Jan J. Borremans

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.

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 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.

Extended long-term (> 5 years) outcome of cerebrospinal fluid shunting in idiopathic normal pressure hydrocephalus.

BACKGROUNDShunt surgery has been established as the only durable and effective treatment for idiopathic normal pressure hydrocephalus. OBJECTIVEWe evaluated the “extended” long-term follow-up (> 5 years) in a prospective study cohort who underwent shunting between 1990 and 1995. A secondary objective was to determine the cause of death in these patients. METHODSFifty-one patients were included after confirmation of the diagnosis by extensive clinical and diagnostic investigations. Surgery included ventriculoatrial or ventriculoperitoneal shunting with differential pressure valves in the majority of patients. For each of the cardinal symptoms, postoperative outcome was assessed separately with the Krauss Improvement Index, yielding a value between 0 (no benefit) and 1 (optimal benefit) for the overall outcome. RESULTSMean age at surgery was 70.2 years (range, 50–87 years). Thirty patients were women, and 21 were men. Short-term (18.8 ± 16.6 months) follow-up was available for 50 patients. The Krauss Improvement Index was 0.66 ± 0.28. Long-term (80.9 ± 51.6 months) follow-up was available for 34 patients. The Krauss Improvement Index was 0.64 ±0.33. Twenty-nine patients died during the long-term follow-up at a mean age of 75.8 years (range, 55–95 years). The major causes of death were cardiovascular disorders: cardiac failure (n = 7) and cerebral ischemia (n = 12). Other causes were pneumonia (n = 2), acute respiratory distress syndrome (n = 1), pulmonary embolism (n = 1), cancer (n = 2), renal failure (n = 1), and unknown (n = 3). There was no shunt-related mortality. CONCLUSIONIdiopathic normal pressure hydrocephalus patients may benefit from shunting over the long term when rigorous selection criteria are applied. Shunt-related mortality is negligible. The main cause of death is vascular comorbidity.

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.

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-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.