Clinical Neuroscience

[ONE-AND-A-HALF SYNDROME - TWO CASES]

ILNICZKY Sándor, KAMONDI Anita, VÁRALLYAY György, GAAL Barbara, PALÁSTI Ágnes, GULYÁS Szilvia, SZIRMAI Imre

NOVEMBER 30, 2007

Clinical Neuroscience - 2007;60(11-12)

[One-and-a-half syndrome is characterized by combination of the clinical features of unilateral horizontal gaze palsy and internuclear ophthalmoplegia. The common symptoms are double vision and oscillopsia. The lesion is located in the paramedian pontine reticular formation, involving the centre of horizontal gaze and medial longitudinal fasciculus. More extensive brainstem damage may result in additional neurological signs. The most frequent underlying diseases are vascular insults, multiple sclerosis, and brainstem tumor. We present two cases of one-and-a-half syndrome. Both patients had lacunar infarction in the paramedian pontine tegmentum, revealed by MRI. The first patient had isolated eye movement disorder, while the second had additional nuclear-type facial paresis. In the first case brainstem evoked potentials indicated brainstem damage, in the second patient it was normal. Ocular symptoms improved within some days in both patients.]

COMMENTS

0 comments

Further articles in this publication

Clinical Neuroscience

[DIFFUSION TENSOR AND FUNCTIONAL MR IMAGING OF SEVERE TRAUMATIC BRAIN INJURY AT LOW MAGNETIC FIELD]

AUER Tibor, SCHWARCZ Attila, EZER Erzsébet, CZEITER Endre, ARADI Mihály, HUDVÁGNER Sándor, JANSZKY József, BÜKI András, DÓCZI Tamás

[Aim of the study - Presentation of diffusion tensor imaging (DTI) performed at low magnetic field (1 Tesla) in the algorithm of work-up of a patient suffering from severe traumatic brain injury (TBI). Method - DTI and functional MRI (fMRI) were applied at 1 Tesla for visualization of neural pathways and examination of sensory functions of a patient with severe TBI. DTI-measurement was also performed on a healthy patient for comparison. Results - DTI acquired at low magnetic field yielded appropriate visualization of neural pathways. DTI confirmed the results of the clinical and fMRI examinations in the patient suffering from severe TBI. Conclusion - An optimized DTI can be useful in the examination of patients with TBI, moreover, it may also help in the establishment of diagnoses of other central nervous system diseases affecting neuronal pathways. The presented results suggest that DTI of appropriate quality can be performed at low magnetic field.]

Clinical Neuroscience

[BILATERAL “OVER THE TOP” DECOMPRESSION THROUGH UNILATERAL LAMINOTOMY FOR LUMBAR AND THORACIC SPINAL CANAL STENOSIS]

BANCZEROWSKI Péter, LIPÓTH László, VERES Róbert

[Objective - The standard surgical procedures used in degenerative thoracic and lumbar spinal canal stenosis allows decompression of the neural structures by unroofing the spinal canal, often resulted in destruction or insufficiency of facet joints, sacrifice the interspinosus/supraspinosus ligament complexes and stripping of the paraspinal muscles altering an already pathologic biomechanical milieu causing segmental instability. Various less invasive techniques exists to save the integrity and prevent the instability of the spine and allow decompression of neural structures located in the spinal canal. The authors discusses the experiences with technique of unilateral laminotomy for bilateral decompression. Methods - The unilateral laminotomy for bilateral decompression technique was performed at 60 levels in 51 patients to decompress the symptomatic degenerative stenosis of the thoracic and lumbar spinal canal. The inclusion criteria were used as follows: symptoms of neurogenic claudication and/or radiculopathy, myelopathy, neuroimaging evidence of degenerative stenosis and absence of instability. Symptoms were considered refractory to nonsurgical conservative management or myelopathy was detected. Results - The distribution of mostly affected segments were the L 4-5 (45%) and L3-4 (28.4%). Neurogenic claudication and walking distance improved during the follow up period in all patients. Seven patients (13.73%) reported excellent, 32 (62.74%) good, 12 (23.53%) fair outcome and no patient a poor overall outcome. The low back pain was the major residual postoperative complaint. 25 (49%) patients were very satisfied with their outcome, 23 (45.1%) were fairly satisfied, 2 (3.9%) were not very satisfied and 1 (2%) patients was dissatisfied. Conclusion - The unilateral laminotomy for bilateral microdecompression technique minimizes resection of and injury to tissues not directly involved in the pathologic process, while affording a safe and through decompression of neural structures located in a degeneratively stenotic spinal canal.]

Clinical Neuroscience

[UNALTERED MRNA EXPRESSION OF CALCITONIN-LIKE RECEPTOR AND RECEPTOR ACTIVITY MODIFYING PROTEINS IN HUMAN ARTERIES IN STROKE AND MYOCARDIAL INFARCTION]

KAREN Eskesen, TAJTI János, HORTOBÁGYI Tibor, SZOK Délia, VÉCSEI László, LARS Edvinsson

[Calcitonin-like receptor (CL-R) is a functional CGRP1- receptor when complexed with RAMP1 or an adrenomedullin-receptor or when complexed with RAMP2 or RAMP3. This study was carried out 1. to set up a method to examine the relative quantity of mRNA of CL-R, RAMP1, RAMP2 and RAMP3 in human coronary (CA), pulmonary (PA) and middle cerebral arteries (MCA), and 2. to examine the level of mRNA expression in cerebro- and cardiovascular diseases. The method was validated with respect to the use of postmortem tissue and we compared β-actin and GAPDH as housekeeping genes. There was no time-dependent change in total RNA and level of mRNA for β-actin or GAPDH could be detected in vessels removed from 1 and 5 days post mortem. The expression of β-actin appears lower in coronary artery than in pulmonary artery and middle cerebral artery with no significant difference for GAPDH; both worked well. There were some differences in mRNA expression for CL-R (higher) and RAMP3 (lower) in middle cerebral artery compared to coronary artery and pulmonary artery. There was no significant difference in mRNA for RAMP1 and RAMP2 in the three types of arteries. We did not observe any difference in mRNA for CL-R and RAMPs in arteries from patients with hemorrhagic stroke, arteriosclerosis and acute myocardial infarction when compared to patients without these diagnoses. Thus the mRNA expression seems to be unaltered in these disorders.]

Clinical Neuroscience

[CONGRESS CALENDAR]

[Congress calendar 2007;60(11-12)]

Clinical Neuroscience

[Professor Béla Halász is 80 years old]

VÉCSEI László

All articles in the issue

Related contents

Clinical Neuroscience

Long-term follow-up results of concomitant chemoradiotherapy followed by adjuvant temozolomide therapy for glioblastoma multiforme patients. The importance of MRI information in survival: Single-center experience

LUKÁCS Gábor, TÓTH Zoltán, SIPOS Dávid, CSIMA Melinda, HADJIEV Janaki, BAJZIK Gábor, CSELIK Zsolt, SEMJÉN Dávid, REPA Imre, KOVÁCS Árpád

Introduction - Glioblastoma multiforme (GBM) is the most common malignant primary anomaly of central nervous system. The GBM infiltrates the nearly sturctures from the initial tumor and its metastatic attribution is well known. The aim of our single-centered retrospective study was to introduce the importance of postoperative medical imaging confirmation of total tumor resection for patient with GBM combined concomitant and adjuvant chemoradiotherapy on a 10 year long patient follow up. Methods - From January 2006 to April 2015 we registered 59 patients with newly diagnosed GBM at the University of Kaposvár Health Center Institute of Diagnostic Imaging and Radiation Oncology. The histological diagnosis was confirmed by a proficient neuropathologist (World Health Organisation WHO; grade IV astrocytoma). According to histological status if the ECOG performance status of patients allowed it the mutidisciplinary oncoteam recommended adjuvant chemoradiotherapy all features strictly by Stupp protocol. (60 Gy dose on the gross tumor volume and 2-3 cm margin for the clinical target volume with parallel 75 mg/m2 TMZ. Four weeks after monotherapial phase patients had to recieve 6 cycles of TMZ first cycle with 150 mg/m2 up to 200 mg/m2). The irradiation was carried out by a conformal three dimensional planning system. Results - 59 patients with the median age of 63 (range 17-84) year. Our sample counted 34 male patients and 25 woman patients. 14 patients underwent gross total tumor resection while, 39 patients underwent partial resection and the rest from our sample 6 patients passed through biopsy. Statistical analysis showed a lengthier survival among males than females, with a median survival of 13 months for males and females, the OS of 26.209 for males, meanwhile 15.625 for females. However, the difference is not considerable (log-rank p=0.203). Our study found that the estimated survival of patients at least 50 years old is significantly shorter at a median survival of 12 months (log rank p=0.027) than that of patients below 50 years of age at a median survival of 23 months. The longest estimated median survival was calculated with patients of ECOG '0' condition (16 months). However, no significant difference was found in the estimated survival of patients of different ECOG conditions (log-rank p=0.146). Based on the extent of surgery, complete resection resulted in the longest average survival of 36.4 months, followed by 21.5 months among patients with biopsy, and 15.8 months among patients with partial resection. Different surgical procedures, however, did not result in significant differences in survival (log-rank p=0.059). The overal survival of patients who had complete resection confirmed by MRI compared with the overal survival of patients with residual tumor confirmed by MRI as well we can estimate that there is significant difference between these two groups (p=0,004). Conclusion - Despite complex and intense treatment, recurrence is inevitable and causes relatively rapid death. In our analysis complete resection, as defined from the neurosurgeon’s report and postoperative MRI, resulted in an independently significant improvement in OS. Our results are the evidences that the treatment of patients with glioblastoma multiforme in Hungary is at least on the same level as any other developed European countries.

Clinical Neuroscience

Cerebral cavernous malformation type 1 with retinal blood vessel tortuosity and KRIT1 gene mutation

KALMÁR Tibor, MARÓTI Zoltán, VADVÁRI Árpád, HALMOSI Ágnes, KÁLOVITS Ferenc, KÁLMÁN Bernadette

Cerebral cavernous malformations (CCMs) represent a relatively rare and heterogeneous clinical entity with mutations identified in three genes. Both sporadic and familial forms have been reported. We present a young female patient with episodic paresthesia and headaches, but without acute neurological deficits. Her mother had a hemorrhaged cavernoma surgically removed 21 years ago. Cranial magnetic resonance imaging revealed multiple cavernous malformations in the size of a few millimeters and the ophthalmologic exam detected retinal blood vessel tortuosity in the proband. Targeted exome sequencing analysis identified a nonsense mutation in exon 16 of the KRIT1 gene, which resulted in a premature stop codon and a truncated protein underlying the abnormal development of cerebral and retinal blood vessels. This mutation with pathogenic significance has been reported before. Our case points to the importance of a thorough clinical and molecular work up despite the uncertain neurological complaints, since life style recommendations, imaging monitoring and genetic counseling may have major significance in the long term health of the patient.

Clinical Neuroscience

[The role of MRI in measuring the effectivity of disease modifying treatments II]

KINCSES Zsigmond Tamás, TÓTH Eszter, FRICSKA-NAGY Zsanett, FÜVESI Judit, RAJDA Cecília, BENCSIK Krisztina, VÖRÖS Erika, CSOMOR Angéla, PALKÓ András, VÉCSEI László

[The paraclinical examinations, principally the MRI have an increasing significance in the diagnosis of multiple sclerosis. However, MRI markers also have a prominent role in monitoring of the disease-course and activity, and also in the planning of possible therapeutic changes. In accordance with previously published international guidelines, in this article we propose a protocol for the monitoring the treatment efficacy in multiple sclerosis. This could be the basis of a consensus based guideline to be implemented in Hungary.]

Clinical Neuroscience

[Childhood sporadic type of hemiplegic migraine with arteria cerebri media hypoperfusion]

NAGY Csaba, BAJZIK Gábor, SKOBRÁK Andrea, CSORBA Eszter, LAJTAI Anikó, BALOGH Gábor, NAGY Ferenc, VAJDA Zsolt

[Hemiplegic migraine is a rare subtype of migraine that is associated with reversible motor weakness in the aura phase. This is an uncommon form of migraine usually starting in childhood. The purpose of this case report is to highlight the differential diagnostic difficulty of the first attack. We describe a case, where the fluctuating unilateral motor weakness and aphasia suggested that the patient had ischaemic stroke. Nevertheless the brain MRI and MR angiography, the measured 5-hydroxyindole acetic acid (5-HIAA) concentration changes and the spontaneously improving clinical status proved the diagnosis of hemiplegic migraine. The MRI and MR angiography was very beneficial in establishing the correct diagnosis in this case. To distinguish between the familiar and sporadic type of hemiplegic migraine further genetic tests can be carried out.]

Clinical Neuroscience

[The role of MRI in measuring the effectivity of disease modifying treatments I]

TÓTH Eszter, BENCSIK Krisztina, VÖRÖS Erika, FRICSKA-NAGY Zsanett, FÜVESI Judit, RAJDA Cecília, CSOMOR Angéla, PALKÓ András, VÉCSEI László, KINCSES Zsigmond Tamás

[MRI has a significant role in the diagnosis of multiple sclerosis. The newer and newer treatment options of the disease make it necessary to monitor the effectiveness of the therapy. Besides the clinical signs (clinical relapses and progression), the different MRI parameters can also reflect the disease activity. In our current article we summarize those MRI markers, which best predict the long-term disability, based on the international standards.]