Cefepime- and cefixime-induced encephalopathy in a patient with normal renal function.
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Biomedical subjects
Publications and source records attributed to R Leiguarda.
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Ideomotor apraxia is defined as a disturbance in timing, sequencing, and spatial organization of gestural movements. Left hemisphere motor dominance reflected by ideomotor apraxia mainly refers to spatially and temporally complex movements performed outside the natural context. While clinicoanatomical studies have failed to unveil a specific lesion correlating with apraxia, white matter damage-interrupting corticocortical and corticosubcortical connections-seems crucial for the deficit to be persistent and severe. Patients with basal ganglia lesions and disorders, such as Parkinson's disease and progressive supranuclear palsy, may exhibit ideomotor apraxia. The putative roles of the basal ganglia in object-oriented action, and therefore in praxis, would include among others (a) the selection of the kinematic parameters and the direction of arm movements, (b) working as an integral part of brain systems involved in timing and representation of action sequences, (c) encoding behavioral context, and (d) working as a subcortical component of the parietofrontal circuits devoted to sensorimotor transformation (e.g., reaching). Several studies suggest that basal ganglia pathology per se may not cause overt apraxia. However, when it is combined with dysfunction of the cortical components of the neural systems involved in sequencing, sensorimotor transformation, and response selection, different types of ideomotor praxis deficits would become clinically manifested.
A wide variety of CNS lesions have been associated with changes in heart rate (HR). However, in epileptic patients their value to lateralize seizure onset remains controversial. This study aims to assess if HR changes associated with partial onset seizures could be useful in lateralizing seizure onset. We analysed HR changes on 100 seizures from 38 consecutive patients (mean age: 27.5 years) admitted for video-EEG telemetry monitoring. We evaluated the R-R interval 30 seconds before the seizure onset and 10, 20 and 120 seconds after the onset. We assessed whether there was a correlation between HR changes and seizure type, left/right differences and different semiological components for each seizure. We recorded 100 seizures. Three non-lateralized seizures were excluded from the analysis; 63/97 (65%) had left hemisphere onset, mainly from the temporal lobe (57.7%). The mean baseline HR was 77 beats per minute Ictal tachycardia (HR: > or = 107.06 beats per minute) was detected in 32 seizures, with ictal onset from the mesial temporal lobe structures in 23/32; 16/32 occurred during the first 10 seconds and 16/32 during the next 20 seconds from the seizure onset independently of the site of origin. Among the different semiological components for each seizure, only dystonic posturing and automatism correlated with HR increments. We did not find bradycardia in our series. Ictal tachycardia occurs most frequently with seizures arising from the mesial temporal lobe and it may not reliably predict the lateralization of seizure onset.
OBJECTIVE: Posteroventral pallidotomy (PVP) has proved to be an effective method for the treatment of Parkinson's disease. However, data on bilateral procedures are still limited. To assess the effects of bilateral globus pallidus (GPi) lesion and to compare it with a combination of unilateral GPi lesion plus contralateral GPi stimulation (PVP+PVS), an open blind randomised trial was designed. METHODS: A prospective series of patients with severe Parkinson's disease refractory to medical treatment, and severe drug induced dyskinesias, were randomised either to simultaneous bilateral PVP or simultaneous PVP+PVS. All patients were assessed with the core assessment programme for intracerebral transplantation (CAPIT), and a comprehensive neuropsychological and neuropsychiatric battery both before surgery and 3 months later. RESULTS: The severe adverse effects found in the first three patients subjected to bilateral PVP led to discontinuation of the protocol. All three patients developed depression and apathy. Speech, salivation, and swallowing, as well as freezing, walking, and falling, dramatically worsened. By contrast, all three patients undergoing PVP+PVS had a significant motor improvement. CONCLUSION: Bilateral simultaneous lesions within the GPi may produce severe motor and psychiatric complications. On the other hand, a combination of PVP+ PVS significantly improves parkinsonian symptoms not associated with the side effects elicited by bilateral lesions.
Twenty-six patients with syringomyelia were studied with polysomnography to determine the frequency of periodic limb movements (PLM) and its relationship to the presence of a Chiari anomaly, the severity of corticospinal tract involvement, and localization of the syrinx. Sixteen patients showed PLM in stages I and II of non-REM sleep and three PLM also while awake. There were no statistically significant differences in overall disability, corticospinal signs, presence of an associated Chiari anomaly, and disease duration between patients with and without PLM, although there was a trend for patients with PLM to have more severe disease. There was preservation of the lumbosacral enlargement of the spinal cord by the syrinx in all patients with PLM. The latency delay between lower and upper limb muscles was suggestive of conduction along propriospinal pathways. Syringomyelia may lead to an abnormal state of spinal hyperexcitability favoring the appearance of PLM. Detailed magnetic resonance image studies of patients with different localizations of the syrinx cavities may help to determine which tracts are involved in the production of PLM.
Patients with basal ganglia diseases may exhibit ideomotor apraxia. To define the nature of the impairment of the action production system, we studied a repetitive gesture of slicing bread by three-dimensional computergraphic analysis in eight nondemented patients with Parkinson's disease in the "on" state, five with progressive supranuclear palsy and four with multiple system atrophy. Two patients with Parkinson's disease and two with progressive supranuclear palsy showed ideomotor apraxia for transitive movements on standard testing. A Selspott II system was used for kinematic analysis of wrist trajectories and angular motions of the shoulder and elbow joints. Patients with Parkinson's disease, progressive supranuclear palsy, and even some with multiple system atrophy exhibited kinematic deficits in the spatial precision of movement and velocity-curvature relationships; in addition, they failed to maintain proper angle/angle relationships and to apportion their relative joint amplitudes normally. Spatial disruption of wrist trajectories was more severe in patients with ideomotor apraxia. We posit that the basal ganglia are part of the parallel parieto-frontal circuits devoted to sensorimotor integration for object-oriented behavior. The severity and characteristics of spatial abnormalities of a transitive movement would therefore depend on the location and distribution of the pathologic process within these circuits.
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Advances in surgical procedures and new immunosuppressor therapies have improved the outcome of renal grafts. However, these changes have been accompanied by infectious, neoplastic and neurologic complications. The purpose of this study was to determine the incidence of neurologic complications among 542 patients receiving a renal transplant (from living or cadaveric donors) at CEMIC between 1970 and 1996. Neurologic complications occurred in 43 patients (8%) as follows: 8 meningitis (1.5%), 8 acute confusional syndrome (1.5%), 7 encephalitis (1.3%), 7 cerebrovascular accidents (1.3%), 6 convulsions (1.1%), 3 tumors (0.5%), 3 femoral nerve lesion (0.5%), and 1 epidural lipomatosis (0.1%). Etiologic agents most commonly observed in meningitis were: Cryptococcus neoformans, Listeria monocytogenes and Mycobacterium tuberculosis. Major difficulties arose in the diagnosis of encephalitis. Diagnosis of the above complications required clinical astuteness and repeated bacteriologic, serologic and imaging studies.
Sensory ataxic polyneuropathies are characterised by the presence of sensory ataxia due to damage to large myelinated sensory fibres, with total or relative preservation of muscle strength, pain and temperature sensation. Hereditary ataxic polyneuropathies are exceptional and very few families with this disorder have been reported so far. We here describe the neurological, electrophysiological and sural nerve biopsy data of four siblings with an ataxic chronic polyneuropathy, starting after age 50. They had an ataxic gait which worsened in darkness, horizontal nystagmus, hypo or areflexia, and severe impairment of limbs' propriocaption. Nerve conduction studies showed absent sensory nerve action potentials in all nerves tested. Somatosensory evoked potentials showed reduced amplitude and prolonged latencies. Sural nerve biopsy showed a severe loss of myelinated and unmyelinated fibres. Symptoms slowly progressed over the years. The recognition of this syndrome is important in the search for the etiology of chronic ataxic neuropathies.
OBJECTIVE: To determine the frequency and types of abnormalities of respiratory control during sleep in syringomyelia and syringobulbia and to provide a basis to predict patients at risk of sudden death. METHODS: Thirty patients (15 male and 15 female; mean age 39.0 +/- 12.6 years) with communicating syringomyelia were divided into two groups: those with evidence of syringobulbia (17 patients) and those without compromise of the medulla or syringomyelia (13 patients). Patients were studied with pulmonary function studies and polysomnography. Respiratory center sensitivity to CO2 (rebreathing technique) was measured in 9 patients. RESULTS: Severely affected patients had mild-to-moderate restriction and individual patients had bilateral diaphragmatic or vocal cord palsy, abnormal respiratory rhythm, prolonged inspiratory time, or an abnormal respiratory response to CO2. Very prolonged central, obstructive, and mixed sleep apneas with low O2 saturation values and a fixed heart rate were recorded in most patients with syringobulbia. Five patients developed severe respiratory complications and died during a follow-up period of 10 years. Respiratory abnormalities failed to correlate with syrinx size. CONCLUSIONS: Severe abnormalities in respiratory rhythm generation during sleep occur in patients with syringobulbia. The respiratory disturbances are not due to muscle weakness and they are not correlated with the size of the cavity. The combination of dysphagia and dysphonia in patients with longstanding syringomyelia and syringobulbia predicted likelihood of respiratory disturbances during sleep.
OBJECTIVE: To determine the effect of a single dose of apomorphine on internal globus pallidus (GPi) neuronal discharge in patients with Parkinson's disease (PD). PATIENTS AND METHODS: Nine PD patients who underwent microelectrode-guided posteroventral pallidotomy (PVP) were studied. After identification of a single GPi unit discharge with sufficient spike S/N ratio to allow reliable thresholding, basal recording was followed by a single 3-mg subcutaneous injection. One-minute samples were recorded 10', 30', and 60' after apomorphine. RESULTS: In four patients, recording was lost after 5-10 minutes. In two, changes were observed at peak-of-dose but recording was then lost, whereas three completed recording and returned to baseline, all five showing significant reduction in GPi firing rate (mean +/- standard deviation for basal and post-apomorphine were 143+/-55.6 and 52+/-19.2, respectively; p <0.002). CONCLUSION: In patients with PD, apomorphine induces changes in GPi spontaneous discharge and modifies firing rates resembling recordings in normal primates. These findings show that clinical improvement as well as induction of dyskinesias following DA administration could be mediated by reduction of GPi outflow.
Microelectrode-guided posteroventral pallidotomy (PVP) has shown to be an effective method in the treatment of a group of patients with advanced Parkinson's disease. A nonlesioning approach by means of deep brain electrodes connected to a programmable neuropacemaker has also been used to inhibit the internal segment of globus pallidus (posteroventral stimulation [PVS]) reporting comparable clinical efficacy to the one obtained with the ablative method. Nevertheless, no controlled studies have been performed to compare the efficacy of both procedures. A prospective series of 13 patients with a clinical indication for globus pallidus surgery was randomized either to a pallidotomy or stimulator implantation, and comparisons on motor and neuropsychologic measurements were made on a 3-month follow-up basis. Primary measurements of efficacy showed a comparable effect on Unified Parkinson's Disease Rating Scale and activities of daily living score after both procedures. Secondary measurements of efficacy showed that although both techniques improve hand tapping score and dyskinesia score, the bilateral improvement in the former was greater after PVS whereas the latter improved more significantly after PVP. No significant changes in neuropsychologic parameters were observed after either PVP or PVS. Side effects and surgery complications occurred in six of 13 patients (three after PVP and three after PVS): they were mild, transient, and unrelated to optic tract injury. In conclusion, the short-time effect and safety of both procedures is comparable.
We present a patient with clinically evident beginning-of-dose motor deterioration who had undergone posteroventral pallidotomy. This patient underwent an intrasurgical apomorphine test followed by single cell recording of the internal globus pallidus (GPi) to determine changes in GPi firing rate during the occurrence of such phenomenon. A significant increase in GPi firing rate coincident with worsening of patient disabilities prior to improvement was found. This finding suggests that beginning-of-dose motor deterioration may be mediated by enhanced thalamic inhibition.
UNLABELLED: Many reports published during the past 5 years have shown evidence of the beneficial effect of posteroventral pallidotomy (PVP) in large groups of patients for up to 3 years, but none of them have compared patients who underwent surgery with a control group. OBJECTIVE: To compare the evolution of Parkinson's disease symptoms at 1-year follow-up between patients who underwent surgery and those who did not. MATERIAL AND METHODS: Ten patients with idiopathic Parkinson's disease refractory to treatment who were included in the Core Assessment for Intracerebral Transplantation program for PVP did not undergo surgery because financial support was lacking. These patients were followed up for 1 year as if they had been operated on and were finally compared with 10 patients having similar characteristics in whom PVP had been performed during the same period of time. RESULTS: There were no significant differences at basal evaluation in the motor section scores of the Unified Parkinson's Disease Rating Scale between those patients who underwent surgery and those who did not, but a significant reduction in Unified Parkinson's Disease Rating Scale motor score in the group who underwent surgery at 1-year evaluation was found (P < 0.006). Dyskinesias, which was nonsignificantly different at basal evaluation, showed, at the 1-year follow-up, a significant reduction in the group who underwent surgery (P < 0.04). Scores from the subsets of the Unified Parkinson's Disease Rating Scale addressing rigidity, tremor, and bradykinesia also proved significantly different at the 1-year follow-up. The slope of the line generated by the two evaluations for each group showed a negative value in the group who underwent surgery (value of -0.21) and a positive value in the group who did not (value of 0.148). CONCLUSION: At the 1-year follow-up, microelectrode-guided PVP produced significant changes in patient motor status and disease progression versus a comparable group of patients who did not undergo surgery during the same period of time.
OBJECTIVE: To examine the differential impairment of implicit and explicit memory systems in cortical and subcortical dementias. BACKGROUND: Whereas verbal priming was reported to be impaired in patients with Alzheimer Disease (AD), patients with Parkinson Disease (PD) may be relatively more impaired on tasks of motor skill learning. METHODS: We examined 15 patients with Alzheimer disease, 10 patients with Parkinson disease and dementia (PD-D), 15 patients with PD but no dementia, and 24 age-comparable normal control subjects with a neuropsychologic battery that included tests of explicit memory (Buschke Selective Reminding Test, Benton Visual Retention Test, Digits Span), and tests of implicit memory (Word-Stem Completion task and the Maze Test). RESULTS: AD and PD-D groups showed similar deficits on all measures of explicit memory, and performed significantly worse than PD patients without dementia and normal control subjects. On the other hand, there were no significant between-group differences in any of the measures of implicit memory. CONCLUSIONS: Our study demonstrated preserved implicit learning in the context of severe explicit learning deficits in patients with dementia, but could not demonstrate a different profile of memory deficits between so-called cortical and subcortical dementias.
We assessed a consecutive series of 398 patients with probable Alzheimer's disease (AD) for the presence of Generalized Anxiety Disorder (GAD) using a standardized neuropsychiatric evaluation. Five percent of patients showed GAD during the 4 weeks preceding the psychiatric evaluation. AD patients with GAD showed significantly higher scores of depression, irritability, overt aggression, mania, and pathological crying than AD patients without GAD. The most severe symptoms of anxiety were those of tension, fears, insomnia, and physical complaints.