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Biomedical subjects

S Giménez-Roldán

Publications and source records attributed to S Giménez-Roldán.

At least 73 records · Page 4Linked to original sources
PubMed1987

Autosomal dominant endosteal hyperostosis. Report of a Spanish family with neurological involvement.

The first reported Spanish family with autosomal dominant endosteal hyperostosis is presented and two members in two different generations studied. Neurological involvement with sensorineural hearing loss, chronic intracranial hypertension, and mild corticospinal tract abnormalities were found in one case with radiological evidence of progressive bone disease at follow-up. In addition to mild hydrocephalus, CT-scan of the head documented a reduction in size of the posterior fossa and encroachment of the foramen magnum. A pattern of selective increase in the bone fraction of serum alkaline phosphatase was also recorded. This family supports the view that severe forms of endosteal hyperostosis are not confined to the autosomal recessive variant, as individuals with the autosomal dominant form may also show relentless progression to neurological involvement during adulthood.

Alkaline Phosphatase↗
PubMed1986

Focal asterixis caused by a small putaminal hemorrhage.

Unilateral asterixis and mild pure motor hemiparesis contralateral to a small putaminal hemorrhage were observed in a 74-year-old hypertensive man. The motor deficit proved to be rapidly reversible, but the focal asterixis remained for some time as the only sign of the intracranial hemorrhage. This case suggests that altered striopallidal inhibitory input to the thalamus probably disturbed the circuit subserving the maintenance of postural tone.

Aged↗
PubMed1985

Tardive dystonia and severe tardive dyskinesia. A comparison of risk factors and prognosis.

Nine tardive dystonia cases were compared with 13 tardive dyskinesia cases selected for the severity and persistence of their involuntary movements. Both groups were neurological referrals from an identical source. While advanced age and female preponderance were prominent features in tardive dyskinesia, onset in most tardive dystonia cases occurred in young adulthood, and the sex distribution showed a slight majority of males. Other differences in the dystonia group included gait abnormalities in four cases, lower tolerance of neuroleptic discontinuance, with the reappearance of psychoses, and a poorer prognosis for reversibility after follow-up. In fact, none of the dystonia patients reversed as opposed to seven of the tardive dyskinesia patients. In order to identify the full spectrum of tardive dystonia and exclude any referral bias, systematic epidemiological studies on psychiatric populations should include young adults of both sexes.

Adolescent↗
PubMed1984

Meige's syndrome: acute and chronic responses to clonazepan and anticholinergics.

Relief of blepharospasm was achieved with clonazepan (CNZ, 1 mg i.v.) and benzhexol (BH, 5 mg i.m.) by acute parenteral administration in 5 cases of Meige's syndrome. Improvement was greater with CNZ, mean value on a quantitative scale 100%, than for BH (84.1%). Both drugs were less effective on the associated oromandibular dystonia (OMD) observed in 3 of the cases, relief again being greater when using CNZ (87.3%) compared to BH (58.3%). Intravenous administration of CNZ predicted the response to prolonged oral medication (6 mg/day) in 3 of the cases. Though both blepharospasm and OMD are thought to represent focal dystonia at different body sites, the extent of improvement achieved with these drugs at the dosage employed differed markedly.

Aged↗
PubMed1981

Early detection of Huntington's disease. Blink reflex and levodopa load in presymptomatic and incipient subjects.

The R2 response on the blink reflex was found to be abnormal in seven out of 17 Huntington's disease descendants. Such abnormalities were present in four untreated subjects and in three further subjects after administration of a single oral dose of levodopa-carbidopa. An increase in latency and differential latency (four cases), or in a single one of these parameters (three cases) were the abnormalities found, resembling findings in four incipient cases detected during routine family surveys. Continuous administration of levodopa-carbidopa over a 10-20 day period did not induce new characteristics in the blink reflex, nor increase those detected previously, and no case developed chorea. We suggest that the analysis of the blink reflex after a single oral levodopa-carbidopa dosage could provide an objective and quantifiable method for the detection of individuals at risk for Huntington's disease.

Adolescent↗
PubMed1981

Tabetic lightning pains: high-dosage intravenous penicillin versus carbamazepine therapy.

The relief of tabetic lightning pains (LP) by carbamazepine (CBZ) was questioned as in some instances penicillin was given simultaneously. Only 1 out of 6 cases became pain-free on high-dosage intravenous penicillin, while CBZ rapidly abolished LP in those unresponsive to penicillin. Gastric crises were similarly abated by CBZ in 1 case under a placebo-controlled study. Inflammatory and degenerative changes in the posterior nerve root and ganglia may be the underlying pathologic basis that explains these two different responses to penicillin.

Administration, Oral↗
PubMed1981

Preclinical EEG abnormalities in subacute sclerosing panencephalitis.

Persistent electroencephalographic (EEG) abnormalities were observed for 4 years before the clinical onset of subacute sclerosing panencephalitis (SSPE) in a girl initially chosen as a "normal" control subject. Neurologic or mental changes were not observed during this period. Initially, there were focal paroxysmal discharges with shifting characteristics. Nonrepetitive generalized slow-wave complexes during wakefulness appeared later and persisted 3 weeks after onset of clinical manifestations. SSPE repetitive complexes were observed 1 month later. Multifocal paroxysmal EEG discharges during the presymptomatic stage of SSPE probably arise from restricted cortical areas containing persistently infected neurons and indicate that despite relative tolerance, the brain is not completely unresponsive to the presence of the virus.

Brain↗
PubMed1980

Startle epilepsy complicating Down syndrome during adulthood.

A woman with Down syndrome--a trisomy 21 karyotype--developed severe startle epilepsy and spontaneous seizures at the age of 20 years. Triggering factors, seizure patterns, and ictal electroencephalograms were the same as in startle epilepsy occurring in children with cerebral palsy but differed in that there was no clinical or radiological evidence of a focal brain lesion in the vicinity of the motor supplementary cortex. A therapeutic trial with clonazepam substantially reduced the frequency of reflex-induced and spontaneous seizures.

Adult↗