Clinical Neuroscience

[Pediatric intraventricular tumors]

MARKIA Balázs, GYORSOK Zsuzsanna, KORDÁS Mariann, BOGNÁR László

DECEMBER 20, 2008

Clinical Neuroscience - 2008;61(11-12)

[Pediatric intraventricular tumors present a well circumscribed group from surgical point of view. These tumors growing in the ventricular system cause hydrocephalus in most of the cases, the presenting symptoms are the signs of raised intracranial pressure. The mass lesion may remain silent for a long period, especially in infancy due to compensatory mechanisms, and the tumor might reach extreme size making the surgery a real challenge. This group has very specific postoperative problems resulting from the disturbance of CSF circulation. In this study we present the retrospective analysis of 55 patient operated for intraventricular tumor in the National Institute of Neurosurgery between 1991 and 2006. Data were analysed regarding histological type, presenting symptoms, type of surgical approach, radicalitiy of the resection and postoperative complications. In addition to our own results brief presentation of the specific histological groups is given based on the available literature.]

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Clinical Neuroscience

[Pneumococcal meningitis in children - 9 1/2-year-experience at Szent László hospital, Budapest, Hungary ]

IVÁDY Balázs, LIPTAI Zoltán, ÚJHELYI Enikő, BALÁZS György

[Background and objective - No recent publications are available about pneumococcal meningitis in Hungarian children. The aim of this study was to collect data of epidemiological, clinical and prognostic features of pneumococcal meningitis in children treated at Szent László Hospital, Budapest, Hungary. Methods - We conducted a retrospective review of medical charts and follow-up records of patients aged 1 to 18 years admitted to our Pediatric and Pediatric Intensive Care Units due to pneumococcal meningitis between 1st Jan 1998 and 30th Jun 2007. Results - 31 children with 34 cases of pneumococcal meningitis were admitted to our hospital in the study period. Two children developed recurrent illness. The mean age was 6 years, 26% were under 1 year of age. The mean duration of hospital stay was 21 days, 97% required intensive care. Frequent clinical symptoms were fever (100%), nuchal rigidity and vomiting (78%), altered mental status (71%), Kernig's and Brudzinski's signs (58%) and seizures (41%). Otitis media, sinusitis, mastoiditis were present in 44%, 58%, 41%, respectively. Subdural effusion, parenchymal cerebral lesion and sinus thrombosis were documented in 5, 3 and 2 cases, respectively. One third of the patients recieved ceftriaxon, two thirds were administered ceftriaxon and vancomycin. Adjunctive therapy with dexamethason was given to 91% of the children. 70% of patients required mechanical ventillation. 9 patients (25%) required endoscopic sinus surgery. In 13 cases (38%) mastoidectomy, in 5 children (15%) neurosurgery was performed. The case fatality rate was 23.5%. 8 (23.5%) patients had mild or moderate, 1 child (3%) developed severe neurological sequelae. Conclusion - Pneumococcal meningitis in children remains a source of substantial morbidity and mortality in childhood. The long hospital stay, the frequent need for intensive care and severe neurologic sequelae emphasize the importance of early diagnosis, early treatment and prevention with pneumococcal conjugate vaccines.]

Clinical Neuroscience

[Changes of the immune functions in patients with eating disorders]

PÁLI Anikó Andrea, PÁSZTHY Bea

[Aims - In this study we investigated whether calorie restriction or redundant food intake influences the function of regulatory T cells (Tregs), and their main regulators (dendritic cells and macrophages), or the targets of Tregs, CD4+ lymphocytes. Patients and methods - We investigated 11 white adolescents (10 girls and 1 boy) with anorexia nervosa, 12 obes adolescents and 10 healthy controlls. With flow cytometry we determined the prevalence of Tregs, myeloid and plasmacytoid dendritic cells. We applied intracellular staining to investigate TNF-alpha and IL-12 production of macrophages, moreover IL-2, IL-4, and IFN-gamma production of CD4+ cells. We also determined calcium flux kinetics upon activation in CD4+ cells. Results - We did not find any difference between obese, anorectic and control individuals in the prevalence of Tregs, dendritic cells, TNF-alpha and IL-12 positive macrophages, IL-4 and IFN-gamma positive CD4+ lymphocytes. We found that the prevalence of IL-2 positive lymphocytes after activation was lower in anorectic than in control subjects [median (range): 11.50 (7.60-15.30) vs. 13.50 (12.00-22.00), p=0.023], and in obese patients, too [12.50 (8.50-15.50) vs. 13.50 (12.00-22.00), p=0.028]. IFN-gamma/IL-4 ratio in CD4+ cells was higher in obese patients compared with control (p=0.046). The calcium flux characteristics of lymphocytes upon activation differed markedly in anorectic and healthy subjects as maximal calcium levels developed later in anorectic patients [86 (45- 232) vs. 215 (59-235) second, p<0.05]. We also tested the association between lymphocyte activation parameters and patients' clinical status, but did not find any association between the variables. Discussion - Our results suggest that the antigen presenting cell - regulatory T cell - CD4+ lymphocyte axis might be affected by calorie and nutritional disturbances, further studies are needed to elucidate the underlying processes.]

Clinical Neuroscience

[Devastating epileptic encephalopathypseudoencephalitis: the new type of catastrophe epilepsy in our department]

NEUWIRTH Magdolna, PARAICZ Éva, LIPTAI Zoltán

[Purpose - Analysis of history of our five patients with intractable epilepsy whose illnes have begun with prolonged status epilepticus (SE) and high-grad fever of unknow cause. Methods - Retrospective study analysis of selected five intractable epileptic patients at a median age of 11.5 (8-14) years. Results - All children had normal development before epilepsy begun. Intractable SE lasted 3-10 (median seven) days by four patients and three months by one patient. The cause of illness was unknow at the beginning and the MRI were normal. Intractable epilepsy followed the SE in all cases without any latent period. Follow-up of the children was 3-15 (median 9.5) years. The seizures came continually with few-day-long breaks, antiepileptic drugs were ineffective. Semiology of seizures, EEG, and functional imaging examinations (PET, SPECT) referred to temporal and frontal lobe damages. Later on, the MR images showed hippocampal sclerosis in one patient and mild generalized brain atrophy in the others. During the years, cognitive deterioration and behavioral problems have been realized. The most severe patient developed tetraparesis, fell in vigil coma and died after five years. Conclusions - The symptoms of our patients fulfilled the criteria of devastating epileptic encephalopathy in schoolaged children.]

Clinical Neuroscience

[How do temporal lobe seizures changeby age?]

FOGARASI András

[Seizure semiology describes different - motor, sensory, autonomic, etc. - aspects of epileptic seizures. Several semiological studies showed already that different epilepsies - especially temporal lobe epilepsy - contain age-dependent features. In our researches, we tried to assess these subjective aspects with as objective methods as possible. We gave a comprehensive (preictal, ictal, and postictal) description of seizure semiology in patients fulfilling the gold standard criteria of semiological studies: being seizure free after temporal lobe resections. Our studies based on a large population, assess epileptic features at different levels of brain maturation. They help to understand why certain semiological axes show special characteristics at different ages. In this review, I summarize the most important results of our seizure semiology studies in temporal lobe epilepsy.]

Clinical Neuroscience

[Clinical and genetic diagnosis of dravet syndrome: report of 20 cases]

SIEGLER Zsuzsa, NEUWIRTH Magdolna, HEGYI Márta, PARAICZ Éva, PÁLMAFY Beatrix, TEGZES Andrea, BARSI Péter, KARCAGI Veronika, CLAES Lieve, DE Jonghe Peter, HERCZEGFALVI Ágnes, FOGARASI András

[Objective and background - Severe myoclonic epilepsy in infancy (SMEI; Dravet's syndrome) is a malignant epilepsy syndrome characterized by prolonged febrile hemiconvulsions or generalized seizures starting in the first year of life. Later on myoclonic, atypical absence, and complex partial seizures appear. When one of these seizure forms is lacking the syndrome of borderline SMEI (SMEB) is defined. Psychomotor delay resulting in mental retardation is observed during the second year of life. In most patients a de novo sodium channel alfa-1 subunit (SCN1A) mutation can be identified. By reviewing the clinical, laboratory, and neuroimaging data of our SMEI patients diagnosed between 2000 and 2008, we would like to share our experiences in this rare but challenging syndrome. Our results will facilitate the earlier and better diagnosis of Hungarian children with SMEI. Patients and methods - Clinical, EEG, MRI and DNA mutation data of 20 SMEI patients treated in the Bethesda Children’s Hospital (Budapest) were reviewed. Results - The first seizure appeared at age 6.3±3.0 months. At least one of the first two seizures were complex febrile seizures in 19/20 and unilateral seizures in 12/20 children. All children except for one showed hemiconvulsions at least once; all children had seizures lasting longer than 15 minutes. Eight of twenty patients had SMEB. DNA diagnostics identified an SCN1A mutation in 17 patients (6 missense, 4 nonsense, 4 frameshift, 2 splice site, 1 deletion) while 3 children had no mutation. Conclusion - Early diagnosis of SMEI is important for the avoiding unnecessary examinations and false therapies as well as for genetic counselling. Typical symptoms of SMEI are early and prolonged febrile hemiconvulsions with neurological symptoms, mental retardation and secondary seizure types later on. The presence of an SCN1A mutation supports the diagnosis. We propose the availability of molecular diagnostics and stiripentol therapy for SMEI children in Hungary.]

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Cases of inborn errors of metabolism diagnosed in children with autism

CAKAR Emel Nafiye, YILMAZBAS Pınar

Autism spectrum disorder is a neurodevelopmental disorder with a heterogeneous presentation, the etiology of which is not clearly elucidated. In recent years, comorbidity has become more evident with the increase in the frequency of autism and diagnostic possibilities of inborn errors of metabolism. One hundred and seventy-nine patients with diagnosis of autism spectrum disorder who presented to the Pediatric Metabolism outpatient clinic between 01/September/2018-29/February/2020 constituted the study population. The personal information, routine and specific metabolic tests of the patients were analyzed retrospectively. Out of the 3261 patients who presented to our outpatient clinic, 179 (5.48%) were diagnosed with autism spectrum disorder and were included in the study. As a result of specific metabolic examinations performed, 6 (3.3%) patients were diagnosed with inborn errors of metabolism. Two of our patients were diagnosed with classical phenylketonuria, two with classical homocystinuria, one with mucopolysaccharidosis type 3D (Sanfilippo syndrome) and one with 3-methylchrotonyl Co-A carboxylase deficiency. Inborn errors of metabolism may rarely present with autism spectrum disorder symptoms. Careful evaluation of the history, physical examination and additional findings in patients diagnosed with autism spectrum disorder will guide the clinician in the decision-making process and chose the appropriate specific metabolic investigation. An underlying inborn errors of metabolism may be a treatable cause of autism.

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

[Effective therapy in highly active pediatric multiple sclerosis ]

MERÔ Gabriella, MÓSER Judit, LIPTAI Zoltán, DIÓSZEGHY Péter, BESSENYEI Mónika, CSÉPÁNY Tünde

[Multiple sclerosis (MS) is typically a disease of young adults. Childhood MS can be defined in patients under 18 years of age, although some authors set the limit un­der the age of 16 formerly known as “early-onset multiple sclerosis” or “juvenile multiple sclerosis”, seen in 3-5% of all MS patients. Nowadays, owing to ever-evolving, better diagnostic tools and well-traced, strictly defined diagnostic criteria, childhood MS is showing an increasing incidence worldwide (0.05-2.85/100 000). MS is characterized by recurrent episodes of the central nervous system with demyelination separated in space and time. In childhood almost exclusively the relapsing-remitting (RR) type of MS occurs. Based on experience in adults, the goal in the pediatric population is also the early diagnosis, to initiate adequate DMT as soon as possible and to achieve symptom relief and good quality of life. Based on efficacy and safety studies in the adult population, inter­feron β-1a and glatiramer acetate were first approved by the FDA and EMA for the treatment of childhood MS also. The increased relapse rate and rapid progression of childhood MS and unfavorable therapeutic response to nearly 45% of the first DMT necessitated the testing of more effective and second-line drugs in the population under 18 years of age (PARADIGMS, CONNECT). Although natalizumab was reported to be effective and well-tolerated in highly active RRMS in childhood, evidence based studies were not yet available when our patients’ treatment started. In this article, we report on the successful treatment of three active RRMS patients with individually authorized off-label use of natalizumab.]

Clinical Neuroscience

[Rehabilitation possibilities and results after neurosurgical intervention of brain tumors ]

DÉNES Zoltán, TARJÁNYI Szilvia, NAGY Helga

[Objectives - Authors examined the rehabilitation possi­bi­lities, necessities, and results of patients after operation with brain tumor, and report their experiences. Method - Retrospective, descriptive study at the Brain Injury Rehabilitation Unit, in National Institute for Medical Rehabilitation. Patients - Patients were admitted consecutively after rehabilitation consultation, from different hospitals, following surgical intervention of brain tumors, between 01 January 2001 and 31 December 2016. Patients participated in a postacute inpatient rehabilitation program, in multidisciplinary team-work, leaded by Physical and Rehabilitation Medicine specialist included the following activities: rehabilitation nursing, physical, occupational, speech, psychological and neuropsychological therapy. Results - At the rehabilitation unit, in the sixteen-year period 84 patients were treated after operation with brain tumor. Patients arrived at the unit after an average of 41 days to the time of the surgical intervention (range: 10-139 days), and the mean length of rehabilitation stay was 49 days (range: 2-193 days). The mean age of patients was 58 years (20-91), who were 34 men and 50 women. The main symptoms were hemiparesis (64), cognitive problems (26), dysphagia (23), aphasia (16), ataxia (15), tetraparesis (5), and paraparesis (1). The mean Barthel Index at the time of admission was 35 points, whereas this value was 75 points at discharge. After the inpatient rehabilitation, 73 patients improved functionally, the status of 9 patients did not show clinically relevant changes, and 2 patients deteriorated. During the rehabilitation 10 patients required urgent interhospital transfer to brain surgery units, 9 patients continued their oncological treatment, two patients continued rehabilitation treatment at another rehabilitation unit, and after rehabilitation 73 patients were discharged to their homes. Conclusions - Inpatient rehabilitation treatment could be necessary after operation of patients with brain tumor especially when functional disorders (disability) are present. Consultation is obligatory among the neurosurgeon, rehabilitation physician and the patient to set realistic rehabilitation goals and determine place and method of rehabilitation treatment, but even at malignancies cooperation with oncological specialist also needed. Authors’ experience shows benefits of multidisciplinary rehabilitation for patients after brain tumor surgery. ]

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