Biomedical subjects
S Giménez-Roldán
Publications and source records attributed to S Giménez-Roldán.
[Aggravation of Parkinson's disease after inadequate use of levodopa in controlled-release preparations].
We describe 16 patients with Parkinson's disease seen during a 6-month period because of aggravation related to the recent introduction of a levodopa/carbidopa controlled-release (CR) preparation, either in combination with or substituting for the standard levodopa preparations. Disease duration was longer than 5 years (mean 8.4 +/- 5.9 years) in most cases at the time the CR preparation was introduced, and the patients were suffering from either severe late levodopa syndrome or progression in disability. For 9 out of 14 patients the CR formula had replaced the older preparation overnight. Though patients often accumulated multiple side-effects, we were able to identify 3 subgroups based on the main problem developing after the CR preparation had been introduced: a) apparent acceleration of the course of disease in patients in advanced stages, even if their response to treatment was otherwise stable (n = 4); b) subacute onset or aggravation of dyskinesias or painful "off"-period dystonia that often prompted reevaluation of the patient (n = 4); c) deterioration of akinetic "off" periods in patients already experiencing response fluctuations (n = 5) or appearance of dose failures in patients who had so far been enjoying stable responses (n = 3). These problems appeared in spite of a significant increase (p = 0.046) in total levodopa daily dose even though there were no differences (p = 0.685) in dose frequency between the CR preparation and standard levodopa.(ABSTRACT TRUNCATED AT 250 WORDS)
Progressive supranuclear palsy and corticobasal ganglionic degeneration: differentiation by clinical features and neuroimaging techniques.
To assess the extent of overlap between clinically diagnosed patients with progressive supranuclear palsy (PSP) and corticobasal ganglionic degeneration (CBGD) we compared clinical scores for rigidity, bradykinesia, supranuclear gaze abnormalities, hemineglect and limb apraxia, postural instability, neck rigidity, and limb dystonia in 15 patients with a degenerative rigid-akinetic syndrome at presentation and at follow-up 3 to 120 months later. Only the presence of hemineglect, usually in combination with limb apraxia, was a reliable and early clinical factor for discriminating between these two conditions. These symptoms were present at admission in all 4 CBGD patients but not in any of the 11 PSP patients either at presentation or later during serial examinations. Though supranuclear ophthalmoplegia, neck rigidity, and postural instability were already observed in most CBGD patients at presentation, their scores remained low compared to those for PSP patients over the longterm. CT-scans and MRI were helpful in supporting clinically-based diagnoses made at presentation in that the vast majority of the PSP patients exhibited various degrees of midbrain atrophy and 50 percent of the CBGD patients exhibited asymmetric pericentral cortical atrophy.
[Patient care in a hospital based unit for treating Parkinson's disease and movement disorders: a 3-year prospective study].
A register covering a 3-year period (1991-1993) was opened for all patients attending a Parkinson's disease and movement disorder clinic which has been running for 15 years in a reference hospital covering a population of 629,555 in Madrid. During the last 2 years, all 5 neurologists serving the reference health area joined to the hospital on a part-time basis. A total of 764 patients were seen during the study period, 56.2% of whom were follow-up patients. About half of the later (49%) were suffering from Parkinson's disease (PD), follow-up duration averaging 4.7 +/- 4.4 years. Follow-up in dystonias (11.8%) and choreas (8.1%) cases was similar and averaged 5.0 +/- 4.2 and 4.5 +/- 4.2 years, respectively. Out of 334 new referrals 192 were new parkinsonism patients, half of them (48.4%) with secondary parkinsonism, particularly drug-induced parkinsonism (32.2%). Incorporation of neurologists serving the reference health area to hospital activities correlated to a decrease in secondary parkinsonism referrals, a slight drop in de novo PD patients, but not in an earlier detection of new PD cases. We conclude that a monographic out-patient clinic may benefit from sharing activities with neurologists serving the referral health area, and from improving the neurological education of general practitioners.
[The startle reflex in humans and its pathology: a proposal for the classification].
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Exposure to well water and pesticides in Parkinson's disease: a case-control study in the Madrid area.
Past exposure to well water and pesticides was assessed in 128 unselected Parkinson's disease (PD) patients and 256 age and sex-matched controls. All were residents in a defined urban area of Madrid, Spain. In keeping with other reports, we found that exposure to well water might be a factor associated with the likelihood of developing PD, though only prolonged exposures of 30 years or longer were significantly different between PD and controls (p less than 0.02). In contrast, past exposure to pesticides did not appear to be associated with an increased risk of developing PD. Prolonged well water drinking antedating the development of PD was not associated with early onset of the disease, nor did such cases progress to greater disability. Future case-control studies addressing prolonged well water consumption as a risk factor in PD should look for differences in the content of substances other than pesticides in the water as determined by the source of water to which patients may have been specifically exposed.
Neuroleptic malignant syndrome related to tetrabenazine introduction and haloperidol discontinuation in Huntington's disease.
We describe the second reported case of neuroleptic malignant syndrome (NMS) related to tetrabenazine therapy in Huntington's disease. In the previously reported case, factors capable of potentiating NMS included a high dosage of tetrabenazine exceeding the accepted therapeutic range, and co-medication with the dopamine-synthesis inhibitor alpha-methylparatyrosine, while in the present case abrupt introduction of the drug and discontinuation of concomitant neuroleptics may have contributed to this important adverse reaction. Uneventful recovery occurred in both cases without the need for drugs specifically enhancing dopaminergic transmission, while rechallenge by tetrabenazine with conventional doses and slow upward titration was not followed by recurrence of the NMS. Tetrabenazine has proved to be a safe and frequently useful drug in the long-term treatment of approximately 400 dyskinetic patients. We believe that NMS related to this drug is rare, provided that it is properly administered.
[The Madrid school of neurology in the old Hospital General de Madrid].
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[Caudate hemorrhage: a frequent diagnostic error].
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[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.
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.
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.
[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.
[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.
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.
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.
[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.
[Neurocysticercosis among Latin American emigrants].
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