Clinical Neuroscience

[Sturge-Weber syndrome: clinical and radiological correlates in 86 patients]

FOGARASI András, TOBIAS Loddenkemper, MELLADO Cecilia, INGRID Tuxhorn, GEORG Evers, DEAN Sarco, BURGESS C. Richard, HALÁSZ Péter, BARSI Péter, GYORSOK Zsuzsanna, GYIMESI Csilla, KÓBOR Jenõ, SIEGLER Zsuzsanna, JANSZKY József, JAKUS Rit

JANUARY 25, 2013

Clinical Neuroscience - 2013;66(01-02)

[Backgrounds and purpose - To correlate the extent of the leptomeningeal angiomatosis with clinical features in Sturge- Weber syndrome (SWS). Methods - The study group consisted of 86 consecutive patients aged two months to 56 (mean 7.9±10.3) years with SWS and epilepsy. Clinical and MRI data were analyzed. Results - Based on the extent of leptomeningeal angiomatosis, patients were divided into two subgroups: 43 patients had hemispheric angiomatosis and atrophy, whereas, another 43 had focal involvement. Nine of the 43 hemispherial patients (10%) showed bilateral involvement: all of these bilateral cases demonstrated dominance in a single side with hemispheric leptomeningeal angiomatosis and contralateral focal extension. Hemispheric and focal subgroups were clinically different. Patients with hemispheric SWS were younger at the age of epilepsy onset (p<0.001) and age at MRI examination (p<0.05). Neither gender, lateralization, duration of epilepsy, appearance of secondarily generalized seizures, nor seizure frequency revealed a significant difference between subgroups. Conclusion - Bilateral involvement is frequent and occurs in cases with a hemisperic involvement on one side. The age of epilepsy onset is related to the extent of leptomeningeal angiomatosis. Patients with hemispheric form of SWS presented with earlier age of seizure onset. Focal pial angiomatoses do not tend to progress (a longer duration is not associated with more frequent hemispheric involvement). Other variables including seizure frequency and secondary generalized tonic-clonic seizures are not associated with the extent of angiomatosis.]



Further articles in this publication

Clinical Neuroscience

[The significance of high-resolution ultrasonography in the diagnosis of peripheral nerve disorders]


[High resolution ultrasonography is an emerging technique for the investigation of peripheral nerves and is increasingly used worldwide in the diagnosis of peripheral nerve disorders, however, until now it is not widespread in Hungary. According to the literature this method is especially useful in entrapment neuropathies, traumatic peripheral nerve injuries, tumors of the peripheral nerves and sonographically guided interventions. Ultrasonography allows precise morphological analysis and quantitative measurements of the nerves providing useful complementary information to electrodiagnostic data. In entrapment neuropathies ultrasound shows nerve swelling mainly proximal to the sites of compression and a focal change of echotexture. On longitudinal scan, an abrupt caliber change and spindle-like swelling of the compressed nerve segment can be seen. Evaluation of the anatomical background and visualisation of the postoperative and posttraumatic changes provide useful information for planning of the therapy. Ultrasound may be of significant help in localizing the pathological nerve segment when it is at an electrophysiologically inaccessible site or when substantial secondary axonal loss precludes precise electrophysiological localization and it might even show pathological changes when nerve conduction studies are normal. Contrary to electrophysiological investigation ultrasonography might discover neurotmesis in the akute phase of traumatic nerve injuries indicating the necessity of surgical intervention. We provide a summary of the main indications and further application areas of this method.]

Clinical Neuroscience

[Mild encephalitis/encephalopathy with a reversible splenial lesion in children]

LIPTAI Zoltán, IVÁDY Balázs, BARSI Péter, VÁRALLYAY György, RUDAS Gábor, FOGARASI András

[Authors, most of them Japanese, have recently published an increasing number of articles on mild encephalitis/encephalopathy with a reversible splenial lesion. We report on two new white European patients and compare published data with our own observations. A 15- year-old girl developed headache, fever, dizziness, vomiting and nuchal rigidity over four days. CSF showed elevated protein and cell count, with the lowest serum Na being 131 mmol/L. MRI on day seven was normal, but she remained febrile, had cerebral edema and episodes of confusion. MRI on day 11 showed a small T2-hyperintense lesion with restricted diffusion in the callosal splenium. Adenoviral infection was proved, and the girl underwent a protracted course of recovery. MRI signal changes improved in six days and disappeared after four months. A 12.5-year-old girl developed headache, lethargy, drowsiness and vomiting. On day five she experienced right-sided numbness, weakness and inability to speak which lasted 12 hours. She was confused and disoriented. MRI disclosed a tiny area of increased T2- signal and restricted diffusion in the splenium. Serum Na was 133 mmol/L, CSF cell count and protein was markedly elevated, and enteroviral infection was detected. Echocardiography showed no changes predisposing to clot formation and no thrombophilia was found. Her symptoms resolved in a week and MRI was normal two months later. These two non-epileptic children increase the small number of white European patients with MERS reported so far. Both had hyponatremia and encephalitis and patient 2 had transient ischemic attack, possibly due to the cerebral edema also resulting in the splenial lesion.]

Clinical Neuroscience

[Aspirin and clopidogrel resistance: possible mechanisms and clinical relevance. Part II: Potential causes and laboratory tests]

VADÁSZ Dávid, SZTRIHA K. László, SAS Katalin, VÉCSEI László

[Recent meta-analyses have indicated that patients with vascular disease demonstrated by laboratory tests to be aspirin or clopidogrel-resistant are at an increased risk of major vascular events. The suggested mechanisms of aspirin resistance include genetic polymorphism, alternative pathways of platelet activation, aspirin-insensitive thromboxane biosynthesis, drug interactions, or a low aspirin dose. Clopidogrel resistance is likely to develop as a result of a decreased bioavailability of the active metabolite, due to genetic variation or concomitant drug treatment. Additional work is required to improve and validate laboratory tests of platelet function, so that they may become useful tools for selection of the most appropriate antiplatelet therapy for an individual patient. Improvements in antiplatelet treatment strategies in the future should lead to a reduction in premature vascular events.]

Clinical Neuroscience

[Experiences with a self developed accelerometer]


[Objective - In neurology the objective evaluation of improvement of paresis on every-day practice. The aim of this study was to develope and test a small 3-d acceleration measuring device and validate its usefulness. Patients and methods - We collected data from 17 mild and medium severity hemiparetic, bedridden acute ischaemic and hemorrhagic stroke patients and compared with data of 22 control subjects. The devices were attached to the paretic and non-paretic extremities and any movements (m/s2) and movement-durations were registered (24h). The data of movement-monitors were compared also with the changes of National Institute of Health Stroke Scale and European Stroke Scale. The electromiograph-sensor of polysomnograph has been used for validation. Results - Mild differences could be found in the use of dominant and non-dominant upper extremities of control persons. The control persons used their upper extremities more frequently than the stroke patients. Our data showed significant correlation with National Institute of Health Stroke Scale. Higher values on the scores were accompanied with less intensive use of extremities. We found a correlation between the consiousness level of patients and their activity of upper extremities. If the patients had severe consiousness disturbances they used significantly less their upper extremities. Conclusion - Our device sensitively detected the movement-differences between paretic and non-paretic extremities and can be used for quantitative evaluation of patient's neurological and consciousness status.]

Clinical Neuroscience

[Early mental test - developing a screening test for mild cognitive impairment]

KÁLMÁN János, PÁKÁSKI Magdolna, HOFFMANN Ildikó, DRÓTOS Gergely, DARVAS Gyöngyi, BODA Krisztina, BENCSIK Tamás, GYIMESI Alíz, GULYÁS Zsófia, BÁLINT Magdolna, SZATLÓCZKI Gréta, PAPP Edina

[Background and purpose - Mild cognitive impairment (MCI) is a heterogenous syndrome considered as a prodromal state of dementia with clinical importance in the early detection of Alzheimer’s Disease. We are currently developing an MCI screening instrument, the Early Mental Test (EMT) suitable to the needs of primary care physicians. The present study describes the validation process of the 6.2 version of the test. Methods - Only subjects (n=132, female 95, male 37) over the age of 55 (mean age 69.2 years (SD=6.59)) scoring at least 20 points on Mini-Mental State Examination (MMSE), mean education 11.17 years (SD=3.86) were included in the study. The psychometric evaluation consisted of Alzheimer’s Disease Assessment Scale Cognitive subscale (ADAS-Cog) and the 6.2 version of EMT. The statistical analyses were carried out using the 17.00 version of SPSS statistical package. Results - The optimalised cut-off point was found to be 3.45 points with corresponding 69% sensitivity, 69% specificity and 69% accuracy measures. The Cronbach-α, that describes the internal consistence of the test was 0.667, which is higher as compared with the same category in the case of the ADASCog (0.446). A weak negative rank correlation was found between the total score of EMT 6.2 and the age of probands (rs=-0.25, p=0.003). Similarly, only a weak correlation was found between the education levels and the total score of EMT 6.2 (rs=0.31, p<0.001). Two of the subtests, the repeated delayed short-time memory and the letter fluency test with a motorical distraction task had significantly better power to separate MCI and control groups than the other subtests of the EMT. Conclusion - The 6.2 version of EMT is a fast and simple detector of MCI with a similar sensitivity-specificity profile to the MMSE, but this version of the test definitely needs further development.]

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Related contents

Clinical Neuroscience

[Zonisamide: one of the first-line antiepileptic drugs in focal epilepsy ]


[Chronic administration of antiepileptic drugs without history of unprovoked epileptic seizures are not recommended for epilepsy prophylaxis. Conversely, if the patient suffered the first unprovoked seizure, then the presence of epileptiform discharges on the EEG, focal neurological signs, and the presence of epileptogenic lesion on the MRI are risk factors for a second seizure (such as for the development of epilepsy). Without these risk factors, the chance of a second seizure is about 25-30%, while the presence of these risk factors (for example signs of previous stroke, neurotrauma, or encephalitis on the MRI) can predict >70% seizure recurrence. Thus the International League Against Epilepsy (ILAE) re-defined the term ’epilepsy’ which can be diagnosed even after the first seizure, if the risk of seizure recurrence is high. According to this definition, we can start antiepileptic drug therapy after a single unprovoked seizure. There are four antiepileptic drugs which has the highest evidence (level „A”) as first-line initial monotherapy for treating newly diagnosed epilepsy. These are: carbamazepine, phenytoin, levetiracetam, and zonisamide (ZNS). The present review focuses on the ZNS. Beacuse ZNS can be administrated once a day, it is an optimal drug for maintaining patient’s compliance and for those patients who have a high risk for developing a non-compliance (for example teenagers and young adults). Due to the low interaction potential, ZNS treatment is safe and effective in treating epilepsy of elderly people. ZNS is an ideal drug in epilepsy accompanied by obesity, because ZNS has a weight loss effect, especially in obese patients.]

Clinical Neuroscience

Effects of valproate, carbamazepine and levetiracetam on Tp-e interval, Tp-e/QT and Tp-e/QTc ratio


Aim - To evaluate P-wave dispersion before and after antiepileptic drug (AED) treatment as well as to investigate the risk of ventricular repolarization using the Tpeak-Tend (Tp-e) interval and Tp-e/QT ratio in patients with epileptic disorder. Methods - A total of 63 patients receiving AED therapy and 35 healthy adults were included. ECG recordings were obtained before and 3 months after anti-epileptic treatment among patients with epilepsy. For both groups, Tp-e and Tp-e/QT ratio were measured using a 12-lead ECG device. Results - Tp-e interval, Tpe/QT and Tp-e/QTc ratios were found to be higher in the patient group than in the control group (p<0.05, for all), while QTmax ratio was significantly lower in the patient group. After 3 months of AED therapy, significant increases in QT max, QTc max, QTcd, Tp-e, Tp-e/QT, and Tp-e/QTc were found among the patients (p<0.05). When the arrhythmic effects of the drugs before and after treatment were compared, especially in the valproic acid group, there were significant increases in Tp-e interval, Tp-e/QT and Tp-e/QTc values after three months of treatment (p<0.05). Carbamazepine and levetiracetam groups were not statistically significant in terms of pre- and post-treatment values. Conclusions - It was concluded that an arrhythmogenic environment may be associated with the disease, and patients who received AED monotherapy may need to be followed up more closely for arrhythmia.

Clinical Neuroscience

Relationship between Status Epilepticus Severity Score and etiology in adult NCSE patients

GENC Fatma, ERDAL Abidin, AKCA Gizem, KARACAY Ertan, GÖKSU Özaydın Eylem, KUTLU Gülnihal, GÖMCELI Bicer Yasemin

Purpose - Nonconvulsive status epilepticus (NCSE) is a heterogeneous, severe neurological disorder of different etiologies. In this study, the outcomes of NCSE episodes was assessed in a large series of adult patients. Our objective was to evaluate relationship between Status Epilepticus Severity Score (STESS) and etiology and the role of etiological factors on predicting the outcomes. Method - In this retrospective study, the medical records of 95 patients over 18 years of age who were diagnosed with NCSE between June 2011 and December 2015 were reviewed. Their treatment and follow-up for NCSE was performed at the Epilepsy Unit in Department of Neurology, Antalya Research and Training Hospital. Etiological factors thought to be responsible for NCSE episodes as well as the prognostic data were retrieved. The etiological factors were classified into three groups as those with a known history of epilepsy (Group 1), primary neurological disorder (Group 2), or systemic/unknown etiology (Group 3). STESS was retrospectively applied to patients. Results - There were 95 participants, 59 of whom were female. Group 1, Group 2, and Group 3 consisted of 11 (7 female), 54 (33 female), and 30 (19 female) patients, respectively. Of the 18 total deaths, 12 occurred in Group 2, and 6 in Group 3. The negative predictive value for a STESS score of ≤ 2 was 93.88% (+LR 2.05 95% CI: 1.44-2.9 and -LR 0.3 95% CI 0.10-0.84 ) in the overall study group. While the corresponding values for Group 1 (patients with epilepsy), Group 2 (patients with primary neurological disorder), and group 3 (patients with systemic or unknown etiology) were 100%, 92.59% (+LR 2.06 95%CI: 1.32-3.21 and -LR 0.28 95% CI 0.08-1.02 ) 83.33% (+LR 1.14 95%CI: 0.59-2.9 and -LR 0.80 95% CI 0.23-2.73). Conclusions - This study included the one of the largest patients series ever reported in whom STESS, a clinical scoring system proposed for use in patients with status epilepticus, has been implemented. Although STESS appeared to be quite useful for predicting a favorable outcome in NCSE patients with epilepsy and primary neurological disorders, its predictive value in patients with systemic or unknown etiology was lower. Further prospective studies including larger NCSE samples are warranted.

Clinical Neuroscience

Thrombocytopenia with gabapentin usage

DILEK Atakli, BURCU Yuksel, PELIN Dogan Ak, HANDE Sariahmetoglu, HÜSEYIN Sari

Gabapentin is an antiepileptic drug approved for adjunctive therapy for partial seizures. We report a case of a patient who had thrombocytopenia with the dose of 2400 mg/day of gabapentin. The causal relationship between gabapentin and thrombocytopenia was revealed by dramatic increase in thrombocyte count following the cessation of the gabapentin treatment. To our knowledge, this is the first case report with a hematopoietic side effect of gabapentin.

Clinical Neuroscience


HORVÁTH A. Réka, KALMÁR Zsuzsanna, FEHÉR Nóra, FOGARASI András, GYIMESI Csilla, JANSZKY József

[Clinical lateralizing signs are the phenomena which can unequivocally refer to the hemispheric onset of epileptic seizures. They can improve the localization of epileptogenic zone during presurgical evaluation, moreover, their presence can predict a success of surgical treatment. Primary sensory phenomena such as visual aura in one half of the field of vision or unilateral ictal somatosensory sensation always appear on the contralateral to the focus. Periictal unilateral headache, although it is an infrequent symptom, is usually an ipsilateral sign. Primary motor phenomena like epileptic clonic, tonic movements, the version of head ubiquitously appear contralateral to the epileptogenic zone. Very useful lateralization sign is the ictal hand-dystonia which lateralizes to the contralateral hemisphere in nearly 100%. The last clonus of the secondarily generalized tonic-clonic seizure lateralizes to the ipsilateral hemisphere in 85%. The fast component of ictal nystagmus appears in nearly 100% on the contralateral side of the epileptic focus. Vegetative symptoms during seizures arising from temporal lobe such as spitting, nausea, vomiting, urinary urge are typical for seizures originating from non-dominant (right) hemisphere. Ictal pallor and cold shivers are dominant hemispheric lateralization signs. Postictal unilateral nose wiping refers to the ipsilateral hemispheric focus compared to the wiping hand. Ictal or postictal aphasia refers to seizure arising from dominant hemisphere. Intelligable speech during complex partial seizures appears in non-dominant seizures. Automatism with preserved consciousness refers to the seizures of non-dominant temporal lobe.]