Acute ischaemic stroke-related choreoathetosis treated with arterial thrombectomy.

A rare case of acute choreoathetosis after acute stroke is presented. This 66-years-old, right-handed Caucasian woman presented with weakness of her right arm and right leg with dysarthria, which resolved by the time she arrived in the emergency department. No obvious focal sign apart from the abnormal choreoathetoid movement of the right arm and leg and of the neck was present. Her medical history included atrial fibrillation without anticoagulation. CT head was nil acute (Alberta Stroke Program Early CT Score of 10). CT angiography of the carotids showed a hyperdense M2 segment of the left middle cerebral artery. Intravenous thrombolysis immediately followed by thrombectomy was decided. Using the Penumbra aspiration device (ACE 68) two clots were removed with two aspirations. A small distal clot remained but partial recanalisation (Thrombolysis in Myocardial Infarction/Thrombolysis in Cerebral Infarction 2b) was achieved. 30 seconds after restoring blood flow, the choreoathetoid movements ceased. The patient was brought to intensive care for further monitoring, which was uneventful.

Neurosurgical treatment of spasticity and other pediatric movement disorders.

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.

Chronic stimulation of the globus pallidus internus for treatment of non-dYT1 generalized dystonia and choreoathetosis: 2-year follow up.

OBJECT: The authors studied the long-term efficacy of deep brain stimulation (DBS) of the posteroventral lateral globus pallidus internus up to 2 years postoperatively in patients with primary non-DYT1 generalized dystonia or choreoathetosis. The results are briefly compared with those reported for DBS in DYT1 dystonia (Oppenheim dystonia), which is caused by the DYT1 gene. METHODS: Enrollment in this prospective expanded pilot study was limited to adult patients with severely disabling, medically refractory non-DYT1 generalized dystonia or choreoathetosis. Six consecutive patients underwent follow-up examinations at defined intervals of 3 months, 1 year, and 2 years postsurgery. There were five women and one man, and their mean age at surgery was 45.5 years. Formal assessments included both the Burke-Fahn-Marsden dystonia scale and the recently developed Unified Dystonia Rating Scale. Two patients had primary generalized non-DYT1 dystonia, and four suffered from choreoathetosis secondary to infantile cerebral palsy. Bilateral quadripolar DBS electrodes were implanted in all instances, except in one patient with markedly asymmetrical symptoms. There were no adverse events related to surgery. The Burke-Fahn-Marsden scores in the two patients with generalized dystonia improved by 78 and 71% at 3 months, by 82 and 69% at 1 year, and by 78 and 70% at 2 years postoperatively. This was paralleled by marked amelioration of disability scores. The mean improvement in Burke-Fahn-Marsden scores in patients with choreoathetosis was 12% at 3 months, 29% at 1 year, and 23% at 2 years postoperatively, which was not significant. Two of these patients thought that they had achieved marked improvement at 2 years postoperatively, although results of objective evaluations were less impressive. In these two patients there was a minor but stable improvement in disability scores. All patients had an improvement in pain scores at the 2-year follow-up review. Medication was tapered off in both patients with generalized dystonia and reduced in two of the patients with choreoathetosis. All stimulation-induced side effects were reversible on adjustment of the DBS settings. Energy consumption of the batteries was considerably higher than in patients with Parkinson disease. CONCLUSIONS: Chronic pallidal DBS is a safe and effective procedure in generalized non-DYT1 dystonia, and it may become the procedure of choice in patients with medically refractory dystonia. Postoperative improvement of choreoathetosis is more modest and varied, and subjective ratings of outcome may exceed objective evaluations.

[A case of chorea-acanthocytosis successfully treated with posteroventral pallidotomy].

We described a 41-year-old man with chorea-acanthocytosis, who presented severe choreoathetosis in all the limbs, neck, face and tongue. Especially, the oral area was affected most strikingly. He had difficulty in eating, because he pushed foods out of the mouth with the tongue. He received left posteroventral pallidotomy (PVP). His involuntary movements became much less enough for the patient to eat with a spoon in a sitting position by himself. Six months after the first operation, although involuntary movement on the right hand remained reduced, choreoballistic movement appeared on the left side. He received PVP on the right side. The choreoballistic movement was remarkably improved after the right PVP. No neurological aggravation has been found for 7 months after the second PVP. Bilateral PVP was fully effective as for the improvement of the activity of daily life. The output from the internal globus pallidus (GPi) is well-known to be inhibitory. Therefore, it is understandable that the coagulation of GPi results in improvement of hypokinesia and rigidity in patients with Parkinson's disease. However, some clinical reports, including ours, have described the significant reduction of hyperkinetic choreoballistic movement after PVP. The exact mechanism of these surgical effects has not been elucidated yet, and further clinical and basic studies are needed to answer this question.

The cervical spine in athetoid cerebral palsy. A radiological study of 180 patients.

We have reviewed the cervical spine radiographs of 180 patients with athetoid cerebral palsy and compared them with those of 417 control subjects. Disc degeneration occurred earlier and progressed more rapidly in the patients, with advanced disc degeneration in 51%, eight times the frequency in normal subjects. At the C3/4 and C4/5 levels, there was listhetic instability in 17% and 27% of the patients, respectively, again six and eight times more frequently than in the control subjects. Angular instability was seen, particularly at the C3/4, C4/5 and C5/6 levels. We found a significantly higher incidence of narrowing of the cervical canal in the patients, notably at the C4 and C5 levels, where the average was 14.4 mm in the patients and 16.4 mm in normal subjects. The combination of disc degeneration and listhetic instability with a narrow canal predisposes these patients to relatively rapid progression to a devastating neurological deficit.

Therapeutic stereotactic procedures on the thalamus for motor movement disorders.

The value of functional neurosurgery in the treatment of motor movement disorders is emphasized. The two methods of stereotactic procedures, namely a destructive one with small lesions centered on specific targets, and a non-destructive one with chronically inserted electrodes connected with an also implanted programmable neuropacemaker are described in detail. The results in Parkinsonian tremor, essential tremor, tremor of multiple sclerosis, post-traumatic tremor and in other involuntary movement disorders are reported and demonstrate that stereotactic neurosurgical treatment of these conditions is a safe and efficacious method.

Image-directed functional neurosurgery with the Cosman-Roberts-Wells stereotactic instrument.

An isocentered system for functional stereotactic procedures with the Cosman-Roberts-Wells frame and a CT localizer that allows extrapolation of target data directly from the CT slice is presented. Based on anatomical landmarks and on the scaled corresponding transverse plates of the Schaltenbrand and Wahren atlas, we delineate the thalamic and cerebellar nuclei. Twenty three image-directed functional procedures were performed in one year on 18 patients (7 with Parkinson's disease, 4 with dystonia, 3 persons with essential tremor, 2 patients with choreo-athetosis and 2 with de-afferentiation pain). The 23 procedures included 19 thalamotomies, two dentatotomies and two stereotactic implantations of deep seated brain electrodes. Successful targeting was verified by intra-operative electrical stimulation and postoperative CT scan. Complete reduction of symptoms was observed in 4 persons with Parkinson's disease and in 2 patients with essential tremor with significant improvement observed in the rest of the patients with the exception of the individual with choreo-athetosis. There were no operation-related complications. The reported technique is safer and less distressing for patients than previous radiological procedures and it makes image-directed stereotactic functional neurosurgery available to many units with the CRW frame.

[Results of hand surgery operations in spastic-athetotic paresis]. / Ergebnisse handchirurgischer Operationen bei spastisch-athetotischen Paresen.

Between 1974 and 1986 a total of 123 patients with a spastic or spastic athetotic paresis of the upper limb underwent surgery. The interval between surgery and follow-up examination was between one and 13 years. There were 73 cases of hemiparesis and 50 dipareses or tetrapareses due to perinatal cerebral paresis and 35 cases due to a variety of causes. The patients were aged between 6 and 58 years, the majority between 8 and 28 years. All contractures in the arm and hand region were treated at a single sitting. The sole exception to this was surgery for swan-neck deformity of the long fingers. In none of the cases was a wrist arthrodesis indicated. As regards the elimination of the previously existing malpositions, some of which were severe, and the cosmetic outcome, the results were good in all cases. The postoperative reduction was also preserved through the subsequent years, until completion of growth. Also, the difference in growth between flexors and extensors had no detectable negative influence on the long-term results of surgery. Only in a few isolated cases was limited revisional surgery necessary to improve the result as regards extension in the elbow joint and the posture of the wrist joint, which it had not been possible to treat satisfactorily at the first sitting. Two patients with a pronounced athetotic component manifested unsatisfactory results in several respects, or overcorrection of extension in the wrist joint: special caution is called for here. As far as necessary, corresponding corrective surgery was performed simultaneously on the lower limbs.

[Use of the microelectrode method for differentiation of white matter from thalamic nuclei during stereotaxic procedures]. / Ispol'zovanie mikroélektrodnogo metoda dlia differentsirovaniia belogo veshchestva i iader talamusa pri stereotaksicheskikh operatsiiakh.

Two hundred and three neurograms were recorded from the ventrolateral and reticular nuclei of the thalamus in 29 patients. Pulsed neuron activity was encountered in 116 cases. Reactions to motor tests were recorded from 26 neurons. The authors constructed maps of the location of the cerebral areas studied in relation to the main intracerebral guiding points. A large number of cells (44%) with rhythmic pulsed discharges with a frequency of 3-7 Hz were found in patients with parkinsonism and double athetosis. A great variability of background and induced activity of the thalamic nuclei under similar conditions of study was revealed.

Primary and revision surgery (selective neurectomy) for facial hyperkinesia.

Symptomatic relief for hyperkinetic movements of facial musculature can be obtained by selective neurectomy of the peripheral divisions of the facial nerve with preservation of the frontal branch. Hemifacial spasm and blepharospasm represent the most common forms of facial hyperkinesia. Satisfactory results using the described technique on 191 patients were achieved in 74% of the patients with hemifacial spasm and blepharospasm in the follow-up period of one to 12 years. In hemifacial spasm, 80% were relieved after a single operation, and 95% were relieved after revision surgery. There were no major postoperative complications. Minor complications included insufficient lid closures in 1.5% of the patients and delayed wound healing with minor salivary fistulas that closed spontaneously in 7% of the patients. Resection of all fibers innervating the orbicularis oculi muscle is essential in preventing recurrence.

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.

[Effect of destruction of the ventrolateral nucleus of the thalamus on the motor analyzer]. / Vliianie destruktsii ventrolateral'nogo iadra talamusa na dvigatel'nyi analizator.

The author examined 100 patients with parkinsonism, Wilson-Konovalov's hepatocerebral dystrophy, atherosis, torsion dystonia and Huntington's chorea. The brain biopotentials were recorded with the use of scalp, cortical and deep leads. Global and stimulation electromyography, as well as chronaximetry were performed. Functional reorganization in the motor analyser was demonstrated which corresponded to the clinical therapeutic effect of stereotaxic operations.

[Therapy of ballistic hyperkinesia (case report)]. / Zur Therapie ballistischer Hyperkinesen (Falldarstellung).

Discussing the example of two cases of patients suffering from mixed choreic-hemiballistic hemi-hyperkinesia, the authors report on successes they obtained in long-term therapy after surgical stereostactic treatment (high-frequency coagultation) in the thalamus or subthalamus. The discussion deals with indications of the etiology, the supposed pathophysiology and pathomorphology of the disease pictures in question.

Long term results of stereotaxic surgery for infantile dystonia and dyskinesia.

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.