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Biomedical subjects

J Ghika

Publications and source records attributed to J Ghika.

72 records · Page 4Linked to original sources

Hyperkinetic motor behaviors contralateral to hemiplegia in acute stroke.

Motor behaviors on the 'good side' of the body, i.e. ipsilateral to the hemispheric lesion, have not been studied systematically. We assessed motor behaviors in 20 consecutive patients during the acute phase (first 1-2 weeks) of a hemispheric stroke with hemiparesis. The behaviors were essentially rotations of the head, neck, eyes and trunk, orofaciopharyngeal or limb stereotypes, compulsive manipulation of the surroundings, or passive mobilization of the paralyzed arm or leg. These behaviors were found only with large infarcts in the territory of the internal carotid artery, middle cerebral artery and/or the anterior cerebral artery (ACA). All but two ACA infarcts involved the internal capsule and basal ganglia. The severity of the motor deficit and the presence of aphasia, neglect, or sensory loss were significantly correlated with the motor behaviors. Although the understanding of these behaviors remains unclear, we suggest that they may represent the clinical expression of early plastic changes of brain maps and circuits after an acute lesion; this is probably an active process induced by disinhibition, in order to establish new compensatory pathways.

Acute Disease↗

The "jerky dystonic unsteady hand": a delayed motor syndrome in posterior thalamic infarctions.

We report the cases of three patients with a thalamic infarct in the territory of the posterior choroidal artery involving the posterior thalamic nuclei. These patients developed delayed complex hyperkinetic motor syndromes, associating ataxia, tremor, dystonia, myoclonus and chorea, which we call "the jerky dystonic unsteady hand". One patient had a severe myoclonic and ataxic-dystonic choreoathetosis; another showed a so-called rubral tremor (myoclonic ataxia with resting, action, and wing-beating tremor) with dystonia; and the third one had a dystonic and ataxic hand with intermittent mild action myoclonus. All of them had sensory dysfunction; two had also presented with a painful Dejérine-Roussy syndrome. All had CT or MRI-proven infarcts in the territory of the posterior cerebral artery involving the posterior choroidal territory with an abnormal signal in the posterior area of the thalamus (pulvinar nucleus) but sparing the other thalamic, subthalamic and midbrain structures. These delayed myoclonic complex hyperkinetic syndromes have not been reported before, and we did not observe them in other topographic forms of thalamic infarcts. They may thus represent a new entity of movement disorders due to lesions in the posterior thalamic nuclei, with specificity for posterior choroidal artery infarcts.

Adult↗

Acute reversible ataxo-myoclonic encephalopathy with flecainide therapy.

A 73 year-old patient with Wolff-Parkinson-White syndrome and paroxysmic supraventricular tachycardia developed an acute reversible encephalopathy within 15 days of initiation of flecainide. The clinical picture was characterized by visual hallucinations, agitation, anxiety, but no disorientation, and a severe cerebello-myoclonic syndrome with total inability to stand up and walk, which was fully reversible on discontinuing the medication.

Aged↗

[Tremors].

Tremor can present a diagnostic problem in family practise. Systematic analysis of this symptom allows rapid classification of the problem without use of rigid definitions. Unilateral or very asymmetric tremor that disappears at the onset of a movement is for example a typical sign for parkinsonism without analysis and differentiation between tremor at rest or position-dependent tremor. Also one may assume, that a symmetric tremor that aggravates during standing or movement in most cases is essential and not of cerebellar origin. Different forms of tremor and their therapies and main tremor types are presented graphically.

Adult↗

Sensory symptoms in cranial dystonia: a potential role in the etiology?

Cranial dystonia is normally considered as a pure movement disorder. Sensory symptoms have not received much attention, but we found ill-defined pain, discomfort, distortion of sensory modalities, 'phantom' kinetic or postural sensations in the orofacial areas subsequently involved by the dyskinesia in all of 11 consecutive patients, preceding by weeks or months the motor syndrome. Physicians were often mislead, initially making diagnoses such as trigeminal neuralgia, dental problems, sicca syndrome, chronic conjunctivitis, glossitis or stomatitis. The patients reported that the orofacial movements were at first willingly performed in order to decrease the discomfort which was felt in these facial areas before the movements finally escaped voluntary control and became socially disturbing. We suspect that the sensory symptoms, for which no objective substrate could be found, and which were always reported before and in the exact location of the subsequent dyskinesia, could be the earliest manifestation of an evolving process in cranial and perhaps other focal dystonias.

Adult↗

Portable system for quantifying motor abnormalities in Parkinson's disease.

In order to quantify motor disabilities in Parkinson's disease (PD), we designed a compact, portable, neurophysiological system based upon a personal computer to measure tremor, bradykinesia, and muscle tone. Tremor was detected by solid state accelerometers and translated into a digital signal. The system displayed the root mean square displacements and frequency distribution of the tremor in the horizontal and vertical planes, along with a reconstructed graphic image of the displacement. Bradykinesia was measured using a panel that detects release and depression of switches in response to auditory and visual signals; the system calculated subjects' reaction times and movement times in milliseconds. Tone at the elbow was measured by strapping the upper extremity to a lightweight low-friction cradle and then passively moving the cradle with an instrumented handle. Signals representing torque and arm angle were processed by the computer and displayed in real time on the screen with stiffness as a mean slope in Nm/degree. In clinical tests, quantitative measures of tremor, movement time and rigidity were significantly abnormal in PD patients compared to control subjects. We conclude that this system is a convenient and accurate method to quantitate important aspects of the parkinsonian syndrome, and may be applied to quantitate other movement disorders.

Adult↗

Electrophysiologic detection of extrapyramidal motor signs in Alzheimer's disease.

We applied quantitative methods to measure extrapyramidal signs in 50 Alzheimer's disease (AD) patients and 40 age-matched control subjects. We measured tremor using accelerometers, bradykinesia using computer-detected reaction times (RTs) and movement times (MTs), and rigidity using a strain gauge linked to a movable arm rest. We excluded subjects with a clinical diagnosis of Parkinson's disease and subjects who required antiparkinsonian, neuroleptic, or anxiolytic medications. Aside from rigidity in two patients, there were no extrapyramidal signs on clinical examination. Based on electrophysiologic measures, however, there was a significant increase in muscle tone (p < 0.001), RT (p < 0.01), and MT (p < 0.03) in AD patients as a group compared with control subjects. Within the AD group, muscle tone and MTs increased across clinical stages of dementia severity (p < 0.05). Tremor frequency and amplitude were normal in AD subjects. These data indicate that quantitative neurophysiologic measures are superior to conventional clinical examinations in detecting extrapyramidal signs in AD. The pathologic substrates of extrapyramidal signs in AD are uncertain but seem to be linked to the degenerative AD process.

Aged↗

L-dopa resistant parkinsonism in an adult woman with a cyst in the posterior fossa.

We report a case of a woman with L-Dopa resistant asymmetrical parkinsonism with a posterior fossa cyst compressing the lower brainstem on MR. She did not show improvement in any of her symptoms after cysto-cardiac derivation. It was not possible to delineate if this was a case of a new malformative syndrome or the coincidence of two different disorders.

Adult↗

[Current aspects of extrapyramidal disorders].

Within the past decade, the harmonious anatomic aspect of the basal ganglia has changed for inhomogeneous structures filled up with a wealth of neurotransmitters and receptor subtypes with massive vertical and horizontal interconnections, asking for new mathematical concepts like parallel distributed processing of computation to approach their respective functions. Moreover, not only the motor control, but also some important aspects of cognition, eye motions, and limbic functions seem to be processed in the basal ganglia. Their overall threshold of activity seems to be under the bipolar control of the substantia nigra and the subthalamic nucleus; this results in hyper or hypokinetic syndromes. Few practical or therapeutical results have come out from the last radiological technologies and biochemical studies of extrapyramidal disorders, but the wealth of information brings hope for new issues from the knowledge in molecular biology, neuropharmacology, and from earlier diagnosis of these diseases in the future.

Basal Ganglia↗

Idazoxan treatment in progressive supranuclear palsy.

To confirm the preliminary report that increases in norepinephrine neurotransmission improve motor performance, we administered the investigational drug idazoxan (IDA) to nine patients with progressive supranuclear palsy (PSP) according to a double-blind crossover protocol. There were seven women and two men, whose mean age was 70 years and mean duration of illness 4 years. All had an advanced parkinsonian syndrome, supranuclear ocular motor palsies, and poor responses to dopaminergic drugs. During administration of 40 mg tid of IDA, the total score and the motor subscale score of the United Parkinson's Disease Rating Scale significantly decreased. Features that improved most included mobility, balance, gait, and measures of digital dexterity. There were no significant changes in any measure during placebo administration. Corticobulbar manifestations and eye movements were not significantly improved during treatment. Side effects of IDA included transient hypertension, tachycardia, action tremor, flushing, and sweating, but none was so severe that any patient withdrew from the study. Among the few attempted treatments of PSP, IDA is the first medication shown in a double-blind study to improve aspects of motor function.

Adrenergic alpha-Antagonists↗

Infarcts in the territory of lenticulostriate branches from the middle cerebral artery. Etiological factors and clinical features in 65 cases.

We studied 65 consecutive patients with a first stroke who had an appropriate CT-proven small infarct in the territory of the lateral (61 patients), medial (3 patients) or both lateral and medial lenticulostriate arteries (1 patient) from the middle cerebral artery. While more than 75% of these patients were either hypertensive or diabetic (having at least one cause for small-artery disease), embolic sources were encountered in 35%, either from large vessels (28%), and/or from the heart (15%). Other causes (angiitis, migraine) were found in only 9%. The neurologic deficit was purely motor in more than 50% of the patients (in half of them with neuropsychological dysfunctions), a sensori-motor deficit was present in 30% (in half of them with neuropsychological dysfunctions), and only 20% had ataxic hemiparesis. No one had pure sensory stroke. None of the classical lacunar syndrome or the modality of sensory, motor or ataxic deficits were specific for any topographic subdivision of LS territory, but there was a tendency for clinical features to be linked with the involved basal ganglia and the topography of pathways in the internal capsule as delineated by anatomical studies. Pure motor deficits were associated with infarcts in the medial and posterior part of LS territory, visual field deficits and hemineglect always corresponded to posteriorly situated infarcts. Neuropsychological deficits were common in infarcts in the anterior and posterior subdivisions of LS territory, with a major effect of the size of infarct. Sensory deficits were not correlated with any location in LS territory, probably because thalamo-efferent fibres have a more diffuse course through the internal capsule.

Aged↗

Infarcts in the territory of the deep perforators from the carotid system.

We studied risk factors and presumed causes of infarct in 100 consecutive patients with a first stroke, who had an appropriate CT-proven infarct in the territory of the deep perforators from the carotid system (ITDPCS). The infarct involved the territory of the lenticulostriate arteries in 65 cases, the anterior choroidal artery in 23 cases, watershed zones between these two territories in four cases, and another territory in eight cases. In 42% of the patients, we felt the cause of the infarct to be small-artery disease. In 36%, at least one source of embolism was present (in 27% from the internal carotid artery, in 17% from the heart), either with (25%) or without (10%) associated hypertension (HT) and diabetes mellitus (DM). Other possible less common etiologies included migraine, syphilitic angiitis, and systemic diseases. We have confirmed that HT or DM are the most common etiologic factors of ITDPCS. However, large-artery disease and cardioembolism may be more important than previously assumed.

Adult↗

[Inability to voluntarily close the eyes. Discussion of supranuclear disorders in palpebral closure based on 2 cases, with a review of the literature].

We studied 2 cases of supranuclear palsy of eye closure in patients who were unable to close their lids under command, but still conserved the possibility of closing them by syncinetic or reflex stimulations as well as during the sleep. The first patient had bilateral infarcts in sylvian watershed zones of fronto-parietal lobe and the second patient had a right cortico-subcortical superficial sylvian infarct. None of the 2 cases had an evolution towards motor impersistence of lid closure. We made a review of the literature and exposed the most debated hypothesis about this phenomenon, i.e. apraxia of lid closure or liberation of "grasping" phenomenon on the face. We expose the possibility of a disconnexion syndrome between motor supplementary area of the right hemisphere, supposed to be dominant for lid closure, and the premotor cortex area, based on reconstructions from CT Scan.

Aged↗

Painful ataxic hemiparesis.

Right hemiparesis with right-sided pain and ataxia developed in a 68-year-old man. Sensation, neuropsychological function, and somatosensory evoked potentials were normal. Computed tomography showed an isolated fresh infarct in the left part of the thalamus. The pain and ataxic disturbances were related to involvement of the thalamus itself, but the hemiparesis with hyperactive tendon reflexes and Babinski's sign was probably due to associated dysfunction in the adjacent internal capsule from compression or edema. In the available clinicopathological reports of cases with hemiparesis and thalamic infarction, contiguous involvement of the internal capsule or no associated lesion has been reported. Because of the occurrence of pain, which is not present in pontine, mesencephalic, or capsular ataxic hemiparesis, we suggest that the syndrome seen in our patient be called "painful ataxic hemiparesis."

Ataxia↗

Internuclear ophthalmoplegia, prenuclear paresis of contralateral superior rectus, and bilateral ptosis.

A patient with infarction of the paramedian part of the lower midbrain on the right side is described, in whom internuclear ophthalmoplegia and bilateral ptosis were associated with limitation of elevation of the contralateral eye from paresis of the superior rectus. Supranuclear paresis was suggested by partial dissociation between phasic and tonic components of upward gaze. It is suggested that the right-sided lesion involved the rostral median longitudinal fasciculus before it reached the oculomotor nucleus, the central caudal nucleus medially situated in the lower end of the oculomotor nucleus, and the prenuclear fibres from the posterior commissure to the ipsilateral nucleus of the contralateral superior rectus. A supranuclear lesion may be suspected when there is a complex association of dysconjugate palsy of oculomotor muscles.

Blepharoptosis↗

Vascular dementia: the role of cerebral infarcts.

Although vascular dementia (VaD) is the second most frequent cause of dementia after Alzheimer disease (AD), the concept remains controversial in terms of delineation. The objective of this review is to investigate, from available literature, the role of cerebral infarcts in the pathogenesis of VaD and to identify areas of interest that need further evaluation and research. The incidence of new onset dementia is increased after stroke. Stroke subtypes, total volume of cerebral infarction and functional tissue loss, and location of the lesions are probably the major determinants of VaD. Any cause of stroke can lead to VaD. In some circumstances the causal relation between stroke and dementia is clear: (1) in young patients who are unlikely to have associated Alzheimer pathology; (2) when the cognitive functioning was normal before stroke, impaired immediately after, and does not worsen over time; (3) when the lesions are located in strategic areas; and (4) when a well-defined vasculopathy known to cause dementia is proven. However, several issues remain unsolved in VaD: lack of specificity of the diagnostic criteria; influence of white matter changes and associated Alzheimer pathology; influence of preexisting cognitive status; possibility of having VaD without stroke and the clinical relevance of silent infarcts to VaD; and best therapeutic strategy to be used to prevent VaD and to prevent stroke in patients with VaD. These questions form the basis for proposals for future research.

Cerebral Infarction↗

Effects of proximal and distal muscles' groups contraction and mental stress on the amplitude and frequency of physiological finger tremor. An accelerometric study.

Physiological finger tremor was assessed by two-dimensional solid accelerometry in 40 healthy normal subjects at rest (R) with the hand hanging over the armrest of a chair, in posture (P) with the arm rested on the armrest but the hand extended from the wrist, and finally adding proximal muscles contraction in extension (E) with the arm extended in front of the patient, each time with and without mental stress. The mean amplitude for physiological tremor, about 30 microns, was almost doubled by hand extension and increased by 4 to 5-fold by arm extension with further increase by mental stress in each position, which gives a good estimation of the contribution of proximal and distal muscles into the amplitude of physiological tremor. There was no significant effect of age between 20 and 60 years on tremor amplitude, but mean tremor frequency decreased significantly between 40 to 60 years. Mental stress increased amplitude but decreased tremor frequency of both across all position, possibly by increasing the synchronization of motor unit firing and by modifying the gain of the motoneurones and the stretch reflex as shown by electrophysiological studies.

Adult↗