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Biomedical subjects

A Guberman

Publications and source records attributed to A Guberman.

18 recordsLinked to original sources
PubMed1992

Neuroblastoma and fetal exposure to phenytoin in a child without dysmorphic features.

Despite the fact that the teratogenic effects of phenytoin have been suggested in several case reports, the evidence for a possible oncogenic potential of phenytoin has not been widely recognized. Recently, neuroblastoma as well as other neuroectodermal and non-ectodermal tumors has been seen in several children exposed to phenytoin prenatally. Previous cases have been almost uniformly associated with the features of "fetal hydantoin syndrome" and none have been developmentally normal. We report a developmentally-normal boy of 21/2 years with an abdominal neuroblastoma whose mother had been on phenytoin (as well as carbamazepine) throughout gestation. We review the various neoplasms which have been reported in the offspring of mothers receiving phenytoin.

Abnormalities, Drug-Induced↗
PubMed1990

Add-on trial of clobazam in intractable adult epilepsy with plasma level correlations.

Clobazam, a novel benzodiazepine, was used as an add-on agent in 47 adult patients with intractable epilepsy and a variety of seizure types. A greater than 75% reduction in seizure frequency was observed in 42% (18) of the patients and a greater than 50% reduction in 65% (29) of the patients which was sustained over a mean follow-up period of 13.3 (+/- 5.6) months. Nine patients had to discontinue the drug due to minor adverse effects or increased seizures. Possible tolerance developed in 6 patients. Plasma levels of the active metabolite N-desmethylclobazam were linearly related to dosage and appeared to correlate with both therapeutic and toxic responses. Clobazam appears to be a safe and effective add-on antiepileptic for a wide variety of seizure types in intractable epilepsy.

Adult↗
PubMed1989

Valproate prevents the recurrence of absence status.

We assessed the value of valproate in the prevention of recurrent attacks of absence status in 25 patients. Eighteen patients had primary generalized epilepsy with a mean frequency of attacks of absence status of 5.7 per year. After a mean follow-up period of 4.4 years, the attack frequency was reduced to 0.6 per year (p less than 0.0005); 14 patients had no recurrence, 3 had rare attacks with noncompliance, and 1 had an incomplete response probably due to gastrointestinal intolerance. Patients with evidence of generalized cerebral damage (n = 2) or with EEG evidence of focalization (n = 5) did not respond as favorably. Valproate is the drug of choice for the prevention of recurrence of absence status. Moreover, the response can be predicted on the basis of the electroclinical subtype of absence status.

Adolescent↗
PubMed1989

Religion and epilepsy.

A study of 57 consecutive patients with epilepsy who were attending a neurological epilepsy clinic at a general hospital, was carried out. A preliminary analysis of the data revealed that 33% had at some time been treated for a psychological disorder, 51% had 'normal' and 60% 'abnormal' interests in religion and related belief systems. Fifty-one percent had undergone a significant change in their interest in these matters in the past (religious conversion). A significant association was found between the 'abnormal' group and psychopathology, and religious conversion. These associations were not found with the 'normal' group.

Adolescent↗
PubMed1988

The neuropsychology of paramedian thalamic infarction.

A longitudinal study of three patients with CT-scan documented paramedian thalamic infarctions (bilateral, primarily right, unilateral left) is reported and the neuropsychology of human paramedian thalamic infarction is reviewed. The neuropsychological deficits following these selected lesions, the nature of the clinical memory disorder, and the neuroanatomy of memory are discussed. The significance of cortical/subcortical relationship in explaining observed behavioral changes is emphasized. Brain damage with maximum involvement in the dorsomedial nuclei and mamillothalamic tracts appears to cause primarily a memory disorder and frontal-limbic behavioral changes, the severity and profile of deficits depending on lesion extent and location. Both anterograde and remote memory loss may be present. Asymmetry in memory at the level of the thalamus was observed, following the left-verbal, right-nonverbal dichotomy.

Aged↗
PubMed1987

Idiopathic facial nerve paralysis (Bell's palsy) in late pregnancy and the early puerperium.

We describe seven patients with idiopathic facial (VII) nerve palsy commencing primarily in late pregnancy or in the immediate postpartum period. One patient had recurrent Bell's palsy in three consecutive pregnancies. Three women developed bilateral palsy. The occurrence of such otherwise rare manifestations of Bell's palsy supports the suggestion that physiologic changes occurring in late pregnancy or the immediate puerperium predispose to Bell's palsy.

Adult↗
PubMed1986

Mollaret's meningitis: a case with increased circulating natural killer cells.

A patient with longstanding Mollaret's meningitis had an increased number of circulating natural killer cells during an acute attack, as evidenced by 73% large granular lymphocytes and 80% Leu-11a-positive peripheral mononuclear cells. Numbers of large granular lymphocytes and Leu-11a-positive cells returned to normal by three months after the attack. Similarity of the morphological features of the Mollaret's cell to those of large granular lymphocytes is noted.

Adult↗
PubMed1986

Swallow syncope.

Swallow syncope is an often misdiagnosed rare disorder due to enhanced vagal tone during eating in patients with underlying esophageal and/or cardiac abnormalities. We present three cases of this disorder, one related to digitalis toxicity and the other two with diffuse esophageal spasm. The investigation, differential diagnosis, prognosis and management of swallow syncope are discussed.

Adult↗
PubMed1986

Bilateral facial paralysis following ethylene glycol ingestion.

Bilateral facial weakness, diminished hearing and dysphagia developed in a patient with a large overdose of ethylene glycol. Our case and two others previously reported draw attention to the fact that cranial nerve dysfunction, especially bilateral facial palsy, may occur in addition to encephalopathy, coma and renal failure with ethylene glycol ingestion.

Adult↗
PubMed1986

Nonconvulsive generalized status epilepticus: clinical features, neuropsychological testing, and long-term follow-up.

We studied 13 episodes of nonconvulsive generalized status epilepticus (NGS) in 10 adults. The syndrome may start in middle or late life and is often mistaken for psychiatric disorders. Some patients have shown good retention of language abilities, except for reduced fluency, despite impairment in other higher functions. In most patients, the syndrome recurs for several years despite anticonvulsant therapy. Transitional cases of NGS with lateralizing EEG features exist, and some cases are probably due to secondary generalization from a temporal or frontal focus.

Adolescent↗
PubMed1985

Substructure of 20 nm filaments of progressive supranuclear palsy.

In contrast to the ultrastructure of Alzheimer's neurofibrillary tangles (NFT), which has been well characterized as accumulations of paired helical 10-nm filaments (PHF) with 80-nm regular constrictions, the morphology of the neurofibrillary changes of PSP remains ill-defined. Until recently, the fine structure of PSP tangles was generally accepted as 15-nm straight filaments or tubules, although many reports describing different electron-microscopic findings have appeared in the literature. In this report, we present morphological data indicating a protofilamentous substructure present in straight filaments of PSP which has some points of similarity with the protofilamentous architectures which have been reported for paired helical filaments of Alzheimer's disease. The straight filaments were found to be composed of six or more helically symmetric 2-5-nm protofilaments. We conclude that despite the varied morphology of filaments in neurofibrillary tangles observed in PSP there may be some underlying identity at the molecular level with the PHF of neurofibrillary tangles of Alzheimer's disease.

Aged↗
PubMed1979

Pseudohypoparathyroidism and epilepsy: diagnostic value of computerized cranial tomography.

Computerized cranial tomograms (CCTs) unexpectedly showed bilateral symmetrical calcifications in the basal ganglia and frontal areas in two unrelated epileptic patients 12 and 13 years of age. The patients presented with a variety of seizures, some with focal features; these seizures were resistant to medication in the first case. Subsequent testing revealed hypocalcemia and other biochemical and radiologic features of pseudohypoparathyroidism, despite absence of the usual phenotypic features, tetanic symptoms, and positive family history. The CCT scan may provide the first clue to an underlying hypocalcemic disorder in an epileptic patient even when the skull X-ray is normal. Early detection of this metabolic condition by CCT scanning allows specific treatment to restore serum calcium levels to normal, which usually eliminates seizures and favors optimal cerebral functioning. Serial CCT scanning also provides a useful means for following the intracerebral calcifications, which remained unchanged after 1 and 2 years of normocalcemia in our 2 patients.

Adolescent↗
PubMed1978

Human neurolymphomatosis.

A patient with a chronically progressive fatal sensorimotor neuropathy showed, at autopsy, extensive and selective lymphocytic infiltration of peripheral and cranial nerves and a segmental demyelinative process. A clinically occult retroperitoneal lymphoma without spread to other systemic organs was also present. The possibility of a selective infiltration of the peripheral nervous system by the retroperitoneal malignancy is rejected as unlikely. Our case bears a strong similarity to 3 other cases previously reported which may be grouped together under the heading "human neurolymphomatosis". The clinical and pathological features of this rare entity are discussed. It bears a resemblance to Marek's disease of chickens and seems to represent an unusual inflammatory neuropathy or form of malignancy.

Brain↗
PubMed1975

Response of generalized penicillin epilepsy in the cat to ethosuximide and diphenylhydantoin.

The effects of ethosuximide and diphenylhydantoin sodium on feline generalized penicillin epilepsy, a model of human generalized corticoreticular (centreccephalic) epilepsy, were significantly reduced following administration of ethosuximide with plasma levels of 60 mug per milliliter, and there was a linear correlation between the plasma level and antiephilepileptic effect (p less than 0.01). Diphenylhydantoin produced a lesser reduction in epileptic activity, and there was no correlation between the plasma level and effect. Four cats that received both drugs successively responded well to ethosuximide, while only two responded to diphenylhdantoin. The good response to ethosuximide is in accord with clinical experience in human generalized corticoreticular epilepsy.

Animals↗
PubMedDate not supplied

Primary reading epilepsy: investigation of critical seizure-provoking stimuli.

Precipitating stimuli were investigated in a 24-year-old woman with primary reading epilepsy. Reading material was presented on a microcomputer video display monitor under controlled conditions while the patient underwent EEG radiotelemetry/video monitoring. We examined the relative contribution of the following variables utilizing a factorial design: eye movements, reading aloud versus reading silently, linguistic complexity, and concentrations. None of these factors acted solely as the critical stimulus in provoking seizures. Seizures were most readily elicited when the patient read aloud, using material of medium or high linguistic complexity with the usual scanning eye movements for reading.

Adult↗