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)

DOI: https://doi.org/10.18071/isz.70.0343

[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.]

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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

Health anxiety mediates the connection between somatosensory amplification and self-reported food sensitivity

ELIESON M. Linn, DÖMÖTÖR Zsuzsanna, KÖTELES Ferenc

Background - The frequency of self-reported food sensitivity (SFS) is increasing, and has a negative impact on the well-being and everyday functioning of the affected people. A considerable proportion of SFS cannot be medically explained. The lack of knowledge of its origin and treatment causes further stress in those affected. Purpose - This study aims to get a better understanding of the psychological background of the condition. Methods - A non-representative community sample (N=335; age: 35.1±13.18 yrs; 75.8% female) completed an English on-line questionnaire assessing somatosensory amplification, health anxiety, modern health worries (MHWs), beliefs concerning the scientific validity of complementary and alternative medicine (CAM), holistic beliefs on health and illness. Results - In multiple binary logistic regression analyses, SFS were associated with CAM related beliefs, somatosensory amplification, and health anxiety after controlling for age and gender. The connection between somatosensory amplification and SFS were completely mediated by health anxiety. No differences between the two groups were found with respect to MHWs, worries about the harmful effects of various artificial components in food, and holistic health beliefs. Discussion: More positive attitudes toward CAM might be based on the lack of conventional treatment, rather than on higher levels of MHWs or a more holistic worldview. Both the existence of symptoms and the presence of health anxiety might be needed for the development and maintenance of SFS. Conclusions - The findings support the notion that somatosensory amplification and health anxiety might play a role in the development and maintenance of SFS.

Clinical Neuroscience

Correlation of vitamin D levels with electrophysiological findings and pain in the patients with Carpal Tunnel Syndrome

DEMIRYÜREK Enes Bekir, SENTÜRK Asli

Objective - This study aimed to assess the correlation between vitamin D deficiency and electrophysiological findings and pain level in patients with symptoms of carpal tunnel syndrome (CTS). Patients and method - A total of 131 patients with symptoms of CTS, 70 with vitamin D deficiency and 61 without vitamin D deficiency, were included in the study. Using demographic data and findings from electrophysiological examinations, the patients were divided into two groups based on their vitamin D level (Group 1: <20 ng/ml; Group 2: ≥20 ng/ml). The Boston Carpal Tunnel Syndrome Questionnaire was used to assess their CTS- related pain level. Results - Although the rate of CTS in the patients with a low vitamin D level was found to be high, no statistically significant correlation was observed between low vitamin D level and the frequency and severity of CTS. Additionally, the pain and functional loss ratio induced by CTS was found to be higher in the group with a lower vitamin D level than in the group with normal levels. Conclusion - Low vitamin D levels may increase the severity of CTS symptoms. Treatment of vitamin D deficiency in patients with CTS can play a role in reducing pain and disability.

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.]

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Clinical Neuroscience

[99-mTc-HMPAO single photon emission computed tomography examinations in genetically determined neurometabolic disorders]

ARANKA László, AMBRUS Edit, VÖRÖS Erika, SVEKUS András, KÓBOR Jenõ, BEREG Edit, PALATKA János, PÁVICS László

[The aim of our study was to determine regional cerebral blood flow (rCBF) abnormalities in different types of enzymopathies. Patients and methods - Among the patients with genetically determined enzymopathies 3 patients had aminoacidopathies, and 11 had different types of encephalopathies, from which 10 had mitochondrial encephalomyopathy (MEMP), and 1 patient had hyperuricaemic encephalopathy. Besides the mentioned 14 patients, 1 had ceroid lipofuscinosis and another patient had tuberous sclerosis. The further distribution of the MEMP patients’ group was the following - 5 patients had MEMP with lactic acidosis, 5 had Leigh’s disease (subacute necrotizing encephalopathy), from which 1 had cytochrome-c-oxidase deficiency (COX). Additionally in all patients were performed cerebral MRI and SPECT examination 10 min. after intravenous administration of 20 Mbq/kg 99 mTc-HMPAO. Results - Fourteen out of 16 SPECT findings were pathologic, showing decreased focal frontal/temporal/temporoparietal cerebral blood perfusion. Aminoacidopathic group - all the 3 patients revealed pathologic signs from the aminoacidopathic patients’ group. Among them the ornithine transcarbamylase (OTC) heterozygous female patient with left-sided hemiparesis caused by hyperammonemic stroke at 10 month-age, showed right sided temporoparietal, occipital and left frontal hypoperfusion, nearly 6 years after the cerebral vascular attack. This finding might be resulted because of diaschisis. Mitochondrial encephalo-myopathic (MEMP) group - all the four patients with MEMP and lactic acidosis showed focal hypoperfusion in the temporal region, while the perfusion was normal in the COX deficient patient and in 2 Leigh’s disease (subacute necrotizing encephalopathy) patients. In the remaining 1 Leigh’s patient frontotemporal hypoperfusion was found. In all patients there were non specific structural abnormalities detected by MRI - cortical and subcortical atrophy, and scattered demyelination foci. In the case of ceroid lipofuscinosis the MRI showed cerebral atrophy and cerebellar hypoplasia, and the SPECT showed right frontal and occipital hypoperfusion, bilateral parietal physiological riping process. The patient with tuberous sclerosis showed bilateral temporo-occipital hypoperfusion. Conclusion - 1. SPECT images demonstrated hypoperfusion rCBF changes in 14 out of all 16 patients. 2. Regional cerebral/cerebellar hypoperfusion was detected by SPECT in mitochondrial encephalomyopathies, with lactate acidosis and aminoacidopathies giving high informative value about the cerebral perfusion.]

Clinical Neuroscience

[Severe intracranial hypotension in an adolescent with Marfan syndrome. Case report]

ROSDY Beáta, KOLLÁR Katalin, MÓSER Judit, VÁRALLYAY György, KORDÁS Mariann

[Spontaneous intracranial hypotension is a rare complication of connective tissue disorders. One of them is Marfan syndrome. It predisposes the patient to meningeal diverticula. Possibly after minor unrecognised head trauma or secondary to Valsalva manoeuvre cerebrospinal fluid leak from meningeal diverticula can happen. It causes postural headache. We describe a 15 year old adolescent female newly diagnosed with Marfan syndrome, who presented with intractable postural headache. Our patient's brain MRI showed bilateral frontal subdural effusion, narrow ventricles, downward displacement of the brainstem, enlarged cervical venous plexi, dural ectasias and wide nerve root sleeves. Radionuclide cisternography demonstrated CSF leaks at multiple sites. She could not be treated conservatively, but was successfully treated by epidural saline injections. Control brain and cervical MRI confirmed her healing, too. At the two and half year follow up visit, she was completely well. So we recommend this easiest technic to use before epidural autolog blood patches.]

Clinical Neuroscience

[Neurological and psychiatrical prospects of apathy]

GYURIS Jenő

[During his long practice as head physician of a neurological and psychiatrical department with over 100 beds performed the examination and department of more than a hundred thousand patients. Based on the acquired experience and the data of the most recent literature he treats every aspect of the apathy syndrome. He emphasizes the multidisciplinary approach during both establishing the causes and the examination and treatment of patients. In order to clarify the diagnosis consultations with other disciplines must be used as well as the the knowledge provided by the now essential CT, MRI, PET, SPECT. The author discusses the international therapeutical possibilities and practice after the recently alredy possible exact diagnosis.]

Clinical Neuroscience

[Posterior ischaemic optic neuropathy]

BOÓR Krisztina, KOVÁCS Krisztina, RÓZSA Anikó, PÁNCZÉL Gyula, SZILVÁSSY Ildikó, GÁCS Gyula

[Here one case report of the posterior ischaemic optic neuropathy, a rare and underdiagnosed form of the non arteritic ischaemic optic neuropathy is presented, to underline the value of the MRI in the diagnosis. The ischaemic optic neuropathy is the infarction of the optic nerve. Depending on the affected segment of optic nerve (optic nerve head or retrobulbar segment) two subclasses exist: the anterior (AION) and the posterior (PION) ischaemic optic neuropathy. Ischaemic optic neuropathy characterized by sudden, painless, mononuclear loss of vision, and/or visual field defect, that is accompanied by a diagnostic picture of the optic disc fundus only in the case of the AION. The diagnosis of the PION is based on a diagnosis of exclusion described by Hayreh in 1981. The macular and retinal laesions, the etiological role of toxic agent, the compression and the inflammation of the optic nerve all have to be excluded. The differential diagnosis between the PION and the retrobulbar neuritis is more difficult. Nowadays, in addition to the case history and the clinical data the diagnosis of the PION could be confirmed with help of VEP (visual evoked potential) and MRI. In the case of an old woman who had a sudden, painless visual loss of left eye we confirmed the diagnosis of PION with MRI which was presumed after had excluding other etiological factors.]

Lege Artis Medicinae

[TAKAYASU’S ARTERITIS DETECTED IN AN EARLY PHASE]

SZÁNTÓ Antónia, VEISZ Richárd, KRENÁCS László, CSIKI Zoltán, GRIGER Zoltán, ZEHER Margit

[INTRODUCTION - Takayasu’s arteritis is an idiopathic inflammatory disease of the great elastic arteries, mainly of the aorta and its primary branches. It primarily affects young women. CASE REPORT - A 20-year-old woman presented with general inflammatory symptoms that had begun 3 to 4 months before. Earlier she underwent a biopsy from a mass on the neck thought to be a lymph node, which on histopathological examination turned out to be a non-necrosing granulomatosis in the wall of a large artery. Based on this finding as well as on the presence of fever, weight loss, carotid murmur, anaemia, elevated platelet count, increased sedimentation rate, the lack of pulse and the findings of imaging examinations, Takayasu’s arteritis was diagnosed. Treatment was started with high-dose steroid monotherapy, which resulted in clinical and laboratory remission after a short time. The imaging findings (neck soft tissue sonogram and MRI) also showed improvement, however, a moderate but definite disease activity was still present, thus steroid treatment was supplemented with azathioprin. CONCLUSION - It is important to follow the disease both clinically and with imaging techniques for an early recognition of the need for changing or adjusting the treatment.]