Clinical Neuroscience

[About childhood polyneuritis ]


OCTOBER 01, 1967

Clinical Neuroscience - 1967;20(10)

[In our presentation of 6 cases of "allergic" polyneuritis in children, we draw attention to the polymorphism of the clinical picture, the frequent involvement of the cranial nerve and the likely frequency of asymptomatic cases. The rare prolonged cases may be confused with heredodegenerative diseases. Their recognition is very important, because their prospects of recovery can be substantially improved by cortisone treatment. ]



Further articles in this publication

Clinical Neuroscience

[Fine-structure analysis of muscle lesions in dystrophia muscularis progressiva]

KORÉNYI Both András, LAPIS Károly, GALLAI Margit

[Authors have used light and electron microscopy to examine material from a biopsy of a sister parathyroid patient with dystrophia musculorum progressiva. The fine structural alterations were consistent with those in the fine structure of neurogenic atrophies, denervated or otherwise damaged striated muscles. In dystrophia musculorum progressiva, the most common lesion affecting most muscle fibres is swelling of the sarcoplasmic reticulum system, which may be a morphological picture of the Richterich stage of decompensated enzyme synthesis. ]

Clinical Neuroscience

[CSF cytology problems in neurological carcinoma]


[Authors describe 3 cases of neurological carcinoma in which suspicious cells were found in the CSF. In patient 1, a brain metastasis of a bronchial carcinoma with normal cell count and lymphocyte-like tumour cells were detected. In patient 2, a malignant papilloma of the plexus papilloma infiltrating the meninges caused chronic meningitis and the CSF cytology picture suggested a tumour-like origin of the disease process. In patient 3, metastasis of cervical carcinoma resulted in cauda syndrome. Epithelial cells showing signs of malignancy were detected in the CSF. Authors have reported polychromasia, anisocytosis, anisonucleosis and polymorphism as the predominant features of CSF tumour cells. The differential diagnostic features of inflammatory and tumorigenic lesions of the CSF cell image are analysed and demonstrated with images. ]

Clinical Neuroscience

[The generalised spike-wave spasm mechanism and the reticular awakening system ]


[In the context of an atypical status epilepticus consisting of partly generalized spike-wave and partly generalized de-synchronizing seizures, the relationship between the generalized spike-wave mechanism and the reticular awakening system is discussed. On the basis of the accumulated experimental data and clinical experience, as well as the lessons learned from our case study, we suggest that cortical inhibition of the facilitatory influence of the reticular waking system plays an essential role in the spike-wave mechanism. ]

Clinical Neuroscience



[The author reports on his experiences at the Psychiatric Research Institute in Prague in June 1967. ]

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Clinical Neuroscience

[Consensus statement of the Hungarian Clinical Neurogenic Society about the therapy of adult SMA patients]

BOCZÁN Judit, KLIVÉNYI Péter, KÁLMÁN Bernadette, SZÉLL Márta, KARCAGI Veronika, ZÁDORI Dénes, MOLNÁR Mária Judit

[Background – Spinal muscular atrophy (SMA) is an autosomal recessive, progressive neuromuscular disorder resulting in a loss of lower motoneurons. Recently, new disease-modifying treatments (two drugs for splicing modification of SMN2 and one for SMN1 gene replacement) have become available. Purpose – The new drugs change the progression of SMA with neonatal and childhood onset. Increasing amount of data are available about the effects of these drugs in adult patients with SMA. In this article, we summarize the available data of new SMA therapies in adult patients. Methods – Members of the Executive Committee of the Hungarian Clinical Neurogenetic Society surveyed the literature for palliative treatments, randomized controlled trials, and retrospective and prospective studies using disease modifying therapies in adult patients with SMA. Patients – We evaluated the outcomes of studies focused on treatments of adult patients mainly with SMA II and III. In this paper, we present our consensus statement in nine points covering palliative care, technical, medical and safety considerations, patient selection, and long-term monitoring of adult patients with SMA. This consensus statement aims to support the most efficient management of adult patients with SMA, and provides information about treatment efficacy and safety to be considered during personalized therapy. It also highlights open questions needed to be answered in future. Using this recommendation in clinical practice can result in optimization of therapy.]

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.]

Lege Artis Medicinae

[The role of intergenerational stress in the background of anorexia nervosa]

TÚRY Ferenc, PÁSZTHY bea

[In childhood and adolescence, family therapy is a crucial therapeutic method in the treatment of anorexia nervosa. Among the family stress factors there are the tensions often too obvious between the parental and grandparental generations. Three case reports are presented, where the transgenerational tensions played an important role in the family of a young patient, suffering from anorexia nervosa. During the family therapy, the treat­ment of interrelated family stress factors and focusing on transgenerational relations proved to be crucial in the successful outcomes. While treating anorexia nervosa by family therapy there are not only important the recognition and treatment of tensions within the nuclear family, but also those of stressed relations emerging from grandparental generation through multigenerational transfer mechanisms. This treatment may be successful either with the grandparent’s inclusion, or exclusion. ]

Clinical Neuroscience

[The long-term follow-up of enzyme replacement treatment in late onset Pompe disease]

MOLNÁR Mária Judit, BORSOS Beáta, VÁRDI Visy Katalin, GROSZ Zoltán, SEBÕK Ágnes, DÉZSI Lívia, ALMÁSSY Zsuzsanna, KERÉNYI Levente, JOBBÁGY Zita, JÁVOR László, BIDLÓ Judit

[Pompe disease (PD) is a rare lysosomal disease caused by the deficient activity of acid alpha-glucosidase (GAA) enzyme due to mutations in the GAA gene. The enzymatic deficiency leads to the accumulation of glycogen within the lysosomes. Clinically, the disease has been classically classified in infantile and childhood/adult forms. Presently cc. close to 600 mutations distributed throughout the whole gene have been reported. The c.-32-13T>G splice mutation that is very common in patients of Caucasian origin affected by the childhood/adult form of the disease, with an allelic frequency close to 70%. Enzyme replacement treatment (ERT) is available for the patients with Pompe disease (Myozyme). In this paper, we are presenting the long term follow up of 13 adult onset cases treated more than 5 years. The longest follow up was 15 years. To evaluate the treatment efficacy, the 6 minutes walking test (6MWT) and the respiratory functions were monitored annually. The analysis revealed that at the beginning of ERT for 3-4 years the 6MWT had been generally increasing, then it declined, and after 10 years it was lower in 77% of the cases than it had been at the start of the treatment. In 23% of the cases the 6MWT increased during the follow up time. Only one of the patients become wheelchair dependent during the follow-up period. The respiratory function showed similar results especially in supine position. A high degree of variability was observed among patients in their responses to the treatment, which only partially associated with the antibody titer against the therapeutic protein. The efficacy of the ERT was associated with the type of the disease causing mutation, the baseline status of the disease, the lifestyle and the diet of the patient. The long-term follow up of the patients with innovative orphan drugs is necessary to really understand the value of the treatment and the need of the patients.]

Clinical Neuroscience

[Personalised epilepsy treatment]


[Epilepsy is one of the most common chronic neurological disease in childhood. Patients with epilepsy – even with so-called benign epilepsy – need medication for years. During this time, children go through a very big change, not only gaining weight and height, but also changing hormonal and metabolic processes. Maturation processes in different brain areas also take place at different rates depending on age. All of these should be considered when preparing a therapeutic plan. In everyday practice after the diagnosis of epilepsy, the applied drug is most often selected based on the shape and type of seizure. However, a number of other factors need to be considered when designing a therapeutic strategy: 1. efficacy (form of epilepsy, type of seizure), 2. age, gender, 3. pharmacological properties of the drug, 4. adverse drug reaction profile, 5. lifestyle (community), figure (skinny, corpulent, obese), 6. other comorbidities (nutrition, behavioral and learning problems, circulatory disorders, kidney or liver disease), 7. expected interactions with other drugs already used, 8. genetics, 9. other aspects (drug registration and prescription rules). The purpose of this article is to help to decide which antiepileptic drugs are expected to have the least side effects in a particular child with different comorbidities and which medications should be avoided if possible.]