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Biomedical subjects

M Y Neufeld

Publications and source records attributed to M Y Neufeld.

At least 19 recordsLinked to original sources

The electroencephalogram in acetazolamide-responsive periodic ataxia.

Acetazolamide-responsive periodic ataxia (ARPA) is a rare movement disorder, characterized by recurrent episodes of vertigo, cerebellar ataxia, and nystagmus, which has recently been characterized genetically. The pathophysiology is unknown, but it is probably not epileptic. By definition, acetazolamide produces an impressive symptomatic relief. Because of the paroxysmal nature of the disorder, EEG tracings were often obtained. We report four new cases (two familial and two sporadic) with typical ARPA (none of whom had metabolic abnormalities or continuous electrical muscle activity) and review the EEG findings associated with this disorder. EEG findings were reported in 18 kindreds and nine sporadic cases (including ours). EEG was described in 54 of the 140 affected cases and was abnormal in 52% (28/54). Most commonly seen was intermittent generalized slow activity, observed in 35% (19/54), frequently intermingled with spikes (10 cases). Other abnormalities included nonspecific mild generalized or focal slowing in seven (13%) and focal epileptic activity in two (4%) patients. The paroxysmal EEG activity frequently seen in ARPA should not establish a diagnosis of epilepsy. Although not specific, it may suggest the correct diagnosis and indicate treatment with acetazolamide.

Acetazolamide

Cognitive effects of scopolamine in dementia.

Cholinergic deficiency was postulated to play an important role in the mental decline observed in Alzheimer's (AD), Parkinson's (PD) and multiinfarct (MID) dementia. In the present study, 11 AD, 8 MID and 7 PD patients (DSM III-R diagnostic criteria for dementia) and 9 healthy age-matched controls (CTRL) were given IV 0.5 mg scopolamine (SCO) or placebo (PLA) in random order (double blind) within one week. The Hebrew Short Mental Test (SMT) and Wechsler Memory Scale (WMS) were administered before and after SCO and PLA in each patient. A comparison of SCO vs. PLA utilizing MANCOVA (the covariate being the basal mental performance [BAS] with SMT or WMS) showed that SCO affected all the groups similarly, except for the Wechsler subtest of logic memory which showed larger deterioration in CTRL compared to demented patients. ANOVA and MANCOVA analyses did not distinguish between the three demented groups. SCO administration does not differentiate between demented patients and CTRL and does not enable discrimination between patients with AD, MID and PD. Moreover, some CTRL with still normal cognitive performance, but lower BAS may be more vulnerable to SCO than others. The integrity of the cholinergic system may be responsible for the different sensitivity to SCO challenge.

Aged

EEG abnormalities in clozapine-treated schizophrenic patients.

A prospective study of EEG findings and occurrence of seizures in patients with refractory schizophrenia treated with clozapine has been conducted. Pretrial EEG and EEG under treatment at a fixed dose of clozapine 300 mg/day were performed. Fifteen patients entered the study, four patients were withdrawn because of side effects or poor compliance. EEG abnormalities appeared in seven of the 11 patients who completed the study (64%): generalized slowing in six of them and epileptic activity in two (one patient had both types of change). None of the patients developed clinical seizures. EEG abnormalities were more frequently observed in those with better clinical response to clozapine and/or shorter duration of disease, although these findings were not statistically significant. We conclude that EEG abnormalities occur frequently (64%) in schizophrenic patients who receive clozapine. However, the EEG changes do not necessarily predict the occurrence of convulsions.

Adult

Electroencephalographic findings with low-dose clozapine treatment in psychotic Parkinsonian patients.

Twenty patients with Parkinson's disease (PD), who developed delusions and psychotic behavior, underwent electroencephalogram (EEG) recordings before and during treatment with low-dose clozapine. Resolution of the psychotic features was observed in all cases. The EEG was unaltered in 15, whereas five patients exhibited increased generalized or focal slowing when compared with the pretreatment tracings. These findings contrast with the high incidence of EEG abnormalities, including epileptiform activity, which are observed when larger doses of clozapine are used in schizophrenic patients, but they underscore that even in low doses, clozapine may cause EEG changes.

Adult

Fatal insomnia in a case of familial Creutzfeldt-Jakob disease with the codon 200(Lys) mutation.

Fatal familial insomnia (FFI) has been exclusively associated with a pathogenic mutation at codon 178 in the PRNP gene coupled with methionine (Met) at codon 129. We now describe a subject with familial Creutzfeldt-Jakob disease, heterozygous for the pathogenic lysine (Lys) mutation at codon 200 and homozygous for Met at codon 129 of the PRNP gene, who was affected by severe insomnia. At autopsy the patient had significant involvement of the thalamus, as previously described in subjects affected by FFI with the codon 178 mutation. This case demonstrates the wide variability of the clinical expressions in patients with the codon 200 mutation, that may include insomnia and thalamic pathology.

Amyloid

Vigabatrin and multifocal myoclonus in adults with partial seizures.

We report the appearance of multifocal myoclonus in two adult patients treated with vigabatrin as an add-on drug for complex partial seizures. The myoclonus subsided after dose reduction or discontinuation of the drug. There were no electroencephalogram correlates during the myoclonic jerks. This phenomenon may represent a apparently dose-related rare adverse drug event, similar to that seen occasionally with other anticonvulsants.

Adult

Low-dose clozapine in the treatment of levodopa-induced mental disturbances in Parkinson's disease.

Delusions and other manifestations of psychotic behavior are common side effects in Parkinson's disease (PD) patients chronically treated with dopaminergic drugs. Clozapine, a dibenzodiazepine derivative, is an antipsychotic drug largely devoid of extrapyramidal side effects. We evaluated the effects of low doses of clozapine on the mental and motor functions in PD patients requiring antipsychotic treatment. Twenty-seven PD patients taking dopaminergic drugs and who had psychotic behavior received clozapine at 12.5 to 75 mg/d. Fifteen patients received clozapine for 1 to 11 months (mean, 6.8 months) and seven received it for 12 to 24 months (mean, 18 months). No patient exhibited motor deterioration, and the psychotic features disappeared immediately, allowing discontinuation of clozapine after several months in 10 patients. Fifteen patients are still receiving clozapine and are free of psychiatric symptoms. The clozapine treatment was discontinued after 5 days (25 mg/d) in two patients because of somnolence. No patient developed neutropenia. Clozapine in low doses is effective in the treatment of drug-induced delusions and hallucinations in PD.

Aged

Should sleep EEG record always be performed after sleep deprivation?

Sleep deprivation (SD) is a known activator of epileptiform EEG activity in patients with epilepsy. In the workup of these patients, EEG recordings are performed following SD both in the awake state and during sleep. The latter significantly increases the duration and the cost of the examination; the specific yield of sleep tracing in single-session wake-sleep record after SD has not been evaluated in adult patients. Our study tried to answer this question, analyzing consecutive recordings of 76 adult patients who had an epileptiform abnormality in the SD record. Thirty-five of the patients were treated with antiepileptic drugs at the time of the study. After SD of 24-26 h, 1000-1500 mg of chloral hydrate were administered; an 18-channel standard awake EEG was performed, followed by 30 min sleep recording. Epileptiform activity was recorded in the wake part only in 7 (9%, 3 focal, 4 generalized); in 39 (51%) the activity was seen in both awake and sleep parts (21 focal, 5 focal with secondary generalization and 13 generalized); and in 30 (40%) it was found in the sleep part only (23 focal, 1 focal with secondary generalization and 6 generalized). Whenever epileptiform activity was apparent in both parts of the recording, its configuration and localization were identical in the sleep and the wake EEGs. This phenomenon was observed in both treated and untreated patients. In combined wake-sleep recording following SD in adults, sleep tracing may reveal epileptiform activity not demonstrated during the preceding wake EEG. However, if epileptiform activity appears already in the wake recording, subsequent sleep tracing may be redundant.

Adolescent

Effects of a single intravenous dose of scopolamine on the quantitative EEG in Alzheimer's disease patients and age-matched controls.

Quantitative EEG (qEEG) was evaluated in Alzheimer's disease (AD) patients and age-matched controls following the administration of a single acute intravenous dose of scopolamine. Eleven AD patients and 8 cognitively intact age-matched controls underwent qEEG in baseline conditions, following double-blind intravenous administration of 0.5 mg scopolamine or placebo. At baseline, AD patients had significantly decreased absolute and relative alpha and increased relative theta amplitudes. In both groups, scopolamine administration was followed by a decrease in absolute and relative alpha amplitude, and increase in the absolute and relative delta activity. The increase in the absolute and relative delta amplitude by scopolamine was significantly more prominent in the controls; the decrease of alpha activity, while larger in controls, was not statistically different from AD. We conclude that scopolamine affects the change in delta amplitude differently in AD patients and controls, probably reflecting the reduced cholinergic tone in AD.

Aged

Stress and epilepsy: the Gulf war experience.

Stress is commonly believed to precipitate seizures in some patients with epilepsy, but direct examination of this assumption is problematic because of the difficulty in defining vague factors such as 'emotional stress'. Using a questionnaire, we have recorded seizure frequency during the 1991 Persian Gulf war, when Israelis were under stress from the threat of Scud missile attacks, in 100 consecutive adult patients with epilepsy. Increased frequency of seizures was reported by eight patients. These were younger than the other patients, the majority showed generalized epileptic EEG activity and all had generalized seizures (secondarily generalized in four). Only four had seizures directly related to the sounding of an alarm and in the others, non-compliance, being off medication at the time and disturbed sleep were probable contributory factors. We conclude that, in this series, epilepsy control was only weakly affected by an acute external emotional stress factor.

Adolescent

EEG as predictor of dementia following first ischemic stroke.

INTRODUCTION: Predictive factors for occurrence of vascular dementia may help identify patients at increased risk of developing this condition. Our purpose was to evaluate the prognostic value of early EEG findings in patients after first ischemic cerebral stroke on the development of dementia. MATERIAL AND METHODS: We performed routine EEG recordings in 199 consecutive non-demented patients with first-ever ischemic stroke, within 48 h of the event. The patients were subsequently followed for their mental state for 2 years. Survival analysis, wherein onset of dementia was the end-point, was performed on the total sample population and conducted separately on those who had normal EEG at time of the event and on those who had abnormal EEG findings (focal or diffuse slowing). RESULTS: Patients with abnormal EEG at baseline had 2.6 times the risk of developing dementia than those who had normal EEG; this odds ratio was statistical significant (CL: 1.3-5.1, p = 0.003). Development of dementia was not related to any specific EEG abnormal pattern. CONCLUSIONS: Abnormal EEG performed close to the first ischemic stroke appears to be an indicator of subsequent cognitive decline, probably because it indicates cortical involvement by the stroke or an underlying indolent cerebral degeneration.

Aged

Exacerbation of focal seizures due to carbamazepine treatment in an adult patient.

Exacerbation of seizures by carbamazepine (CBZ) has been well documented in children. These were mainly generalized convulsions and only rarely was an increase in previous focal convulsions observed. We report an adult epileptic patient, who had an increased frequency of focal seizures following treatment with CBZ. The exacerbation occurred with high CBZ blood levels, but there were no other signs of encephalopathy. Lowering the dose of CBZ was followed by dramatic clinical improvement. This report points to the possibility that increased focal seizure frequency under CBZ treatment in adults may imply drug effect, rather than worsening of the underlying disease.

Adult

Demyelinating peripheral neuropathy in Creutzfeldt-Jakob disease.

We describe 2 patients of Jewish Libyan descent, who presented with a clinical syndrome compatible with Creutzfeldt-Jakob disease and who were found to have a mutation of codon 200 in the prion protein. The patients developed symptoms and signs of peripheral nerve involvement diagnosed by electrodiagnostic and histopathological studies as demyelinating neuropathy. This may be a rare manifestation of Creutzfeldt-Jakob disease.

Action Potentials

Topographic distribution of the periodic discharges in Creutzfeldt-Jakob disease (CJD).

A characteristic EEG abnormality in patients with Creutzfeldt-Jakob disease (CJD) consists of periodic high voltage bilateral diphasic or triphasic sharp waves widely distributed over both hemispheres. The mechanism underlying these electrographic manifestations is, however, unclear. To increase our understanding of these phenomena, we have employed the technique of microcomputer brain mapping, to analyze the electrical fields of the bilaterally-distributed complexes in four patients with CJD. Ten generalized discharges were studied in each patient. The EEG analysis demonstrated variability in the area of onset as well as the field maxima between the different complexes among patients as well as between discharges in each patient. The generalized pattern seen in routine EEG was clearly shown on brain mapping to be not strictly generalized from onset, nor bilaterally symmetrical. These findings may suggest multifocal areas of onset of the generalized discharges, or alternating pathways of activation of the cortical areas by a subcortical pacemaker.

Aged

Coma with triphasic wave pattern in EEG as a complication of temporal arteritis.

Coma is rare in temporal arteritis (TA). We describe a patient with TA who suddenly developed coma with a triphasic EEG pattern that resolved after initiation of steroid treatment. Although the arterial lesions in TA are segmental in nature, TA may present with signs of diffuse neurologic deficit; early treatment can result in complete resolution.

Aged