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Biomedical subjects

J Artieda

Publications and source records attributed to J Artieda.

At least 19 recordsLinked to original sources

Reflex myoclonus in olivopontocerebellar atrophy.

The presence of reflex myoclonus in response to touching and pin-pricking the wrist or stretching the fingers and to photic stimulation was assessed in 24 patients with a presumed diagnosis of olivopontocerebellar atrophy (OPCA) and in 30 age matched control subjects. Reflex myoclonus to soma-esthetic stimulation was found in 23 patients and in none of the controls. Photic myoclonus was present in 12 patients and in none of the controls. Electrophysiological study of the reflex myoclonus showed enhanced (> 10 microV) somatosensory evoked potentials and an associated reflex electromyographic discharge (C-wave) in 15 patients. These findings indicate that reflex myoclonus is common in OPCA and probably of cortical origin.

Atrophy

[Olivopontocerebellar atrophy and Parkinson's disease: diagnostic problems].

Clinical diagnoses of Parkinson's disease (PD) is highly inaccurate. Olivopontocerebellar atrophy (OPCA) is a major source of diagnostic confusion. We have studied the clinical characteristics of 50 patients with PD, 24 patients with OPCA judged by the presence of selective atrophy of the cerebellum and brainstem (diagnosed by CT brain scan) and 30 normal controls of similar age. The typical triad of PD, tremor, rigidity and akinesia, did not distinguish among patients from either group. The presence of severe postural imbalance and reflex myoclonus (in OPCA but not in PD) were the 2 most highly discriminative clinical features.

Aged

The pathophysiology and pharmacology of photic cortical reflex myoclonus.

We studied 6 patients with myoclonus elicited by flash stimulation (1-15 Hz). Multichannel electromyographic recording showed a rostrocaudal recruitment order for the generalized myoclonic jerk. In all patients, each flash induced a large (36.9 +/- 5.7 microV) frontal biphasic wave with an onset latency of 42 msec that preceded the earliest muscle response in the face by at least 4 msec (range, 4-7 msec), the activity in the biceps by 11 to 14 msec, and the activity in tibialis anterior by 26 to 34 msec. Occipital potentials evoked by the same flash stimulation had a latency of 33.7 msec and were of normal amplitude (2.1 +/- 1.2 microV). Brain-mapping analysis indicated that the frontal activity correlated with the myoclonus originated in the premotor and motor cortices. These findings provide evidence for a cortical origin of this form of stimulus-sensitive myoclonus in humans. Administration of apomorphine and lisuride (intravenously) and levodopa-carbidopa (orally) abolished the photic myoclonus. Intravenous 5-hydroxytryptophan plus carbidopa (orally) and piracetam (orally) were also effective against photic myoclonus. The wide range of drugs active against photic cortical myoclonus suggests the participation of several biochemical mechanisms in its origin.

5-Hydroxytryptophan

Cortical mechanisms mediating asterixis.

We describe a patient with chronic renal failure who suffered multifocal action-induced jerks. Electromyography (EMG) recorded the typical silence of asterixis. Back-averaging the EEG activity preceding the EMG silent periods in the forearm showed a biphasic wave antedating the asterixis by 23 ms. Somatosensory evoked potentials (SEPs) after median nerve stimulation were pathologically enlarged on both hemispheres. Brain-mapping of the biphasic wave preceding asterixis and the giant SEPs indicated a common origin in the sensorimotor cortex. This observation provides further documentation of a cortical origin for some types of asterixis in humans.

Brain Mapping

Temporal discrimination is abnormal in Parkinson's disease.

Temporal discrimination thresholds (TDT) for recognition of paired sensory (tactile, auditory and visual) stimuli given over a wide range of time intervals were assessed in 44 patients with Parkinson's disease (PD) and 20 age-matched normal subjects. A significant increment in TDT for all three sensory modalities was found in PD patients compared with controls. This abnormality was greatly attenuated for about 2 h by a single levodopa/carbidopa (250/25 mg) tablet. A significant correlation was found between disease severity as assessed clinically and TDT. Patients with more severe PD had higher TDT values. The study of the peripheral median nerve and cortical somatosensory evoked potential recovery curves following double electrical stimulation of the index finger showed no differences between patients and control subjects, nor changes from 'off' to 'on' motor state which could explain the findings. These results indicate the existence of an abnormality of timing mechanisms in PD.

Action Potentials

Time estimation and reproduction is abnormal in Parkinson's disease.

We compared the performance of 44 patients with Parkinson's disease (PD), tested 12-24 h after withdrawal of dopaminergic medication, with 20 age-matched controls, on a verbal test for time estimation and in several time reproduction tasks. Patients with PD underestimated the duration of a time interval in the verbal time estimation task and showed overproduction of time intervals when required to reproduce a short time sample. Absolute errors were greater in the reproduction of longer time intervals in both control and PD patients, but especially in the latter. The presentation of time markers at faster rates had a detrimental effect on the performance of the patients but not of the controls. Patients with more severe PD performed worse on the time estimation and reproduction tasks compared with those with milder disease. Administration of levodopa-carbidopa (205/25 mg, p.o.) significantly reduced absolute errors in time estimation and reproduction in conditions where time markers were presented at the two faster rates of 5 Hz and 3.3 Hz. Performance in these two latter tests best discriminated patients and controls and had a positive significant association with simple reaction time and movement time. These results lead us to suggest that time estimation, i.e. the 'internal clock', is abnormally slow in PD.

Adult

Performance of repetitive wrist movements in Parkinson's disease.

The timing of repetitive alternating 80 degrees flexion-extension movements of the right wrist was studied in 42 patients with Parkinson's disease, tested while not receiving dompaminergic medication and 20 age- and sex-matched controls. Five rates of movement (0.5 Hz, 1 Hz, 1.5 Hz, 2 Hz, 2.5 Hz) were examined. The interval between two successive flexion movements, as measured from the electromyography (EMG) records was taken as the unit of analysis or inter-response interval (IRI). At 0.5 Hz, 1 Hz and 1.5 Hz there were no differences between groups in mean IRIs. At higher rates of movement (2 Hz and 2.5 Hz), however, the controls were significantly more accurate in timing of repetitive movements than the patients. At all five frequencies, the patients with moderate or severe Parkinson's disease were less accurate in timing of repetitive movements than those with mild disease, although the differences were not significant. In nine patients tested in the 'on' and 'off' medication states, administration of 250 mg of levodopa/carbidopa resulted in significantly more accurate timing of repetitive movements. Wing and Kristofferson's (1973b) two-process model of repetitive movements was applied to the IRI data. At various rates of movement, the primary prediction of the model that lag 1 autocorrelations should be in the 0 to -0.5 range was violated in 40-70% of the patient and controls. For those subjects who had lag 1 autocorrelations in the expected range, IRI variability was to break down into a timekeeper and a motor delay variance. At all frequencies, the patients had significantly higher variance for IRI, timekeeper and motor delay than the controls. Although not significant, more severe Parkinson's disease was associated with greater IRI, timekeeper and motor delay variance, while administration of levodopa resulted in reduction of the three types of variance. The validity of the Wing and Kristofferson model for the analysis of this type of movement is discussed.

Acoustic Stimulation

Neurologic complications after sublaminar wiring. An experimental study in lambs.

To study possible neurologic complications, five lambs were operated on by sublaminar wiring at the thoracolumbar spine. Monitoring of the central motor pathway was carried out by percutaneous electrostimulation. A comparative anatomic study was designed to compare the magnitude of the spinal canal and the area occupied by the spinal cord at the low thoracic and lumbar level in 15 lamb, 8 pig, and 8 human spines. The following parameters were measured by slide caliper: the anteroposterior diameter of the spinal canal and the spinal cord, the length and thickness of the laminas, and the distance between each consecutive lamina. All five operated lambs showed major neurologic deficits after surgery. The results of the anatomic study suggest that there exists in humans a sufficiently ample "safety zone" that permits wire insertion without risking injury to neurologic structures. Such a "safety zone" is nonexistent in lambs and pigs unless an extensive laminectomy is performed to decrease the depth of wire penetration.

Animals

Abnormal muscle and skin mitochondria in family with myoclonus, ataxia, and deafness (May and White syndrome).

A mother and two of her daughters had deafness and cortical reflex myoclonus; the mother also had mild truncal ataxia. Muscle and skin biopsy specimens revealed abundant ragged-red fibres and abnormal mitochondria. The son of one of the daughters had sensorineural deafness. Three other grandchildren were asymptomatic. The two daughters also had diabetes mellitus, hypertension and cardiomyopathy. Another daughter died of renal failure. The mother lost her hearing in her 70s, one daughter in her 30s, and the other daughter and the grandson in their 20s. The mother has had transient episodes (24-48 hours) of temporal disorientation, severe action myoclonus, and ataxia for about eight years. This is the first reported family with inherited deafness, myoclonus, and ataxia with mitochondrial pathology.

Adolescent

Hereditary paroxysmal ataxia with neuromyotonia.

The clinical manifestations of a patient with hereditary paroxysmal ataxia and neuromyotonia are described. Generalized tremor, triggered by sudden movements, and spasms of hand and foot muscles were the main clinical findings. Electromyogram (EMG) and nerve blocking studies led to the diagnosis of neuromyotonia. Treatment with acetozolamide was of no therapeutic value, confirming previous observations about the difference in response of paroxysmal ataxia with and without neuromyotonia.

Adult

Cortical activity preceding self-initiated and externally triggered voluntary movement.

The cortical electromyogram (EMG) activity, preceding voluntary movements, was recorded in 12 normal subjects in two different situations: first, when movements were self-induced by the subjects by their own will; and second, in response to threshold electrical stimulation of the index finger, a brief flash of a light-emitting diode (LED), and a click. Four types of movements were studied: (a) fast extension of the right wrist, (b) fast supination of the left wrist, (c) either movement depending on the subject's own decision or on which index finger was stimulated, and (d) fast sequential right and left wrist extension. In all subjects, self-initiated movements were preceded by a typical Bereitschaftspotential (BP) starting 1,290 +/- 208 ms before the EMG discharge. When the same movements were triggered by an external clue, there was no BP. The BP was present, although with a shorter duration, when subjects were asked to wait for a brief period after index finger stimulation, before extending the right wrist. From these results, we conclude that the BP is closely associated with the timing of internally generated movements, and that different cortical areas are probably involved in the generation of self-induced and externally referenced movements in humans.

Adult

The anatomical basis of somaesthetic temporal discrimination in humans.

Somaesthetic temporal discrimination (STD) is the ability to perceive as separate two successive somaesthetic stimuli applied to the same or different parts of the body. Paired electrical stimuli were applied to the index finger, using different time-intervals, to study the STD threshold (STDT) in 84 normal subjects and 51 patients with focal cerebral lesions. Abnormal STDT values were found on the affected side of patients with a lesion of the primary somatosensory cortex, and internal capsulethalamus. Lesions which did not produce sensory impairment but caused abnormal STDT were located in the posterior parietal cortex, the head of the caudate nucleus, the putamen, the medial thalamus and the lenticular nucleus. Frontal, temporal and occipital cortex lesions did not produce any abnormality in the STDT, but one patient with a bilateral lesion of the supplementary motor area (SMA) had abnormal STDT. These results indicate that normal perception of two somaesthetic stimuli as separate in time depends not only upon the integrity of the somatosensory pathway and primary somaesthetic cortex, but also of the posterior parietal cortex, SMA and subcortical structures such as the striatum and thalamus.

Adolescent

Reciprocal inhibition between forearm muscles in spastic hemiplegia.

We studied reciprocal inhibition of H-reflexes in the forearm flexor and extensor muscles in 14 patients with spastic hemiplegia secondary to a focal cerebral lesion and 14 normal volunteers. In the spastic limb, the Hmax/Mmax ratio was increased in both flexor and particularly extensor wrist muscles. The 3 normal inhibitory phases of reciprocal inhibition between extensor and flexor forearm muscles were markedly reduced on the spastic side of patients. The early disynaptic phase showed the greatest alteration. Reduced or absent inhibition between forearm muscles associated with increased spinal motoneuron excitability may be typical to spastic hemiplegia.

Brain Diseases

The blink reflex in patients with idiopathic torsion dystonia.

The blink reflex and its recovery cycle were examined in 57 patients with idiopathic dystonia affecting different parts of the body. The group comprised 9 patients with generalized and 15 with segmental forms, 19 with torticollis, and 14 with focal arm dystonia. None had blepharospasm. The duration and amplitude of the R2 component of the blink reflex showed only minor changes. However, its recovery cycle to paired supraorbital nerve stimuli was abnormal in all groups of patients, except those with focal arm dystonia. These findings may be interpreted as showing abnormal control of the interneuronal networks mediating the blink reflex in patients with dystonia affecting sites other than the facial muscles. The fact that the principal changes were seen in patients with torticollis, and generalized or segmental dystonia, suggests that the extent of dystonia (rather than the severity) and, therefore, the close proximity to the cranial muscles was important in determining the extent of the abnormal interneuron function.

Arm

Generalized reflex myoclonus in a patient with alcohol-sensitive spontaneous myoclonus and an abnormal gait.

A patient with alcohol-sensitive spontaneous, action- and stimulus-sensitive generalized reflex myoclonus is reported. Gait was abnormal but could not be clearly classified as dystonic. No other neurological abnormality was present. The possible relationship between alcohol-sensitive myoclonic dystonia and this case is discussed. Reflex myoclonus may serve as an additional clinical marker in the study of families with alcohol-sensitive myoclonus, dystonia, or both.

Adult

Severe polyneuropathy and motor loss after intrathecal thiotepa combination chemotherapy: description of two cases.

Two cases of severe delayed neurologic toxicity related to the administration of intrathecal (IT) combination chemotherapy including thiotepa (TSPA) are presented. Both cases developed axonal neuropathy with motor predominance in the lower extremities 1 and 6 months after IT chemotherapy was administered. Neurologic toxicities have been described with IT-methotrexate, IT-cytosine arabinoside and IT-TSPA. To our knowledge, however, axonal neuropathy following administration of these three agents has not been previously described. In spite of the fact that TSPA is a useful IT agent, its combination with MTX, ara-C and radiotherapy could cause severe neurotoxicity. This unexpected complication indicates the need for further toxicology research on IT-TSPA.

Adolescent

Clinical and CT scan findings in a case of cyanide intoxication.

A 39-year-old man showed a combination of severe parkinsonism and progressive dystonia following attempted suicide with sodium cyanide. Computed tomography (CT) scan showed bilateral lucencies in the putamen and external globus pallidus. The topography of lesions on CT scan closely correlated with the pathological findings described in a previous report of cyanide-induced parkinsonism. This is the first reported case of cyanide intoxication with delayed-onset dystonia.

Adult