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The frequency spectrum of the elbow movements of one normal subject and six handicapped subjects were obtained in order to investigate the possibility of using this technique in quantifying athetosis. The frequency spectrum technique appears to be useful but data from more subjects must be obtained and specific details regarding scaling need to be investigated.
Movement disorders developed in five children, ages 6 to 21 months, during the course of bacterial meningitis caused by Hemophilus influenzae (one), Streptococcus pneumoniae (one), Neisseria meningitidis (one), or Mycobacterium tuberculosis (two). Athetosis, choreoathetosis, and hemiballismus occurred, ranging in duration from hours to months. Cranial computed tomography, performed in four cases, showed no lesion of the basal ganglia. The movements were of such abrupt onset and severity that in four cases they were initially misinterpreted as seizures, and anticonvulsant therapy was contemplated. It is important to recognize the potential development of movement disorders during the acute phase of bacterial meningitis to preclude the inappropriate administration of anticonvulsant medication.
Neurological findings, motor symptoms, mental abnormality and dysarthria were examined in 28 children with lesions in the thalamus, putamen, and/or peri-Rolandic area. The thalamus and putamen were involved in eight, and only the thalamus in ten of the children. Most of these 18 children had mild disabilities; they did not have severe mental retardation and could walk alone, speak words, and grasp an object. Dominant flexion of the hips was observed in many of the children who could walk. Two-thirds of these children had athetotic involuntary movement and the remaining had gross or fine motor abnormalities although they had no involuntary movement. In most of these children, reaching patterns were abnormal and were affected by shoulder retraction. Their abnormal movements were thought to be inappropriate muscle activity brought about by voluntary movements. In the remaining ten children, the thalamus, putamen, and peri-Rolandic area were all involved. Many had severe disabilities such as severe mental retardation and the inability to sit, speak words, or grasp an object. All had athetotic involuntary movements. Three children had spasticity of the lower extremities. Five children with severe disabilities and no spasticity were thought to have apparent weakness with athetosis.
The H-reflex was studied in 53 children with cerebral palsy, and the results were compared with those obtained for 56 normal control subjects. Pairs of identical stimuli were delivered and the time course of recovery of the amplitude of the H-reflex was determined. Recovery of the H-reflex was increased in the normal control group aged 0 to 12 mos compared to that in the normal control group aged 1 to 9 yrs, especially at interstimulus intervals from 100 to 800 msec. In children with spasticity, marked recoveries of the H-reflex were observed in both age groups, 0 to 12 mos and 1 to 9 yrs, compared to those in normal controls. In the cases of athetosis, recovery of the H-reflex was also marked. In children with ataxia, it was not pronounced and similar to that in normal controls. From these findings, an H-reflex study was considered to be useful for evaluation of central nervous system function in childhood.
Denervation, provosed at the turn of the century and abandoned because of inconstant results, has again been taken up in principle, but with a notable change in technique. It is necessary to do a careful examination of the muscles that are to be denervated (of the median and ulnar nerves) and to determine the nature of the spasticity. The incision, longer than in the original technique, allows a better view of the median and ulnar nerves. The nerve branches to each muscle are thus easily identified, and by electric stimulation during the operation one judges how much denervation to subject the muscles to while taking into account the phenomenon of the take-over of the denervated muscles by the remaining nerve fibers. Part of the nerve branches are then sectioned under microscopic control near the muscle, taking care to cauterize the proximal stump. It may be necessary to do associated tenotomies or capsulotomies. This method gives good results if the technique and the contraindications, (athetosis, chorea and cerebral deficits) are respected. It must be emphasized that the results depend on the degree of denervation, which is hardly quantifiable, and is subject to the operator's experience.
Chorea and athetosis are rare presenting symptoms in childhood. Chorea can be a presenting symptom in a number of hereditary diseases, including neurodegenerative diseases, paroxysmal diseases, and metabolic diseases. In these situations, family history, associated symptoms, and other physical findings will often enable a correct diagnosis. Benign childhood chorea is probably a genetically heterogeneous group of disorders, generally without other symptoms. Clinical aspects of these disorders are reviewed here.
This report presents cinefluorographic data on the articulation of isolated vowels, VCV nonsense utterances, and short sentences by five subjects with athetoid cerebral palsy. Articulatory abnormalities were identified from tracings of vocal tract shapes and from displacement-by-time plots of articulatory events. The most frequent abnormalities were large ranges of jaw movement, inappropriate positioning of the tongue for various phonetic segments (especially because of a reduced range of tongue movement in the anteroposterior dimension), intermittency of velopharyngeal closure caused by an instability of velar elevation, prolonged transition times for articulatory movements, and retrusion of the lower lip. The speech disorder associated with athetosis is considered with respect to a model of motor learning.
Our experience is based on a series of 25 patients suffering from infantile dyskinesias and dystonias who underwent stereotaxic surgery of basal ganglia. We first note the immediate good results (77%) obtained by thalamo-sub-thalamotomy. Concerning dyskinesia this type of surgery enjoys in an opinion a place of choice. Secondly we have been able to estimate secondary deterioration on long term results in patients examined 2 to 15 years post-operatively. In contrast to various results reported in the literature good long term results are not superior to 50%. Some deterioration is noted in patients operated on for choreo-athetosis over the age of 20. A pallido-subthalamic lesion is efficient at the beginning of the disease course since it improves motor performance and thereby helps possibilities of intellectual acquirement. Bilateral lesions have after improved I.Q. Effects of this treatment specially on spasticity must be discussed among other types of surgery.
The aim of this study was to compare the pattern of motor dysfunction in Hong Kong Chinese children with spastic diplegic and the athetoid type of cerebral palsy (CP) by the Gross Motor Function Measure (GMFM). The GMFM was first tested on its content validity in differentiating the items suitable for testing on upper and lower limb function, followed by test-retest and inter-rater reliability studies, before it was used in a within-subject experiment comparing upper and lower gross motor function in two types of CP, respectively. Children with spastic diplegia (n=18) and with athetosis (n=19) were recruited from three pre-school centres, schools for physically handicapped children and centres of the Hong Kong Spastic Association in Hong Kong. The GMFM, a standardized outcome measure of the motor function in children with CP, was used in comparing mean scores of motor tasks involving upper limbs and those not involving upper limbs in the athetoid and spastic cerebral palsied children. The scores were significantly higher for the children in the spastic diplegic group, but there was no significant difference in the performance of static and dynamic motor tasks of the GMFM between the children in the two groups.
Two-staged anterior and posterior spine fusion with instrumentation was evaluated in 17 cerebral palsy patients, ages 11 to 30 years. Functional benefits included improved sitting balance and, in ambulators, no loss of independence. Curve correction averaged 60%. The pseudarthrosis rate of 18% was lower than previously reported for patients with anterior or posterior fusions alone. Instrumentation complications occurred in 50% of patients with athetosis. The combined anterior and posterior fusion for thoracolumbar and lumbar curves appeared to give the best correction, highest rate of fusion, and fewest complications in this complex patient group.
Patients with cerebral palsy and fixed hip extension are often unable to sit and therefore lose social and educational opportunities. At Texas Scottish Rite Hospital (Dallas, TX, U.S.A.), 29 patients had hip extension-abduction contractures. Eleven patients with mild involvement were managed with physical therapy. Six patients had moderate involvement and required wheelchair modification to maintain sitting. Twelve patients demonstrated severe contractures that required major soft tissue releases and often femoral shortening osteotomies to regain flexion. Patients with athetosis or rigidity were identified as those most likely to develop extension-abduction contractures either spontaneously or following adductor releases.
A review was made of 88 adult institutionalized patients with spastic cerebral palsy and contractural deformity of the hips. 21 were untreated for dislocated hip, and 11 of these suffered from hip pain. The degree of pain was directly related to neurological maturity and to the coexistence of athetosis and spasticity. Decubitus ulcers and perineal care problems were more associated with contractures than with dislocation alone. It is concluded that dislocation and subluxation should be prevented by surgical means, but that surgical treatment of the already dislocated hip should be reserved for the neurologically mature and athetoid patient.
A third case of hyperargininaemia occurring in one family was studied from birth. In cord blood serum arginine concentration was only slightly raised, but arginase activity in red blood cell haemolysates was very low. In the urine on day 2 a typical cystinuria pattern was present. Arginine concentration in serum increased to 158 mumol/100 ml on the 41st day of life. Later determinations of the arginase activity in peripheral blood showed values below the sensitivity of the method. Blood ammonia was consistently high, and cystinuria was present. The enzymatic defect was further displayed by intravenous loading tests with arginine. Serum urea values were predominantly normal or near the lower limit of normal, suggesting the presence of other metabolic pathways of urea synthesis. In urine there was no excretion of guanidinosuccinic acid, while the excretion of other monosubstituted guanidine derivatives was increased, pointing to a connexion with hyperargininaemia. Owing to parental attitude, a low protein diet (1-5 g/kg) was introduced only late. The infant developed severe mental retardation, athetosis, and spasticity.
An electromyographic technique was used to record the tonic stretch reflex (TSR) responses to passive sinusoidal stretching of biceps brachii muscle in athetotic patients. The TSR transmission characteristics measured while the patient was in a relaxed state were compared with those measured while the patient was voluntarily active. The resting TSR and action TSR were found to differ markedly. Sensitivity, pattern, duration, and timing of the TSR responses were all changed during voluntary activity. The results suggest that the TSR in athetosis is functionally reorganized by supraspinal influences during voluntary activity. It is proposed that athetoid movements and action tremor may be a manifestation of abnormal action TSR caused by faulty supra-spinal control during activity.
Visual tracking tests have been used to obtain a quantified statistical description of the involuntary movements of the arm about the elbow joint in a group of patients suffering from athetoid cerebral palsy. Three separate components of involuntary activity can be recognized and it is possible that each may be a different physiological mechanism. First there are irregular movements which are represented by a continuous power spectrum which decreases with increasing frequency, reaching a negligible value between 2-3 Hz. The second component is a rhythmical low frequency movement which is indicated by a predominant peak in the power spectrum at a frequency of 0·3-0·6 Hz. The presence of this peak was predicted in a previous paper because of underdamping demonstrated in the closed loop voluntary control of movement in athetosis. The third component is the athetoid action tremor in which both agonist and antagonist muscle groups contract vigorously but asynchronously at a frequency of 1·5-4 Hz.
For children whose spasticity and movement disorders are inadequately treated by oral medications and botulinum toxins, neurosurgical procedures are now available to effectively treat spasticity, tremor, and many cases of dystonia. Spastic diplegia can be treated with selective lumbar rhizotomies, which significantly decrease spasticity, increase range of motion, and improve Gross Motor Function Measure scores. Children with spastic quadriparesis and those with secondary dystonia can be treated with intrathecal baclofen, which diminishes both spasticity and dystonia and is associated with improved function and quality of life. Children with primary dystonia and those with tremor can be treated with deep brain stimulation of the internal globus pallidus and thalamus, respectively. Some children with chorea respond to deep brain stimulation. There are no effective neurosurgical treatments for athetosis or ataxia. The effectiveness of neurosurgical treatments of pediatric movement disorders has increased significantly in the past 15 years.
Data are presented for the first 50 patients with cerebral palsy who underwent chronic cerebellar stimulation for symptom alleviation. We observed significant shorter and longer term improvement in spasticity as well as athetosis, speech, and functional status. Continuing increments in improvement were noted as a function of time on stimulation. In many instances, psychometric test scores and behavior also were improved. There was one death in this series. There were no neurologic complications due to cerebellar stimulation. The results of this study warrant the judicious use of cerebellar stimulation for symptomatic and functional relief in cerebral palsy.
We report a patient with a cervical spinal astrocytoma who presented with athetotic hand movements. We postulate that acute pain, related to a syrinx around the operative site, contributed to the athetosis. These movements occur only after protracted, severe loss of proprioception, often in conjunction with an acute, generally painful crisis. Athetotic movements can disappear without change in proprioceptive or motor deficits when pain lessens.