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Biomedical subjects

A Molins

Publications and source records attributed to A Molins.

At least 19 recordsLinked to original sources
PubMed2002

[Status cataplecticus induced by abrupt withdrawal of clomipramine].

INTRODUCTION: Cataplexy is one of the main narcoleptic symptoms and is characterized by sudden loss of muscle tone triggered by emotional stimuli while consciousness is mantained. Clomipramine is an effective treatment of cataplexy. Cataplexy that occurs repeatedly for hours or days is referred to as status cataplecticus. PATIENTS: We report three adults with narcolepsy in whom cataplexy was chronically and effectively treated with clomipramine (75-150 mg/day). For diverse reasons, these three patients had an abrupt withdrawal of clomipramine, and after 2-9 days patients showed an invalidant status cataplecticus characterized by a marked increase of the frequency, duration and severity of their cataplectic attacks that were now elicited by mild emotional stimuli. After introduction of anticataplectic agents (clomipramine in two patients and fluoxetine in one patient), status cataplecticus was resolved in less than a week. CONCLUSION: In patients with narcolepsy, abrupt withdrawal of chronic treatment with clomipramine may be associated with status cataplecticus. This condition may be resolved with the reintroduction of anticataplectic agents.

English Abstract↗
PubMed1998

The need to quantify right-to-left shunt in acute ischemic stroke: a case-control study.

BACKGROUND AND PURPOSE: Although right-to-left shunt (RLSh) has been reported to be significantly more frequent in young stroke patients with cryptogenic stroke, its relevance in a nonselected population of acute ischemic stroke is not well known. The aim of this study was to determine the importance of the RLSh magnitude as a risk factor for stroke in nonselected patients. METHODS: Two hundred eight patients hospitalized consecutively with transient ischemic attack or acute cerebral infarction and 100 healthy control subjects were studied. Transcranial Doppler ultrasonography (TCD) was performed in both middle cerebral arteries (MCAs) after intravenous application of agitated saline solution. The magnitude of RLSh was quantified by counting the number of signals in 1 MCA during a Valsalva maneuver. RLSh was classified as "no shunt," "small" (< 10 signals), and "large" (> 10 signals), with the latter including the "shower" (> 25 signals) and "curtain" (uncountable signals) patterns. Extensive investigations, including contrast transesophageal echocardiography, were carried out on patients diagnosed as suffering from stroke of an uncertain etiology. The importance of RLSh for stroke was assessed by logistic regression analysis. RESULTS: Contrast TCD detected a large RLSh in 40 (19.7%) patients and in 21 (21%) control subjects, all with cardiac RLSh characteristics. A large RLSh was present in 4.7% of atherothrombotic strokes, 10.5% of cardioembolic strokes, 15.4% of lacunar strokes, and 45.3% of cryptogenic strokes (P<0.001). Although the overall frequency of RLSh was not significantly different between patients and control subjects, the detection of curtain or shower patterns by contrast TCD was associated with a higher risk of stroke (odds ratio, 3.5; 95% confidence interval, 1.29 to 9.87), particularly with cryptogenic stroke (odds ratio, 12.4; 95% confidence interval, 4.08 to 38.09) after adjustment for concomitant vascular risk factors. CONCLUSIONS: It is essential to quantify RLSh by contrast TCD during the Valsalva maneuver given that only those with shower and curtain patterns are associated with a higher risk of ischemic stroke in a nonselected population.

Acute Disease↗
PubMed1997

Wolfram syndrome: a neuropathological study.

Neuropathological examination was carried out on a patient aged 37 years who had suffered from Wolfram syndrome. Atrophy of the olfactory bulbs and tracts, atrophy of the optic nerves and chiasm, loss of neurons in the lateral geniculate nuclei mainly affecting the small cell layers, atrophy of the superior colliculus, loss of fibers in the cochlear nerve and mild loss of neurons in the cochlear nuclei and inferior colliculus, mild olivopontocerebellar atrophy, and demyelination of the pyramidal tracts were the main neuropathological findings. These correlated with anosmia, loss of vision, loss of hearing, cerebellar symptoms and signs, Babinski sign, and clonus, respectively, clinically observed in this patient. Mild neuron loss and gliosis in the preoptic and paraventricular area of the hypothalamus and mild motor neuron loss in the spinal cord did not reach thresholds of impaired function, although loss of neurons in discrete bulbar nuclei might have accounted for the late episode of food aspiration and suffocation. The relationship between memory loss, personality disturbances, and signs of prefrontal release and mild loss of neurons in the anterior and dorso-medial nuclei of the thalamus remains unclear.

Adult↗
PubMed1996

Effect of malnutrition after acute stroke on clinical outcome.

BACKGROUND AND PURPOSE: Malnutrition has received little attention in acute stroke, although it represents a risk of decreased immunity and nosocomial infections. Our objectives were to determine the prevalence of malnutrition after 1 week of hospitalization in acute stroke and to establish its relation to the stress response and neurological outcome. METHODS: The study included 104 patients with an acute stroke of less than 24 hours' duration. Nutritional parameters (triceps skinfold thickness, midarm muscle circumference, serum albumin, and calorimetry) were evaluated at admission and after 1 week. Stress response (free urinary cortisol) was measured daily during the first week. Neurological deficit was evaluated by the Canadian Stroke Scale. Clinical outcome was estimated by the Barthel Index 1 month after the acute stroke. Patients received an oral standard diet or polymeric enteral nutrition when they had swallowing difficulties. RESULTS: Protein-energy malnutrition was observed in 16.3% of patients at inclusion and in 26.4% after the first week, with a significant decrease in fat (P = .002) and visceral protein compartments (P = .049). Malnourished patients showed higher stress reaction and increased frequency of infections and bedsores in comparison with the appropriately nourished group. Multiple logistic regression analysis showed that malnutrition after 1 week (odds ratio, 3.5; 95% confidence interval, 1.2 to 10.2) and elevated free urinary cortisol (odds ratio, 3.3; confidence interval, 1.05 to 10.2) increased the risk of poor outcome (death or Barthel Index < or = 50 on the 30th day of follow-up) independently of age and nutritional status at admission. CONCLUSIONS: Our findings suggest that protein-energy malnutrition after acute stroke is a risk factor for poor outcome. Early appropriate enteral caloric feeding did not prevent malnutrition during the first week of hospitalization.

Acute Disease↗
PubMed1995

Clinical, neuropathologic, and genetic studies of a large spinocerebellar ataxia type 1 (SCA1) kindred: (CAG)n expansion and early premonitory signs and symptoms.

We report the clinical, neuropathologic, and genetic studies of a large kindred (family M-ADCA1) with autosomal dominant spinocerebellar ataxia type 1 (SCA1), ascertained in 41 members, with clinical data available in twenty-two. The mean age of onset was 36.3 +/- 6.2 years (ages, 26 to 52), the mean duration of the disease was 15.8 +/- 6.5 years (range, 10 to 28 years), and the mean age at death was 54.1 +/- 9.5 years (ages, 39 to 72). Premonitory signs and symptoms appeared earlier than the usual onset symptoms in many of the clinically unaffected patients who inherited the mutated SCA1 gene. Anticipation was present when we compared the seventh and eighth generations. A more severe course of the disease occurred in offspring of affected males. Neuropathologic examination, performed on three patients, showed the usual findings of SCA1; Golgi and immunocytochemistry studies suggested primary damage of the Purkinje cells. We analyzed the CAG-repeat mutation responsible for the SCA1 phenotype in a total of 41 family members. There was expansion in 19 subjects (10 clinically affected, seven with early signs and symptoms, and two asymptomatic individuals), and all showed heterozygosity, with one allele between 41 and 59 repeats (SCA1 mutation) and the other in the range of 6 to 39 repeats (normal range). The clinical analysis of "at risk" patients with the SCA1 mutation showed that minor signs and symptoms begin before full clinical diagnosis, and these premonitory manifestations can herald full development of SCA1 by years.

Adult↗
PubMed1994

Iron-related damage in acute ischemic stroke.

BACKGROUND AND PURPOSE: Although iron-mediated mechanisms are important in experimental brain injury after carotid occlusion, their clinical role in acute ischemic stroke has not been determined. We evaluated the influence of iron stores, measured as serum ferritin, on the outcome of acute cerebral infarct. METHODS: Admission and fasting glycemia, glycosylated hemoglobin, serum cortisol, serum ferritin, and 24-hour urinary free cortisol levels were measured on the first day of hospitalization in 67 patients admitted with an acute ischemic stroke of less than 24 hours' duration. Patients were classified into two groups according to their Canadian Stroke Scale (CSS) score on day 30: good outcome group (alive and CSS score > 7 points) and poor outcome group (dead or CSS score < or = 7 points). RESULTS: Thirty-three patients (49%) had good outcome and 34 (51%) poor outcome. Fasting glycemia (P = .001), serum cortisol (P < .001), and urinary free cortisol (P = .001) but not admission glycemia and glycosylated hemoglobin had higher levels in patients with poor outcome. Serum ferritin values were greater in the poor outcome group (218 +/- 156 micrograms/L versus 133 +/- 125 micrograms/L; P = .004), and a correlation between ferritin values and degree of worsening or improvement of the CSS score on day 30 was found (P = .002). Serum cortisol (odds ratio [OR], 6.7; 95% confidence interval [CI], 1.7 to 26), fasting glycemia (OR, 5.4; 95% CI, 1.2 to 24), and serum ferritin (OR, 4.6; 95% CI, 1.1 to 19) were independently related to poor outcome in a logistic regression analysis. CONCLUSIONS: High serum ferritin levels within the first 24 hours of hospitalization for an acute ischemic stroke are related to a poor prognosis, independent of the stress response. More research is needed to determine the origin of increased serum ferritin levels and the therapeutic implications.

Acute Disease↗
PubMed1993

[Value of the transcranial Doppler examination in the diagnosis of brain death].

BACKGROUND: Transcranial Doppler examination (TCD) is a non invasive method capable of detecting the interruption of cerebral flow in patients with criteria of brain death. Its recognition as an alternative to isoelectric EEG for the diagnosis of brain death requires previous validation. METHODS: Twenty-six patients in profound coma were examined by TCD. Of 23 patients with technically adequate study 13 manifested clinical criteria and EEG of brain death. Of these patients 9 had received barbiturate treatment and 4 had not. RESULTS: Changes in the flow waves of the TCD were observed in the form of diastolic reflux or systolic points of slight amplitude without diastolic flow in at least 2 arteries in 12 of 13 patients with criteria of brain death (sensitivity = 92%) and in none of the 10 patients without criteria of brain death (specificity = 100%). The use of barbiturates did not modify the normal anterograde flow detected by TCD in the absence of criteria of brain death. CONCLUSIONS: Transcranial Doppler is a good method for confirming the clinical diagnosis of brain death, fundamentally in patients undergoing treatment with drugs depressing the central nervous system.

Adolescent↗
PubMed1993

Presymptomatic analysis of spinocerebellar ataxia type 1 (SCA1) via the expansion of the SCA1 CAG-repeat in a large pedigree displaying anticipation and parental male bias.

Autosomal dominant cerebellar ataxia type 1 (ADCA1) is a clinical and genetic heterogeneous neurodegenerative disorder which leads to progressive cerebellar ataxia. One defective gene responsible for the disease was first localised to 6p (SCA1, spinocerebellar ataxia type 1) and the mutation has been more recently characterised. We have analysed the CAG-repeat mutation responsible for the SCA1 phenotype in a large Spanish kindred with 41 affected members, in which positive linkage with D6S89 was previously shown. All (10) clinically affected members analysed were heterozygous with one disease allele being between 41 to 57 CAG repeats, and the other in the normal range, from 6 to 39 repeats. Nine clinically unaffected individuals who were between the ages of 18 and 40, were found to have expansions of the CAG repeat (41 to 59), and 22 other 'at risk' individuals were found to have inherited the SCA1 gene with copies of the CAG repeat in the normal range. We have also observed that affected fathers passed on the mutated SCA1 gene with larger increases in the number of CAG repeats than affected mothers did. In one case a decrease in the number of CAG repeats (51 to 50) was detected in the transmission from the affected mother, and in two cases no change was observed in the transmission of a 41 allele repeat by a mother. As in the other disorders in which knowledge of the mutation has been obtained, analysis of the repeat expansion dramatically changes diagnosis of SCA1.

Adolescent↗
PubMed1993

[Migraine-infarct in patients treated with beta-blockers].

Migraine in itself is a potential cause of cerebral infarct in young adults. However the mechanism of migrainous ictus is open to discussion. Some drugs (oral anticonceptives, ergotaminics) could favor its appearance. Three cases has described in the medical literature of migraine patients in whom betablockers administration has been related with onset of an ischemic ictus. We present two new cases of this association. Nonselective betablockers provoke an increase in platelet activity by stimulation of platelet alpha-2 receptors; this together with their vasoconstrictor effect could favor the apparition of cerebral infarction in patients with migraine. We suggest, following other authors, to include non selective betablockers in the group of drugs which have to be carefully managed in the patients with migraine, especially in those with the classical type (migraine with aura) in whom an initial vasoconstriction has been suggested.

Acute Disease↗
PubMed1992

[Idiopathic icepick-like headache].

Two cases of idiopathic icepick-like headache are presented. This entity is clinically well defined but the appearance of accesses of intense, extremely brief pain commonly located at the same point in the territory of the first trigeminal branch followed at variable intervals and responding almost exclusively to treatment with indomethacin is little known. The appearance of pain crisis at regular intervals in one case lead to the differential diagnosis of cluster headache of a habitually different treatment: correct diagnosis requires a detailed account of the clinical characteristics of the pain with special emphasis on chronopathology.

Adult↗
PubMed1992

Neurokinin concentrations in cerebrospinal fluid. A preliminary study in Parkinson's disease.

Immunoreactive neurokinin A was measured in the cerebrospinal fluid of twelve patients with Parkinson's disease and eleven normal subjects, using a sensitive and precise extraction/concentration radioimmunoassay method. The mean value obtained in Parkinson's disease patients (13.2 +/- 4.6 pmol/l) was lower than that of the controls (17.4 +/- 5.9). The tendency toward a significant decrease (p = 0.085) found in this preliminary study could indicate that neurokinin A containing neurons are involved in the pathophysiology of Parkinson's disease. In addition, the establishment of reference values for neurokinin A in cerebrospinal fluid may provide a basis for further studies of this neuropeptide in neurological disorders.

Aged↗
PubMed1991

Somatostatin cerebrospinal fluid levels in dementia.

Somatostatin levels were measured in cerebrospinal fluid of patients with Alzheimer's disease, multi-infarct dementia and normal pressure hydrocephalus and compared with levels from a normal control group. All pathological groups showed a statistically significant decrease of somatostatin with respect to the control group, but no significant differences were found amongst them. A negative correlation was found between the Mini Mental State Test and the somatostatin levels in Alzheimer's disease patients but not in the other groups. Our results confirm that the lower levels of somatostatin in cerebrospinal fluid are not specific to Alzheimer's disease and indicate that the decrease found in all the groups is probably the result of neuronal destruction or damage in the diseases examined.

Adult↗
PubMed1991

Reappraisal of the intracranial pressure and cerebrospinal fluid dynamics in patients with the so-called "normal pressure hydrocephalus" syndrome.

Fifty-four shunt-responsive patients were selected from a prospective protocol directed to study patients with suspected normal pressure hydrocephalus (NPH). Patients with gait disturbances, dementia, non-responsive L-Dopa Parkinsonism, urinary or faecal incontinence and an Evans ratio greater or equal to 0.30 on the CT scan were included in the study. As a part of their work-up all patients underwent intracranial pressure monitoring and hydrodynamic studies using Marmarou's bolus test. According to mean intracranial pressure (ICP) and the percentage of high amplitude B-waves, patients were subdivided in the following categories: 1) Active hydrocephalus (mean ICP above 15 mmHg), which is in fact no tone normal pressure hydrocephalus; 2) Compensated unstable hydrocephalus, when mean ICP was below 15 mmHg and B-waves were present in more than 25% of the total recording time and 3) Compensated stable hydrocephalus when ICP was lower or equal to 15 mmHg and beta waves were present in less than 25% of the total recording time. The majority of the patients in this study (70%) presented continuous high or intermittently raised ICP (active or unstable compensated hydrocephalus group). Mean resistance to outflow of CSF (Rout) was 38.8 mm Hg/ml/min in active hydrocephalus and 23.5 mm Hg/ml/min in the compensated group (Students t-test, p less than 0.05). Higher resistance to outflow was found in patients with obliterated cortical sulci and obliterated Sylvian cisterns in the CT scan. No statistically significant correlation was found when plotting the percentage of beta waves against pressure volume index (PVI), compliance or Rout. An exponential correlation was found when plotting beta waves against the sum of conductance to outflow and compliance calculated by PVI method (r = 0.79). Patients with the so-called normal pressure hydrocephalus syndrome have different ICP and CSF dynamic profiles. Additional studies taking into consideration these differences are necessary before defining the sensitivity, specificity and predictive value of ICP monitoring and CSF studies in selecting appropriate candidates for shunting.

Adult↗