Disappearance of hemichorea as the initial presentation of moyamoya disease after revascularization surgery regardless of residual hypoperfusion evaluated by quantitative N-isopropyl-p-123I-iodoamphetamine SPECT.

BACKGROUND AND IMPORTANCE: Although hypoperfusion of the basal ganglia or the frontal subcortical matter is suspected, the pathology of chorea in moyamoya disease remains unclarified. Herein, we report a case of moyamoya disease presenting with hemichorea and evaluate pre- and postoperative perfusion using single photon emission computed tomography with N-isopropyl-p-123I-iodoamphetamine (123I-IMP SPECT). CLINICAL PRESENTATION: An 18-year-old woman presented with choreic movement of her left limbs. Magnetic resonance imaging revealed an ivy sign, and 123I-IMP SPECT demonstrated decreased cerebral blood flow (CBF) and cerebral vascular reserve (CVR) values in the right hemisphere. The patient underwent direct and indirect revascularization surgery to improve cerebral hemodynamic impairment. The choreic movements entirely resolved immediately after surgery. Although CBF and CVR values in the ipsilateral hemisphere demonstrated by quantitative SPECT increased, these did not reach the normal values threshold. CONCLUSION: Choreic movement in moyamoya disease may be related to cerebral hemodynamic impairment. Further studies are required to elucidate its pathophysiological mechanisms.

Medical Management of Movement Disorders.

Pharmacological treatment is the cornerstone in the management of movement disorders. Although most available treatment options have no impact on the underlying process of each movement disorder, symptomatic therapies can significantly improve patient's quality of life and level of disability. Here, we review the current knowledge on clinical symptomatic management of Parkinson's disease (both early and advanced stages), essential tremor, dystonia, and chorea. Ideally, treatment should be carried out by specialists with reasonable experience in movement disorders, as it needs to be tailored for each patient depending on several appraisals, including but not limited to patients' needs, compliance issues, potential side effects, caregiver support, and presence of comorbidities. When medications fail to improve patient's disability, stereotactic surgery is a well-established option for most of these disorders.

The Pioneering and Unknown Stereotactic Approach of Roeder and Orthner from Göttingen. Part II: Long-Term Outcome and Postmortem Analysis of Bilateral Pallidotomy in the Pre-Levodopa Era.

Before the advent of levodopa, pallidotomy was initially the most effective treatment for Parkinson disease, but it was soon superseded by thalamotomy. It is widely unknown that, similar to Leksell, 2 neurologists from Göttingen, Orthner and Roeder, perpetuated pallidotomy against the mainstream of their time. Postmortem studies demonstrated that true posterior and ventral pallidoansotomy sparing the overwhelming mass of the pallidum was accomplished. This was due to a unique and individually tailored stereotactic technique even allowing bilateral staged pallidotomies. In 1962, the long-term effects (3-year follow-up on average) of the first 18 out of 36 patients with staged bilateral pallidotomies were reported in great detail. Meticulous descriptions of each case indicate long-term improvements in parkinsonian rigidity and associated pain, as well as posture, gait, and akinesia (e.g., improved repetitive movements and arm swinging). Alleviation of tremor was found to require larger lesions than needed for suppression of rigidity. No improvement in speech, drooling, or seborrhea was observed. By 1962, the team had operated 13 patients with postencephalitic oculogyric crises with remarkable results (mean follow-up: 5 years). They also described alleviation of nonparkinsonian hyperkinetic disorders (e.g., hemiballism and chorea) with pallidotomy. The reported rates for surgical mortality and other complications had been remarkably low, even if compared to those reported after the revival of pallidotomy by Laitinen in the post-levodopa era. This applies also to bilateral pallidotomy performed with a positive risk-benefit ratio that has remained unparalleled to date. The intricate history of pallidotomy for movement disorders is incomplete without an appreciation of the achievements of the Göttingen group.

Chorea in the clinical presentation of moyamoya disease: results of surgical revascularization and a proposed clinicopathological correlation.

OBJECT: Chorea is a movement disorder characterized by brief, irregular, involuntary contractions that appear to flow from 1 muscle to another. There are a limited number of reports in the literature that have linked moyamoya disease and chorea. The authors describe their experience in treating moyamoya disease in patients in whom chorea developed as part of the clinical presentation. METHODS: The authors conducted a retrospective review of a consecutive series of 316 children who underwent pial synangiosis revascularization for moyamoya disease at the Boston Children's Hospital. RESULTS: Of 316 surgically treated patients with moyamoya disease, 10 (3.2%; 6 boys and 4 girls) had chorea as a part of their presentation. The average age at surgical treatment was 9.9 years (range 3.8-17.9 years). All patients had evidence of hypertrophied lenticulostriate collateral vessels through the basal ganglia on preoperative angiography and/or MRI on affected sides. Two patients had cystic lesions in the basal ganglia. Nine patients underwent bilateral craniotomies for pial synangiosis, and 1 patient underwent a single craniotomy for unilateral disease. Follow-up was available in 9 patients (average 50.1 months). The mean duration of chorea was 1.36 years (range 2 days to 4 years), with resolution of symptoms in all patients. One patient developed chorea 3 years after surgical treatment, 4 patients had transient chorea that resolved prior to surgery, and 5 patients experienced resolution of the chorea after surgery (average 13 months). CONCLUSIONS: The authors describe children with moyamoya disease and chorea as part of their clinical presentation. The data suggest that involvement of the basal ganglia by the hypertrophied collateral vessels contributes to the development of chorea, which can wax or wane depending on disease stage or involution of the vessels after revascularization surgery. In most patients, however, the chorea improves or disappears about 1 year after presentation.

Hemi-chorea: an unusual presentation of brainstem glioma.

Brainstem gliomas are neoplasms primarily affecting children. Depending on the type, they usually present with varying combination of multiple bilateral or unilateral cranial nerve palsies, long-tract signs, ataxia and sometimes hydrocephalus. Though movement disorders have been reported in association with basal ganglia and thalamic tumours, hemi-chorea, as a presentation of brainstem glioma, has not been reported till date. I present a case of a 9-year-old female child, who presented with complaints of difficulty in walking and involuntary movements affecting the left upper and lower limb. On examination, she had chorea involving left side of the body, bilateral lateral rectus palsy, with spasticity of right upper and lower limb. CT scan and MRI of the brain showed focal glioma involving the upper pons and midbrain. The patient underwent surgery in the form of sub-occipital craniotomy and tumour removal. Postoperative imaging studies showed no residual tumour. Histopathological examination was suggestive of pilocytic astrocytoma grade 1. Postoperatively hemi-chorea decreased in intensity, but did not disappear completely.

Posteroventral pallidotomy in medically intractable postapoplectic monochorea: case report.

BACKGROUND: Posteroventral pallidotomy is a widely accepted surgical procedure for treating medically intractable Parkinson's disease and Levo-dopa induced dyskinesia. In the surgical treatment of hyperkinetic movement disorders, generalized dystonia has recently become a favorable indication of posteroventral pallidotomy. However, a commonly recognized surgical procedure for treating choreiform movement disorders has not yet been established. Here we present an unusual experience of a posteroventral pallidotomy performed to treat a medically intractable monochorea caused by a vascular insult on the basal ganglia. METHODS: A 63-year-old female presented with choreiform movement of the left upper limb that she had suffered for 5 months. She was found to have a hemorrhagic infarction in the right putaminal area. No other abnormal lesions were shown by magnetic resonance imaging except for a widening of the right cerebellopontine cistern because of an acoustic neurinoma removed 5 years previously. Despite medication with a dopamine antagonist, choreiform movement of the left limb had not improved, and the patient complained of rigidity and slowness of ambulation owing to the side effects of the medicine. A right posteroventral pallidotomy was performed with macrostimulation for a physiologic confirmation of the globus pallidus internus (GPi), which is the conventional target for Parkinson's disease. After coagulating the GPi target, the choreiform movement of the contralateral upper limb was completely abolished. RESULTS: The postoperative course was uneventful and no recurrence of chorea was observed over a follow-up period of 6 months. CONCLUSIONS: Stereotactic surgery for hyperkinetic movement disorders is not as common a procedure as that used for treating Parkinson's disease. Furthermore, there have been few reports of pallidal surgery for treating the chorea caused by an ischemic insult. However, on the basis of the current concept that varying types of hyperkinetic disorders may have a common pathophysiological mechanism, a posteroventral pallidotomy may be an alternative surgical procedure for treating medically intractable postapoplectic chorea like in an occasion of dystonia.

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.

Hemichorea as an initial manifestation of moyamoya disease: reversible striatal hypoperfusion demonstrated on single photon emission computed tomography.

We describe a case with unilateral moyamoya disease that showed progressive hemichorea as an initial manifestation. Single photon emission computed tomography showed perfusion defect in the contralateral basal ganglia although magnetic resonance imaging was unremarkable. Hemichorea improved along with normalization of perfusion after bypass surgery, suggestive of striatal hypoperfusion as the cause of hemichorea.

Use of selective thalamotomy for various kinds of movement disorder, based on basic studies.

Methods of selective thalamotomy with microrecording for several kinds of movement disorder are described. Precise thalamotomy (Vim, VO or Vim+VO) depends on the understanding of the functional organization of the human thalamus on the one hand, and the patient's specific feature of movement disorders on the other. To realize selective thalamotomy, microrecording and a computerized planning system are essential. Thus, a properly selected thalamotomy for movement disorders is quite useful.

Thalamotomy for the alleviation of levodopa-induced dyskinesia: experimental studies in the 1-methyl-4-phenyl-1,2,3,6-tetrahydropyridine-treated parkinsonian monkey.

This work set out to test the hypothesis that thalamotomy in the area of the thalamus which receives the input from the medial segment of the globus pallidus would decrease or prevent levodopa-induced dyskinesia. Peak dose dyskinesia is a major problem in the treatment of parkinsonian patients with levodopa therapy but this remains the best pharmacological agent for treating the condition. The hypothesis was derived from previous work which has suggested that reduced pallidal inhibition of the thalamus results in dyskinesia [Crossman (1990) Movement Dis. 5, 100-108]. A neuroanatomical tracing study was carried out prior to the thalamotomy work, using the anterograde tracer wheatgerm-agglutinin conjugated to horseradish peroxidase. This delineated the anterior part of the ventrolateral thalamus in the primate in terms of its afferent inputs. Wheatgerm agglutinin-horseradish peroxidase was injected into the medial segment of the globus pallidus bilaterally in three Macaca fascicularis and traced to terminals in the ventral thalamus and other brain areas. The appropriate thalamic area involved was plotted on atlas sections in preparation for stereotactic thalamotomy. Previous studies of neuronal input to the ventral thalamus are confusing due to the different nomenclatures used by different workers. Early workers used cytoarchitectonic boundaries which do not correspond with function. There are also differences in nomenclature between man, monkey and other animals. The current study maps the pallidal terminal territory within the thalamus in terms of stereotactic co-ordinates related to a published macaque atlas [Shantha et al. (1968) A Stereotaxic Atlas of the Java Monkey Brain. S. Karger, Basel] and can thus be used by other workers in the field. A well-established primate model of Parkinsonism was used for the thalamotomy study. Eight monkeys (Macaca fascicularis) were rendered parkinsonian with 1-methyl-4-phenyl-1,2,3,6-tetrahydropyridine. Regular dosing with levodopa or apomorphine reliably resulted in peak-dose dyskinesia which was scored in terms of its choreic and dystonic components. A radiofrequency electrode was used to create the ablative lesions. Chorea was always reduced and frequently abolished by a thalamotomy located in the pallidal terminal territory. This result was obtained after 10 thalamotomies in a total of six animals. Four animals received bilateral lesions, with an interval between operations and two animals underwent unilateral surgery.(ABSTRACT TRUNCATED AT 400 WORDS)

Periventricular hydatid cyst presenting with hemichorea.

A case of periventricular hydatid cyst presenting with only hemichorea is reported. The unusual clinical course is described.

Bilateral ballismus in children.

Bilateral ballismus is extremely rare. We found 23 reported cases, and only 2 of these were in children. In older patients the movement disorder is usually due to cerebrovascular disease, but in younger patients a variety of aetiopathological causes may be found. There are few data regarding medical treatment. There have been no previous reports on stereotactic operations. We report on two severely disabled children who underwent stereotactic surgery. A 9-year-old boy suffering from bilateral ballismus after meningoencephalitis was operated on bilaterally (two operations 1 year apart). Another 9-year-old boy, who was suffering from progressive, presumably degenerative, basal ganglia disease, was operated on unilaterally. The nosological and conceptual controversies differentiating bilateral ballismus as a phenomenological entity are reviewed. The therapeutic options, indications, and special problems of stereotactic surgery in these rare cases are discussed.

Neurosurgical treatment of the dyskinesias.

I have attempted to review what I consider to be the principal conclusions of a 25 year study of the neurosurgical treatment of the dyskinesias. The concept that the pathological disinhibition which is responsible for the production of these syndromes can be modified by destructive lesions within the thalamus or by prosthetic mobilization of inhibitory mechanisms of the cerebellum is supported by the results presented in this report. Neurosurgical treatment of the dyskinesias is potentially a relatively safe and efficacious means of alleviating incapacitation of a large group of patients. However, meticulous attention to patient selection as well as adherence to the general principles of surgical technique which have been reviewed are essential if the full potential of these approaches is to be realized.

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.