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Biomedical subjects

S Giménez-Roldán

Publications and source records attributed to S Giménez-Roldán.

At least 55 records · Page 3Linked to original sources
PubMed1992

[Arnold-Chiari malformation with multiple paroxysmal manifestations induced by coughing].

We present a 66-year old woman suffering from a chronic disorder characterized by multiple paroxysmal symptoms precipitated by coughing. These included cephalalgia, syncope, binocular photopsia phenomena with blurred vision, and an "electric-like" paroxysmal tingling of the hands. In addition to a central spinal cord cavity and hindbrain herniation, magnetic resonance imaging showed multiple skeletal anomalies and the craniospinal junction which included a narrow clivo-axial angle, basilar impression of the skull and a tight foramen magnum. Resonance magnetic imaging showed a high-signal intensity lesion on T2-weighted images at the posterior medullo-spinal junction suggesting focal demyelination. We propose that paroxysmal symptoms induced by coughing in patients bearing hindbrain ectopia and skeletal anomalies at the foramen magnum region may involve different pathogenetic mechanisms, including ectopic axonal activity and ephaptic transmission at the sensory pathways. This caused a Lhermitte-like phenomenon precipitated by coughing, rather than by forward flexion of the neck. However, increased pressure at the posterior fossa presumably underlies all these phenomena, and may therefore be potentially relieved by suboccipital decompressive craniotomy.

Arnold-Chiari Malformation↗
PubMed1991

Cinnarizine-induced parkinsonism. Susceptibility related to aging and essential tremor.

Age at onset in 24 consecutive cinnarizine-induced parkinsonism (CIP) patients referred during a 2-year period was compared with 102 newly referred cases of Parkinson's disease (PD) examined during the same period. Not only did CIP onset occur at a greater age than PD (70.6 + 1.4 years versus 60.1 + 1.1 years), but the number of CIP cases increased steadily with age, whereas the incidence of PD patients peaked between the ages of 55 and 60 years, as is usually the case. At the time of referral, 62% of CIP cases and 14% of PD cases were over the age of 70, suggesting that advanced age was not a source of referral bias. A structured questionnaire prospectively given to 24 CIP patients revealed a history of tremor in at least one family member in 56% of the cases, whereas the incidence was much lower in 124 PD cases (17%) and 102 hospitalized nonneurological patients aged over 65 (6%). Moreover, three of the CIP patients themselves had a history of essential tremor previous to the onset of parkinsonism. CIP patients had frequently been exposed to the drug for years before developing any extrapyramidal symptoms (mean exposure, 4.1 +/- 4 years; range 4 months to 15 years). Though controlled epidemiological studies are needed to evaluate the possibility that cinnarizine is increasingly prescribed in the general population with advancing age, our data suggests that aging plus a background of genetically determined essential tremor represented critical risk factors for development of this drug side effect.

Adult↗
PubMed1991

Predicting beneficial response to a protein-redistribution diet in fluctuating Parkinson's disease.

To identify factors that might help in predicting the benefit to be gained from a protein-redistribution diet (PRD) we subjected 26 parkinsonian patients with motor fluctuations refractory to optimal timing and dosage of levodopa plus bromocriptine to a 2,000-2,500 Kcal., 65-80 g/d protein containing diet maintained for 8-12 weeks. Fifteen patients were eligible for evaluation, 10 of whom being benefited by the PRD (79 percent reduction in the mean "off" time in "on-off" charts). When the duration and pattern of the fluctuations were compared in the 10 diet-benefit patients with those in 5 diet-failure patients only dose-failures consistently occurring postprandially were resolved by the PRD. Wearing-off failures responded unpredictably while random "on-off" fluctuations were present only in the diet-failure group. Nocturnal akinesia and peak-dose dyskinesias often worsened. In no patient "on"-time quality was modified by the diet. In addition, the diet-failure group was characterized by a younger mean age at onset (p less than 0.05) and by longer duration in their fluctuations (p less than 0.001). Though requiring confirmation in a larger series, our results suggest that parkinsonian patients showing fluctuations over prolonged periods, particularly those having a pattern of random "on-off" oscillations in motor performance and dose-failures unrelated to meals are unlikely to benefit from a PRD.

Aged↗
PubMed1991

[Advances in the treatment of the dystonias].

Except in Wilson's disease, few secondary dystonias are susceptible te benefit from an etiological treatment. The somatic distribution of dystonia often determines the therapeutic strategy. Thus, stereotactic surgery may be the treatment of choice for hemidystonia while anticholinergic medication may alleviate generalized dystonia, particularly in childhood. Finally, local infiltrations of botulinum toxin are particularly useful for various forms of local and segmental dystonia. Certain subsyndromes as myoclonic dystonia, levodopa sensitive dystonia and paroxysmal choreoathetosis may benefit from relatively specific treatment strategies.

Blepharospasm↗
PubMed1991

[Proposal for a protein redistribution diet in the control of motor fluctuations in Parkinson's disease: acceptance and efficacy].

Some patients with Parkinson's disease (PD) and fluctuations of motor response to levodopa therapy may benefit by avoiding proteins during daytime meals, while leaving them unrestricted until bedtime. The acceptance and benefits of a protein redistribution diet (PRD) was studied in 26 PD patients whose fluctuations were refractory to current medications. Only 15 patients (57.2%) were still adhered to the diet 3 months later. Non compliance was more often justified on the basis of the changes in alimentary habits, as a too heavy supper (37%), scanty variation of meals (27%) and difficulties in preparing the diet (18%), rather than do to adverse effects of the diet on PD which occurred in 2 patients (exacerbation of the dyskinesias and lack of effectiveness, respectively). The PRD proved beneficial to 67% of those patients able to keep adhered to it, 4 patients shifting to stable responses. Five diet-benefit patients who performed daily "on-off" charts decreased their mean daily "off" time from 13 to 3 % (p less than 0.05), but "on" time quality remained unimproved by the diet. The PRD introduces a heavy change in dietary habits which is not readily accepted by many PD patients. However, the fact that fluctuations disappeared in one fourth of those able to maintain the diet warrants a closely supervised, short-time trial to identify those in whom benefits override the inconveniences of such new changes in the patient's way of life.

Aged↗
PubMed1990

High-dose intravenous-penicillin in neurosyphilis: effect on intrathecal synthesis of IgG, IgM, IgA and IgD.

We evaluated occurrence and levels of intrathecal synthesis of IgG, IgM, IgA and IgD by calculating corresponding index values in 8 patients with definite and 4 with suspected or possible neurosyphilis, prior to, during and after high-dose intravenous penicillin therapy. Four patients with active neurosyphilis displayed intrathecal synthesis of IgG, IgM and IgA. Only 2 of them showed elevated IgD index, and both had taboparesis, pleocytosis and positive VDRL in CSF, and simultaneous elevation of the IgG, IgA and especially of the IgM indices. This suggests that intrathecal synthesis of IgD may occur in patients with severe CNS inflammation in response to diffuse CNS treponemal damage. Penicillin therapy incited transitory elevation of one or more of the immunoglobulin index values in most patients, possibly in response to massive treponemal lysis inside the CNS. Thereafter, the values became mostly normalized but exceptions occurred, including one patient who had elevated IgG and IgM index which persisted 31 months after therapy. Our data indicate that determinations of immunoglobulin indices performed on consecutive specimens from individual patients with neurosyphilis may be helpful in the evaluation of treatment.

Adult↗
PubMed1990

Sensory ataxic hemiparesis in thalamic hemorrhage.

Ataxic hemiparesis with cerebellar-like features has been reported following infarction or hemorrhage of the thalamus. We describe five patients with incoordination and corticospinal tract signs in the limbs opposite a dorsolateral thalamic hemorrhage. In four patients the hemorrhage extended superiorly into the lateral ventricle, the adjacent paraventricular region, and the upper and medial parts of the posterior limb of the internal capsule. Instead of cerebellar dysfunction, these patients exhibited directional errors of movement that improved distinctly when performed under visual guidance. We explain the incoordination on the basis of conscious deep sensory loss in the involved limbs. Perception of light touch, painful pinprick, and temperature were preserved in all patients. We use the term "sensory ataxic hemiparesis" to distinguish these patients from those with "classic" ataxic hemiparesis and cerebellar-like features, a distinction that may be important when planning functional rehabilitation.

Adult↗
PubMed1990

[Familial spastic paraparesis: phenotypic variant of adrenoleukodystrophy].

Two brothers developed a slowly progressive paraparesis during adulthood, lately associated in one to primary adrenal and gonadal insufficiency but no electrophysiological evidence of peripheral nerve involvement. Both patients and an asymptomatic sister had increased plasma concentrations of very-long chain fatty acids (VLCFA). A CT-scan of the propositus was initially normal but showed at follow-up diffuse white matter hypodensities consistent with severe demyelination of the centrum semiovale. The appearance of the lesions remained unchanged for the next two years without clinical evidence of supraspinal changes in despite of progression of the paraparesis. We believe that these observations further support a link between the severe infantile variety of adrenoleukodystrophy and the more protracted adult-onset variants. Further, CT-scan in families with spastic paraparesis may be of help in detecting early evidence of an underlying diffuse white matter disorder, eventually supported by more conclusive studies as VLCFA determinations.

Adrenoleukodystrophy↗
PubMed1989

Life-threatening cranial dystonia following trihexyphenidyl withdrawal.

A 64-year-old woman with blepharospasm, sustained clenching of the jaw, antecollis, and a strained, high-pitched phonation continued chronic trihexyphenidyl therapy despite the lack of any obvious benefit. Abrupt, accidental withdrawal of trihexyphenidyl triggered severe exacerbation of the cranial dystonia associated with inspiratory stridor and acute respiratory difficulties, prompting emergency admission. On indirect laryngoscopy, hyperadduction of the vocal folds was not the cause of the upper airway obstruction. A more likely cause of the inspiratory obstruction appeared to be forward bending of the neck combined with mouth-clenching spasms. Reinstitution of intravenous anticholinergic medication provided relatively prompt relief. We caution against abrupt interruption of anticholinergics in patients with severe segmental cranial dystonia, even in those cases in which no benefit is apparent to observers.

Aged↗
PubMed1989

[Huntington disease: tetrabenazine compared to haloperidol in the reduction of involuntary movements].

Dopaminergic oversupply relative to intrinsic striatal cell loss is thought to underlie the involuntary movements of Huntington's disease (HD). Tetrabenazine (TBZ) and haloperidol, two antidopaminergic compounds differing in their mechanism of action on the nigrostriatal pathway were compared in 11 HD patients in their ability to decrease the choreatic movements in a single-blind cross-over study. Though improvement in chorea scores over baseline values was greater under TBZ (46.3 +/- 23.4) than haloperidol (28.6 +/- 47.7), the difference did not reach statistical significance (Student t tests). All 11 patients improved to some extent during the TBZ phase, and in 6 it was greater than while under haloperidol. However, haloperidol reduction in the chorea scores was significantly greater in 3 patients, while there was no difference in 2 other cases. Severe depression occurred in 3 patients under TBZ, in one leading to attempting suicide, while tardive dyskinesia complicated haloperidol therapy in 3 patients. The later drug could be an alternative therapy for those patients in whom TBZ provides unsatisfactory control of the choreatic movements, but it might be the drug of choice in cases with associated depressive illness or organic psychosis.

Adult↗
PubMed1989

[Essential tremor in the elderly: incidence in hospital admissions for non-neurologic reasons].

The frequency of essential tremor (ET) was determined in 102 unselected elderly patients aged 65 or over (average 73.9 +/- 9.1) admitted to the medical and surgical wards of a general hospital for non-neurological conditions. Patients bearing potential causes for symptomatic tremor were excluded. ET was observed in 10 individuals (9.8%) and in one a previously unrecognized Parkinson's disease (PD) was detected. The frequency of the ET increased linearly by age groups up to the age of 80 to decrease thereafter. In 30% of the cases the tremor did not involve the limbs, five had pathologically enhanced physiological tremor, one had type 2 ET and in one other a type 3 ET was combined with tremor of the head. No case had previously consulted because of the tremor and a positive family history for this was found in only 2 cases. In the overall group, a 4.9% affirmed to have a relative with tremor and in a further 3.9% a specific diagnosis of PD had been established in at least one relative. The occurrence of ET in first-degree family members was considerably lower, with a history of ET found in 1.5% out of 197 parents, 0.8% of 473 brothers and sisters and 0.3% out of 277 living sons and daughters. Our study indicates that a positive family history for tremor is commonly encountered in the general population, and a high percentage of elderly individuals may themselves exhibit ET albeit mild enough usually as not to warrant neurological consultation.(ABSTRACT TRUNCATED AT 250 WORDS)

Aged↗
PubMed1988

Involuntary closure of eyelids in parkinsonism. Electrophysiological evidence for prolonged inhibition of the levator palpebrae muscles.

Involuntary closure of eyelids (ICE), a phenomenon variously interpreted as blepharospasm and apraxia of lid opening, is occasionally observed in parkinsonism. Nine patients (4 with Parkinson's disease, 2 with post-encephalitic parkinsonism, and 3 with supranuclear palsy) with prominent ICE, were studied by electromyographic recording of the eye muscles. ICE episodes were shown to be dependent upon prolonged, irregular inhibition of the normal tonic activity of the levator palpebrae superioris (LPS) muscle causing drooping of the upper eyelid without any corresponding activation of the orbicularis oculi (OO) muscle. Nevertheless, some degree of excessive, widely fluctuating OO activity was present in seven of the patients. Blepharocolysis (from Gr. blepharon, eyelid, and kolysis inhibition) is put forward as the term to designate ICE episodes resulting from abnormally long inhibition of the LPS muscles and should be differentiated electrophysiologically from blepharospasm, excessive OO muscles activity. Abnormal influences from basal ganglia acting on brainstem structures that regulate blinking may falicitate either of the two components of normal blinking resulting in ICE due to the predominance of LPS inhibition (blepharocolysis), the predominance of OO activation (blepharospasm) or a combination of the two.

Adult↗
PubMed1988

Clinical, biochemical, and pharmacological observation in a patient with postasphyxic myoclonus: association to serotonin hyperactivity.

Posthypoxic action myoclonus is usually associated with impaired serotonin (5-HT) neurotransmission but in some patients 5-HT precursors aggravate and 5-HT blockers improve action myoclonus. We studied a 65-year-old man who presented with action myoclonus following a prolonged episode of moderate hypoxia and severe hypercarbia. The myoclonus increased with 5-hydroxytryptophan (5-HTP) 1,200 mg/day plus carbidopa 300 mg/day and sodium salt of valproic acid (SVA) 800 mg/day, and improved with 1 mg of clonazepam (CNZ) in an intravenous bolus. Biochemical analysis of the cerebrospinal fluid (CSF) prior to any drug therapy did not reveal abnormalities in the levels of homovanillic acid (HVA) and methoxyhydroxyphenylglycol (MHPG) but 5-hydroxyindoleacetic acid (5-HIAA) levels were elevated in comparison with controls (33 versus 21 ng/ml). SVA therapy produced a moderate increase and 5-HTP plus carbidopa a threefold elevation of 5-HIAA in CSF and marked aggravation of action myoclonus. Methysergide (3 mg/day) totally suppressed myoclonus and decreased CSF 5-HIAA to undetectable levels. Methysergide also reduced CSF tryptophan to 40% of baseline levels. Discontinuation of methysergide and substitution by placebo was followed by reappearance of myoclonus. A partial and incomplete spontaneous remission of symptoms took place 7 months after the asphyxic episode. Action myoclonus and enhanced 5-HT neurotransmission may be present in patients in which acidosis reverses the effects of hypoxia on 5-HT neurotransmission.

5-Hydroxytryptophan↗