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

SEPTEMBER 30, 2017

Clinical Neuroscience - 2017;70(09-10)


[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.]



Further articles in this publication

Clinical Neuroscience

[Diabetes, dementia, depression, distress]

SZATMÁRI Szabolcs, ORBÁN-KIS Károly, MIHÁLY István, LÁZÁR Alpár Sándor

[The number of people living with diabetes continues to rise. Therefore neurologists or other health care practitioners may be increasingly faced with comorbid neuropsychiatric disorders commonly presented by diabetic patients. More recently there has been an increasing research interest not only in the interactions between diabetes and the nervous system, the fine structure and functional changes of the brain, but also in the cognitive aspects of antidiabetic treatments. Patients with both types of diabetes mellitus may show signs of cognitive decline, and depression. Comorbid insomnia, anxiety, and distress may also occur. The bi-directional relationships between all these phenomena as well as their connection with diabetes can lead to further health and quality of life deterioration. Therefore it is important that all practitioners involved in the care of diabetic patients recognize the presence of comorbid neuropsychiatric disturbances early on during the healthcare process. Identifying higher risk patients and early screening could improve the prognosis of diabetes and may prevent complications.]

Clinical Neuroscience

[The Multiple Sclerosis Registry of Szeged]

BENCSIK Krisztina, SANDI Dániel, BIERNACKI Tamás, KINCSES Zsigmond Tamás, FÜVESI Judit, FRICSKA-NAGY Zsanett, VÉCSEI László

[Multiple sclerosis (MS) is a rare disease of the central nervous system considering the total population, the prevalence in Hungary is 83.9/100.000. The first MS registry was established in Denmark in the middle of the 1950’s. This was followed by the establishment of several national, then international databases with the number of enrolled patients in the hundred-thousands. At the beginning, the primary goal of the registries were the epidemiological surveys, focusing on the number of patients, the prevalence, the incidence, the mortality and the co-morbidity. As of today, however, with the rapid advancement and development of new disease modifying therapies (DMT) with different effectiveness and adverse reactions, the therapeutic use of the registries became even more essential: the modern, up-to-date, well established registries become integral part of the DMTs’ monitorization. The Multiple Sclerosis Registry of Szeged was first established as a “paper-based” database, then, in 2012, it was upgraded to an electronic, easily contactable and useable internet-based registry. As of today, it contains the socio-demographic and clinical data of more than 600 patients; we constantly add new patients as well as keep the registry up-to-date with the refreshment of old patients’ data. Aside from the “classical” clinical data, it can be used for the recording and assessment of the MRI scans and the data on psychopathological and quality of life assessments, which are becoming more and more important in everyday MS management. The establishment of the internet-based registry incredibly helped both the monitorization of the effectiveness of DMTs, and the success of the new epidemiological and psychopathological surveys. ]

Clinical Neuroscience

Title of the article: Bilateral acute angle-closure glaucoma induced by duloxetine

ATUM Mahmut, VILDAN Tunc, ENES Demiryurek, ALAGOZ Gursoy

Introduction - To present a rare case of bilateral acute angle-closure glaucoma secondary to duloxetine administered for the treatment of depression. Case presentation - A 46 year old woman developed bilateral closed angle glaucoma after 15 days of duloxetine usage. Intraocular pressures (IOP) were 52 and 55 mm Hg in right and left eyes respectively, with shallow anterior chamber and angle closure on gonioscopy. Discontinuing duloxetine treatment, initiation of antiglaucomatous treatment and bilateral Nd:YAG Laser iridotomy obtained normalized IOP and anterior chamber depth. Conclusion - Duloxetine, used in the treatment of depression can be responsible for acute angle-closure glaucoma by leading to mydriasis and ciliary effusion.

Clinical Neuroscience

[Current diagnosis and treatment of idiopathic intracranial hypertension]

SALOMVÁRY Bernadett, PÁNCZÉL Gyula, MARKIA Balázs, NAGY Gábor

[Background - Idiopathic intracranial hypertension is cha-racterized by raised intracranial pressure of unknown origin, leading to persisting visual loss if left untreated. Purpose - We assessed timing of surgery, and the efficacy and safety of ventriculo-peritoneal shunt. Methods - Retrospective analysis of 65 patients treated at our Neuro-ophthalmology Clinic between 2009 and 2017. Patients - We treated 15 children and 50 adults, 42 patients conservatively, and 23 surgically. The median age at presentation was 27 years for adults, 88% were obese, and 86% female. The age of children was 5-17 years, 40% were obese, and 53% girl. The commonest presentation symptom was headache in both groups (64%), followed by obscuration (33%), and double vision (22-31%). Subjective visual loss was only experienced in the surgical group (50%). The time until diagnosis was 2 weeks in both groups. However, the conservative group presented to our institute significantly earlier (3 weeks), than the surgical group (8 weeks). The follow-up time was 25 months. Results - In the conservative group papilla edema was 2D, visual acuity ≥0.7, and visual field loss was only mild. Time to cure was 3 months. In the surgical group both preoperative papilla edema (3D), and visual function were significantly worse. Indications for surgery were papilla edema, deteriorating visual function or relapse resistant to conservative treatment. Papilla edema disappeared 3 months after surgery, and visual field deficit improved significantly. We detected significant improvement in all aspects of visual function even at first neuro-ophthalmic control 4 days after surgery. However, visual acuity only improved in cases of preoperative acuity ≥0.3. Shunt revision occurred in 17%, and shunt infection in 8.5%. One patient suffered from persistent visual deterioration after surgery, and asymptomatic complication (epidural hematoma) was found in another patient. There was no surgical mortality. Conclusions - This is a curable condition with early diagnosis and adequate treatment, and persistent visual loss can be prevented. Surgery is effective and safe, close neuro-ophthalmic monitoring is mandatory for its optimal timing. Visual function of all patients can be preserved when operated on in time, whereas severe visual loss appears to be irreversible despite surgery.]

Clinical Neuroscience

[Administration of idarucizumab in spontaneous intracerebral hemorrhage under dabigatran-therapy]


[Introduction - Among antidotes in development for reversal of novel oral anticoagulants, dabigatran-specific idarucizumab was the first one to reach the market. Case presentation - We present the first Hungarian case of intracerebral hemorrhage under treatment with dabigatran, where idarucizumab was administered to suspend anticoagulation. Discussion - Our report is concordant with prior publications, confirming the efficacy of the antidote in reversing the effect of dabigatran, and thus, preventing intracerebral hematoma progression in the acute phase. Conclusion - Since there is no proven alternative to idarucizumab, conducting randomized clinical trials would be unethical. Therefore, besides case reports, positive results of prospective studies could help us revise therapeutic guidelines, and thus, improve the prognosis of dabigatran-associated intracerebral hemorrhages.]

All articles in the issue

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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

[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.]

Clinical Neuroscience

[Simultaneous occurrence of cavernous angioma and developental venous anomaly]

KUNCZ Ádám, VÖRÖS Erika, BODOSI Mihály

[The significance of the simultaneous occurrence of cavernous angioma and developmental venous anomaly, the diagnostic possibilities and the surgical treatment are discussed based upon three cases. Angiography and magnetic resonance imaging together are necessary to get an accurate diagnosis. According to the literature and to the fact that it is the cavernous angioma that mainly causes the symptoms, the cavernosus angioma should be resected, while the developmental venous anomaly should be spared.]