Clinical Neuroscience

[Angioneuritic edema in ischaemic stroke patients treated with rt-PA]


JULY 30, 2016

Clinical Neuroscience - 2016;69(07-08)


[Data of our 254 patients who were treated with rt-PA between 1st of Jan, 2011 and 31st of Dec, 2014 were processed. We focused on angioneurotic oedema as allergic complication of thrombolysis which caused life threatening respiratory obstruction in two cases. We describe these two patients’ history. Out of 254 patients six (2.3%) suffered angioneurotic edema caused respiratory obstruction in two (0.90%) cases. This occurrence is approximately 1.3-5.1% in literature. Five, out of six patients who suffered from angioneurotic oedema, had been treated with ACE inhibitors or ARB before. The role of ACE inhibitors is known in metabolism of bradykinin cascade. Plasmin which present during thrombolysis, precipitates biochemical mechanisms of this potential life threatening complication. Therefore rt-PA alone can be the cause of angioedema, but it can be more frequent together with ACE inhibitors therapy.]



Further articles in this publication

Clinical Neuroscience

Association of anterior thoracic meningocele and azygos lobe of the lung

DENIZ Ersay Fatih, SENAYLI Atilla, BICAKCI Ünal

Here we report an anterior thoracic meningocele case. Twoyears- old female patient was presented with kyphosis. Azygos lobe of the lung was also demonstrated during radiological studies. Posterolateral thoracotomy incision and extralpeural approach was performed for excision of the anterior meningocele to untether the cord. Although both anomalies are related to faulty embryogenesis and it is well known that faulty embryogenesis may also reveal coexisting abnormalities, we could not speculate a common mechanism for anterior thoracic meningocele and azygos lobe of the lung association.

Clinical Neuroscience

[Meningioma and pregnancy]


[Meningiomas are one of the most frequent primary intracranial tumours, representing one-third of all intracranialneoplasms. The vast majority of meningiomas are histologically benign, but recurrence and progression is quite frequent. They occur usually between the 6th and 7th decade, the female/male ratio is 3:2. Although rare in pregnancy, when occurring, they can cause serious, life-threatening complications due to rapid growth and unfavourable localisation. There are two dominant hypothesis explaining rapid growth in pregnancy: the role of hormonal effects and hemodynamic changes. Several studies tested these theories but none provided unequivocal answer probably because the pathomechanism is complex and multifactorial. We provide an overview of the pathomechanism of meningiomas in pregnancy with emphasis on data obtained by advanced neuropathological, molecular biological, bioinformatic, imaging and epidemiological methods. A better understanding of the processes leading to meningioma development and growth in pregnancy will help us to design personalized therapy and reduce morbidity and mortality.]

Clinical Neuroscience

Syndrome of trephined-underestimated and poorly understood complication after decompressive craniectomy


Decompressive craniectomy (DC) is still a matter of debate, with a numerous complications as expansion of haemorrhagic contusions, external cerebral herniation, subdural hygromas, post-traumatic hydrocephalus (HC). The often overlooked “syndrome of the trephined” (ST) as a delayed complication of DC also known as sinking skin flap sy initially described in 1939.ST is characterised by the neurological changes associated with alteration of the pressure/volume relationship between intracranial pressure (ICP), volume of cerebrospinal fluid (CSF), blood, and brain tissue in patients with large bone defects. This review aims at elucidating the mechanisms responsible for the development of ST, and providing useful tips and red-flag signs for healthcare professionals involved with care of post DC patients. Symptoms identified on time could help to develop appropriate treatment strategies for this suddenly deteriorating, but possible reversible condition. Although the treatment strategy is straightforward, calling for a prompt cranioplasty, the correction of HC through CSF diversion devices might require a lengthy optimisation period. Continuous changes in the setting of the shunting systems or spinal tap might lead to dangerous swinging of the midline structures causing further neurological deterioration. Thus, finding the right balance in terms of clinical management often represents a significant challenge.

Clinical Neuroscience

[The role of zonisamide in the management of pediatric partial epilepsy]

ROSDY Beáta, KOLLÁR Katalin, MÓSER Judit, MELLÁR Mónika

[In our review we discuss the group of approved antiepileptic drugs for children in Hungary. We cite the results of the review conducted by the International League Against Epilepsy on antiepileptic drug efficacy and effectiveness as initial monotherapy for newly diagnosed epileptic seizures and syndromes in pediatric age group. 25% of pediatric epilepsy is therapy resistant, so we further need new drugs, which must be investigated according to the rules of the European Medicine Agency. The ethical dilemmas of childhood drug studies lead to the situation that the new antiepileptic drugs, approved as monotherapy in adult epilepsies, are in the majority just in add-on regimen tested in pediatric patients. As clinicians we appreciate open label extension safety studies. An old-new antiepileptic drug in Europe is zonisamide. Though it was approved for first line monotherapy in pediatric and adult patients with partial and generalised epilepsy in 1989 in Japan, the European Medicine Agency licensed its use as adjunctive therapy in children aged 6 years or older with partial seizures (with or without secondary generalisation) just in 2013. The results of the openlabel extension study appeared in 2014. The mean dose received was 7.5 mg/kg/day. During the open label phase 11% of the patients achieved seizure freedom and it was maintained throughout the study. The drug was generally well tolerated. The most frequently reported treatment-related adverse events were decreased weight (6%), decreased appetite (4%), and headache (2%). No new or unexpected side effects emerged. In conclusion oral zonisamide as adjunctive therapy in pediatric patients aged 6-17 years with partial seizures demonstrated an acceptable safety and tolerability profile and efficacy over a period at least 1 year. So it is a good treatment option in this population.]

Clinical Neuroscience

[Transthyretin familial amyloid polyneuropathy - three Hungarian cases with rare mutations (His88Arg and Phe33Leu)]

CSILLIK Anita, POZSONYI Zoltán, SOÓS Krisztina, BALOGH István, BODÓ Imre, ARÁNYI Zsuzsanna

[Introduction - Transthyretin familial amyloid polyneuropathy is a rare autosomal dominant progressive systemic disesase of adults caused by endoneural amyloid deposition due to point mutations of the transthyretin gene. It is the most severe form among hereditary polyneuropathies, being fatal within 10 years if left untreated. The disease is underdiagnosed, the late onset forms (above the age of 50) being probably more widespread than previously thought. Early diagnosis is essential as the early introduction of causal therapy (tafamidis) slows progression and prolongs survival. Patients - We report here three non-related Hungarian cases of transthyretin familial amyloid polyneuropathy with non- Val30Met mutations (His88Arg in two cases, Phe33Leu in one case). They were all characterized by late-onset, progressive, length-dependent, axonal, sensorimotor polyneuropathy and the simultaneous presentation of severe restrictive cardiomyopathy. In all three cases, clinical and electrophysiological signs of myopathy were also present, suggesting the involvement of skeletal muscles as well. In two cases, high resolution ultrasound of the peripheral nerves was also performed, which showed segmental structural alterations (change or loss of fascicular structure) and some increase of echogenicity of the interfascicular epineurium, without substantial enlargement of the nerves. Conclusion - In Hungary, mainly the rare, non-Val30Met mutation forms of transthyretin familial amyloid polyneuropathy are encountered, as in our cases. As opposed to the Val30Met forms, these mutations are characterized by late onset and simultaneous presentation of severe cardiomyopathy. Our report highlights the importance of considering transthyretin familial amyloid polyneuropathy in the differential diagnosis of late-onset, progressive, axonal polyneuropathies of unknown etiology, particularly if associated with cardiac disease.]

All articles in the issue

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Lege Artis Medicinae

[Notes on the management of hypertension in chronic kidney disease ]


[The prevalence of hypertension among pa­tients with chronic kidney disease is high, reaching more than 80%. Hypertension is both one of the main causes and also the most common consequence of chronic kidney disease. It is also a main factor responsible for the high cardiovascular morbidity and mortality in this patient population. Blood pressure control can improve patient outcomes, lower cardiovascular risk and slow down the progression of kidney dis­ease, irrespective of the underlying cause. The optimal therapy should therefore focus not only on blood pressure reduction but also on renoprotection. Basic understanding of the renal pathophysiology in hypertension and renal effects of various medications is of paramount importance. In this review, we summarized cornerstones of the antihypertensive therapy in patients with chronic kidney disease. The management of patients receiving kidney replacement therapies, such as hemodialysis, peritoneal dialysis or transplanta­tion requires special knowledge and expe­rience, therefore it is not discussed here. The aim of this review was to allow non-nephrologist physicians to take care of their kidney patients with more confidence and effectiveness.]

Clinical Neuroscience

[Clinical neurophysiological methods in diagnosis and treatment of cerebrovascular diseases]

NAGY Ildikó, FABÓ Dániel

[Neurophysiological methods are gaining ground in the diagnosis and therapy of cerebrovascular disease. While the role of the EEG (electroencephalography) in the diagnosis of post-stroke epilepsy is constant, quantitative EEG para-meters, as new indicators of early efficiency after thrombolysis or in prognosis of patient’s condition have proved their effectiveness in several clinical studies. In intensive care units, continuous EEG monitoring of critically ill patients became part of neurointenzive care protocols. SSEP (somatosesnsory evoked potencial) and EEG performed during carotid endarterectomy, are early indicative intraoperativ neuromonitoring methods of poor outcome. Neurorehabilitation is a newly discovered area of neurophysiology. Clinical studies have demonstrated the effectiveness of repetitive transcranial magnetic stimulation (rTMS) in the rehabilitation of stroke patients. Brain computer interface mark the onset of modern rehabi-litation, where the function deficit is replaced by robotic tehnology. ]

Clinical Neuroscience

[Dizziness - vertigo Warning symptoms in vertebrobasilar ischemia - Part I. ]


[Dizziness and vertigo - like headache - are the most common complaints which leads patients to visit the doctor. In spite of the headache - which may be primary (e.g. migraine) or symptomatic - dizziness and vertigo do not appear to be a separate nosologic entity but rather the symptoms of several neurological disorders. For differential diagnosis, interdisciplinary thinking and activity is needed because the vestibular, neurological and psychiatric disorders might have a common role in the development of symptoms and further overlapping can also occur. The vascular disorders of the vertebrobasilar system are discussed in detail in this review. The importance, occurrence and causes of vertigo as a warning symptom is in the focus. The author draws attention to life-threatening conditions with acute onset in cases of the posterior scale ischemia and emphasizes the importance of the correct and early diagnosis. The author tries to clear up the nihilistic aspect in treating of stroke and stresses the necessity of thrombolysis and interventional radiological procedures which may be the only chance for the recovery of the patients. The pharmacological prevention of recurrent vascular events is also important and obligatory for the clinicians.]

Hungarian Immunology

[Patomechanism of hereditary angioneurotic oedema and provoking factors of oedematous attacks]

FARKAS Henriette

[The author describes the genetic background of hereditary angioneurotic edema, an autosomal dominant disorder. The pathomechanism of edemaformation and the significance of major mediator substances are explained along with clinical manifestations and their management. A special emphasis is placed on prophylaxis, the mainstay of which is the elimination of precipitating factors. The latter include mechanical trauma, diagnostic and therapeutic interventions performed in the cephalic-cervical region, mental stress, and sex hormones. The effect of endocrine therapies, ACE inhibitors, and infections - Helicobacter pylori in particular - on the natural course of the disease is also discussed.]

Clinical Neuroscience

Is stroke indeed a “Monday morning disease”?


Introduction - The therapeutic time window of acute stroke is short. Decision on the use of intravenous thrombolysis is based on well-defined criteria. Any delay in the transport to a designated stroke centre decreases the odds of therapeutic success. In Hungary, the admission rate of stroke patients peaks on Monday, the number gradually decreasing by the end of the week. This phenomenon has long been suggested to be due to the lack of emergency care approach. According to the literature, however, returning to work following a holiday is a risk factor for acute stroke. A similar phenomenon is well-known in veterinary medicine, a condition in horses referred to as ‘Monday morning disease’. In our study, we analysed the distribution of admissions due to acute stroke by the day of the week in 4 independent data sources. Patients and methods - The number of patients admitted to the Szent János Hospital, Budapest, Hungary with stroke and that of emergency ambulance transports in the whole city of Budapest due to acute stroke were analysed in the period between January 1 and March 31, 2009. The distribution of thrombolytic interventions reflecting hospitalizations for hyperacute stroke was analysed based on data of the Szent János Hospital in 2009-2012, and on national data from 2006-2012. Descriptive statistics was used to present the data. The variation between daily admission was compared by chi-square test. Results - The proportion of daily admission of stroke patients admitted to the Szent János Hospital was the highest at the beginning of the week (18% on Monday, and 21% on Tuesday) and the lowest on the weekend (9% and 9% on Saturday and Sunday, respectively). The distribution of ambulance transports in Budapest due to acute stroke tended to be similar (15% and 15% on Monday and Tuesday, whereas 13% and 12% on Saturday and Sunday, respectively) on different days of the week. No such Monday peak could be observed in a single centre regarding thrombolytic interventions: 18% and 19% of the total of 80 thrombolytic interventions in the Szent János Hospital were performed on Monday and Sunday, respectively. At the national level the higher Monday rate is obvious: during a 7-year period 16.0%, 12.7%, and 13.5% of all thrombolytic interventions in Hungary were performed on Monday, Saturday and Sunday, respectively. Conclusion - Monday preference of stroke is not exclusively caused by the lack of emergency care approach, and the phenomenon is not consistent at the individual hospital level in cases undergoing thrombolysis.