[Double athetosis with marble state in a brother and sister of the Van Hoe family, with a note on the development of our knowledge concerning double athetosis].
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Athetosis↗
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[Clinical study of two cases of involuntary withdrawing movement in one leg indicating athetosis and localized choreo-athetosis].
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Athetosis↗
[Effect of music on the rehabilitation of children with choreo-athetosis. I. Effect of rhythmic stimuli on the frequency of involuntary movements in choreo-athetosis syndrome].
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Athetosis↗
[Athetosis].
Athetosis is a peculiar involuntary movement resulting from pathologic involvement of the basal ganglia. Although mechanism of this movement is still far from established, athetosis is clinically differentiated from chorea and dystonia. The purpose of this article is to review and summarise the classification of this involuntary movement disorder. This movement disorder is classified into double athetosis, chorea-athetosis, unilateral athetosis and pseudo-athetosis. The double athetosis is featured by increased muscle tonus and irregular small amplitude movement, which appears the most frequently in patients of cerebral palsy. In chorea-athetosis, irregular abnormal movement is more prominent and larger than double athetosis. This type of movement appears commonly in patients other than cerebral palsy. Unilateral and pseudo-athetosis are derived not from disturbance of the basal ganglia but from impared sensory pathways of the deep sensation due to cerebro-vascular lesion. Stereotactic VL-thalamotomy is effective to relieve increased muscle tonus but not to decrease involuntary movement.
Athetosis↗
[Athetosis or dystonia?].
The term athetosis has progressively disappeared from the anglo-saxon literature which considers that athetosis is part of the spectrum of dystonia. These two clinical entities can be distinguished, however. Athetosis can be identified, searching for subtle semiological traits, in particular at the level of the hand. The earlier appearance of athetosis may be result from its onset during the early phases of development of the central nervous system. Despite its rarity, the clinical diagnosis of athetosis is important to consider from a prognostic point of view. Indeed, it results from brain lesions, and is therefore not a hereditary disorder as it may be the case for dystonia, and its evolution is relatively stable. The efficacy of treatments used in patients with dystonia, in particular high frequency pallidal stimulation, remains to be assessed in patients with athetosis. The concept of athetosis is still helpful in clinical practice.
Athetosis↗
[Chorea-athetosis in the anti-Hu syndrome].
INTRODUCTION: Paraneoplastic choreo-athetoses are rare. We report a case of anti-Hu syndrome with choreo-athetosis. CASE REPORT: A 48-year-old woman developed a small-cell lung carcinoma revealed by an anti-Hu syndrome. The neurological features included choreo-athetosis predominating in the upper limbs, chronic sensorimotor axonal polyneuropathy, and opsoclonus. The cerebrospinal fluid was acellular and contained several oligoclonal IgG bands, not found in the corresponding serum. Magnetic resonance imaging revealed bilateral high-intensity lesions on T2/FLAIR sequence in the corona radiata. Moderate transitory improvement of the paraneoplastic neurological syndrome was observed after several carboplatin-etoposid cycles. CONCLUSION: A paraneoplastic origin must be considered in all cases of unexplained choreo-athetosis. Paraneoplastic choreo-athetosis is most often associated with other neurological symptoms. The most frequent associated tumor is a small-cell lung carcinoma with anti-CRMP5 and/or anti-Hu antibodies. Our patient developed paraneoplastic choreo-athetosis related to an anti-Hu syndrome in the absence of anti-CRMP5/CV2 antibodies. Paraneoplastic choreo-athetosis might result from a central lesion, and/or from proprioceptive deafferentation subsequent to peripheral neuropathy.
Antineoplastic Combined Chemotherapy Protocols↗
Athetosis-dystonia in intramedullary lesions of spinal cord.
Athetosis and dystonia are well known clinical signs, described in disorders of basal ganglia. As opposed to pseudoathetosis, true athetosis was hitherto not reported in cord lesions. We here report three patients with athetosis and dystonia of hands due to intramedullary lesions of cervical cord: two patients with syringomyelia and one with glioma. Even though pseudoathetosis can be produced by lesions of posterior columns and likely to be confused with the involuntary movements of our patients, they had clinical and EMG findings consistent with true athetosis. A possible explanation for the athetosis and dystonia due to cord lesion is being postulated.
Adult↗
[A case of Creutzfeldt-Jakob disease exhibiting athetosis in the early stage].
A 68-year-old man was hospitalized on 24 June, 1998 because of visual and gait disturbance. A month before admission, he had been aware of blurred or double vision while watching TV. A few days later, he developed dysphagia and clumsiness in the fingers. His gait became unstable and he exhibited restless finger movements. His shoulders and trunk showed torsion while walking. On admission, he became disoriented and showed rigidity in the legs and athetosis in the bilateral fingers. Routine laboratory findings, thyroid function data, and the serum levels of vitamin B1, B12, Cu, and ceruloplasmin were within the normal ranges. Periodic synchronous discharges (PSD) were observed on electroencephalography. MRI showed T2-high intensity and atrophy of the bilateral caudate nucleus and putamen in addition to the cerebral cortex. 99mTc-ECD-SPECT showed a decrease of local blood flow in the bilateral frontal, right temporal, and bilateral parietal lobes and bilateral thalami. Athetosis became exacerbated and was observed for a month, overlapping with myoclonus. We diagnosed the patient as having CJD because of progressive dementia, myoclonus and PSD. Analysis of the prion protein revealed that codon 129 was Met/Met and codon 219 Glu/Glu by DNA sequences. The patient developed akinetic mutism and rigid contracture, and died of pneumonia on 5 September, 1998. Because athetosis is thought to involve the bilateral caudate nucleus, putamen and thalamus, the findings of diagnostic imaging in this patient might be relative to the clinical symptoms.
Aged↗
[Primary Sjögren's syndrome presenting with choreo-athetosis].
A 69-year-old man presenting with choreo-athetosis was proved to have primary Sjögren's syndrome. Choreo-athetosis suddenly appeared in the bilateral legs and the right arm; it was predominant in the right limbs. On the neurological examinations, there was no abnormal finding except for this involuntary movement. Brain MRI showed no abnormal finding. 18F-Fluorodeoxyglucose PET revealed that glucose metabolism was relatively increased in the bilateral striatum and thalamus (left side dominant). Dry eye and thirst were present for 4 years, and a diagnosis of primary Sjögren syndrome was confirmed by ophthalmological examinations, lip biopsy and sialography. Choreo-athetosis disappeared after thioridazine hydrochloride was started, while steroid was not effective.
Aged↗
[Double athetosis with Bielschowsky bodies--their histological features and distribution in the lateral pallidum].
This report analyzes a rare case of double athetosis with Bielschowsky bodies. These bodies are pleomorphic intra-neuronal PAS positive deposits mainly found in the lateral palladium of both sides. Clinically the patient was diagnosed as "double athetosis" and mild mental retardation. In her childhood she went through seizure attacks several times. The degree of athetosis was more severe in the upper extremities than in the lower ones. At the age of 25 she died from suffocation. Post-mortem findings: The brain weighed 1520 g. The cerebral cortex and cerebellum were not atrophic and externally not remarkable. Microscopically the most remarkable finding was PAS positive intra neuronal inclusions mainly restricted to the lateral pallidum, which are known as "Bielschowsky bodies." They varied in size and shape, and divided into 2 types according to their structural features. One is rather round type mostly in the intra-neuronal perikarya, and the other is small round but sometimes sausage-like in shape which is thought to be intra-axonal. We investigated the distribution of these deposits in and around the lateral pallidum. The distribution was different between these 2 types, that is, small and intra-axonal inclusions were seen "diffuse" all over the lateral pallidum and a little were in medial lamina which lies between lateral and medial pallidum, while large intra-perikaryal type were strictly restricted in the lateral pallidum and dominantly found near the internal capsule. This patient experienced generalized convulsion several times in her childhood but severe ischemic change was not seen in cerebral structures, especially in the hippocampus. Electron microscopically these bodies consisted of the accumulation of irregular fine fibrils.(ABSTRACT TRUNCATED AT 250 WORDS)
Adult↗
Athetosis increases resting metabolic rate in adults with cerebral palsy.
OBJECTIVES: To determine whether resting metabolic rate (RMR) is higher or lower in adults with cerebral palsy compared with the RMR of control subjects and to further examine physical characteristics of cerebral palsy that might affect RMR. DESIGN: Twenty-one adults with cerebral palsy (9 women, 12 men) were compared with 50 control subjects (25 men, 25 women) within the same age range (18 through 50 years). The following measurements were made: RMR by indirect calorimetry, anthropometrics, body composition, and habitual physical activity patterns. The study was conducted at the University of Vermont General Clinical Research Center and the Ball State University Human Performance Laboratory. STATISTICAL ANALYSES: Mean values +/- standard deviations, t tests, Pearson product-moment correlation coefficients, analysis of covariance, and stepwise multiple correlation regression analysis were used to examine the relationships among variables of interest. RESULTS: No significant differences were found in body weight, body mass index, fat mass, percentage body fat, and measured RMR between the two groups. The subjects with cerebral palsy were significantly shorter, had less fat-free mass, and expended fewer kilocalories in leisure time activities than the control subjects. After statistical adjustment for differences in fat-free mass, the subjects with cerebral palsy had a 14% (P < .001) higher adjusted RMR (1,742 kcal/day) compared with that of the control subjects (1,534 kcal/day). According to stepwise regression analysis, RMR was best predicted in the entire sample by fat-free mass and the presence or absence of athetosis (multiple R = .83, P < .001). The presence of cerebral palsy alone was not significantly correlated with RMR. CONCLUSIONS: The increased energy requirements of adults with cerebral palsy can be partially explained by athetotic movements. In this sample, the presence of athetosis increased RMR by an average of 524 kcal/day.
Adolescent↗
Botulinum toxin for spasticity and athetosis in children with cerebral palsy.
Botulinum toxin A has been used therapeutically in humans for a variety of conditions since 1980. Over the past few years, it has been used more frequently for spasticity management. We describe the use of botulinum toxin for spasticity or athetosis management in three children with cerebral palsy. Two of these children had severe spasticity or athetosis that was unresponsive to other forms of treatment. The injection of botulinum toxin decreased pain and improved ease of care in these two children. Another child with left hemiparetic cerebral palsy underwent injection of botulinum toxin into upper and lower limb muscles to improve function. In this patient, the injections were combined with other forms of treatment. In this patient spasticity was decreased and function was increased temporarily. The use of botulinum toxin in general is discussed and related to these three cases.
Adolescent↗
Familial paroxysmal kinesigenic choreo-athetosis in a child with visual hallucinations and obsessive-compulsive behaviour.
A teenage male is described, in whom Tourette Syndrome was suspected, which was later replaced by attacks of paroxysmal kinesigenic choreo-athetosis. He also exhibited bizarre, episodic perceptual distortions of his visual environment and manifestations of an obsessive-compulsive disorder. Carbamazepine treatment not only completely eliminated the recurring attacks of his choreo-athetosis but also, contrary to expectations, the visual disturbances and even the symptoms of his obsessive-compulsive disorder. Obsessive compulsive disorder should be searched for by direct questioning in all patients with basal ganglia disorders.
Adolescent↗
Electromyographic study of the rigidospasticity of athetosis.
The static and dynamic components of the tonic stretch reflex and shortening reactions have been studied in 10 patients with athetosis. EMG activity could be recorded only from the biceps muscle when the patient was at rest. The dynamic stretch reflex increased with the velocity of stretching in all muscles examined except the biceps. The biceps stretch reflex was found to be inhibited by increasing muscle length, whereas the stretch reflexes of triceps, hamstrings, and quadriceps muscles were facilitated by increasing muscle length. Reinforcement increased resting activity in the biceps and the dynamic shortening reaction of the triceps muscle. Both these effects were suppressed by the action of phenoxybenzamine. Although phenoxybenzamine was shown to reduce muscle tone in a double-blind controlled trial, no corresponding improvement was detected in involuntary movements or the patients' performance in a tracking test. The differences between the pattern of hypertonus in athetosis, Parkinson's disease, spasticity, and activated normal subjects are presented in discussion.
Adult↗
Attempted rapid elbow flexion movements in patients with athetosis.
Voluntary rapid elbow flexion movements were studied in 14 patients with athetosis on the basis of cerebral palsy. When the movement was attempted with one arm, other muscles inappropriate for the task, such as muscles in the opposite limb, were also activated. EMG activity of the biceps and triceps was analysed in detail, and the patterns seen in the different patients were divided into six groups: (1) The normal "ballistic" triphasic pattern, with bursts of normal duration, alternating in biceps and triceps, but the triceps might be activated first, causing the limb to extend rather than flex, (2) The triphasic pattern, with bursts of long duration, (3) Repetitive cycles of the triphasic pattern with particularly long antagonist bursts, apparently limiting the movement in each cycle, (4) Long bursts synchronous in agonist and antagonist muscles, (5) Continuous activity of the agonist, with reduction in activity of the antagonist, (6) Failure to be able to do the task. The pathophysiology of athetosis is that voluntary movement is characterised by excessive muscular activity, most prominently in inappropriate muscles, both extraneous to the task and directly antagonistic.
Adult↗
Prevalence of chorea, dystonia and athetosis in Assiut, Egypt: a clinical and epidemiological study.
Involuntary movements originate from different parts of the nervous system. The character of movements depends upon the site of the lesion and the type of pathological change. The presence of more than one type of involuntary movement in a patient can cause confusion and difficulty in the proper classification of movement disorders, which then leads to problems in the differential diagnosis and appropriate treatment. This work was planned to estimate the prevalence and to study the aetiological factors of chorea, dystonia, athetosis and hemiballismus in Assiut, a representative community of Upper Egypt. This study was carried out on 7,000 families (42,000 subjects) representing different types of communities (2,000 families from urban, 2,000 families from suburban and 3,000 families from rural communities). All members of these samples were personally interviewed at home. Full clinical assessment and special investigations required for the diagnosis of different types of chorea, dystonia and athetosis were carried out in Assiut University Hospital. The prevalence rate for rheumatic chorea was 62/100,000 population and it was significantly higher (p < 0.01) among rural than urban and suburban populations, whereas Huntington's chorea had a prevalence rate of 21/100,000 with no significant difference between different areas studied (urban, suburban and rural). The other two common types of chorea were reported with prevalence rates of 12/100,000 for the encephalitic type and 17/100,000 for the atherosclerotic type. No single case of generalized dystonia was recorded and all cases were of the focal type of dystonia with a prevalence rate of 26/100,000 population. No significant differences were recorded between the different areas studied (urban, suburban and rural).(ABSTRACT TRUNCATED AT 250 WORDS)
Adolescent↗
[Levodopa treatment of a case of torsion spasm with athetosis].
The result about Levodopa treatment in torsion dystonia and athetosis are quite contradictory. We report an interesting case, significant for the results that we obtained in a long treatment period. A girl was suffering from age of 8 years of torsion dystonia associated with athetosis. We made our first clinical examination when 12 years old: she was not able to stand-up and to walking, neither was she able to be sitting in a chair without arms. In two months of treatment with Levodopa 3.000 mg. by day, the symptoms completely disappeared. During the first two years of treatment, at every effort to decrease the therapy we observed an important increase of symptoms. Successively it was possible to decrease the therapy and now, after 8 years of treatment, with a minimal dose, the girl doesn't show any symptoms, so that she can manage a normal relative life. Probably these good results depend on the fact that was a sporadic recessive form of torsion dystonia case.
Athetosis↗
Acute athetosis as a result of phenytoin toxicity in a child.
A case of acute athetosis in a child was found to be related to phenytoin toxicity. Symptoms were intermittent and coincided with drug administration. More common toxic effects were not noted on initial evaluation of the patient.
Athetosis↗