Magdalena Wójcik-Pędziwiatr

Small volume of the posterior cranial fossa and arterial hypertension are risk factors of hemifacial spasm

OBJECTIVES So far, there are only two studies evaluating the relation between the small volume of the posterior cranial fossa (VPCF) and the occurrence of HFS, both on Asian population. The aim of the study was to determine small VPCF and arterial hypertension (AH), as risk factors for hemifacial spasm (HFS) and their relation to neurovascular conflict (NVC) in Polish Caucasian-origin patients. MATERIALS AND METHODS The study included 60 patients with idiopathic HFS and 60 healthy volunteers matched by sex and age. AH was defined according to WHO. The VPCF measured the volume of the prepontine, prespinal and both cerebellopontine angle cisterns in MRI scans. RESULTS There were no significant differences between occurrence of AH and the VPCF of patients and controls but the mean VPCF in women was significantly smaller than in men, In the multivariate regression analysis model only NVC was the statistically significant. In the subgroup of >50-year-old patients the most dominant risk factor was NVC (OR 71.09; 95% CI 21.08-239.77; p=0.0000), followed by the AH duration (OR 1.07; 95% CI 1.00-1.16; p=0.047). In the subgroup of <50 years, NVC was also the dominant risk factor, followed by the lower VPCF (Walad test: OR 0.4; 95% CI 0.16-1.04; p=0.045). CONCLUSION There was no significant difference in VPCF and in frequency of AH diagnosis in HFS patients and age- and sex-related controls, but the logistic regression analysis showed that small VPCF and AH duration are risk factors of HFS in younger and older patients respectively.

Saccadic eye movements in juvenile variant of Huntington disease

BACKGROUND AND PURPOSE Huntington disease (HD) is a neurodegenerative disease leading to involuntary movements, cognitive and behavior decline. The juvenile variant of HD (JHD) manifests in people younger than 21 and is characterized by a different clinical presentation, i.e. rigidity and bradykinesia. Rapid eye movements were not extensively studied in patients with JHD. Aims of our study were to describe the saccadic eye movements in JHD patients and to find a correlation between the saccade abnormalities, severity of the disease and cognitive and behavior deterioration. MATERIALS AND METHODS We studied 10 patients with JHD and 10 healthy subjects. Reflexive and volitional saccades were assessed with the Saccadometer Advanced. The battery of cognitive and behavior tests was performed as well. RESULTS We found a prolonged latency, slowness and decreased velocity of reflexive and voluntary saccades and reduced amplitude of voluntary saccades. Moreover, patients with JHD executed a significantly lower number of volitional saccades and made more incorrect cued saccades than controls. We noted a significant correlation between prolonged latency of reflexive saccades with gap task and disease severity and significant inverse correlation between prolonged latency of reflexive saccades with overlap task, an increased number of incorrect saccades made on a cue and impairment in working memory. CONCLUSION Abnormalities of saccade eye movements in patients with JHD were similar to those reported in patients with HD. Our findings did not confirm abnormalities previously reported in patients with early onset HD. Abnormal saccade parameters correlated also with a disease severity and cognitive deterioration.

Is hypertension a risk factor of hemifacial spasm

OBJECTIVES The published data on the relation between arterial hypertension (AH) and hemifacial spasm (HFS) are controversial. The aim of the study was to determine the prevalence of AH in HFS patients and the relation of AH and compression of the brainstem at the region of vasomotor center. MATERIALS AND METHODS The study included 60 of primary HFS patients and 60 healthy controls matched by age. AH was defined according to WHO criteria. The vessel compression of the brainstem was measure on MRI scans in selected region of vasomotor center located in the ventro-lateral medulla (VLM), between the pontomedullary junction, retro-olivary sulcus and the root entry zone (REZ) of the IX and X nerves. Modeling and compression severity of the VLM was graded in the 0-3 scale. RESULTS The prevalence of AH in HFS patients did not differ significantly from the control group (61.6% vs 45.0%, p=ns). VML compression by vessel was frequently found in HFS patients with AH than without AH (97.2% vs 60.9%, χ(2)=11.0, p=0.0009). A similar relation was also found in the control group. The higher rate of VML vascular compression was related to the presence of AH in both, HFS patients and control group. CONCLUSION The prevalence of AH in HFS patients does not differ from controls. The VLM compression in HFS patients and controls is related to AH diagnosis. The association between AH and VLM compression is stronger in patients with higher degree of VLM compression.

Eye movement abnormalities in essential tremor

Abstract Essential tremor (ET) is the most prevalent movement disorder, characterized mainly by an action tremor of the arms. Only a few studies published as yet have assessed oculomotor abnormalities in ET and their results are unequivocal. The aim of this study was to assess the oculomotor abnormalities in ET patients compared with the control group and to find the relationship between oculomotor abnormalities and clinical features of ET patients. We studied 50 ET patients and 42 matched by age and gender healthy controls. Saccadometer Advanced (Ober Consulting, Poland) was used to investigate reflexive, pace-induced and cued saccades and conventional electrooculography for evaluation of smooth pursuit and fixation. The severity of the tremor was assessed by the Clinical Rating Scale for Tremor. Significant differences between ET patients and controls were found for the incidence of reflexive saccades dysmetria and deficit of smooth pursuit. Reflexive saccades dysmetria was more frequent in patients in the second and third phase of ET compared to the first phase. The reflexive saccades latency increase was correlated with severity of the tremor. In conclusion, oculomotor abnormalities were significantly more common in ET patients than in healthy subjects. The most common oculomotor disturbances in ET were reflexive saccades dysmetria and slowing of smooth pursuit. The frequency of reflexive saccades dysmetria increased with progression of ET. The reflexive saccades latency increase was related to the severity of tremor.

Eye movements in essential tremor patients with parkinsonian and cerebellar signs

Apart from intention tremor essential tremor (ET) patients may display other cerebellar signs, like dysmetria or tandem gait disturbances as well as parkinsonian signs like resting tremor, cogwheel sign, subtle bradykinesia. Previous reports claimed the occurrence of the eye movement abnormalities characteristic for dysfunction of cerebellar dorsal vermis in ET patients with concomitant cerebellar signs. There are no previous reports evaluating the eye movement abnormalities in ET patients with concomitant parkinsonian signs. The objective of this study was to determine the relationship between the occurrence of parkinsonian and cerebellar signs and the oculomotor abnormalities in ET patients. METHOD Fifty ET patients including 6 (12.0%) patients with concomitant parkinsonian signs (ET-P), 20 (40.0%) patients with cerebellar signs (ET-C), 7 (14.0%) with mixed parkinsonian and cerebellar signs (ET-M), 17 (34.0%) patients with the only tremor (ET-T) together with 42 healthy controls were included to the study. Reflexive, pace-induced and cued saccades were recorded using Saccadometer Advanced. Smooth pursuit and fixation were tested using EOG. RESULTS Latency of pace-induced saccades was significantly longer in ET-C and ET-M patients compared to ET-T and ET-P patients. Latency of cued saccades was significantly longer in ET-M patients compared to ET-T. There were no significant differences of the eye movement parameters between ET-P patients compared to ET-T patients. CONCLUSION In ET patient with concomitant cerebellar signs prolonged volitional saccades latency was detected. There are no particular differences in the eye movements in ET patients with concomitant parkinsonian signs compared to ET patients without concomitant signs.

Invasive treatment for carotid fibromuscular dysplasia.

Introduction Fibromuscular dysplasia (FMD) is an infrequent non-inflamatory disease of unknown etiology that affects mainly medium-size arteries. The prevalence of FMD among patients scheduled for endovascular treatment of carotid artery stenosis is unknown. Aim To evaluate the prevalence and treatment options of carotid FMD in patients scheduled for carotid artery stenting (CAS). Material and methods Between Jan 2001 and Dec 2013, 2012 CAS procedures were performed in 1809 patients (66.1% men; age 65.3 ±8.4 years, 49.2% symptomatic). In case of FMD suspicion in Doppler-duplex ultrasound (DUS), computed tomography angiography was performed for aortic arch and extracranial and intracranial artery imaging. For invasive treatment of FMD carotid stenosis, balloon angioplasty was considered first. If the result of balloon angioplasty was not satisfactory (> 30% residual stenosis, dissection), stent placement was scheduled. All patients underwent follow-up DUS and neurological examination 3, 6 and 12 months after angioplasty, then annually. Results There were 7 (0.4%) (4 symptomatic) cases of FMD. The FMD group was younger (47.9 ±7.5 years vs. 67.2 ±8.9 years, p = 0.0001), with higher prevalence of women (71.4% vs. 32.7%, p = 0.0422), a higher rate of dissected lesions (57.1% vs. 4.6%, p = 0.0002) and less severe stenosis (73.4% vs. 83.9%, p = 0.0070) as compared to the non-FMD group. In the non-FMD group the prevalence of coronary artery disease was higher (65.1% vs. 14.3% in FMD group, p = 0.009). All FMD patients underwent successful carotid artery angioplasty with the use of neuroprotection devices. In 4 cases angioplasty was supported by stent implantation. Conclusions Fibromuscular dysplasia is rare among patients referred for CAS. In case of significant FMD carotid stenosis, it may be treated with balloon angioplasty (stent supported if necessary) with optimal immediate and long-term results.

Neuroacanthocytosis - Clinical variability (a report on six cases)

Objective: To explore the association between striatal dopaminergic deficits and cognitive impairment within a large cohort of early, drug-naive Parkinson’s disease patients and to test the hypothesis that executive dysfunction in Parkinson’s disease is caused by striatal dopaminergic depletion. Background: Cognitive impairment in Parkinson’s disease is common and influences patients’ everyday functioning, but the mechanisms of early cognitive decline are not known. Understanding of these mechanisms is important for the development of methods preventing cognitive decline in Parkinson’s disease. Previous studies suggest that the dopaminergic system influences cognition in PD. Methods: Neuropsychological and cerebral dopamine transporter SPECT imaging data of 339 Parkinson’s disease patients and 158 Healthy controls of the Parkinson’s Progression Markers Initiative study were analysed. Neuropsychological evaluation consisted of standardized tests of memory, visuospatial and executive function. SPECT imaging was performed with [123I]FP-CIT and specific binding ratios in left and right putamen and caudate nucleus were calculated. The association between specific binding ratios and cognition was performed using a cognitive composite z-score, domain z-scores and individual test scores. Multivariate general linear model regression analyses were performed including age, gender, education, and laterality as predictors and specific binding ratios as dependent variables. Results: Uncorrected analysis showed no associations between dopamine transporter imaging and memory and visuospatial domains. A small but significant positive association between specific binding ratios and the attention/executive domain was found, which was not significant after adjusting for age. However, in a moderated mediation model, we found that cognitive executive differences between controls and patients with Parkinson’s disease were mediated by an age-moderated dopaminergic deficit in the left caudate nucleus. Conclusions: Our findings support the hypothesis that nigrostriatal dopaminergic deficit contributes to executive impairment, but not to memory or visuospatial impairment in early Parkinson’s disease.Objective: To investigate whether spirography-based objective measures of motor dysfunctions are able to discriminate between Parkinson’s disease (PD) patients with different motor states (Off and ...Objective: This study aims to determine PPN’s electrophysiological activities in rats to help future studies and to investigate the effect of subthalamic nucleus stimulation on PPN. Background: Long-duration medical treatment of Parkinson patients causes complications and morbidity. Risks in destructive surgery are releatively high, new treatment methods such as stereotactic functional surgery has been proposed recently. While sensory and behavioral processes of pedunculopontine nucleus (PPN) are well known as a locomotor center, its role on initiating and sustaining motion function in primates or rats has been also demonstrated. All functions of PPN are not fully known yet, and its DBS has been proposed as an alternative therapeutic target in treating gait problems of Parkinson’s disease recently. Methods: In this study, 14 male wistar type healthy rats with average 292 (284-317) gram weight and with the same age group were used. In the sham group, two probes were inserted, one to the STN bilaterally and the other to the right PPN to record PPN’s electrophysiological activities. In the experiment group, in addition to the same procedures used in the sham group, STN was stimulated bilaterally at 0.5 Hz, 10 Hz, 60 Hz ve 130 Hz and PPN’s electrophysiological activities were recorded before and after bilateral STN stimulations. Results: Analyzing the neural activity after the 60 Hz stimulation, it revealed that STN has a stimulus effect on PPN neurons increasing the firing rate. The PPN neurons demonstrated three different patterns of firing, burst random and regular. The majority of the neurons (68%) exhibited a regular pattern of firing in the sham group. After bilateral STN stimulation with very low (0,5 Hz and 10 Hz) and high (130 Hz) frequencies the PPN neurons maintained their firing patterns. However, after 60 Hz stimulation of STN a significant percentage of neurons (82,1 %) fired with a more regular pattern. Conclusions: The results of this study provides additional information to our understanding on PPN’s electrophysiological activities. 60 Hz STN stimulation can increase the firing rates and changes the behaviour of the PPN neurons.Objective: To analyze the relationship between the electric field and the volume of tissue activated (VTA) during model-based investigations of deep brain stimulation (DBS).Background: An important ...This journal suppl. entitled: Supplement: Abstracts of the Eighteenth International Congress of Parkinsons Disease and Movement Disorders / Poster Presentation