Clinical Neuroscience

[Spontaneous intracerebral haematomas in children]

VELKEY Imre1, DOBAI József2, GYARMATI János3

MARCH 20, 1993

Clinical Neuroscience - 1993;46(03-04)

[The authors report two cases of spontaneous intracerebral haematomas in children. Both children presented with serious symptoms of acute intracerebral haemorrhage. Investigations do not reveal the cause of the haemorrhage in either of the cases and angiography detected no arteriovenous malformation. The term ,,occult” is reserved for these cases, as spontaneous intracerebral haematomas do not result in space-occupying process or CSF passage blockage and may be treated medically.]

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  1. Borsod-Abaúj-Zemplén Megyei Kórház-Rendelőintézet Gyermekegészségügyi Központ
  2. Borsod-Abaúj-Zemplén Megyei Kórház-Rendelőintézet Gyermekegészségügyi Központ I. Ideg-Idegsebészeti Osztály
  3. Borsod-Abaúj-Zemplén Megyei Kórház-Rendelőintézet Gyermekegészségügyi Központ Radiológiai és Izotóp Diagnosztikai Intézet

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Clinical Neuroscience

[Post-stroke depression]

ASZALÓS Zsuzsa, PATAKY Ilona, SIMON Andrea, NAGY Zoltán

[The pathogenesis of depression following cerebrovascular accident (post-stroke depression) is poorly understood. Thirty seven (28%) out of 134 patients from the „Budapest Stroke Data Bank" were found to be depressed. Thirty two patients (86%) in this group were affected by depression within three months or the stroke. Diagnosis of depression was bades on CES-D, Ham-D and Zung scales, and a word fluency test was performed with 11 depressed and 11 non-depressed patients. Comparing the frequency of post-stroke depression in groups with ischemic damage of the carotid versus the vertebrobasilar system, as well as the left versus right middle cerebral artery, no significant differences were found. Depression reduced the activities of daily living (score: 7.8) compared to the activities of non-depressed patients (score: 3.8) at the same score of stroke – severity (4.7 versus 4.1). These observations suggest that post-stroke depression influences the rehabilitation of stroke patients, therefore the diagnosis and treatment of post-stroke depression may increase the efficacy of stroke rehabilitation.]

Clinical Neuroscience

[The role of brainstem auditory evoked potentials in the diagnosis of vertebrobasilar insufficiency]

MOGYORÓS Ilona

[The diagnostic usefulness of brainstem auditory evoked potentials was compared with other diagnostic possibilities. Brainstem auditory evoked potential was examined in patients with vertebrobasilar insufficiency. Based on the duration of clinical symptoms, patients were divided into three groups: 85 patients with transient ischaemic attack (TIA), 31 patients with prolonged ischaemic symptoms, and 35 patients with chronic symptoms were examined. The latency and interpeak latency of the auditory evoked potentials increased in 49,3 per cent. A subgroup within the TIA group was specially tested. The patients belonging to this group were admitted to the clinic soon after the onset of TIA. Acoustic evoked potentials showed increased latencies and interpeak latencies in 45,8 per cent of this group. The increasing latencies of the waves I. and III. and the interpeak latancies of I-II. and I-V. waves were the most frequent differences. Otoneurology demonstrated more frequent functional disturbances in brainstem than in acoustic evoked potentials in the second and third group. Computerized tomography is a really helpful only in examining chronic cases, while the CT reports 3,6 per cent hypodensities in the first group and 17,3 per cent in the third group. Brainstem auditory evoked potential testing is the most sensitive diagnostic method in the diagnosis of transient ischaemic attack.]

Clinical Neuroscience

[The effect of cerebrospinal fluid drainage on middle cerebral artery blood flow velocity in conditions with raised intracarnial pressure]

BARZÓ Pál, DÓCZI Tamás, KOPNICZKY Zsolt, ROTYIS Mária, BODOSI Mihály

[Middle cerebral artery (MCA) blood flow velocity and intracranial pressure (ICP) were recorded in 42 patients suffering from raised ICP. A major (ICP25 mmHg) or moderate (251CP15 mmHg) degree of intracranial hypertension was reduced by means of either continuous or intermittent CSF drainage. Measurements of MCA blood flow velocity were carried out with transcranial Doppler sonography (TCD). Three types of reactions were observed with regard to cerebral blood flow velocity (CBFV) changes in response to CSF drainage. Patients in Group 1 demonstrated pressure passive CBFV changes throughout the observed cerebral perfusion pressure (PP) range. In Group 2, cerebrospinal fluid (CSF) drainage brought about a transitory increase in CBFV for a few minutes. In Group 3, the reduction of ICP to the normal level did not influence CBFV at all. The pulsatility index (PI) of the cerebrovascular reserve capacity (CVR), which was highest in Group 1, changed in contrast with the CBFV changes in Groups 1 and 2. The pressure-passive velocity pattern (in Group 1) suggests that the blood vessels were at nearly maximum dilatation and were perhaps failing to constrict properly in response to increased PP. As recovery proceeded (Group 2), the mechanism became effective, thereby reestablishing autoregulation. Whereas clinical signs and computed tomography reveal only the trend of the ICP, TCD provides the possibility of a semi-quantitative evaluation of ICP changes and seems especially promising in the rapid assessment of the efficacy of treatment aimed at ICP reduction.]

Clinical Neuroscience

[Measurements of regional cerebral blood flow and blood flow velocity in experimental intracarnial hypertension: infusion via the cisterna magna in rabbits]

BARZÓ Pál, DÓCZI Tamás, CSETE Klára, BUZA Zoltán, BODOSI Mihály

[The cerebral blood flow velocity (CBFV), as measured by transcranial Doppler sonography (TCD) via the transorbital route in the intracranial segment of internal carotid artery (ICA), and the regional cerebral blood (volume) flow (rCBF) in the corresponding cortical areas, as measured by the hydrogen clearance technique, were determined in 8 New Zealand White rabbits undergoing cisterna magna infusion for elevation of the intracranial pressure (ICP). In the lower range of autoregulation, i. e. at perfusion pressures (PP) between 80 and 40 mm of mercury and even below this, the changes in (CBFV) and CBF showed a strong correlation (0,86) under conditions with standard PCO2 (PaCO2)=35+2 mm of mercury). Autoregulation was exhausted at 40 mm of mercury and the cerebrovascular resistance (CVR) was minimal. Below this PP, the CBF and CBV dropped sharply, whereas CVR, gradually increased, indicating that, despite the maximally dilated resistance vessels, the intracranial hypertension causes the vascular resistance to increase, possibly via blocking of the venous outflow. Our results confirmed that non-invasive and easily (even at the bedside) applicable measurements of CBFV changes could substitute the cumbersome and expensive isotope measurements of CBF in intracranial hypertension.]

Clinical Neuroscience

[Benign focal amyotrophy]

DIÓSZEGHY Péter, MOLNÁR Mária, MOLNÁR Tibor, MECHLER Ferenc

[A 36 year old female is presented with focal segmental atrophy and weakness of the right lower limb affecting predominantly the lower leg. The slight asymmetry presentsince in her childhood started to progress at the beginning of the fourth decade. The EMG and the muscle histology revealed chronic neurogenic changes, which were consistent with spinal motoneuron lesions. The electrophysiological investigations verified the focal segmental localisation of the involvement. The benign course and the good prognosis of this clinical syndrome point out the importance of its recognition and separation from other motoneuron diseases. The cause of this disorder is unknown; probably, it has heterogeneous etiology.]

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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

[Is the implementation of Vojta therapy associated with faster gross motor development in children with cerebral palsy? ]

SANZ-MENGIBAR Jose Manuel , MENENDEZ-PARDIÑAS Monica , SANTONJA-MEDINA Fernando

[Vojta therapy has been reported as clinically beneficial for strength, movement and gross motor activities in individual cases and is being included within the second of three levels of evidence in interventions for cerebral palsy. The goal of this study is to understand the effect of Vojta therapy on the gross motor function. Our clinical trial followed a one group, pre-post design to quantify rates of changes in GMFM-88 after a two-months period undergoing Vojta therapy. A total of 16 patients were recruited. Post-intervention acceleration rates of GMFM-88-items acquisition (0.005; p<0.001) and Locomotor Stages (1.063; p<0.0001) increased significatively following Vojta the­rapy intervention. In this study, Vojta therapy has shown to accelerate the acquisition of GMFM-88-items and Loco­motor Stages in children with cerebral palsy younger than 18 months. Because functional training was not utilised, and other non-Vojta therapy intervention did not influence the outcome, Vojta therapy seems to activate the postural control required to achieve uncompleted GMFM-88-items. ]

Clinical Neuroscience

[Family planning in multiple sclerosis: conception, pregnancy, breastfeeding]

RÓZSA Csilla

[Family planning is an exceptionally important question in multiple sclerosis, as women of childbearing age are the ones most often affected. Although it is proven that pregnancy does not worsen the long-term prognosis of relapsing-remitting multiple sclerosis, many patients are still doubtful about having children. This question is further complicated by the fact that patients – and often even doctors – are not sufficiently informed about how the ever-increasing number of available disease-modifying treatments affect pregnancies. Breastfeeding is an even less clear topic. Patients usually look to their neurologists first for answers concerning these matters. It falls to the neurologist to rationally evaluate the risks and benefits of contraception, pregnancy, assisted reproduction, childbirth, breastfeeding and disease modifying treatments, to inform patients about these, and then together come to a decision about the best possible therapeutic approach, taking the patients’ individual family plans into consideration. Here we present a review of relevant literature adhering to international guidelines on the topics of conception, pregnancy and breastfeeding, with a special focus on the applicability of approved disease modifying treatments during pregnancy and breastfeeding. The goal of this article is to provide clinicians involved in the care of MS patients with up-to-date information that they can utilize in their day-to-day clinical practice. ]

Clinical Neuroscience

[Disease burden of Duchenne muscular dystrophy patients and their caregivers]

PÉNTEK Márta, HERCZEGFALVI Ágnes, MOLNÁR Mária Judit, SZŐNYI László Pál, KOSZTOLÁNYI György, PFLIEGLER György, MELEGH Béla, BONCZ Imre, BRODSZKY Valentin, BAJI Petra, SZEGEDI Márta, POGÁNY Gábor, GULÁCSI László

[Background and purpose - Data on the disease burden of Duchenne Muscular Dystrophy are scarce in Hungary. The aim of this study was to assess patients’ and their caregivers’ health related quality of life and healthcare utilisations. Methods - A cross sectional survey was performed as part of the European BURQOL-RD project. The EQ-5D-5L and Barthel Index questionnaires were applied, health care utilisations and patients’ informal carers were surveyed. Results - One symptomatic female carer, 50 children (boys 94%) and six adult patients (five males) participated in the study, the latter two subgroups were included in the analysis. The average age was 9.7 (SD=4.6) and 24.3 (SD=9.8) years, respectively. Median age at time of diagnosis was three years. The average EQ-5D score among children and adults was 0.198 (SD=0.417) and 0.244 (SD=0.322), respectively, the Barthel Index was 57.6 (SD=29.9) and 53.0 (SD=36.5). Score of satisfaction with healthcare (10-point Likert-scale) was mean 5.3 (SD=2.1) and 5.3 (SD=2.9). 15 children were hospitalised in the past 12 months for mean 12.9 (SD=24.5) days. Two patients received help from professional carer. 25 children (mean age 11.1, SD=4.4 years) were helped/supervisied by principal informal carer (parent) for mean 90.1 (SD=44.4) hours/week and further family members helped in 21 cases. Correlation between EQ-5D and Barthel Index was strong and significant (0.731; p<0.01) as well as with informal care time (-0.770; p<0.01), but correlation with satisfaction with health care was not significant (EQ-5D: 0.241; Barthel Index: 0.219; informal care: -0.142). Conclusion - Duchenne muscular dystrophy leads to a significant deterioration in the quality of life of patients. Parents play outstanding role in the care of affected children. This study is the first in the Central and Eastern European region that provides quality of life data in this rare disease for further health economic studies.]

Clinical Neuroscience

[Sleep habits among preschool- and schoolchildren]

FUSZ Katalin, RITECZ Bernadett, BALOGH Brigitta, TAKÁCS Krisztina, SOMLAI Eszter, RAPOSA L. Bence, OLÁH András

[Objective - Our aim is to evaluate sleep habits, sleep quality and influencing factors among preschool- and schoolchildren. Method - Two questionnaires were recorded. Questionnaire 1 dealt with sleeping habits, breastfeeding and health behavior of preschool children and infant, and it contained the abbreviated version of the Children’s Sleep Habits Questionnaire. Questionnaire 2 dealt with health behavior and the application of sleep hygiene rules, as well as it contained the Athens Insomnia Scale. Subjects - We assessed a total of 1063 questionnaires: 516 kindergarten children participated in our online survey across the country; 547 primary and secondary school students participated in the 2nd questionnaire survey in Szolnok. Results - Parents’ observation shows that the average nighttime sleeping time of kindergarten children is 10 hours 20 minutes on weekdays and 10 hours 36 minutes on weekends. The most popular sleeping habits in kindergarten age: teal reading (65.1%) and co-sleeping (42.8%). Parents of infants used breastfeeding (50.4%) and rocking (43.2%) most frequently before sleep. Co-sleeping has a positive influence on the length of lactation. Among the preschool sleeping habits we have proved a number of positive effects of teal reading, while watching television have negative effects. The sleep quality of school-age children according to the Athens Insomnia Scale is 6.11 points (SD: 4.11), 19% of the children are insomniac. Their sleep time is 7 hours 31 minutes on weekdays and 9 hours 30 minutes on weekends. The usage of good health behavior and sleep hygiene rules positively influence sleep quality and sleep duration. Conclusions - With our results, we would like to draw the attention of children and parents to the importance of sleeping and using sleep hygiene rules.]