Joachim K. Krauss

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.

The relation of intracranial pressure B-waves to different sleep stages in patients with suspected normal pressure hydrocephalus

SummaryThe interpretation of data from continuous monitoring of intra-cranial pressure (ICP) in patients with suspected normal pressure hydrocephalus (NPH) is the subject of controversy. Despite the fact that overnight ICP monitoring is widely used for the diagnosis of NPH, normative criteria are poorly defined. The present study demonstrates that there is a relationship between the relative frequency, the absolute amplitude, the wavelength and the morphology of B-waves and different sleep stages.Intraventricular intracranial pressure was recorded continuously overnight in 16 patients with suspected normal pressure hydrocephalus. Simultaneous polysomnography was performed to investigate the relation of spontaneous ICP oscillations to different sleep stages. A correlative analysis was done with the data of 13 patients. Three patients were excluded, one who was awake throughout the night and two in whom polysomnography was incomplete due to technicai reasons. The mean resting cerebrospinal fluid (CSF) pressure was 12.87 cm CSF. B-waves were observed in the ICP recordings of all patients. They were present for a mean of 72% of the total recording time. The relative frequency of B-waves was higher during REM sleep and sleep stage 2 as compared to wakefulness (87.8% and 83.2% vs. 56, p < 0.05). The absolute amplitude was higher during REM sleep than in wakefulness (9.56 vs. 3.44 cm CSF, p < 0.05). Wavelengths were longer in REM sleep than in wakefulness and stages 1 and 2 (62.4 vs. 42, 40.7 and 44.8 sec, p < 0.05). The morphology of B-waves was also related to different sleep stages. Ramp-type B-waves were associated with REM sleep in six patients, however, were also present in sleep stage 2 in three of them.Knowledge of the relation of spontaneous ICP oscillations to different sleep stages may help to establish physiological foundations and alterations. Furthermore, polysomnography may be useful to avoid erroneous interpretation of ICP recordings due to sleep stage related variability.

CT-stereotactic fibrinolysis of spontaneous intracerebral hematomas

CT-stereotactic fibrinolysis is an effective alternative to surgical and conservative therapies for intracerebral hematoma. The method consists of stereotactically puncturing and partially evacuating the hematoma. After fibrinolysis using urokinase, the residual hematoma is completely evacuated through a catheter inserted in the cavity of the hematoma. The operation is usually performed under local anesthesia. Stereotactic methods are safer and less invasive than other methods. Since October 1985, a total of 85 patients have been treated with this method in the Department of Stereotaxy and Neuronuclear Medicine at the University of Freiburg Medical School. Although 25 patients died (29.4%) during the mean follow-up period of 20 months, only 16 (18.8%) died in the acute postoperative phase or within the first 60 days after evacuation. Eighteen patients (21.2%) had died six months after the operation. The quality of life of the 60 surviving patients, as measured on the Karnofsky Scale at follow-up, was very good to good in 70% and moderate in 23.3%. Only 6.7% of the patients were so disabled that they required special care and assistance or had to be placed in a nursing home. The long-term results are thus very encouraging.

Parkinsonism and rest tremor secondary to supratentorial tumours sparing the basal ganglia.

SummaryIntracranial neoplasms are an uncommon cause of symptomatic Parkinsonism and rest tremor. We found an incidence of 0.3% in a prospective evaluation of 907 patients with supratentorial tumours. Eight patients with Parkinsonism and rest tremor secondary to supratentorial tumours sparing the basal ganglia are reported.Neuro-imaging revealed compression and distortion of the basal ganglia by large tumours which were identified histopathologically as meningiomas in four patients and as an epidermoid, a fibrillary astrocytoma, an anaplastic oligodendroglioma and a glioblastoma.Six patients underwent tumour removal by craniotomy, in two the histopathology was obtained by stereotactic biopsy. Four patients were free of Parkinsonian symptoms and signs on long-term follow-up.The possible pathophysiological mechanisms involved are discussed. Since some of these patients closely resemble cases of idiopathic Parkinsons disease, and the movement disorder can precede other symptoms and signs or will remain isolated in the further course, the diagnosis of an intracranial neoplasm was generally delayed in these patients. Increased awareness of this rare entity may lead to an earlier diagnosis. Early computed tomography in patients with Parkinsonism might help to detect these patients with a potentially curable cause of their condition.

Hemidystonia due to a contralateral parietooccipital metastasis Disappearance after removal of the mass lesion

A patient presented with left-sided hemidystonia. CT revealed a contralateral parieto-occipital mass lesion compressing the basal ganglia, which were spared by the mass. After microsurgical resection of the tumor, which was verified histologically as a metastasis of a large-cell anaplastic carcinoma, the movement disorder dissolved completely.A patient presented with left-sided hemidystonia. CT revealed a contralateral parieto-occipital mass lesion compressing the basal ganglia, which were spared by the mass. After microsurgical resection of the tumor, which was verified histologically as a metastasis of a large-cell anaplastic carcinoma, the movement disorder dissolved completely.

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.

Hemifacial spasm due to posterior fossa tumors: The impact of tumor location on electrophysiological findings

Ephaptic transmission is one of the electrophysiological hallmarks of hemifacial spasm. It is generally accepted that in the majority of patients with idiopathic hemifacial spasm, microvascular compression of the facial nerve at the site where the nerve exits the brain stem is the underlying cause. Whether the actual site of the ephapse is at the site of the lesion or at a nuclear level due to hyperexcitability of the facial motor nucleus is still controversial. Rarely, hemifacial spasm may be due to space occupying lesions in the cerebellopontine angle or in the brain stem. We report the electrophysiological findings of four patients with hemifacial spasm due to extra-axial tumors in different locations of the posterior fossa. The location of the tumor was intrameatal in one patient, in the cerebellopontine angle in two patients and in the brain stem in another patient. Facial nerve motor neurographies including transcranial magnetic stimulation revealed abnormal findings in two patients. Selective stimulation of facial nerve branches demonstrated delayed (ephaptic) responses in all but one patient whose hemifacial spasm had disappeared after treatment with carbamazepine. The latencies of the delayed responses did not correlate with the tumor location. In sum, the site of ephaptic transmission cannot be reliably determined by latency measurements of the delayed response because of its variability which is probably caused by the different size and diameter of the axons participating in ephaptic transmission as well as by the extent of focal demyelination at the site of the lesion. A neuroradiological work up including MR imaging should be mandatory in all patients with hemifacial spasm because electrophysiological studies fail to differentiate between idiopathic and symptomatic hemifacial spasm.

Subdural hematoma as a cause of contralateral dystonia

A 72-year-old patient was operated on for a right-sided chronic subdural hematoma. After removal of the drain he became somnolent and developed dystonia of the left arm. Computerised tomography demonstrated a new right-sided subdural hematoma with compression of the right hemisphere and a midline shift to the contralateral side. Within hours after evacuation of the recurrent hematoma the dystonia disappeared. Rapid development and reversal is unusual in secondary dystonia. Mechanical compression, altered vascular supply, and metabolic changes may underlie the pathophysiology of this rare symptomatic movement disorder.

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.