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Biomedical subjects

L Rumbach

Publications and source records attributed to L Rumbach.

At least 19 recordsLinked to original sources

[Role of immunity in the development of progressive multifocal leukoencephalopathy: a report of three patients with type B lymphoma and humoral immunodeficiency and six others with acquired immunodeficiency syndrome].

INTRODUCTION: Progressive multifocal leukoencephalopathy (PML) caused by JC virus (JCV) mostly occurs in different conditions of impaired cellular immunity like acquired immunodeficiency syndrome (AIDS) and rarely when humoral immunity is involved. PML remains unusual although there is a high prevalence of JCV among the population and immunosuppression is not rare because of chemotherapies. METHODS: We present two groups of patients: first, we studied reports of three patients suffering from lymphoma type B who developed a PML, proved by cerebral biopsy. The second group included six HIV-infected patients who developed a PML. No biopsy was made but MRI and the physical examination suggested strong arguments for the diagnosis. RESULTS: In the first group, PML was furthered by humoral immunosuppression (rate of immunoglobulin G under 4 g/l). Average survival was five months. In the second group, HIV-infected patients had a survival range from 2 to 58 months after the first PML symptoms and one of them is still alive. CONCLUSION: Humoral immunosuppression in lymphoma can contribute to the development of PML. PML prognosis is often severe but prolonged survivals were described. So it is necessary to restore a sufficient immunity level. But immunity failure may be insufficient to lead to PML. In the case of lymphomas, the role of malignant lymphocytes in multiplication and mutation of JCV might be an interesting pathophysiological hypothesis.

Adult↗

[Acute leukaemia in two multiple sclerosis patients treated with mitoxantrone].

INTRODUCTION: Mitoxantrone (Mx) is used as a second-line treatment in multiple sclerosis. Since 1998, eight cases of acute leukemia (AL) have been described. We report two new cases of myeloid AL that occurred during treatment with Mx. OBSERVATIONS: The first case concerned a women who was treated with Mx for 3 months. In spite of a very low total dose (58.32 mg), she developed promyelocytic AL. The second patient died of myeloid AL, 27 months after the last injection of Mx. DISCUSSION: All the reported cases of AL occurring after Mx respond to the criteria of leukemia induced by anti-topoisomerases II. Epidemiological data and those from animal experiments suggest that Mx has direct role in the occurrence of leukemia. CONCLUSION: It must be remembered that even if the risk of Mx-induced leukemia is low, blood cell counts must be closely monitored for at least five years after the last injection of this treatment.

Acute Disease↗

Modafinil for fatigue in MS: a randomized placebo-controlled double-blind study.

OBJECTIVE: To assess whether modafinil, a wakefulness-promoting agent, is useful for fatigue in patients with multiple sclerosis (MS). METHODS: Patients with MS with stable disability, and a baseline score of 45 or more on the Modified Fatigue Impact Scale (MFIS), were eligible for the 5-week randomized, double-blind, placebo-controlled, parallel group study. The initial daily dose of modafinil was 200 mg for 1 week. Depending on tolerance, the dose was increased by 100 mg every week up to 400 mg/day and remained unchanged between day 21 and day 35. The primary outcome variable was the change of MFIS score at day 35. RESULTS: A total of 115 patients with MS were enrolled in the study and in the intention to treat analysis. The mean MFIS score at baseline was 63 +/- 9 in the placebo group and 63 +/- 10 in the modafinil group. MFIS scores improved between day 0 and day 35 in both placebo-treated and modafinil-treated groups, but no significant difference was detected between the two groups. There was no major safety concern. CONCLUSIONS: There was no improvement of fatigue in patients with multiple sclerosis treated with modafinil vs placebo according to the Modified Fatigue Impact Scale.

Adult↗

Impaired cardiovascular autonomic control in newly and long-term-treated patients with Parkinson's disease: involvement of L-dopa therapy.

In idiopathic Parkinson's disease (PD), autonomic dysfunction is frequent, causing orthostatic hypotension. The respective roles of disease progression and dopaminergic treatment remain unclear. In this study, we investigated the autonomic control of cardiovascular functions and its relation to L-dopa therapy in both newly diagnosed (ND) and long-term-treated (LT) patients. Study subjects were: (1) nine ND patients never having undergone treatment with L-dopa; (2) 18 LT patients who had been receiving L-dopa treatment for a long period. ND patients were investigated before L-dopa treatment and after stabilization of their L-dopa dosage. LT patients were investigated once with their regular treatment and once after a 12-h interruption of L-dopa treatment; (3) nine healthy subjects served as controls. At each test session, blood pressure (BP), heart rate (HR), plasma catecholamines, heart rate variability (HRV), and spontaneous baroreflex sensitivity were assessed in the supine and upright positions. Before receiving L-dopa medication, ND patients had reduced E/I ratios (HR response/deep breathing) and lowered HRV when compared to controls; this was evidence of early effects of the disease on autonomic HR control. Introduction of L-dopa treatment reduced BP, HR, and plasma levels of adrenaline and noradrenaline. Similar changes were found in LT patients when contrasting the short-term treatment interruption and the usual L-dopa dosage. The treatment-linked increase in plasma dopamine also correlated with the decrease in noradrenaline. These results showed that mild impairment of autonomic cardiovascular control occurred early in the course of PD. They also provided evidence that the side effects of L-dopa aggravated the impairment of the autonomic control of BP and HR.

Aged↗

Acute aphasia in multiple sclerosis: A multicenter study of 22 patients.

Aphasia is usually considered to be rare in multiple sclerosis (MS). To determine the clinical and radiologic characteristics of MS patients with acute aphasia, the authors investigated data from 2,700 patients from three MS centers and found 22 patients with acute aphasia (0.81%). Aphasia was the first clinical manifestation of MS in eight patients (36%). Brain MRI showed giant plaques in eight cases (40%). A full recovery was observed in 14 patients (64%). Furthermore, acute aphasia did not appear to be a criterion for poor prognosis.

Acute Disease↗

[Isolated pontine infarction: vascular anatomy helps understand clinical signs].

Clinical findings of pontine infarcts are more frequently described as classical crossed-syndrome. The aim of this study was to analyse clinical presentation of isolated pontine infarcts. From the Besancon Stroke Registry, we retrospectively studied 34 patients with isolated pontine infarcts documented on MRI with a strict centro-bicommissural plane as the reference. MRI were analyzed using an arterial territories mapping of brainstem. Among our 34 pts, none developed a classical crossed-syndrome. A thorough knowledge of vascular anatomy of brainstem is necessary to understand clinical findings. Moreover, this knowledge allow to explain the rare occurrence of pontine crossed-syndrome.

Adult↗

[Acute polyradiculoneuritis during primary herpes simple virus infection].

The herpes virus family, particularly cytomegalovirus and Epstein-Barr virus, are often associated with acute polyradiculoneuritis (APRN). APRN following primary herpes simplex virus infection is much more uncommon, viral reactivation generally being involved. We report a patient who developed APRN following herpes simplex virus primary infection, probably HSV II.

Adolescent↗

Atypical MELAS syndrome associated with a new mitochondrial tRNA glutamine point mutation.

The authors studied a 47-year-old patient who presented with an association of deafness, acute cerebral stroke-like episode, leukoencephalopathy, and extensive basal ganglia calcifications. Late onset and neuroradiologic findings were atypical for MELAS syndrome (Mitochondrial Myopathy, Encephalopathy, Lactic Acidosis, and Strokelike episodes). A heteroplasmic G to A transition at nucleotide 4332 in the tRNA glutamine gene was identified in the patient's muscle mitochondrial DNA. The pathogenicity of the mutation was shown in single muscle fibers by the correlation between high mutation load and cytochrome c oxidase defect.

DNA, Mitochondrial↗

[Therapeutic indications targeting etiology of progressive multiple sclerosis].

The indication for a long-term treatment in secondary or primary progressive multiple sclerosis is raised in more than half of the patients with MS. The fact that the pathogenic mechanisms involved in these two entities are unknown makes it difficult to predict the clinical course. The still insufficient knowledge of the MRI parameters is another obstacle to overcome. Nevertheless, the results of a certain number of clinical trials have, despite the methodological inadequacies, provided some insight. Immunomodulators, such as interferon-beta, have shown some efficacy, particularly in patients with a still inflammatory form of secondary progressive MS. Massive short-term immunosuppression does not appear to affect the course of progression but prolonged immunosuppression appears to be able to retard progression, at least in certain patients. This points out the importance of starting treatment as early as possible, before the development of irreversible damage. A better analysis of results already published, and most importantly, of results currently under way, should be most helpful in terms of therapeutic management if not in terms of our understanding of the pathophysiological processes involved.

Clinical Trials as Topic↗

Unilateral pure thalamic asterixis: clinical, electromyographic, and topographic patterns.

Eleven patients (nine with infarctions and two with primary hematomas) with isolated thalamic lesions and contralateral asterixis were examined using a standard electromyographic and neuroimaging protocol. Asterixis was a short-duration phenomenon associated with a hemiataxia hypesthesia syndrome in all patients. Electromechanical synchronization was constant for the two silent period types. The anatomic data strongly suggest that ventral lateral or lateral posterior thalamus are concerned in the pathophysiology of thalamic asterixis.

Adult↗

Status epilepticus in stroke: report on a hospital-based stroke cohort.

OBJECTIVE: To evaluate occurrence rate, clinical data, and prognostic factors of status epilepticus (SE) after stroke. METHODS: From 1984 to 1994, 3,205 patients were admitted to the Department of Neurology at our institution with first-time strokes. A total of 159 of these patients had first-time poststroke seizures. Among these 159 patients, cases of SE were identified and evaluated. RESULTS: SE was recognized in 31 patients (19%). In 17 patients, SE was the first epileptic symptom (initial SE), and in 4 patients, stroke began with SE (S-SE). In the 14 remaining patients, SE occurred after one or more seizure(s). After a mean follow-up period of 47 months, neurologic deterioration occurred after SE in 15 patients. This deterioration was permanent in two patients. Fifteen patients died; in five patients, death was directly related to SE. Eight of the 17 patients with initial SE and all 14 patients with SE after one or more seizure(s) developed other seizures or SE. S-SE, however, was not a predictive factor for additional seizure(s). CONCLUSIONS: Status epilepticus is common among patients with poststroke seizures. Although the immediate prognosis of patients with status epilepticus is poor, status epilepticus as the presenting sign did not necessarily predict subsequent epilepsy.

Adolescent↗

Seizures and epilepsy following strokes: recurrence factors.

BACKGROUND AND PURPOSE: Though there have been many reports on poststroke seizures, there is still much we do not know about them. Using a large cohort of stroke patients we analyzed the characteristics of the seizure(s) and the rate and factors involved in seizure recurrence. METHODS: Out of the 3,205 patients admitted for a first-ever stroke to our department between 1984 and 1994, we retrospectively studied the data of all patients with a first-ever seizure and analyzed their evolution. Two types of seizure(s) were defined: 'early-onset' seizures (occurring within the 14 days following the stroke) and 'late-onset' ones (after the 14th day). RESULTS: 159 patients were included in the study, i.e. 4.96%. There were 116 ischemic strokes and 43 primary hematomas. Cortical involvement was found in 87% of the patients. Early-onset seizures occurred in 57 patients and late-onset ones in 102 patients, 76% of which were observed within 2 years. Follow-up was performed in 135 patients with a mean follow-up period of 47 months; 68 of them presented a seizure recurrence. A 2nd seizure occurred more often in the patients with late-onset seizures (p < 0.01); recurrence was either single (24 patients) or multiple (44 patients). Univariate analysis demonstrated 3 factors for multiple recurrences: hemorrhagic component, low Rankin scale after the initial seizure and occipital involvement. Multivariate analysis determined 2 factors: occipital involvement and late onset of the 1st seizure as a predictive model of multiple recurrences. CONCLUSIONS: This study confirms that poststroke seizures are frequent and must be divided into 2 types: early-onset (</=14 days) and late-onset seizures. It demonstrates that a significantly lower rate of patients with early-onset seizures develop another seizure, i.e. epilepsy, than do patients with late-onset seizures. Other factors are involved in recurrence suggesting that poststroke epilepsy probably occurs in a chronically injured brain. The problem of treatment remain unanswered.

Acute Disease↗

Primary intracerebral hemorrhages in the Besançon stroke registry. Initial clinical and CT findings, early course and 30-day outcome in 350 patients.

The purpose of this study was to estimate the risk factors, early course, outcome and neuroimaging patterns in primary intracerebral hemorrhages (PIH). Using the Besançon Stroke Registry, 350 patients with first PIH documented by computed tomography (CT) between 1987 and 1993 were included in the present study. Patients with hemorrhage secondary to traumatism, brain tumor, thrombolytic treatment, vascular malformation or with hemorrhagic infarction were excluded. All CTs were evaluated to define the location, extension and volume of bleeding (55% of CT were performed within the first 12 h). Causes of death were classified and the 30-day outcome survival was evaluated with a modified Rankin scale (40 patients underwent a noncodified surgical procedure and were excluded from the outcome evaluation). Locations were lobar (36.5%), lenticular (32%), thalamic (15.7%), cerebellar (8.8%), midbrain and pons (2%), intraventricular (2%), caudate (1%) and multiple (2%). Risk factors included hypertension (54.8%), alcohol (18%) anticoagulant treatment (8.8%) and none (31.2%). The largest mean volume was in putaminal (41.7 ml) and lobar (39.8 ml) locations. Among 191 patients admitted before the 12th hour of evolution, 51 (26.7%) experienced an early clinical worsening. In this group, the percentage of patients with anticoagulant treatment (19.6%) was significantly higher (p<0.0001). PIH enlargement was documented in 3 patients. Overall, the 30-day mortality rate was 24.2% with 48% of all deaths occurring in the first 3 days. Death and 30-day survival status were closely associated with PIH volume (p<0.0001). Our study provides information on the natural history of PIH and especially on initial evolution. PIH volume seems to be an interesting indicator for death and functional status at 30 days.

Adolescent↗

[Emergency neurology consultations in the university hospital setting: contribution of the neurologist to inpatient management].

While outpatient management for chronic neurological diseases is well-established, the impact of inpatient neurological examination in emergency room and university hospital remain largely underestimated. We prospectively studied the role of the neurologist in patient management, in a primary care university hospital. Over a period of 12 months, we prospectively recorded the demographics of patients requiring examination in the emergency room, the initial suspected neurological diagnosis of the emergency room, the final diagnosis of the neurology team, and the patients' outcomes. For each patient, the time between admission, the call and the neurological examination were recorded. Neurological examinations were performed in 2220 patients in whom 75.6 p.100 were performed in the emergency room. These latter patients corresponded to 14 p.100 of all patients admitted in the emergency room. Of examined patients, 52 p.100 were male and mean age was 56.9 +/- 21 years. The time between admission and examination was 32 min. (+/- 36 min), irrespective of the day of the week, and depended on the suspected diagnosis: shorter in stroke and status epilepticus (p<0.05), and longer in loss of consciousness and vertigo (p<0.01). Forty-four percent of the examinations took place in the evening and night. The reasons for examinations were: stroke (28.3 p.100), epilepsy (17.7 p.100), headaches (8.4 p.100), loss of consciousness (7.9 p.100), cognitive dysfunctions (4.1 p.100), neuropathies (4 p.100) and miscellaneous (8.1 p.100). Neurological examinations modified neurological diagnosis and treatment in more than 86 p.100 of the patients. Following neurological examination, 17.2 p.100 of the patients were able to go home, while the rest were admitted to the stroke unit (27.2 p.100), the general neurological unit (27.3 p.100) or in other departments (28.3 p.100), of which intensive care unit (5.3 p.100) or neurosurgery (5.9 p.100). Emergency neurologic examination improves neurological diagnosis and has a positive impact both on treatment and, more globally, in patient management.

Consultants↗