Clinical Neuroscience

[The role of Hungarian-rooted scholars in the development of Otoneurology ]

TAMÁS T. László1, GARAI Tibor1, TOMPOS Tamás1, MAIHOUB Stefani2, SZIRMAI Ágnes2

SEPTEMBER 30, 2019

Clinical Neuroscience - 2019;72(09-10)

DOI: https://doi.org/10.18071/isz.72.0295

[Despite of the symptoms of vertigo have been known since thousands of years, it was evident by the research of the pioneer scientists of the 19th century (Flourens, Ménière, Breuer and others) that dizziness can also be attributed to inner ear disfunctions. The discovery of the vestibulo-ocular reflex was an important milestone (Endre Hőgyes, 1884). The vestibulo-ocular reflex stabilizes images on the retina by rotating the eyes at the same speed but in the opposite direction of head motion. The milestone discovery of Hőgyes by stimulating individual labyrinth receptors and recording the activity of eye muscles were verified by János Szentágothai in 1950. Low-frequency lesions of the angular vestibulo-ocular reflex can be investigated by caloric test (Robert Bárány,1906), high-frequency lesions by head impulse test (Gabor Michael Halmagyi and Ian Stewart Curthoys, 1988).]

AFFILIATIONS

  1. Dr. Petz Aladár Megyei Oktató Kórház, Fül-Orr-Gégészeti és Fej-Nyaksebészeti Osztály, Gyôr
  2. Semmelweis Egyetem, Általános Orvostudományi Kar, Fül-Orr-Gégészeti és Fej-Nyaksebészeti Klinika, Budapest

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Drop foot is defined as difficulty of dorsiflexion of the foot and ankle due to weak anterior tibial, extensor hallucis longus and extensor digitorum longus muscles. Cauda equina syndrome, local peroneal nerve damage due to trauma, nerve entrapment, compartment syndrome and tumors are common etiologies. A 32-year-old male patient was applied with difficulty in dorsiflexion of both of his toes, feet and ankles after he had squatted in toilette for 6-7 hours (because of his haemorrhoid) after intense alcohol intake 2 weeks before. Acute, partial, demyelinating lesion in head of fibula segment of peroneal nerves was diagnosed by electromyography. This case was reported since prolonged squatting is an extremely rare cause of acute bilateral peroneal neuropathy. This type of neuropathy is mostly demyelination and has good prognosis with physical therapy and mechanical devices, but surgical intervention may be required due to axonal damage. People such as workers and farmers working in the squatting position for long hours should be advised to change their position as soon as the compression symptoms (numbness, tingling) appear.

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Coexistence of cervical vertebral scalloping, pedicle deficiencies and dural ectasia in type I neurofibromatosis

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Neurofibromatosis type 1 (NF-1; also known as Von Recklinghausen’s disease) is a common autosomal dominant disease that occurs in the general population at the rate of 1 in 3000. Many NF-1 patients present with spinal malformations. A 54-year-old female patient was admitted to the Outpatient Clinic of Dermatology with gradually increasing swelling and spots on the body that had been present for a long period of time. Cervical vertebral scalloping, pedicle deficiencies and dural ectasia (DE) were also detected. She was diagnosed with NF-1. NF-1 is routinely seen in dermatology practice. Coexistence of NF-1 with vertebral scalloping, pedicle deficiencies and DE rarely occurs. Our case is the second reported instance in the literature of NF-1 with a spinal anomaly in the cervical region, and the first reported instance of the coexistence of NF-1 with cervical vertebral scalloping, pedicle deficiencies and DE.

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[The Expanded Disability Status Scale scoring in patients with multiple sclerosis]

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[Gait disturbance is a major symptom in patients with multiple sclerosis. The Expanded Disability Status Scale (EDSS) was first used in clinical trials of multiple sclerosis for the assessment of disability, however it has become more and more widely used in clinical practice as well. Nowadays its use is essential in application of the new diagnostic criteria, the new clinical form classification and in monitoring the efficacy of therapies. EDSS is based on a standardised neurological examination, but focuses on those symptoms that are frequent in multiple sclerosis. Based on the examination it assesses seven functional systems: visual, brainstem, pyramidal, cerebellar, sensory, bowel-bladder and cerebral functions. EDSS scores can be determined based on the scores given in the functional systems and on testing the walking distance. In newer versions the “Ambulation score” has been added. This chapter clarifies the scores based on the maximal walking distance and the need for a walking aid to walk this distance. The Neurostatus/EDSS training method improves the reproducibility of the standardised neurological examination that forms the basis of the EDSS scoring. Of the tests assessing walking, the Timed-25 Foot Walk Test and the self-administered 12-Item Multiple Sclerosis Walking Scale are suitable for routine evaluation of walking performance. An increase of more than 20% in the Timed-25 Foot Walk may be considered a significant change in gait. ]

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Modern health worries in patients with affective disorders. A pilot study

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Background - Modern health worries (MHWs) are asso­ciated with various indicators of negative affect, conspiracy theories, and paranormal beliefs in healthy individuals. Purpose - The current pilot study aimed to assess MHWs and indicators of negative affect in patients with affective disorders (N = 66), as well as the possible associations between MHWs and paranoid and schizophrenic tendencies. Results - Compared to somatic patients, psychiatric patients showed higher levels of MHWs, somatosensory amplification, health anxiety, and somatic symptoms. Medium level associations between MHWs and paranoid (r = 0.35, p < 0.01) and schizophrenic (r = 0.37, p < 0.01) tendencies were also revealed. Somatosensory amplification (β = 0.452, p < 0.001) and paranoia (β = 0.281, p < 0.01) significantly contributed to MHWs in multiple linear regression analysis (R2 = 0.323, p < 0.001). Discussion - High (i.e. pathological) levels of negative affect can impact a number of related characteristics. Non-pathological paranoid tendencies might contribute to MHWs. The identification of paranoid tendencies seems to be relevant for the treatment of psychiatric patients exhibiting MHWs. Conclusion - Patients with affective disorders are characterized by higher levels of modern health worries, health anxiety, and somatosensory amplification. Modern health worries are associated with paranoid tendencies.

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[Newer studies on the strong link between sleep and epilepsy: Epilepsy as an epileptic transformation of sleep plastic functions]

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[Aims - Overview of the new data about the strong link of sleep and epilepsy and conjoining cognitive impairment. Methods - Search for relevant references and summary of our own research activity on the topic. Results - Strong interrealtionship exists between epilepsy and plastic brain functions (memory processing and synaptic homeostasis) and the working modes of NREM sleep. In the most frequent childhood and adult epilepsy networks responsible for plastic functions can be derailed to an epileptic level of excitability, and suffer a transitory or permanent epileptic transformation. Exampling on the three big epilepsies: absence epilepsy; medial temporal lobe epilepsy; and childhood idiopathic focal age dependent epilepsy spectrum we demonstrate the most important features of this epileptic transformation. The association of cognitive impairment to certain sleep dependent epilepsies gains explanation by the epilepsy caused interference with slow wave decline (ICFE) and memory consolidation (MTLE) during NREM sleep. This paper serves also to introduce the concept of sleep dependent system epilepsies. Conclusions - We provide evidences about shared mechanisms among sleep related epilepsies being the derailment of sleep plastic funcions toward exaggerated excitability determined by the inherent possibilities of the signal transduction properties. ]

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Clinical Neuroscience

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Purpose - Methylation is a key epigenetic modification of DNA and regarding its impact on epilepsy, it is argued that “DNA methylation may play an important role in seizure susceptibility and maintenance of the disorder”. DNA methylation status of KCC2 (SCL12A5) and NKCC1 (SCL12A2) associated with refractory temporal lobe epilepsy was investigated in our study. Materials and methods - Thirty-eight patients with temporal lobe epilepsy (TLE) who were diagnosed by video EEG monitoring and 32 healthy control subjects were included in the study. Twenty-three patients in TLE group were men and the remaining 15 were women. Among them, 27 had unilateral temporal focus (9 with right; 18 with left) and 11 patients had bilateral TLE. We analyzed promoter region methylation status of the KCC2 (SCL12A5) and NKCC1 (SCL12A2) genes in the case and control groups. Gene regions of interest were amplified through PCR and sequencing was accomplished with pyro-sequencing. Results - We found a significant relationship between TLE and methylation on the NKCC1. However, there was no association between TLE and methylation on the KCC2 gene. Also, we found no association between right or left and unilateral or bilateral foci of TLE. There was no relationship between TLE and methylation on the NKCC1and KCC2 genes in terms of mesial temporal sclerosis in cranial MRI, head trauma or febrile convulsions. Conclusion - The methylation of NKCC1 can be a mecha­nism of refractory temporal lobe epilepsy. There are limited findings about DNA methylation in TLE. Therefore, further studies with large sample sizes are necessary.