Clinical Neuroscience

[Chosen, possessed, epileptic]

JERNEY Judit

MARCH 30, 2008

Clinical Neuroscience - 2008;61(03-04)

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Clinical Neuroscience

[Evidence for the expression of parathyroid hormone 2 receptor in the human brainstem (in English language)]

BAGÓ Attila G., PALKOVITS Miklós, TED B. Usdin, SERESS László, DOBOLYI Árpád

[Background and purpose - The parathyroid hormone 2 receptor (PTH2R) is a G protein coupled receptor. Pharmacological and anatomical evidence suggests that the recently identified tuberoinfundibular peptide of 39 residues is, and parathyroid hormone and parathyroid hormone-related peptide are not, its endogenous ligand. Initial functional studies suggest that the PTH2R is involved in the regulation of viscerosensory information processing. As a first step towards clinical applications, herein we describe the presence of the PTH2R in the human brainstem. Material and methods - Total RNA was isolated from postmortem human cortical and brainstem samples for RT-PCR. Good quality RNA, as assessed on formaldehyde gel, was reverse transcribed. The combined cDNA products were used as template in PCR reactions with primer pairs specific for the human PTH2R. In addition, PTH2R immunolabelling was performed on free floating sections of the human medulla oblongata using fluorescent amplification immunochemistry. Results - Specific bands in the RT-PCR experiments and sequencing of PCR products demonstrated the expression of PTH2R mRNA in the human brainstem. A high density of PTH2R-immunoreactive fibers was found in brain regions of the medulla oblongata including the nucleus of the solitary tract, the spinal trigeminal nucleus, and the dorsal reticular nucleus of the medulla. Conclusion - Independent demonstration of the presence of PTH2R mRNA and immunoreactivity supports the specific expression of the PTH2R in the human brainstem. The distribution of PTH2R-immunoreactive fibers in viscerosensory brain regions is similar to that reported in mouse and rat suggesting a similar role of the PTH2R in human as in rodents. This finding will have important implications when experimental data obtained on the function of the TIP39-PTH2R neuromodulator system in rodents are to be utilized in human.]

Clinical Neuroscience

[Prevalence of stroke/cardiovascular risk factors in rural Hungary - A cross-sectional descriptive study (in English language)]

BODO Michael, THURÓCZY György, PÁNCZÉL Gyula, SIPOS Kornél, ILIÁS Lajos, SZÕNYI Péter, MIKE Bodó Jr, NEBELLA Tamás, BÁNYÁSZ Attila, NAGY Zoltán

[A multi-faceted survey was conducted in 1992-94 to ascertain the somatic, mental and socio-economic conditions of the residents of a village in eastern Hungary. Here we report data on prevalence of somatic disorders from the survey. Objectives - a) To collect and compare prevalence of known cardiovascular disease, including stroke risk factors, in a specific population (a Hungarian village); b) to test a computer-based mass screening device ("Cerberus") designed to identify individuals in the test population at high risk for stroke; c) to compare results obtained with Cerberus with known stroke risk/cardiovascular disease factors and traditional medical records. Methods - A cross-sectional survey (546 subjects) was conducted in Csengersima in the early 1990s, using the Cerberus screening system, which includes: 1. a questionnaire addressing the risk factors for stroke/cardiovascular disease; 2. amplifiers to record the pulse waves of cerebral (rheoencephalography) and peripheral arteries, electrocardiogram and electroencephalogram. Additionally, subjects were measured for carotid stenosis by Doppler ultrasound and 12-lead electrocardiogram; they were also screened for blood cholesterol, glucose, and triglyceride levels. Findings - Prevalence of the following stroke risk factors was identified: overweight, 63.25%; sclerotic brain arteries by rheoencephalogram, 54.29%; heart disease, 37.92%; pathologic carotid flow, 34.24%; smoking, 30.55%; high blood cholesterol, 28.70%; hypertension, 27.83%; high triglyceride, 24.35%; abnormality of electrocardiogram, 20%; high glucose, 15.95%; symptoms of transient ischemic attack, 16.07%; alcohol abuse, 6.74%; and diabetes, 4.53%. Conclusion - The study demonstrates a possible model for primary cardiovascular disease/stroke prevention. The simple, noninvasive test uses the bioimpedance method of measurement. This method offers a standardizable, costeffective, practical technique for mass screenings by identifying the population at high risk for cardiovascular disturbances, especially cerebrovascular disease. In this model, the rheoencephalogram can detect cerebrovascular arteriosclerosis in the susceptibility/presymptomatic phase, earlier than the Doppler ultrasound technique. The method also provides a model for storing analog physiological signals in a computer-based medical record and the first steps of turning it into an expert system also tested.]

Clinical Neuroscience

[Risk of mental disorders, their changes and somatic consideration in rural Hungary (in English language)]

SIPOS Kornél, BODO Michael, MAY Zsolt, LENDVAI Balázs, PIROS Andrea, SPITZER Nóra, PATAKY Ilona, NAGY Zoltán, BÁNYÁSZ Attila

[Objective - Although the primary purpose of the study reported here was to identify stroke risk factors among the residents of a village in eastern Hungary, the study also included a multi-faceted survey conducted in 1992-94 to ascertain the somatic, mental and socio-economic conditions of the residents. Here we report data from the survey on prevalence of mental disorders (a cross-sectional descriptive study). Method - The screenings included the following tests administered to 535 subjects: anxiety, depression, dementia, neurosis were measured; recent medical records were compared to survey data for 330 of the same subjects. Findings - The summary of prevalence of mental disorders measured in this study was as follows: anxiety 34.7% (severe), dementia 44.68% (mild), depression 66% (mild), 15.94% (medium), 7.88% (severe), neurosis 66.73% (mild, medium, and severe). Medical records maintained by village physicians since 1960 differed from the results obtained in the present study. A treatment gap was observed between mental health treatment for neurosis, as indicated by medical records, and the diagnostic prevalence of neurosis as measured by the survey instruments: there were three times as many people diagnosed as neurotic in the survey as had been noted in village medical records. Additionally, the unique position of cerebrovascular alteration was established between the mental and somatic factors. Conclusion - The study demonstrates the successful simultaneous collection of a wide spectrum of data on somatic conditions, mental disorders, and socio-economic status of the subjects. The study showed that 1. patientcentered medical care can simultaneously address both somatic and mental factors; 2. it is possible to decrease the treatment gap in mental health; 3. there is value in systematic collection of data in order to optimize the planning of prevention, health care costs and decision making.]

Clinical Neuroscience

[Szepesváraljai Sarbó Artúr (1867-1943)]

EMED Alexander

Clinical Neuroscience

[REMOVAL OF INTRASPINAL SPACE-OCCUPYING LESIONS THROUGH UNILATERAL PARTIAL APPROACH, THE “HEMI-SEMI LAMINECTOMY”]

BANCZEROWSKI Péter, VAJDA János, VERES Róbert

[Objective - The conventional dorsal surgical approaches used in removal of intraspinal space-occupying lesions by unroofing the spinal canal, often result the destruction of dorsal bony structures, sacrifice the interspinosus/supraspinosus ligament complexes and stripping of the paraspinal muscles causing a pathologic biomechanical milieu may lead to spinal deformities, instability. Various less invasive techniques exist to save the integrity and to prevent the instability of the spinal column and allow removal of intraspinally located space-occupying lesions at the same time. The authors discuss the experiences with unilateral partial laminectomy approach in removal of intraspinally, mainly lateral, intra- or extradurally located pathologic lesions. Methods - The unilateral partial laminectomy, in which the laminas were preserved (hemi-semi laminectomy) was performed in 86 symptomatic patients to remove space-occupying intra- or extradurally located lesions of the cervical, thoracic and lumbar spinal canal. Symptoms were local or radicular pain, motor, sensory and vegetative disturbances. Results - Adequate surgery of the lesions located within the spinal canal was achieved in all patients using this approach. The hemi-semi laminectomy was performed at one spinal level in 68 patients, two levels in 15 and three levels in 3. The affected spine was the cervical in 16, the cervico-thoracic in 6, the thoracic in 35, the thoraco-lumbar in 10 and lumbar region in 19 cases. Histological results were as follows: 32 intradural meningeomas, 27 neurinomas, 10 ependymomas, 3 arachnoid cysts, 2 cavernomas and extradurally 4 epidural haemorrhage, 5 epidural abscesses and 3 dural vascular malformations. Conclusion - The unilateral partial laminectomy (named hemi-semi laminectomy) approach for the mainly laterally located intra- or extradural lesions, confined to one side, allow to minimize resection of and injury to tissues not directly involved in the pathologic process, while affording a safe and thorough removal of space-occupying pathologies and decompression of neural structures located in a spinal canal. Two additional advantages come from this technique in cases of misjudged level or at re-operation.]

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Clinical Neuroscience

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

JANSZKY József, HORVÁTH Réka, KOMOLY Sámuel

[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

Simultaneous subdural, subarachnoideal and intracerebral hAemorrhage after rupture of a peripheral middle cerebral artery aneurysm

BÉRES-MOLNÁR Anna Katalin, FOLYOVICH András, SZLOBODA Péter, SZENDREY-KISS Zsolt, BERECZKI Dániel, BAKOS Mária, VÁRALLYAY György, SZABÓ Huba, NYÁRI István

The cause of intracerebral, subarachnoid and subdural haemorrhage is different, and the simultaneous appearance in the same case is extremely rare. We describe the case of a patient with a ruptured aneurysm on the distal segment of the middle cerebral artery, with a concomitant subdural and intracerebral haemorrhage, and a subsequent secondary brainstem (Duret) haemorrhage. The 59-year-old woman had hypertension and diabetes in her medical history. She experienced anomic aphasia and left-sided headache starting one day before admission. She had no trauma. A few minutes after admission she suddenly became comatose, her breathing became superficial. Non-contrast CT revealed left sided fronto-parietal subdural and subarachnoid and intracerebral haemorrhage, and bleeding was also observed in the right pontine region. The patient had leucocytosis and hyperglycemia but normal hemostasis. After the subdural haemorrhage had been evacuated, the patient was transferred to intensive care unit. Sepsis developed. Echocardiography did not detect endocarditis. Neurological status, vigilance gradually improved. The rehabilitation process was interrupted by epileptic status. Control CT and CT angiography proved an aneurysm in the peripheral part of the left middle cerebral artery, which was later clipped. Histolo­gical examination excluded mycotic etiology of the aneu­rysm and “normal aneurysm wall” was described. The brain stem haemorrhage – Duret bleeding – was presumably caused by a sudden increase in intracranial pressure due to the supratentorial space occupying process and consequential trans-tentorial herniation. This case is a rarity, as the patient not only survived, but lives an active life with some residual symptoms.

Clinical Neuroscience

A case report of Morvan syndrome

AYTAC Emrah, ACAR Türkan

Morvan syndrome is a rare disease characterized by peripheral nerve hyperexcitability, encephalopathy, dys­autonomia and significant insomnia. The patient, who was included in the present study, was followed-up at our clinics for confusion, myokymia, hyperhidrosis, epileptic seizures, tachycardia, agitation, hypokalemia, and hyponatremia. The cranial MRI of the patient demonstrated hyperintensities at the T2 and FLAIR sections of the medial temporal lobe and insular lobes. Electromyography and neurotransmission examination results were concordant with peripheral nerve hyperreactivity. Contactin-associated protein-like 2 antibodies and leucine-rich glioma inactivated protein 1 antibodies were detected as positive. The patient was diagnosed with Morvan syndrome; intravenous immunoglobulin and corticosteroid treatment was started. Almost full remission was achieved. This very rare syndrome implies challenges in diagnosis and treatment; however, remission can be achieved during the follow-up. In addition, caution is needed in the long-term follow-up of these patients regarding the development of malignancies.

Clinical Neuroscience

[Sturge Weber type 3 presenting with occipital epileptic seizure: case report ]

SERİNDAĞ Cansu Helin, EREN Fulya, KARAHAN Gökçen Muazzez, GUL Gunay, SELCUK Hakan, KARA Batuhan, SOYSAL Aysun

[Sturge Weber syndrome is the third most common neurocutaneous syndrome after neurofibromatosis and tuberous sclerosis. Three distinct types were identified. Type 3 with leptomeningeal involvement alone is the rarest among other types. The reported case is a 21-years-old female patient without any known chronic disease. She admitted to the emergency department after visual symptoms and headache, followed by generalized tonic clonic seizure. EEG of the patient showed left occipital seizure activity. The contrast enhanced magnetic resonance imaging (MRI) showed left occipital leptomeningeal angioma. Digital substraction angiography (DSA) revealed minimal blushed contrast enhancement on late venous phase and lack of superficial cortical veins. Her focal seizures were under control with levatiracetam and lacosamide treatment. The reported case is unique because of the late onset presentation with focal seizure without mental retardation.]

Clinical Neuroscience

Valproic acid associated pleuropericardial effusion: case report

DEMIR Figen Ulku

Introduction - Valproic acid is an effective antiepileptic and mood stabilizer used in the treatment of many neurological and psychiatric disorders. Although there are frequently seen side effects, effusions between layers of pleural and pericardial membranes are rare to be seen. Case - Pleuropericardial effusion was detected in a 23 years old woman who was under valproic acid treatment because of epileptic seizure. After 1 year of valproic acid treatment, patient complained of dyspnea. As all the researches intended on etiology were usual, valproic acid has been thought to be responsible for the matter. Control examination after 1.5 months regarding the end of treatment revealed complete recovery of pleuropericardial effusion. Discussion - Pleural and pericardial effusions are rarely seen complications related to the use of valproic acid. It must also be kept in mind that valproic acid causes a potential for such side effects which can be blamed etiologically when the other possibilities for patients are excluded.