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Assessment of upper-limb function and movement in children with cerebral palsy wearing lycra garments.

It has recently been suggested that lycra garments are helpful for children with cerebral palsy (CP). Twelve children, with athetosis, ataxia, and spasticity, were fitted with lycra garments (Kendall-Camp UK Ltd). Scores on the Paediatric Evaluation of Disability Inventory (PEDI) scales were determined before and after wearing the garment for at least 6 hours a day for 6 weeks. Five children with motor problems representative of the whole group were investigated during a reach-and-grasp task by kinematic motion analysis; reflective markers were used with and without the garment. Carers were given a questionnaire concerning the practicalities of using the garments. All 12 children made improvements in at least one of the functional scales of the PEDI, and scores for the whole group showed significant gains (Wilcoxon chi2 test, self-help p<0.01; mobility p<0.5; social p<0.1). These changes were usually slight, although noticed by carers. Six children made gains of at least one scale of the caregiver assistance scores, two of the children showed losses (due to difficulties removing the garment for toileting), and four showed no change. Motion analysis indicated that (1) two children with athetosis had improved proximal stability in sitting and in smoothness of arm movements, (2) one child with ataxia had improved in proximal and distal stability, and (3) two children with spasticity had more jerky movements, although one improved in proximal stability. All children had problems in wearing the garments, including problems with toileting and incontinence of urine; the parents of only one child wanted to continue using it. Results suggest that the functional benefit of lycra garments for children with CP is mainly due to improvements in proximal stability but this should be weighed against the inconvenience and loss of independence.

Activities of Daily Living↗

Delayed onset mixed involuntary movements after thalamic stroke: clinical, radiological and pathophysiological findings.

Although occurrence of involuntary movements after thalamic stroke has occasionally been reported, studies using a sufficiently large number of patients and a control population are not available. Between 1995 and 1999, the author prospectively identified 35 patients with post-thalamic stroke delayed-onset involuntary movements, which included all or some degree of dystonia-athetosis-chorea-action tremor, occasionally associated with jerky, myoclonic components. A control group included 58 patients examined by the author during the same period who had lateral thalamic stroke but no involuntary movements. Demography, clinical features and imaging study results were compared. There were no differences in gender, age, risk factors, side of the lesion and follow-up periods. During the acute stage of stroke, the patients who had involuntary movements significantly more often had severe (< or = III/V) hemiparesis (50 versus 20%, P < 0.05) and severe sensory loss (in all modalities, P < 0.01) than the control group. At the time of assessment of involuntary movements, the patients with involuntary movements significantly more often had severe sensory deficit (in all modalities, P < 0.01) and severe limb ataxia (60 versus 5%, P < 0.01) than the control patients, but neither more severe motor dysfunction (7 versus 0%) nor more painful sensory symptoms (57 versus 57%). The patients with involuntary movements had a higher frequency of haemorrhagic (versus ischaemic) stroke (63 versus 31%, P < 0.05). Further analysis showed that dystonia-athetosis-chorea was closely associated with position sensory loss, whereas the tremor/myoclonic movements were related to cerebellar ataxia. Recovery of severe limb weakness seemed to augment the instability of the involuntary movements. Persistent failure of the proprioceptive sensory and cerebellar inputs in addition to successful, but unbalanced, recovery of the motor dysfunction seemed to result in a pathological motor integrative system and consequent involuntary movements in patients with relatively severe lateral-posterior thalamic strokes simultaneously damaging the lemniscal sensory pathway, the cerebellar-rubrothalamic tract and, relatively less severely, the pyramidal tract.

Adult↗

Stereotactic selective thalamotomy for the treatment of tremor type cerebral palsy in adolescence.

6 cases with tremor-athetotic type cerebral palsy and 2 cases with moderate dystonia-tremor type cerebral palsy were treated by selective stereotactic thalamotomy. In the former group, postural-movement type tremor in the upper limb gradually progressed with age while athetosis remained unchanged. In the latter group, dystonia in the truncal muscles predominated over the irregular tremulous movement of the upper limbs. In all cases, the intelligence was almost normal. Stereotactic selective thalamotomy (Vim for tremor athetosis, VL-Vim for dystonia tremor) was performed under local anesthesia with the aid of radiological and neurophysiological control methods. The results of the operations were satisfactory in regard to the tremor relief and concomitant improvement of motor performances in most of the cases. Stereotactic treatment might be an effective way to make possible a one-step progress in these handicapped cases. The importance of postoperative physical therapy is also emphasized.

Adolescent↗

[Pseudoathetosis after medullar and pontine hemorrhage].

INTRODUCTION: Athetosis is a slow and purposeless involuntary movement which changes in frequency, severity. The term pseudoathetosis is used when this movement is associated with proprioceptive sensory impairment. CLINICAL CASE: A 63 year old man developed right medulla and pons hemorrhage and presented loss of proprioception in left hemibody and athetosis movements in left hand. CONCLUSION: We report a case of pseudoathetosis associated with loss of proprioceptive sensation.

Athetosis↗

Follow-up study of patients with cerebral palsy.

Of 319 patients with cerebral palsy recalled for reevaluation 15 years after the initial visit, 10 percent had died. Of the living, 55 percent had spasticity, 32 percent had athetosis, 4 percent had ataxia and 9 percent had mixed spasticity and athetosis; 38 percent had an intelligence quotient (IQ) less than 50, 24 percent between 50 and 79, and 38 percent had IQ above 80. There was a high correlation between overall functional outcome and intellectual level. Severity of physical disability, as measured by hand use, mobility and speech, also correlated with dependence, in part because increased severity of the disability was associated with decreased intellectual capacity generally.Twenty-five years after the initial visit, parental attitudes and personality intactness were evaluated (using the Minnesota Multiphasic Personality Inventory [MMPI]) and were correlated with satisfaction with status in life in 28 persons predicted to be independent on the 15-year study. Twenty (72 percent) of the 28 were satisfied with their status in life and of these, 16 were evaluated (with the MMPI) with 70 percent scoring in the normal range; 13 (65 percent) had parents with a positive attitude. Positive attitude was defined as parental feelings that the handicapped child was a worthy, valuable person, to be encouraged and assisted but not isolated from the world of nonhandicapped people. Careful serial assessment by professional teams combined with repeated long-term counseling of families can result in optimal outcome for the disability level involved, due to the primary role parents play in the development of a child's character and behavior.

Adult↗

[Acupuncture needles, straying in the central nervous system and presenting neurological signs and symptoms].

Acupuncture has been practiced in the treatment of many diseases in Japan. "Okibari" is one of the procedures in acupuncture treatment: a fine stainless steel or silver needle is inserted into the subcutaneous tissue through the skin, to remain in the subcutaneous tissue. A 57-year-old pharmacist was knocked down by a motorcycle in 1971, since then moderate weakness of left extremities and stiffness of muscles have remained as sequelae. She was consequently treated with acupuncture. Many small needles were inserted permanently in the nuchal, occipital and other areas of the body ten to twelve years before she developed gradual clumsiness and dysesthesia in her right hand in 1984. When she was admitted for the first time in 1985, neurological examination revealed left Horner's syndrome and diminished deep sensation in her right extremities with pseudo-athetosis of her right hand, along with spastic paresis of left extremities and right carpal tunnel syndrome. An old needle which had strayed into left dorsal medulla was considered to be responsible for these symptoms. In 1988 loss of pain and temperature sensation in the right side of her body below the shoulder, and diminished deep sensation of left extremities were appended, and weakness of her left extremities became aggravated. Pseudo-athetosis of her right hand was seen less prominently. In plain X-ray films many needle shadows were visualized. On CT scan needle shadows could be seen also in the left dorsal medulla, right cerebellum and in the subarachnoid space of left dorsal C1-C2 level.(ABSTRACT TRUNCATED AT 250 WORDS)

Acupuncture Therapy↗

Cervical radiculopathy or myelopathy secondary to athetoid cerebral palsy.

Radiculopathy or myelopathy often occurs during adult life in patients who have athetosis. Herniation of an intervertebral disc, spondylosis, malalignment or instability of the cervical spine, or a combination of these lesions, can develop because of the athetoid hyperactivity. We reviewed the cases of ten patients who had cervical radiculopathy or myelopathy, or both, secondary to athetosis and who were surgically treated between the ages of thirty and fifty-eight years. The surgery consisted of discectomy, removal of osteophytes, and anterior interbody fusion. When several segments were involved, an extensive subtotal resection of the vertebrae and discs, followed by strut bone-grafting, was done.

Adult↗

[The possibility of pharmacologic therapy of extrapyramidal hyperkineses with tiapride].

A novel substance, tiaprid (Tiapridex), from the family of the substituted benzamides, can be used in an attempt to block the dopaminergic receptors of the corpus striatum without inducing the psychiatric side effects on the mesolimbic system associated with, for instance, neuroleptics. Since hyperkinetic illness of the brain-stem ganglions accompanied by myoclonia, athetosis and dystonia are believed to stem from a state of imbalance between dopaminergic and cholinergic processes in which dopaminergic activity predominates, the application of tiaprid was tested on patients with etiologically different illnesses in an open clinical study. The course of treatment was documented by measurements and a record of clinical findings. No tolerance problems were encountered, and the substance had no grave side effects. Its effects proved to be good to very good on myoclonic hyperkinesis and good to moderate on athetosis, but dystonia seemed to be resistant to this form of treatment.

Adult↗

[A sibling of delayed post-anoxic encephalopathy after strangulation].

A sibling of three year old girl and a year old boy, showed delayed post-anoxic encephlopathy after strangulation. After three days of the accident, the girl developed tetraplegia and choreo-athetosis. Her brother also developed choreo-athetosis two weeks after strangulation. T2 weighted MRI revealed a high signal intensity in the bilateral putamen and caudate nucleus. After hyperbaric oxygen therapy for two months, their symptoms diminished. We hypothesize that the functional damage of the neurons occurred in the bilateral basal ganglia as delayed neuronal death because of their vulnerability and peculiarity of the local circulation. Hyperbaric oxygen therapy may be effective in rescuing the neurons from hypoxia.

Asphyxia↗

Deep brain and motor cortex stimulation for post-stroke movement disorders and post-stroke pain.

Our experience of deep brain stimulation (DBS) and motor cortex stimulation (MCS) in patients with post-stroke movement disorders and post-stroke pain is reviewed. DBS of the thalamic nuclei ventralis oralis posterior et intermedius proved to be useful in more than 70% of patients with post-stroke involuntary movements (hemiballismus, hemichoreo-athetosis, distal resting and/or action tremor, and proximal postural tremor). The effect of DBS of the thalamic nucleus ventralis caudalis or internal capsule on post-stroke pain was usually disappointing. Excellent pain control can be achieved by MCS in approximately 50% of patients with post-stroke pain. In the course of clinical trials on MCS for the control of post-stroke pain, it was found that co-existent post-stroke involuntary movements (hemichoreo-athetosis and resting tremor) could also be controlled by MCS. Post-stroke involuntary movements, especially those in thalamic syndrome, are sometimes associated with post-stroke pain. In such disorders, involuntary movements are attenuated, but the pain in the same patients is often exacerbated by DBS of the thalamic nuclei ventralis oralis posterior et intermedius. MCS could be the therapy of choice under such circumstances. Subjective improvement of voluntary motor performance, which had been impaired in association with mild or moderate hemiparesis, was reported during MCS by approximately 20% of patients with post-stroke pain. Such an effect on voluntary motor performance appears to be caused by an inhibition of their rigidity. The reversibility of DBS and MCS makes them an important option for the control of post-stroke movement disorders and post-stroke pain.

Electrodes, Implanted↗

Chronic cerebellar stimulation for cerebral palsy--five-year study.

Two hundred sixty-two patients were implanted with cerebellar stimulator systems since February 1974. Cerebral Palsy (CP) patients constituted 88% (230) of this series. The age range was 3 to 53 years with 70% under 20 years of age. Half the CP series were severely affected with the rest being moderately to mildly involved. Athetosis was present in 50%. The primary effect of CCS has been a lowering of spastic muscle tone in 90% of the patients. Improvements in control of immature reflexes such as startle response, head control, scissoring, balance and sitting occur in the first month. Athetosis progressively decreases to a 50% level. In the moderate mild CP group abilities improve over the first six months leading to better feeding, dressing, and ambulation with clearer speech and less drooling. After six months, 25 of 48 patients were out of wheelchairs, walking. A further 47 patients were ambulating better. No deaths from surgery. Five have died during the 5 years from other causes. Eleven patients (4%) have had infected systems. Equipment problems especially with malfunctioning radio receivers (40%) have served as blind controls--spasticity returning and abilities decreasing. Replacements with another receiver or with a totally implantable lithium powered pacemaker (May 1979) have lead to a return of benefits. Post-mortem findings indicate the low current levels (0.8 uC/sqcm) applied intermittently produced no appreciable damage to the cerebellar cortex.

Adolescent↗

Treatment of involuntary movement disorders with tetrabenazine.

Seventeen patients with choreiform, athetoid, or ballistic involuntary movements, or with spasmodic torticollis, were treated with tetrabenazine in doses of 25 to 200 mg daily for periods varying from two weeks to more than six months. Randomized ciné film of the patients' involuntary movements, taken before, during, and after treatment was assessed individually by seven `blind' observers. Eight patients were judged improved; two had Huntington's chorea, two athetosis, two dystonia musculorum deformans, one hemiballismus, and one spasmodic torticollis. Four of the eight improved patients have continued taking the drug for longer than six months. In a second study seven patients with Huntington's chorea were treated for two weeks each with tetrabenazine (50 mg t.d.s.) and with amantadine (100 mg t.d.s.) and the results assessed by the same method. The choreiform movements of six of these patients were strikingly improved with tetrabenazine therapy, but amantadine had no effect. Tetrabenazine is an effective agent for the suppression of choreiform and ballistic involuntary movements. It is only slightly effective in the treatment of athetosis and spasmodic torticollis. Drowsiness, insomnia, and depression were the most conspicuous unwanted effects, and these may limit the clinical usefulness of the drug.

Adolescent↗

[Statistical study of the relationships between the etiology and clinical picture of cerebral palsy].

846 children with cerebral palsy were examined and 642 were selected for a statistical study by correspondence analysis. The aim was to identify without any prior assumptions, the relationships between the aetiological factors and the clinical findings. The study was completed by conventional statistical analysis of 584 of the cases. Small birth weight and a history of abnormal pregnancies was associated with a cerebral palsy affecting both legs, and often with a squint. Resuscitation was associated with athetosis and abnormalities of posture and behaviour. If the resuscitation lasted for more than 15 minutes or there were certain problems during delivery, severe abnormalities of both arms and major speech difficulties were observed. Resuscitation for less than 15 minutes or for an unknown time or intractable vomiting during pregnancy was associated with quadraplegia. Hemiplegia was related to post natal events but the aetiology was not always known. Foeto-maternal incompatibility was associated with athetosis, deafness, severe speech problems and ophthalmoplegias.

Abortion, Threatened↗

[Magnetic resonance imaging findings in children with cerebral palsy].

OBJECTIVE: To study the clinical and neurological abnormalities in children with cerebral palsy (CP) and to attempt to correlate the types of CP and the gestational age at birth with radiological abnormalities detected by magnetic resonance imaging (MRI) of the brain. METHODS: This is a hospital-based study, the subjects included 104 children with cerebral palsy who were hospitalized in the Qingdao Rehibilitation Center For Disabled Children. All the 104 hospitalized CP cases (47 with spastic diplegia, 9 with tetraplegia, 15 with hemiplegia, 22 with athetosis, and 11 with ataxia) were examined neurologically and their perinatal history was reviewed. Their cranial MRI findings were studied. The association between the gestational ages, CP types, and the radiological findings were studied. RESULTS: The type distribution was significantly different between term- and preterm- infants. Spastic diplegia was the main type in preterm infants while hemiplegia and ataxia were mainly seen in term infants. MRI abnormalities were found in 88 of the 104 cases and abnormal rates of spastic diplegia, tetraplegia, hemiplegia, athetosis, and ataxia were 89.4%, 100%, 100%, 54.5% and 90.9%, respectively. There was no significant difference in abnormal rates between term and preterm groups. Thirty-one of 42 (73.8%) children with spastic diplegia had significant periventricular leukomalacia (PVL), which was more common among preterm-born children (90%). Of the 15 children with hemiplegia, 13 had unilateral lesions on neuroimaging. Spastic tetraplegia was associated with extensive, bilateral, diffuse brain damage. The abnormalities in term-born infants with athetoid cerebral palsy were mainly located in the basal ganglia region whereas the major abnormality in premature infants was PVL. Of the 11 children with ataxic cerebral palsy, 8 cases showed congenital cerebellum dysplasia on brain imaging. CONCLUSIONS: Radiological abnormalities of the brain were correlated with CP types and the gestational age at birth; MRI scan was useful in revealing underlying brain abnormalities and speculating on the etiology of cerebral palsy.

Cerebral Palsy↗

[Vegetative disorders in children with cerebral palsy. Results of an inquiry of parents].

Many of the disturbances resulting from dysregulations in the autonomous nervous system of children with cerebral palsy are rarely discussed in the doctor's praxis. Nevertheless, they are causes of trouble and worry for the parents. For this reason we started an inquiry into this matter. Questionnaires were sent to the parents of 452 C.P. patients. 374 were answered with sufficient care. The following factors were evaluated: sleep, bladder and bowel activity, temperature regulation, vomiting, sweating, blood circulation, growth. The C.P. children were compared to their own siblings especially to the next younger ones. The diagnoses were as follows: Spastic tetraplegia 197 patients. Spastic hemiplegia 44 patients, Athetosis 33 patients, Mixed cases of spasticity and athetosis 82 patients, Other 15 patients. The degrees of handicap in terms of motor development were: severe (unability to sit unsupported) 166 cases, moderate (unability to kneel or walk unsupported) 118 cases, mild (ability to kneel and/or walk unsupported) 87 cases. Summarized, the statements of the parents gave the following results: sleep disturbances: 169 cases (46%), constipation: 145 cases (39%), tendency towards temperature dysregulation: 112 cases (30%) , tendency towards increased vomiting: 91 cases (25%), sweating increased or decreased: 110 cases (30%), irregular and frequent voiding of bladder: 92 cases (25%), unstable regulation of blood circulation: 101 cases (27%), cold skin: 264 cases (71%), body-length deficit: 119 cases (32%), low-weight: 177 cases (48%), feet too small for age: 252 cases (68%). Results are related to diagnosis and severeness of handicap. In addition, it is discussed, whether there are relations between several of the investigated factors. The influence of the patients sex is discussed.

Adolescent↗

Pseudochoreoathetosis. Movements associated with loss of proprioception.

OBJECTIVE: To describe seven patients with proprioceptive sensory loss and choreoathetoid movements. DESIGN: Case series. SETTING: Outpatient and inpatient university referral. PATIENTS: Patients with sensory loss and abnormal movements. INTERVENTION: None. MAIN OUTCOME MEASURE: None. RESULTS: One patient had a parietal cortex injury, one had a thalamic infarction, two had spinal cord lesions, two had dorsal root ganglion neuronopathies, and one had an ulnar neuropathy. In each case, the duration of abnormal movements correlated with the duration of proprioceptive sensory loss, and the abnormal movements were restricted to body parts with proprioceptive sensory loss. The movements varied from chorea and athetosis to dystonia. CONCLUSIONS: These cases suggest that proprioceptive sensory loss can lead to a movement disorder, termed pseudochoreoathetosis, which occurs following the appearance of lesions anywhere along proprioceptive sensory pathways, from peripheral nerves to the cerebral cortex. It is hypothesized that pseudochoreoathetosis occurs because of the failure to process limb proprioceptive information in the striatum. Therefore, both choreoathetosis and pseudochoreoathetosis may be manifestations of the failure of the striatum to properly integrate cortical motor and sensory inputs.

Adult↗

Ventricular somatostatin-like immunoreactivity in patients with basal ganglia disease.

The concentrations of somatostatin-like immunoreactivity (SLI) in lateral ventricular fluid of patients with extrapyramidal motor disease were determined by specific radio-immunoassay. Mean SLI levels were significantly lower in patients with Parkinson's disease (mean +/- SEM); 42.9 +/- 2.9 fmol/ml) and in patients with dystonic syndromes (39.4 +/- 3.2) than in patients with benign essential tremor (65.3 +/- 9.7). The lowest levels were found in patients with athetosis (34.7 +/- 5.4). In parkinsonian patients somatostatin levels correlated with the degree of akinesia, rigidity and autonomic disturbances.

Adult↗

[Non-epileptic paroxysmal movement disorders].

Non-epileptic paroxysmal dyskinesias present with different forms of extrapyramidal hyperkinesias (dystonia, chorea, athetosis, ballism) in variable combinations and with cerebellar signs, respectively. They may be classified as: 1. paroxysmal dystonias/choreoathetoses (paroxysmal dystonic choreoathetosis = PDC), paroxysmal kinesigenic choreoathetosis = PKC, intermediate form) and 2. paroxysmal ataxias (PA) (PA with myokymia and neuromyotonia, azetazolamide-responsive PA). Nocturnal paroxysmal dystonia is now regarded as one form of nocturnal frontal lobe epilepsy. Research in molecular genetics has substantially contributed to the etiologic understanding of paroxysmal dyskinesias: In different families linkage has been successfully completed for PDC (chromosome 2q) and PA (chromosomes 12p, 19p). PA are now identified as channelopathies with mutations in the potassium channel (PA with myokymia and neuromyotonia) and the calcium channel gene (azetazolamide-responsive PA).

Athetosis↗