 patients. Also, 113 patients had lateralized EEG abnormalities on the side of defective reactivity and on the side in which cerebral lesions or disorders of cerebral function were present. The unilateral failure of alpha attenuation with mental concentration represents a subtle lateralized electrographic manifestation of cerebral dysfunction on the side of defective reactivity.

Adolescent↗

Subclinical rhythmic electrographic discharges of adults (SREDA) revisited: a study using digital EEG analysis.

Previous descriptions of the subclinical rhythmic electrographic discharges of adults (SREDA) have been based entirely on visual analysis of analog electroencephalographic (EEG) recordings. The introduction of digital electroencephalograms (EEGs) and advances in digital signal processing provide an opportunity to restudy in more depth the nature of SREDA. We identified nine patients who had SREDA diagnosed on a routine EEG recording since the introduction of digital EEG to our laboratory in August 1995. Following careful rereview using standard montages, six of these patients were determined to fulfill the traditional requirements for the diagnosis of SREDA, whereas three were believed to have other benign discharges. Review with Laplacian montages demonstrated that the site of the SREDA activity was maximal in the parietal region or parietocentrotemporal regions, whereas it was maximal in the temporal or frontotemporal regions in the non-SREDA discharges. Frequency analysis, using both the conventional fast Fourier transform (FFT) and time-frequency mapping with the Wigner FFT variant, demonstrated that the SREDA consisted of a complex mixture of multiple rapidly shifting frequencies which showed little spatial and temporal correlation. In contrast, the non-SREDA all consisted of a single dominant well-organized rhythmic frequency spectrum that remained stable throughout space and time.

Adult↗

The EEG findings in extratemporal seizures.

Extratemporal seizures originate from the frontal, central, parietal, occipital, and midline regions of the brain. The scalp EEG can show various types of interictal and ictal discharges consisting of spikes, spike and wave sharp waves, paroxysmal fast activity, or rhythmic activity in the beta, alpha, theta, or delta frequency ranges. The discharges can occur as focal, regional, lateralized, or secondarily generalized discharges. Discharges arising from the frontal region are varied and at times complex. Centro-temporal spikes associated with benign epilepsy of childhood have a characteristic blunt spike and wave appearance. Centro-parietal spikes can occur in children with benign childhood epilepsy or in association with symptomatic epilepsies at any age. Occipital spike discharges have been seen in young children with visual problems, benign occipital epilepsy of childhood, the Sturge-Weber syndrome, and other symptomatic or structural lesions involving the occipital lobe. There may be problems with detection of the source of origin of seizures secondary to the anatomy of the various regions, deep foci, small restricted foci, rapid spread of epileptiform discharges, and contaminating effects of muscle and movement artifact. Depth or intracranial recordings may help in further localization of foci.

Adult↗

Unusual variants of subclinical rhythmic electrographic discharge of adults (SREDA).

Since 1981, when we first described a group of patients with subclinical rhythmic electrographic discharge of adults (SREDA), we have seen 108 patients with SREDA. Nineteen of these patients had unusual or atypical features of SREDA. The typical pattern consists of a non-evolving theta rhythm occurring in a widespread manner but maximal over the parietal and posterior temporal regions and lasting for a few seconds to a minute without clinical manifestations or symptoms. The unusual variants consist of predominantly delta frequencies, notched waveforms, a frontal or more focal distribution, a more prolonged duration, and presence during sleep. There was no significant difference in the patient group that had the atypical pattern compared with those with the typical pattern. Although the mechanism of the SREDA is unclear, it appears to represent a benign EEG phenomenon that has little diagnostic significance and should be distinguished from seizure discharges.

Adult↗

Angelman's syndrome: clinical and electroencephalographic findings.

Angelman's syndrome is a rare genetic disorder characterized by developmental delay, craniofacial abnormalities, ataxia, paroxysmal laughter, and seizures. The diagnosis is suspected in infants who have the characteristic clinical features and electroencephalographic (EEG) abnormalities and is confirmed by the genetic identification of a maternally derived 15q11-13 deletion. We report on 3 patients with genetically confirmed Angelman's syndrome who had the characteristic clinical and EEG features. The EEGs demonstrated high-amplitude 2- to 3-Hz delta activity, with intermittent spike-and-slow-wave discharges maximal in the occipital region in 2 patients and generalized sharp-and-slow-wave discharges, occipital spikes, and electrographic status epilepticus during slow-wave sleep in the other patient. The findings of generalized high-amplitude delta slowing and occipital spike-and-wave discharges, facilitated by eye closure, in children with developmental delay and seizures suggest the diagnosis of Angelman's syndrome and should lead to genetic testing.

Angelman Syndrome↗

Clinical accompaniments of the burst-suppression EEG pattern.

A burst-suppression pattern on the EEG following anoxic insult is usually associated with a poor prognosis. Myoclonic jerks may accompany the electrographic burst. On rare occasions oral, ocular, or appendicular movements can be associated with bursts of EEG activity recorded following cerebral anoxic insult. At times these movements may cause confusion about the patient's state of consciousness or the purposefulness of the movements; they may mimic volitional movements in response to external stimuli. To better describe the variety of movements that may accompany bursts of EEG activity in comatose patients, we review 12 cases where movements occurred in association with the electrographic bursts.

Adult↗

Epileptiform abnormalities in hepatic encephalopathy.

Epileptiform abnormalities are uncommon in patients with hepatic encephalopathy. A review of EEGs in patients with hepatic encephalopathy over a 10-year period identified 18 (15%) with epileptiform abnormalities. Thirteen patients had interictal discharges consisting of focal spike and sharp wave discharges, bilateral independent discharges, and generalized spike and wave discharges. A total of 10 patients had electrographic seizure discharges, focal in 6 and generalized in 5 (some patients had more than one abnormality). Twelve patients had clinical seizures, partial in four and generalized in eight. Neuroimaging failed to provide an etiology for the generation of epileptiform discharges in most patients, including those with focal abnormalities. Most patients with epileptiform discharges died or deteriorated. We conclude that epileptiform can be seen in patients with hepatic encephalopathy, and when present imply a poor prognosis.

Adult↗

Periodic epileptiform discharges in the midline.

Five patients had electroencephalograms (EEGs) that showed periodic epileptiform discharges in the midline (PEDIM); other than location, this activity has the same characteristics as periodic lateralized epileptiform discharges (PLEDs). All 5 patients had acute onset of partial motor seizures involving the lower extremity. The location of the PEDIM corresponded to the seizure type and focal neurologic deficits. All 5 patients had sustained a cerebrovascular insult, either old or new, and the PEDIM and seizures suggested an origin from the watershed area between the anterior, middle, and posterior cerebral arteries, involving predominantly the parasagittal region of the cerebral hemisphere.

Aged↗

Periodic lateralized epileptiform discharges in multiple sclerosis.

We report an unusual cause of periodic lateralized epileptiform discharges (PLEDs) in a young man with a long history of multiple sclerosis. Two exacerbations of multiple sclerosis, 3 years apart, were complicated by complex partial status epilepticus. After each episode, serial electroencephalograms revealed PLEDs (right frontal PLEDs in the first episode and right frontotemporal and bifrontal PLEDs in the second one), which resolved within 2 weeks. With the first episode, magnetic resonance imaging revealed an enhancing white matter lesion in the right frontal area, which improved after 3 weeks. We concluded that in each instance an exacerbation of multiple sclerosis caused the seizures.

Adult↗

Epileptiform electroencephalographic patterns.

Electroencephalography (EEG) is the most useful test for assessment of patients with epilepsy. It can help establish the diagnosis of epilepsy and determine the type of seizure disorder and its site of origin. Epileptiform abnormalities in the EEG tracing may be focal or generalized. The main types of focal epileptiform discharges arise from the temporal, frontal, occipital, centroparietal, centrotemporal, and midline regions of the brain. Generalized epileptiform discharges consist of the 3-Hz spike-and-wave, slow spike-and-wave, atypical spike-and-wave, paroxysmal fast activity, and hypsarrhythmic patterns. Status epilepticus is manifested by continuous epileptiform discharges or recurrent seizure activity without interim recovery, which can occur in a generalized or focal manner. Benign epileptiform variants unassociated with seizures can also be present in the EEG. Included in this category are the "14 & 6" positive bursts, small sharp spikes, wicket waves, 6-Hz spike-and-wave discharges, and rhythmic temporal theta activity. The EEG findings should be interpreted in the context of the overall clinical picture.

Electroencephalography↗

Chronic inflammatory meningoencephalitis should not be mistaken for Alzheimer's disease.

We describe two patients with a chronic encephalopathy that clinically resembled dementia but that resolved after oral administration of high-dose corticosteroid therapy. Both patients had serologically documented Sjögren's syndrome, a diagnosis that was further supported by biopsy of a salivary gland in one. Neither patient had radiologic evidence of vasculitis of the central nervous system. In one patient, meningeal and brain biopsy specimens showed perivascular inflammatory lymphocytic infiltrates. Chronic inflammatory meningoencephalitis is a treatable cause of chronic encephalopathy that should be clinically distinguished from dementia associated with Alzheimer's disease.

Aged↗

Effects of midazolam on electroencephalograms of seriously ill patients.

Midazolam hydrochloride, a water-soluble benzodiazepine for intravenous injection, is frequently used to provide sedation to mechanically ventilated patients in the critical-care unit. Although the effects of midazolam on the electroencephalograms (EEGs) of healthy volunteers have been reported previously, to our knowledge such effects in ill patients have not been documented. Herein we describe the effects of intravenously administered midazolam on the EEGs of eight seriously ill patients (age range, 49 to 80 years; mean, 69 years). The EEGs showed drug-induced relatively fast (alpha or beta) frequency activity superimposed on delta slowing in six patients and mild to moderate voltage attenuation in three patients. An alpha frequency coma pattern, a transient burst-suppression pattern, and a spindle coma pattern were also seen. Thus, the effects of intravenously administered midazolam on the EEGs of seriously ill patients are similar to those noted after the administration of other benzodiazepines. These potential effects should be considered when their EEG tracings are interpreted.

Aged↗

Arthur Conan Doyle, Joseph Bell, and Sherlock Holmes. A neurologic connection.

Neurologists, like physicians in several other medical specialties, can lay claim to Sherlock Holmes as one of their own. This assertion is validated by the number of neurologic conditions, such as seizures, stroke, syncope, encephalopathies, and head trauma, that are mentioned in the stories and novels. In addition, the article reviews the powers of observation and the deductive approach utilized by Conan Doyle and Joseph Bell, the models for Sherlock Holmes, and how these skills can be applied to medical problems.

Drama↗

Epileptiform electroencephalographic abnormalities in liver transplant recipients.

We retrospectively studied patients who had undergone orthotopic liver transplantation and who also had electroencephalography to determine whether epileptiform changes were associated with a poor neurological outcome. Study groups were 36 patients who died after transplantation (141 electroencephalograms) and underwent neuropathological examination, 11 who died (18 electroencephalograms) but did not have autopsy, and a third group of 34 (62 electroencephalograms) who remained alive. Epileptiform activity was seen in electroencephalograms of 14 of the patients who died (11 from the autopsy group) and in 2 of those who remained alive. All had multiple epileptiform abnormalities and clinical or subclinical seizures. The incidence of epileptiform activity after orthotopic liver transplantation was fivefold higher in the nonsurvivors. Serious cerebral structural changes were found in 10 of the 11 patients who underwent autopsy. Epileptiform activity in the electroencephalograms of patients who had undergone orthotopic liver transplantation indicates a poor prognosis. It should alert the clinician to investigate further for potentially treatable causes.

Adolescent↗