Cerebral arteriovenous malformations and movement disorders.

A series of six patients with movement disorders associated with cerebral arteriovenous malformations (AVM) is reported. The AVMs were classified according to the Spetzler-Martin classification as grade V (one patient), grade IV (four patients), and as grade III (one patient). One patient had action-induced hemidystonia caused by a contralateral frontoparietal AVM which compressed the putamen and was supplied partially by enlarged lenticulostriate arteries. Two patients presented with unilateral cortical tremor associated with contralateral high-frontal cortical/subcortical AVMs sparing the basal ganglia. Another patient developed hemidystonia and hemichorea-hemiballism after bleeding of a contralateral temporooccipital AVM and subsequent ischemia. Two patients had focal dystonia after thalamic and basal ganglia hemorrhage from AVMs. Five patients were operated on. The movement disorder was abolished in one patient postoperatively. Different mechanisms were identified that are relevant for the development of AVM-related movement disorders: mass effect, diaschisis, local parenchymal altered cerebral blood flow, and hemorrhagic or ischemic structural lesions.

Hemichorea and hemiballism associated with contralateral hemiparesis and ipsilateral basal ganglia lesions.

We report on two patients with unilateral hyperkinetic movement disorders associated with contralateral hemiparesis and ipsilateral basal ganglia lesions. The first patient, a 47-year-old woman, had a low-grade astrocytoma located in the right basal ganglia extending into the subthalamic area and the cerebral peduncle. She presented with left hemiparesis, right hemichorea, and intermittent right-sided tremor at rest. The second patient, a 85-year-old woman, had hypertensive hemorrhage to the right posterior basal ganglia, the posterior limb of the internal capsule, the lateral thalamus, and the subthalamic region with accompanying intraventricular bleeding. She developed right-sided transient hemichorea-hemiballism. A videotape illustration of one of the patients is provided. The literature on the rare occurrence of ipsilateral hemichorea-hemiballism is discussed and possible pathomechanisms are reviewed. We postulate that hemiparesis contralateral to basal ganglia lesions might have a conditioning effect on the appearance of ipsilateral dyskinetic movement disorders.

Movement disorders following nonfunctional neurosurgery.

OBJECT: Knowledge is scarce about movement disorders that follow neurosurgical operations other than functional stereotactic surgery. The cases of 14 patients who suffered from movement disorders secondary to craniocerebral or spinal surgery are analyzed. None of these patients was initially treated by any of the authors. METHODS: Twelve patients underwent surgery for cerebral diseases. Nine of these patients harbored tumors and three patients had neurovascular disorders. Two patients underwent spinal surgery for cervicothoracic ependymoma or for multiple cervical disc herniations. Twelve of the 14 patients had immediate postoperative side effects such as hemiparesis, ataxia, and somnolence. In all but two patients, movement disorders became manifest only after a delay. Dystonic movement disorders developed in eight patients, unilateral tremors in three patients, unilateral facial myokymia in one patient, and hemichorea-hemiballism in two patients. The mean delay of onset for tremor was 5 weeks and that for dystonic movement disorders was 5.5 months. Movement disorders were transient in three patients; however, they were persistent in 11 patients at a mean follow-up period of 5 years. These movement disorders caused marked persistent disability in four patients. Lesions of the contralateral striatum were identified in patients with dystonic syndromes and lesions of the dentatothalamic outflow in patients with tremors. In three patients who had postoperative basal ganglia lesions after partial removal of astrocytomas, tumor regrowth was later documented. Medical treatment in patients with persistent movement disorders rendered only limited benefit. Two patients improved with botulin injections. In one patient postoperative hemidystonia was alleviated by contralateral thalamotomy. CONCLUSIONS: Dystonic syndromes and tremors are the most common movement disorders that occur after craniocerebral and spinal surgery. Postoperative movement disorders can lead to various degrees of functional disability. The pathoanatomical correlations are similar to those described in other patients with secondary movement disorders.

Movement disorders in adult hydrocephalus.

In a prospective series of symptomatic adult hydrocephalus characterized by gait disturbance, cognitive impairment, and/or urinary incontinence, 88 of 118 patients (75%) had additional akinetic, tremulous, hypertonic, or hyperkinetic movement disorders. Their prevalence was highest in patients with idiopathic normal pressure hydrocephalus (NPH) of the elderly (56/65 patients, 86%), and they were less frequent in patients with secondary NPH (10/15, 66%), with nonhydrodynamic atrophic/other hydrocephalus (20/33, 61%), and with obstructive hydrocephalus/aqueductal stenosis (2/5, 40%). Akinetic symptoms were found in 73 of 118 patients (62%), and the most frequent movement disorder was upper extremity bradykinesia (55%). Akinetic, tremulous, hypertonic, and hyperkinetic movement disorders were exclusively secondary to causes not related to hydrocephalus in 24 of 118 patients (20%). The proportion of patients with movement disorders not attributable to only such causes was highest in the idiopathic NPH group (44/65, 68%). Thirteen of 118 patients (11%) presented with a parkinsonian syndrome. There was evidence for coexistent Parkinson's disease in four of these patients. Parkinsonism was found to be secondary to NPH in five patients and was found improved after shunting. Akinetic symptoms in patients with NPH generally responded favorably to CSF diversion, which was evident in 80% of a subset of this group. Various other movement disorders did not show definite improvement. The high prevalence of bradykinesia and other akinetic symptoms in NPH and the beneficial effect of shunting on such symptoms suggest that NPH may cause a more generalized disorder of motor function.

Cerebrospinal fluid shunting in idiopathic normal-pressure hydrocephalus of the elderly: effect of periventricular and deep white matter lesions.

OBJECTIVE: We investigated the effect of periventricular and deep white matter lesions (DWMLs) on outcome after cerebrospinal fluid shunting in a prospective series of elderly patients with idiopathic normal-pressure hydrocephalus. METHODS: White matter lesions were assessed with T2-weighted magnetic resonance scans according to a standard protocol in 41 patients with idiopathic normal-pressure hydrocephalus of the elderly who underwent subsequent shunting. In all patients, the diagnosis of idiopathic normal-pressure hydrocephalus had been established preoperatively by clinical and diagnostic investigations. RESULTS: At a mean follow-up of 16 months, clinical improvement was observed in 37 of 41 patients (90%). There was no persistent morbidity related to surgery. The degree of overall clinical improvement was negatively correlated with the extension of periventricular lesions (correlation coefficient r = -0.324 [P = 0.04]) and DWMLs (correlation coefficient r = -0.373 [P = 0.02]). This negative correlation was also noted when the analysis was conducted separately for each of the cardinal symptoms (gait disturbance, cognitive impairment, and urinary incontinence). There was no consistent pattern of periventricular and DWMLs in the four patients who failed to respond to shunting. CONCLUSION: Periventricular and DWMLs of varying degrees are common findings on magnetic resonance scans of patients with idiopathic normal-pressure hydrocephalus of the elderly. After careful preoperative selection of patients with idiopathic normal-pressure hydrocephalus, individuals with DWMLs suggestive of concomitant vascular encephalopathy may also benefit from cerebrospinal fluid diversion. Although, in general, the degree of improvement depends on the severity of periventricular and DWMLs, patients with more extensive WMLs still may derive clinical benefit from the operation.

Hemichorea-hemiballism associated with an ipsilateral intraventricular cyst after resection of a meningioma.

We report a patient who developed hemichorea-hemiballism ipsilateral to an intraventricular cyst after resection of a meningioma of the lateral ventricle while under medication with phenytoin. Magnetic resonance imaging demonstrated distortion of the upper brainstem and diencephalon compatible with functional involvement of the contralateral subthalamic nucleus. The movement disorder disappeared after drainage of the cyst. Hemichorea and hemiballism have been reported to be associated with ipsilateral cerebral lesions in rare cases. Review of the literature raises some doubt about the validity of assumptions drawn from previous reports. There is little evidence that hemichorea-hemiballism may be caused by an isolated ipsilateral lesion.

Vascular risk factors and arteriosclerotic disease in idiopathic normal-pressure hydrocephalus of the elderly.

BACKGROUND AND PURPOSE: There is some evidence from previous studies that idiopathic normal-pressure hydrocephalus (NPH) of the elderly might be linked to vascular leukoencephalopathy. The purpose of this study was to examine the prevalence and impact of vascular risk factors and vascular diseases in idiopathic NPH compared with a control cohort. METHODS: The prevalence of arterial hypertension; diabetes mellitus; hypercholesterolemia; hyperlipidemia; smoking; obesity; and cardiac, cerebrovascular, and other arteriosclerotic diseases was assessed in 65 patients with idiopathic NPH. The findings were compared with those of 70 patients with comparable age distribution. To describe the differences of the prevalences of vascular risk factors, odds ratios were obtained by univariate and multivariate analyses. RESULTS: The univariate analysis revealed significant associations between idiopathic NPH and arterial hypertension (prevalence, 54 of 65 [83%]; control group, 25 of 70 [36%]; P < .001) and diabetes mellitus (prevalence, 31 of 63 [49%]; control group, 20 of 70 [29%]; P < .015) but not with other vascular risk factors. After multivariate regression analysis, only hypertension remained significantly associated with NPH (P < .0001). There was also a significant association between NPH and cardiac (P < .001), cerebral arteriosclerotic (P = .007), and other arteriosclerotic diseases (P = .001). A positive association was found between the severity of clinical symptoms of NPH and the presence of hypertension, especially for gait disturbance. The presence of hypertension was not related to the duration of NPH. CONCLUSIONS: Our data show a highly significant association between idiopathic NPH and arterial hypertension. Arterial hypertension might be involved in the pathophysiological mechanisms promoting idiopathic NPH.

Ballism not related to vascular disease: A report of 16 patients and review of the literature.

Sixteen patients with ballism not related to vascular disease are reported. Ballism was caused by subthalamic metastases and cerebral tumours in four patients, lesions after functional stereotaxy in three, presumed neurodegenerative disease in two, and by an ipsilateral intraventricular cyst after resection of a meningioma, cerebral toxoplasmosis with AIDS, severe head trauma and sepsis, late recurrence of rheumatic fever, meningoencephalitis, perinatal hypoxia, and conversion syndrome in one, respectively. Two patients had bilateral ballism, 11 had hemiballism, and three had monoballism. Involvement of the contralateral subthalamic nucleus was found in 10/13 patients with symptomatic unilateral ballism. One patient with presumed neurodegenerative disease had bilateral alterations of caudate and putamen on MR. The effect of different treatment strategies was evaluated. Treatment was directed to the underlying disease and/ or to the movement disorder. Response to pharmacotherapy was poor except in one patient. Five patients underwent various neurosurgical interventions. Functional stereotactic operations were performed in eight patients. Lesions were placed in the contralateral ventrolateral thalamus and the zona incerta, the internal pallidum, and in the zona incerta and the pulvinar thalami. Four out of eight patients had complete sustained relief of hemiballism after the operation. No patient was lost to follow-up, which ranged from 3 months to 27 years. After various therapeutic strategies ballism was no longer present in 10 patients and had improved in three, while another three patients did not benefit from therapy. Review of the literature illustrates the shift of aetiologic factors over decades underlying this rare symptom. A multidisciplinary approach should be considered in these patients to alleviate the severe and disabling movement disorder.