Clinical Neuroscience

[A CASE OF FRONTOTEMPORAL LOBAR DEGENERATION WITH UBIQUITIN-POSITIVE INTRANEURONAL INCLUSIONS]

LEEL-ŐSSY Lóránt, RÉVÉSZ Tamás, ALMÁSI Kálmán, SZŰCS Iván, SZABÓ Éva

JULY 30, 2007

Clinical Neuroscience - 2007;60(07-08)

[The case of a 57 year-old-man is reported who has been treated several times because of his relatively expedite mental decline which has begun four years before his death. His first complaints were forgetfulness, mild changes in his behaviour, confusion and difficulty in speech. The neuropsychiatric examinations displayed a mild difficulty in naming and sometimes comprehension of words, although his speech was gramatically correct. Furthermore the patient presented a very severe decrease in short term memory with dementia and confusion. These symptomes together with the results of CT and test examinations established the diagnosis of Alzheimer's disease. Finally pneumonia afflicted the patient during the last hospitalization and he died. Histopathological examinations of the brain showed a severe, mainly temporofrontal atrophy caused by an extensive cortical neuronal loss and gliosis without neurofibrillar degenerations and senile plaques which characterize the Alzheimer's disease. Tau-positivity Pick- and Lewy-bodies may not be found. The loss of neurons associated in some places with spongiosity of laminar form. The ubiquitine-positive intracytoplasmic inclusions proved to be the most characteristic feature in the swollen neurons. These mainly occurred in the gray matter of the mediobasal part of the temporal lobe. The positivity of GFAP immunocytochemistry revealed a definite astrocytosis in the affected parts of the gray matter. In the temporal and frontal cortex scattered ballooned cells (achromatic or Pick cells) were seen in αB-crystallin immunohistochemistry. These findings confirmed the diagnosis of the case of frontotemporal lobar degeneration with ubiquitin-positive intraneuronal inclusions (FTLD-U) without tau-positivity. The separation of the different forms in the group of the frontotemporal dementias is recommended by means of the modern immunocytochemical examinations.]

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Clinical Neuroscience

[DYT1 POSITIVE GENERALISED DYSTONIA: A CASE STUDY OF TWO SIBLINGS]

BEREZNAI Benjámin, BARACZKA Krisztina, NAGY Zoltán, MOLNÁR Mária Judit

[The early-onset generalised dystonia is a dyskinetic movement disorder with a wide variety in phenotype and poor response to pharmacological treatment. A mutation on the DYT1 gene is responsible for the disease in more than 50% of cases with typical early-onset dystonia beginning in a limb. We describe the medical history of two brothers with first signs of focal dystonia at age 12 starting with right side lower limb dystonia of the older brother and writers cramp of the younger one. In both over a period of 6 and 10 years dystonia generalised. The negativ results of MRI, electrophisiological testing and muscle biopsy corroborate the diagnosis of primary dystonia. The DNA from the older patient was tested for the 3 bp deletion in exon 5 of the DYT1 gene by restriction enzyme. The positive result confirmed the diagnosis of early-onset primary dystonia. A short synopsis of routine molecular genetic tests indications and treatment options is outlined.]

Clinical Neuroscience

[INVESTIGATION OF THE EFFECT OF VINPOCETINE ON CEREBRAL BLOOD FLOW AND COGNITIVE FUNCTIONS]

VALIKOVICS Attila

[Introduction - Vinpocetine has been widely used in the treatment of ischaemic cerebrovascular diseases and dementias of vascular type. Chronic cerebral hypoperfusion plays an important role in the development of certain types of dementia. In consequence of complex mode of action vinpocetine plays a significant role in the improvement of cerebral hypoperfusion. The symptoms of mild cognitive impairment considered as “predementia” are similar to those of dementia, although milder. Aims - The authors investigated the characteristics of the blood flow parameters of patients with ischemic stroke and mild cognitive impairment both in resting conditions or following chemical stimulus as well as they investigated the severity of mental deterioration in the two patient groups. In a pilot study the authors examined the influence of 12-week long oral vinpocetine therapy on the blood flow parameters and cognitive functions in the two patient groups. Methods - The authors studied the blood flow velocity of a. cerebri media in resting conditions and after 30 sec of breath holding with transcranial Doppler before treatment and after a 12-week long oral vinpocetine treatment. At the same time psychometric tests (MMSE, ADAS-Cog) were used in order to examine cognitive functions, while the general condition of the patients were scored by Clinical Global Impression (CGI) scale. Results - After a 12-week long oral vinpocetine treatment the increase of blood flow velocity in resting conditions compared to the baseline values was significant in the vascular group. The percent increase of mean velocity after the breath holding TCD test showed a significant increase compared to the baseline in both patient groups. The authors found a significant improvement of cognitive functions after a 12-week long oral vinpocetine therapy using psychometric tests. The improvement was identical in both groups. The general condition of patients improved significantly according to both the investigator's and the patients' opinion; patients with mild cognitive impairment judged the improvement higher. Conclusions - Vinpocetine improved the cerebrovascular reserve capacity in both patient groups and favourably influenced the cognitive status and general condition of patients with chronic hypoperfusion. The authors recommend the use of vinpocetine for the treatment of patients with mild cognitive impairment.]

Clinical Neuroscience

[In memoriam István Somogyi MD]

SZILÁRD János, JÁRDÁNHÁZY Tamás

Clinical Neuroscience

[CONGRESS CALENDAR]

Clinical Neuroscience

[PROTEIN BIOMARKERS IN EXPERIMENTAL MODELS AND IN THE CLINICAL CARE FOR TRAUMATIC BRAIN INJURY]

LÜCKL János, FARKAS Orsolya, PÁL József, KÖVESDI Erzsébet, CZEITER Endre, SZELLÁR Dóra, DÓCZI Tamás, KOMOLY Sámuel, BÜKI András

[Traumatic brain injury is the leading cause of mortality in Hungary in the population under 40 years of age. In Western societies, like the United Sates, traumatic brain injury represents an extreme social-economic burden, expected to become the third leading cause of mortality until 2020. Despite its’ epidemiological significance, experimental therapeutic modalities developed in the last few decades did not prove efficient in the clinical care of severe traumatic brain injury. The reason for such a lack of success in terms of translating experimental results to clinical treatment at least partially could be explained by the paucity and the low sensitivity and specificity of clinical parameters endowing us to monitor the efficacy of the therapy. The drive for finding clinical parameters and monitoring tools that enable us to monitor treatment efficacy as well as outcome focused recent attention on biomarkers (and) surrogate markers that are based on rational pathological processes associated with/operant in traumatic brain injury. This review summarizes those biomarkers that could purportedly be used to monitor the treatment of the severely head injured while also providing information on salvageability facilitating the conduction of more rationally designed clinical studies.]

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Cyanocobalamin and cholecalciferol synergistically improve functional and histopathological nerve healing in experimental rat model

ALBAY Cem, ADANIR Oktay, AKKALP Kahraman Asli, DOGAN Burcu Vasfiye, GULAEC Akif Mehmet, BEYTEMUR Ozan

Introduction - Peripheral nerve injury (PNI) is a frequent problem among young adults. Hopefully, regeneration can occur in PNI unlike central nervous system. If nerve cut is complete, gold standard treatment is surgery, but incomplete cuts have been tried to be treated by medicines. The aim of the study was to evaluate and compare clinical and histopathological outcomes of independent treatment of each of Vitamin B12 (B12) and Vitamin D3 (D3) and their combination on sciatic nerve injury in an experimental rat model. Materials and methods - Experimental animal study was performed after the approval of BEH Ethics Committee No. 2015/10. 32 rats were grouped into four (n=8) according to treatment procedures, such as Group 1 (controls with no treatment), Group 2 (intraperitoneal 1 mg/kg/day B12), Group 3 (oral 3500 IU/kg/week D3), Group 4 (intraperitoneal 1 mg/kg/day B12+ oral 3500 IU/kg/week D3). Sciatic Functional Index (SFI) and histopathological analysis were performed. Results - SFIs of Group 2, 3, 4 were statistically significantly higher than controls. Group 2 and 3 were statistically not different, however Group 4 was statistically significantly higher than others according to SFI. Axonal degeneration (AD) in all treatment groups were statistically significantly lower than in Group 1. AD in Group 4 was significantly lower than in Group 2 and 3; there was no significant difference between Group 2 and 3. There was no significant difference between Group 1,2 and 3 in Axonolysis (A). But A of Group 4 was significantly very much lower than all others. Oedema- inflammation (OE-I) in all treatment groups were significantly lower than in Group 1; there was no significant difference between Group 2 and group 4. OE-I in Group 2 and 4 were significantly lower than in Group 3. There were no significant differences between Group 1, 2 and 3 in damage level scores; score of Group 4 was significantly lower than of Group 1. Conclusions - B12 and D3 were found effective with no statistically significant difference. But combined use of B12 and D3 improve nerve healing synergistically. We recommend combined use of B12 and D3 after PNI as soon as possible.

Clinical Neuroscience

Delirium due to the use of topical cyclopentolate hydrochloride

MAHMUT Atum, ERKAN Çelik, GÜRSOY Alagöz

Introduction - Our aim is to present a rare case where a child had delirium manifestation after instillation of cyclopentolate. Case presentation - A 7-year old patient was seen in our outpatient clinic, and cyclopentolate was dropped three times at 10 minutes intervals in both eyes. The patient suddenly developed behavioral disorders along with gait disturbance, and complained of visual hallucinations 20-25 minutes after the last drop. The patient was transferred to intensive care unit and 0.02 mg/kg IV. physostigmine was administered. The patient improved after minutes of onset of physostigmine, and was discharged with total recovery after 30 minutes. Conclusion - Delirium is a rare systemic side effect of cyclopentolate. The specific antidote is physostigmine, which can be used in severely agitated patients who are not responding to other therapies.

Clinical Neuroscience

Evaluation of anxiety, depression and marital relationships in patients with migraine

DEMIR Fıgen Ulku, BOZKURT Oya

Aim - The aim of this study was to evaluate the frequency and characteristics of attacks in patients with migraine, to determine the effects of anxiety or depressive symptoms, and to evaluate the marital relationships of patients with migraine. Method - Thirty patients who were admitted to the neurology outpatient clinic of our hospital between July 2018 and October 2018 and were diagnosed with migraine according to the 2013 International Headache Society (IHS) diagnostic criteria were included in this cross-sectional study. Age, sex, headache frequency and severity, depressive traits, marital satisfaction and anxiety status were examined. We used the Beck Depression Inventory (BDI), State-Trait Anxiety Inventory (STAI), Maudsley Marital Questionnaire (MMQ) and Visual Analogue Scale (VAS) for measuring relevant parameters. Results - The mean severity of migraine pain according to VAS scale was 6.93 ± 1.41 and the mean number of migraine attacks was 4.50 ± 4.24. The mean BDI score of the patients was 12.66 ± 8.98, the mean MMQ-M score was 19.80 ± 12.52, the mean MMQ-S score was 13.20 ± 9.53, the mean STAI-state score was 39.93 ± 10.87 and the mean STAI-trait score was 45.73 ± 8.96. No significant correlation was found between age, number of migraine attacks, migraine duration, migraine headache intensity, and BDI, STAI and MMQ scores (p>0.05). But there was a positive correlation between MMQ-S and scores obtained from the BDI and STAI-state scales (p<0.05). Conclusion - In this study more than half of the migraine patients had mild, moderate or severe depression. A positive correlation was found between sexual dissatisfaction and scale scores of depression and anxiety.

Clinical Neuroscience

Isolated hypoglossal nerve palsy due to a jugular foramen schwannoma

ÖZTOP-CAKMAK Özgür, VANLI-YAVUZ Ebru, AYGÜN Serhat, BASTAN Birgül, EGEMEN Emrah, SOLAROGLU Ihsan, GURSOY-OZDEMIR Yesemin

Introduction – Although the involvement of the hypoglossal nerve together with other cranial nerves is common in several pathological conditions of the brain, particularly the brainstem, isolated hypoglossal nerve palsy is a rare condition and a diagnostic challenge. Case presentation – The presented patient arrived to the hospital with a history of slurred speech and an uncomfortable sensation on his tongue. Neurological examination showed left-sided hemiatrophy of the tongue with fasciculations and deviation towards the left side during protrusion. Based on the clinical and MRI findings, a diagnosis of hypoglossal nerve schwannoma was made. Discussion – Hypoglossal nerve palsy may arise from multiple causes such as trauma, infections, neoplasms, and endocrine, autoimmune and vascular pathologies. In our case, the isolated involvement of the hypoglossal nerve was at the skull base segment, where the damage to the hypoglossal nerve may occur mostly due to metastasis, nasopharyngeal carcinomas, nerve sheath tumors and glomus tumors. Conclusion – Because of the complexity of the region’s anatomy, the patient diagnosed with hypoglossal nerve schwannoma was referred for gamma knife radiosurgery.

Clinical Neuroscience

To handle the HaNDL syndrome through a case: The syndrome of headache with neurologic deficits and cerebrospinal fluid lymphocytosis

ÇOBAN Eda, TEKER Ruken Serap, SERİNDAĞ Helin, SAKALLI Nazan, SOYSAL Aysun

The syndrome of headache with neurologic deficits and cerebrospinal fluid lymphocytosis (HaNDL) is a rare entity. This disease has been related to migrainous headaches. It is a benign, self-limited disorder, which is characterized by fluctuating neurological symptoms and cerebrospinal fluid lymphocytosis. We describe a case of a 47 years old man with acute onset of headache and aphasia. Cerebrospinal fluid analysis revealed a lymphocytic pleocytosis (25 cells/μl, 100% lymphocytes). Electroencephalogram showed moderate slow rhythm in the left hemisphere, with temporoparietal predominance, and without epileptiform activity. His blood tests as well as magnetic resonance imaging (MRI) results were normal. With the diagnosis of HaNDL syndrome the patient was accepted in the Department of Neurology and discharged with full recovery.