Clinical Neuroscience

Subgroups of brain stem tumors in childhood

PARAICZ Ervin, VAJDA János, CZIRJÁK Sándor, TÓTH Katalin

MAY 20, 1994

Clinical Neuroscience - 1994;47(05-06)

Neuroimaging (MR, CT) and surgical (microsurgery, CUSA, laser) techniques have revolutionised the neurosurgeon's view on tumours within the brain stem and emanated new perspective in their management. With the growing experience, it has nowadays become evident that the wide variety of tumours here and the very different ways they are treated call for a detailed classification of these tumours. Features such as MR and CT appearance, contrast uptake, exact localisation and also the neurological signs they evoke might be the sound basis for creating subgroups in this special cohort of brain tumour patients. Exophitic tumours growing dorsally into the 4th ventricle24 and intrinsic tumours of the cervicomedullary junction' have a favourable prognosis while the diffusely growing hypodens gliomas (high grade in many cases) or exophitic tumours growing laterally do not exhibit follow up longer than 6–15 months.



Further articles in this publication

Clinical Neuroscience

Epidermoid tumours of the posterior fossa

K. Bálint, F. Slowik, M. Kordás, J. Juhász, J. Julow

In opposite of the benign biological behaviour of the posterior fossa epidermoids the operation of these tumours a great challenge for the surgeon both theoretical and surgical point of view. We analysed our 14 operated cases clinico pathologically in this retrospective study.

Clinical Neuroscience

Transoral and anterolateral surgery of the cervical spine in ventral extradural pathological processes

EMIL Pásztor

This paper is a shortened version of an invited lecture given at the Karolinska Institute, Stockholm, on 17th September, 1993, illustrated with 120 slides.

Clinical Neuroscience

Correlation of clinical and molecular genetic findings in malignant brain stem tumors

V. K. Ammon, U. Sure, DN Louis, V. Deimling A.

Brain stem gliomas are rare, predominantly pediatric tumours. Histologically, they are comparable to adult supratentorial astrocytomas. Most of the pediatric brain stem tumours were classified as low-grade astrocytoma (WHO II), anaplastic astrocytoma (WHO III) or glioblastoma multiforme (GBM, WHO IV). Survival of patients with malignant brain stem gliomas as WHO grade III and IV rarely exceeds more than two years. Recently developed molecular genetic techniques gave new insights in tumour biology. Oncogenes and tumour suppressor genes are genetic alterations which can cause tumorous transformation and furthermore malignant progression. Molecular genetic studies of malignant brain stem gliomas have rarely been investigated. Therefore, we set out to study 12 such tumours clinically and 2 by molecular biological methods.

Clinical Neuroscience

Simultaneous occurence of unilateral multiplex meningiomas and syringomyelia

BÜKI András, MÉSZÁROS István, KÖVÉR Ferenc, KASÓ Gábor

Long lasting intracranial hypertension is considered to be a major pathogenic factor of syringomyelia in patients with a Chiari malformation or posterior fossa tumor.

Clinical Neuroscience

Different autoregulatory responses in the cerebellar cortex, neocortex and subcortical gray matter of the rat to systemic hypo- and hypertension


Cerebral autoregulation was investigated in the cerebral and cerebellar cortex, and subcortical gray matter (caudate nucleus) of the rat by means of Laser-Doppler flowmetry. As the vascular architecture of the basal ganglia, the cerebral cortex and the cerebellar cortex have substantial geometrical, onto genetical and pathological differences (3), we tested the working hypothesis that autoregulation of the blood supply to these areas may also be different. Laser-Doppler flowmetry has an ideal time resolution, and it enables analysis of flow-pressure curves (1, 2). The dependency of autoregulation on the rate of change in systemic blood pressure (SABP) in all three regions were confirmed. Control of CBF was significantly different in the subcortical gray matter and the neocortex. Interestingly, no autoregulatory capacity of the cerebellar vasculature was found.

All articles in the issue

Related contents

Clinical Neuroscience

Late simultaneous carcinomatous meningitis, temporal bone infiltrating macro-metastasis and disseminated multi-organ micro-metastases presenting with mono-symptomatic vertigo – a clinico-pathological case reporT

JARABIN András János, KLIVÉNYI Péter, TISZLAVICZ László, MOLNÁR Anna Fiona, GION Katalin, FÖLDESI Imre, KISS Geza Jozsef, ROVÓ László, BELLA Zsolt

Although vertigo is one of the most common complaints, intracranial malignant tumors rarely cause sudden asymmetry between the tone of the vestibular peripheries masquerading as a peripheral-like disorder. Here we report a case of simultaneous temporal bone infiltrating macro-metastasis and disseminated multi-organ micro-metastases presenting as acute unilateral vestibular syndrome, due to the reawakening of a primary gastric signet ring cell carcinoma. Purpose – Our objective was to identify those pathophysiological steps that may explain the complex process of tumor reawakening, dissemination. The possible causes of vestibular asymmetry were also traced. A 56-year-old male patient’s interdisciplinary medical data had been retrospectively analyzed. Original clinical and pathological results have been collected and thoroughly reevaluated, then new histological staining and immunohistochemistry methods have been added to the diagnostic pool. During the autopsy the cerebrum and cerebellum was edematous. The apex of the left petrous bone was infiltrated and destructed by a tumor mass of 2x2 cm in size. Histological reexamination of the original gastric resection specimen slides revealed focal submucosal tumorous infiltration with a vascular invasion. By immunohistochemistry mainly single infiltrating tumor cells were observed with Cytokeratin 7 and Vimentin positivity and partial loss of E-cadherin staining. The subsequent histological examination of necropsy tissue specimens confirmed the disseminated, multi-organ microscopic tumorous invasion. Discussion – It has been recently reported that the expression of Vimentin and the loss of E-cadherin is significantly associated with advanced stage, lymph node metastasis, vascular and neural invasion and undifferentiated type with p<0.05 significance. As our patient was middle aged and had no immune-deficiency, the promoting factor of the reawakening of the primary GC malignant disease after a 9-year-long period of dormancy remained undiscovered. The organ-specific tropism explained by the “seed and soil” theory was unexpected, due to rare occurrence of gastric cancer to metastasize in the meninges given that only a minority of these cells would be capable of crossing the blood brain barrier. Patients with past malignancies and new onset of neurological symptoms should alert the physician to central nervous system involvement, and the appropriate, targeted diagnostic and therapeutic work-up should be established immediately. Targeted staining with specific antibodies is recommended. Recent studies on cell lines indicate that metformin strongly inhibits epithelial-mesenchymal transition of gastric cancer cells. Therefore, further studies need to be performed on cases positive for epithelial-mesenchymal transition.

Clinical Neuroscience

Electrophysiological investigation for autonomic dysfunction in patients with myasthenia gravis: A prospective study


Myasthenia gravis (MG) is an autoimmune disorder of neuromuscular transmission. Autonomic dysfunction is not a commonly known association with MG. We conducted this study to evaluate autonomic functions in MG & subgroups and to investigate the effects of acetylcholinesterase inhibitors. This study comprised 30 autoimmune MG patients and 30 healthy volunteers. Autonomic tests including sympathetic skin response (SSR) and R-R interval variation analysis (RRIV) was carried out. The tests were performed two times for patients who were under acetylcholinesterase inhibitors during the current assessment. The RRIV rise during hyperventilation was better (p=0.006) and Valsalva ratio (p=0.039) was lower in control group. The SSR amplitudes were lower thereafter drug intake (p=0.030). As much as time went by after drug administration prolonged SSR latencies were obtained (p=0.043).Valsalva ratio was lower in the AchR antibody negative group (p=0.033). The findings showed that both ocular/generalized MG patients have a subclinical parasympathetic abnormality prominent in the AchR antibody negative group and pyridostigmine has a peripheral sympathetic cholinergic noncumulative effect.

Clinical Neuroscience

Atypical presentation of late-onset Sandhoff disease: a case report

SALAMON András , SZPISJAK László , ZÁDORI Dénes, LÉNÁRT István, MARÓTI Zoltán, KALMÁR Tibor , BRIERLEY M. H. Charlotte, DEEGAN B. Patrick , KLIVÉNYI Péter

Sandhoff disease is a rare type of hereditary (autosomal recessive) GM2-gangliosidosis, which is caused by mutation of the HEXB gene. Disruption of the β subunit of the hexosaminidase (Hex) enzyme affects the function of both the Hex-A and Hex-B isoforms. The severity and the age of onset of the disease (infantile or classic; juvenile; adult) depends on the residual activity of the enzyme. The late-onset form is characterized by diverse symptomatology, comprising motor neuron disease, ataxia, tremor, dystonia, psychiatric symptoms and neuropathy. A 36-year-old female patient has been presenting progressive, symmetrical lower limb weakness for 9 years. Detailed neurological examination revealed mild symmetrical weakness in the hip flexors without the involvement of other muscle groups. The patellar reflex was decreased on both sides. Laboratory tests showed no relevant alteration and routine electroencephalography and brain MRI were normal. Nerve conduction studies and electromyography revealed alterations corresponding to sensory neuropathy. Muscle biopsy demonstrated signs of mild neurogenic lesion. Her younger brother (32-year-old) was observed with similar symptoms. Detailed genetic study detected a known pathogenic missense mutation and a 15,088 base pair long known pathogenic deletion in the HEXB gene (NM_000521.4:c.1417G>A; NM_000521:c.-376-5836_669+1473del; double heterozygous state). Segregation analysis and hexosaminidase enzyme assay of the family further confirmed the diagnosis of late-onset Sandhoff disease. The purpose of this case report is to draw attention to the significance of late-onset Sandhoff disease amongst disorders presenting with proximal predominant symmetric lower limb muscle weakness in adulthood.

Hypertension and nephrology

[Association between cyclothymic affective temperament and hypertension]


[Affective temperaments (cyclothymic, hypertymic, depressive, anxious, irritable) are stable parts of personality and after adolescent only their minor changes are detectable. Their connections with psychopathology is well-described; depressive temperament plays role in major depression, cyclothymic temperament in bipolar II disorder, while hyperthymic temperament in bipolar I disorder. Moreover, scientific data of the last decade suggest, that affective temperaments are also associated with somatic diseases. Cyclothymic temperament is supposed to have the closest connection with hypertension. The prevalence of hypertension is higher parallel with the presence of dominant cyclothymic affective temperament and in this condition the frequency of cardiovascular complications in hypertensive patients was also described to be higher. In chronic hypertensive patients cyclothymic temperament score is positively associated with systolic blood pressure and in women with the earlier development of hypertension. The background of these associations is probably based on the more prevalent presence of common risk factors (smoking, obesity, alcoholism) with more pronounced cyclothymic temperament. The scientific importance of the research of the associations of personality traits including affective temperaments with somatic disorders can help in the identification of higher risk patient subgroups.]

Clinical Neuroscience

TikTok and tics: the possible role of social media in the exacerbation of tics during the COVID lockdown

NAGY Péter , CSERHÁTI Helga , ROSDY Beáta , BODÓ Tímea, HEGYI Márta , SZAMOSÚJVÁRI Judit , FOGARASI Joseph Dominic , FOGARASI András

Over the past year, many cases with newly onset or significantly exacerbated tic disorders were observed worldwide, where some aspects of the clinical presentation or the symptomatology were atypical for established tic diagnoses. Our purpose was to describe the atypical cases and raise relevant diagnostic issues. Consecutive cases with atypical tic presentations were documented. Five atypical tic cases are described. These cases shared some common characteristics, most notably the fact that all of them had been exposed to online presentation of ticking behaviour on social media platforms prior to the de novo development or exacerbation of their tics. Even though the order of events suggests causality and therefore the diagnosis of a functional tic disorder, unambiguous criteria for classifying atypical tics as functional symptoms are lacking. Differentiating neurodevelopmental and functional tics in childhood is currently problematic. Based on the currently unresolved issues in differential diagnosis, the importance of watchful waiting and behavioural interventions is highlighted to avoid unwarranted pharmacotherapy.