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Biomedical subjects

R Mayeux

Publications and source records attributed to R Mayeux.

9 recordsLinked to original sources
PubMed1992

Behavioral syndromes in Alzheimer's disease.

The Behavioral Syndromes Scale for Dementia (BSSD) is a new instrument that showed strong internal consistency and interrater reliability in an outpatient sample of 106 patients with probable Alzheimer's disease. Factor analysis provided support for a priori symptom groupings, particularly the syndromes of disinhibition and apathy-indifference. Dependency (87%), denial of illness (63%), and motor agitation (55%) were common, while sexual disinhibition (2.9%) and self-destructive behaviors (2.9%) were rare. Virtually all symptoms were predominantly minimal to mild in severity. Patients with longer illness duration were more apathetic. Disinhibited behaviors and apathy-indifference increased with greater severity of dementia. Catastrophic reactions, aggression, and agitation were associated with greater functional impairment. There was great heterogeneity in symptom presentation. In Alzheimer's disease, several behavioral changes might be direct manifestations of underlying brain pathology, rather than being solely secondary to cognitive impairment.

Activities of Daily Living↗
PubMed1979

Poriomania.

Three patients with complex partial seizures experienced prolonged episodes of aimless wandering followed by retrograde amnesia for the experience, a phenomenon called poriomania. Our experience and the literature suggest that this behavior is a prolonged postictal automatism and is not psychogenic. Adjustments of anticonvulsant therapy eliminated this behavior in all three of our patients.

Amnesia, Retrograde↗
PubMed1979

Phantom limb and multiple sclerosis.

Phantom limb phenomena are most often associated with amputations, although they may accompany many neurologic disorders. In the neurologic variety, expression of illusory limbs varies with the level at which the somesthetic pathway is interrupted, and with changes in consciousness. Phantom limb illusions are rarely reported in patients with multiple sclerosis (MS). The patient reported here complained of a supernumerary phantom limb during an exacerbation of MS. This case suggests that in neurologic diseases, phantom limb illusions may result from distorted perception of limbs that are subject to both sensory loss and such recurrent spontaneous sensations as paresthesias.

Adult↗
PubMed1978

Strokes and ulcerative colitis.

One adolescent and two young adults had ulcerative colitis and cerebral thrombosis. All survived with neurologic deficit. These patients had no other predisposing factor for cerebrovascular disease. Systemic arterial and venous thromboembolic complications occur often in ulcerative colitis, but stroke is uncommon. Abnormalities in the early stages of clotting may be responsible, and the risk of thromboembolic phenomena in young patients seems to increase with exacerbations of this form of chronic inflammatory bowel disease, and possibly with regional enteritis as well.

Adolescent↗
PubMed1978

Cerebral cysticercosis.

Five patients with cerebral cysticercosis, two within the year preceding the date of this article, were seen at the New York Neurological Institute. The patients presented with mental changes, seizures, and symptoms of increased intracranial pressure, and had a history of having immigrated from an area endemic for cysticercosis. They were found to have parenchymal or intraventricular cysticercosis cysts. The interval from immigration to onset of symptoms was as long as 3 years. Plain radiograms of the skull and soft tissues, ventriculograms, and especially the CT scan, as well as the CSF examination, were useful in making the diagnosis. Surgical removal of an intraventricular cyst was curative in two patients and seizures were controlled with anticonvulsants in the other three.

Adult↗
PubMed1978

Complex partial status epilepticus: case report and proposal for diagnostic criteria.

A child had complex partial status epilepticus after resection of a craniopharyngioma. The status epilepticus was manifested by confusion and documented electroencephalographically by continuous focal seizure patterns in the right temporal region. Complex partial status epilepticus is an electroclinical syndrome of prolonged or repetitive complex partial seizures (with continuous interictal confusion) accompanied by electroencephalographic seizure patterns, which are either focal (usually temporal lobe) or secondarily generalized from a focal pacemaker.

Child, Preschool↗
PubMedDate not supplied

Lipid storage myopathy, ichthyosis, and steatorrhea.

A 41-year-old man had ichtyrosis, ectropion, steatorrhea, and slowly progressive proximal limb weakness. Biopsies showed abnormal lipid accumulation in muscle, liver skin, leukocytes, and gastric mucosa. Lipid storage was particularly marked in cultures of skin and muscle, and it increased in subsequent cell generations. By electron microscopy, the lipid globules showed no limiting membranes. The stored lipid was identified by thin-layer chromatography as triglyceride; there was no excess of cholesterol or cholesteryl esters. Muscle carnitine concentration and activities of carnitine palmityltransferase and acid lipase were normal; 14CO2 production from labeled palmitate in leukocytes was not impaired. The excessive accumulation of triglyceride in different tissues and in the progeny of cells in tissue culture suggests a genetic error of lipid metabolism.

Adult↗