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Neurologia Croatica 2018, 67 (1-4) ORIGINAL SCIENTIFIC PAPER CASE REPORT 15 Neurologia Croatica 2017, 66 (1-4) ORIGINAL SCIENTIFIC PAPER CASE REPORT BOOK REVIEW 17 NEWS 19 21 Neurologia Croatica 2016, 65 (1-4) ORIGINAL SCIENTIFIC PAPER CASE REPORT 25 Neurologia Croatica 2015, 64 (3-4) ORIGINAL SCIENTIFIC PAPER CASE REPORTS IMAGES IN NEUROLOGY 61 63 Neurologia Croatica 2015, 64 (1-2) CLINICAL REVIEWS 5 13 CASE REPORTS 33 Neurologia Croatica 2014, 63 (3-4) ORIGINAL SCIENTIFIC PAPER CASE REPORTS CASE RECORDS OF THE ZAGREB UNIVERSITY HOSPITAL CENTER IMAGES IN NEUROLOGY 97 99 101 103 107 109
Neurologia Croatica 2014, 63 (1-2) ORIGINAL SCIENTIFIC PAPER CLINICAL REVIEWS 19 CASE REPORTS 41 CASE RECORDS OF THE ZAGREB UNIVERSITY HOSPITAL CENTER IMAGES IN NEUROLOGY 63
Neurologia Croatica 2013, 62 (3-4) ORIGINAL SCIENTIFIC PAPER 41 CLINICAL REVIEW 49 CASE REPORTS 63 CASE RECORDS OF THE ZAGREB UNIVERSITY HOSPITAL CENTER IMAGES IN NEUROLOGY 77 78
Neurologia Croatica 2013, 62 (1-2) CLINICAL REVIEWS 3 CASE REPORT CASE RECORDS OF THE ZAGREB UNIVERSITY HOSPITAL CENTER IMAGES IN NEUROLOGY 31
Neurologia Croatica 2012, 61 (3-4) CLINICAL REPORTS 53 CASE REPORTS Book review 73 75 85 89
Neurologia Croatica 2012, 61 (1-2) PRELIMINARY SCIENTIFIC COMMUNICATION 3
ABSTRACT - Survival rate after operative treatment of malignant glioma combined with chemotherapy and
radiotherapy significantly depends on the extensiveness of tumor resection. Therefore, any procedure that
can assure better intraoperative tumor identification is useful. We present our experience with intraoperative
usage of 5-aminolevulinic acid (5-ALA), a fluorescent porphyric substance that accumulates in tumor tissue
and is visible under blue light incorporated in the operative microscope. Three hours after anesthesiology
induction, the patient orally received a solution of 20 mg/kg 5-ALA in 50 mL of water. Intraoperatively, a red
fluorescent porphyrin excitation was visible between tumor margins under violet-blue light. The fluorescent
central part and also the marginal part of the tumor were histopathologically examined. Resection result was
verified with early post enhancement computed tomography and magnetic resonance imaging. Porphyric
fluorescence does not appear in normal brain tissue, whereas in malignant gliomas it stands out extremely
red. Less intensive red color is seen at tumor margins. This was confirmed by biopsies obtained in many different
parts of the operation field, depending on fluorescence intensity. There was no fluorescence bleaching
during the surgery. In conclusion, the usage of 5-ALA during operative treatment of malignant gliomas leads
to safer and more thorough tumor resection. Further improvement of contrast usage should enable lower
stage glioma identification, which would certainly contribute to more successful treatment of this type of
tumors.
CLINICAL REVIEWS 11
ABSTRACT - Parkinson’s disease (PD) is a chronic neurodegenerative disorder which affects 1% of the
population aged 60 and over. The etiology of PD has not yet been fully clarified, although it is known for
years that the patients suffer from degeneration of dopaminergic, primarily nigrostriatal neurons. It is assumed
that the disease results from interaction of genetic and environmental factors. An important role is
attributed to the action of free radicals, mitochondrial dysfunction and oxidative stress mechanism. Therefore,
previous research on the connection between the influence of different environmental factors and the
development of PD is reviewed, with emphasis on the evaluation of current views and comparison of different
results. While clear links to rural living, farming, well water consumption, metals, solvents, coffee and tea
drinking, weight, physical activity, use of nonsteroidal anti-inflammatory drugs and narcotics have not been
established, there is general agreement that exposure to pesticides and smoking increase the probability of
developing PD. Insufficiency of accurate and relevant opinions on this problem indicates the need of further
research with clearly defined criteria in terms of ‘evidence-based medicine’. Reliable data are essential in order
to harmonize opinions, make major contribution and serve as a starting point to explore the possible
neuroprotective mechanisms and more effective treatments of PD.
23
ABSTRACT - Various studies have attempted to determine the incidence and prevalence of seizures in multiple
sclerosis (MS), although the exact prevalence of epileptic seizures is still controversial. The aim of this
study was to assess the association between MS and epilepsy, to analyze clinical findings of epilepsy in MS
patients, to estimate electroencephalography (EEG) and magnetic resonance imaging findings of the central
nervous system (CNS), response to antiepileptic drug treatment and possible association of the prevalence of
epileptic seizures with MS severity and duration. Medical records of 224 MS patients diagnosed at Pula General
Hospital during a 25-year period (1985-2010) were reviewed. Seizures apparently occurred more commonly
in MS patients than in the general population. Partial seizures with or without secondary generalization
appeared to be more common. Primary generalized epilepsy has also been reported, although less frequently.
The onset of epileptic seizures preceded the diagnosis of MS, occurred in parallel or within five years
of MS onset. The occurrence of epileptiform EEG abnormalities in MS patients was rare. The lesions involved
in the pathogenesis of epilepsy were plaques or demyelination affecting cortical or subcortical areas (predominantly
frontal and temporal). Generally, seizure control with standard antiepileptic drugs was good.
CASE REPORT 33 35
Neurologia Croatica 2011, 60 (3-4) CLINICAL REVIEWS 101
ABSTRACT – Although palliative medicine has been advanced rapidly during the last few decades as an
institutionalized response to the emergence of oncologic and other chronic diseases carrying the huge health
burden, the Croatian medical system finds itself at the beginning after years of neglecting the palliative
medicine problems. One of the goals of this short overview is to describe the basic principles of palliative
medicine and the area of its practice. While assessing the various paradigms of palliative medicine, varying
from hospice movement to palliative medicine integrated into the three levels of health care, the authors will
try to clarify conceptual dilemmas in the area. Second goal arises in presenting the palliative care needs in
the non-oncologic patients suffering from various chronic diseases and assessing the possible distinctions in
these populations. Third goal is to bring an overview of palliative care in Croatia with the possible practical
solutions for the implementation of palliative medicine in daily practice. From these particular goals, the
ultimate goal of this overview arises, i.e. making the Croatian medical society familiar with the basic palliative
medicine principles in order to additionally sensitize it for the overwhelming needs of palliative patients.
107
ABSTRACT – The main aim of this review is to give an overview of up-to-date tendencies in communication
skills and theory with neurological patients. The authors characterized neurological disorders as the major
public health problem and, therefore, present the results of various studies indicating insufficient communication
skills with this group of patients. Also, we identified another vulnerable group, the caregivers of neurological
patients suffering from various depressive and anxiety disorders associated with the burden of
daily emotional and physical management of their significant others. Various factors possibly related to the
inappropriate communication are stressed out from the physicians’ and patients’ point of view. In response
to these needs, the authors present modern patient-oriented communication paradigms based on specific
goals and various outcomes, applying the conceptual framework to Alzheimer disorder, focusing on the
various dilemmas arising from the diagnosis disclosure to the disease progression. The need for personalized
psychosocial intervention is stressed out in order to improve the quality of life of patients and their significant others.
113
ABSTRACT – Palliative care team is a multidisciplinary team that usually includes physician, clinical psychologist,
nurses, social worker, occupational therapist, and number of other experts, offering support, help,
counseling and advice designed to meet the individual needs of each patient and his family referred to the
service. Clinical psychologist is an expert who is trained and professionally qualified to provide help to palliative
patients with emotional and behavioral difficulties, as well as to members of palliative care team (to
prevent burn out, to help them cope appropriately with daily stressful situations, etc.). Clinical psychologist
working with palliative patients and with members of his family provides psychological assessment, counseling,
psychotherapy, motivational interviewing to encourage intrinsic motivation to reach behavioral
changes and better adaptation to the disease.
119
ABSTRACT – Today when efforts are made to diagnose dementia in the early course of disease, it is highly
desirable to offer a high quality and continuous program of treatment and care to persons with dementia and
their families. Although there is no cure for Alzheimer’s disease and dementia, the natural course of the disease
can be slowed down and better quality of life offered to persons with dementia and their caregivers using
standard (symptomatic) pharmacological treatment with anti-dementia drugs and other psychopharmaceuticals.
In the management of dementia, non-pharmacological interventions should also be used because they
may prove beneficial. Croatia as a country with one of the oldest population in Europe should develop an
action plan to fight Alzheimer’s disease; in other words, we should be aware of the upcoming Alzheimer’s
disease epidemic in the near future. Good quality palliative care is of great value in Alzheimer’s disease management,
so all programs for palliative medicine in Croatia, such as those launched at the University but also
in the field should be encouraged.
125
ABSTRACT – The methods of palliative care have long been defined exclusively in the frame of care for patients
with malignant diseases. Recently, however, it has been realized that the group of patients requiring
these methods of medical treatment is by far greater. In neurologic casuistics, the percentage of incurable
diseases is very high. Curiously enough, palliative care has come into the focus of interest of this medical
profession pretty late. Among neurologic diseases, cerebrovascular diseases definitely are of high medical
and socioeconomic importance, as they still are the leading cause of disability and the third most common
cause of mortality in the world, with palliative care being frequently necessary in their terminal stage. Statistical
analysis of patients in the chronic stage of cerebrovascular diseases (post-stroke state with the grade of
disability preventing independent mobility) revealed the patients and/or their families to complain mostly of
the following: swallowing difficulties (dysphagia), speech disturbances (dysphasia/dysarthria), constipation,
confusion, psychomotor agitation, incontinence and pain. One of four stroke patients is concerned for fear
from pending death. A high proportion of patients with cerebrovascular diseases are also concerned about
their disease impact on their close environment, while developing fear from disability, solitude and dependency.
When talking about palliative care, special attention should be paid to patients in permanent vegetative
state, which mostly develops following traumatic brain injury but may also be due to particular cerebrovascular
diseases (e.g., subarachnoid hemorrhage). The management and palliative care for these patients should
include therapy for pain, spasticity, muscle contractures, pathologic fractures, decubitus ulcers, or neuropathic
pain.
131
AABSTRACT – Many neurologic diseases are progressive and incurable. The optimal care of such patients
requires that neurologists understand and apply the principles of palliative medicine. Indeed, the principles
of palliative care are relevant to the management of many patients because minimizing distress and controlling
pain and other symptoms are important even for patients with curable disease. Palliative care is an approach
that improves the quality of life of patients and their families facing the problems associated with a
life-threatening disease. The aim of palliative care is prevention and relief of suffering by means of early identification, impeccable assessment and treatment of distressing symptoms and other problems, physical, psychosocial
and spiritual. Although the term palliative care is often used to mean ‘end of life care’, palliative care
is often very appropriate and applicable earlier in the course of disease, in conjunction with other therapies
that are intended to prolong life. In this context, palliative care will enhance the patient’s quality of life and
offer a support system that helps the patient live as actively as possible until death, thus affirming life while
regarding dying as a normal process. Palliative care is a generic term which, in describing an approach to
care, highlights the responsibility carried by all health care professionals to provide holistic care for their
patients with progressive neurologic disease.
137
ABSTRACT – Palliative care is defined as an approach aimed at upgrading the quality of life of patients facing
a lethal disease and of their families. The main principle and purpose of palliative care in a health care
institution is providing due care for such patients through offering high-quality services on special wards or
ward units. These services include prevention and alleviation of symptoms by early detection, assessment
and management of pain, while alleviating other mental and psychosocial problems. It implies use of appropriate
palliative interventions, which may include disease-modifying treatments, such as surgery, radiotherapy,
chemotherapy, hormonal procedures, etc. Rehabilitation has been generally accepted as a major form of
active palliative care. At this stage, the patient and his family are faced with numerous and complex issues
that go beyond professional abilities of any individual profession; therefore, modern palliative care is inconceivable
without the interdisciplinary team engagement, its members having the goal in common, i.e. to reduce
suffering and to upgrade the quality of life of the patient and his family. The composition of the interdisciplinary
team depends on the level of program development, program goals, and individual patient
needs; however, the majority of programs include a physician, nurse, social worker, clerical person and volunteers.
The patient and his family also are members of the team; it is of utmost importance that they feel
acceptance and empathy from other team members, and to be free to speak out their feelings, with due respect
for their informed consent. Currently, owing to the great advances in diagnostic and therapeutic options
for patients with epilepsy, the role of palliative care is declining in this patient population. However,
progressive myoclonus epilepsies make a special clinical entity in epileptology, which requires palliative care.
The most important clinical entities of progressive myoclonus epilepsy for which palliative treatment is one
of the major approaches included in therapeutic algorithm are presented in this report.
151
ABSTRACT – Palliative medicine is comprehensive care for patients and their family members, with the aim
of improving their quality of life, according to the patients’ needs and desires. Although palliative medicine
is traditionally associated with oncologic diseases, it is no less important in the care of patients suffering from
chronic neurologic diseases such as multiple sclerosis. As there is no curative therapy for secondary progressive
and primary progressive forms of multiple sclerosis, palliative medicine is crucial in the treatment of
these forms of the disease and for relief of symptoms such as pain, spasticity, tremor, ataxia, sphincter and
bowel disturbances, and mental disorders. Unfortunately, for the majority of symptom relieving drugs in
multiple sclerosis, there is a lack of randomized studies to confirm their efficacy, thus their effectiveness has
to be judged from the experiences of other medical branches. Good palliative care improves the quality of life
of both the patients and their families.
155
ABSTRACT – Although new treatments for neuromuscular diseases are being developed, the palliative care
approach to the care of neuromuscular patient is outlined and the role of the multidisciplinary team emphasized.
Therefore, the palliative care approach to the care of neuromuscular patients and their families should
be in the center of good clinical practice. Neuromuscular disease with the most demanding and challenging
palliative care is probably a motor neuron disease. The term motor neuron disease covers a range of conditions
in which the motor neuron cell bears the brunt of the disease process. The most severe complications
of neuromuscular diseases are progressive muscle weakness, respiratory failure and dysphagia, but palliative
care should be focused on speech and language therapy, and on emotional, spiritual and social aspects of the
disease. Such a comprehensive palliative care should be focused on patient and all caregivers.
165
ABSTRACT – Palliative medicine implies care offered to patients in the advanced, terminal stage of oncologic
and other chronic diseases. The goal of palliative care is symptom control and consequential improvement
of the patient’s quality of life. Pain is one of the most common symptoms in the advanced stage of many
chronic diseases including neurologic ones. Neuropathic pain caused by the lesion and disease of the nociceptive
system, with a prevalence of 8% in the general population of Europe, requires specific treatment
which is frequently missed or inadequately applied. Current principles in the clinical approach and therapy
for neuropathic pain in the frame of palliative care are described.
171
ABSTRACT – Palliative care integrates holistic and multidisciplinary approach to patient, implying care offered
to patient by a team of physicians, nurses, physical therapists, social workers, spiritual counsel and
volunteers. From the aspect of social work, palliative care includes activities of all those involved in the team
approach to care of patients whose disease has become unresponsive to treatment, all this in line with the
patient’s needs and desires. Improving the quality of life of these patients and their families is the main goal
of social workers’ activities.
177 181
Neurologia Croatica 2011, 60 (2) ORIGINAL SCIENTIFIC PAPER SCIENTIFIC COMMUNICATION CLINICAL REVIEW
Neurologia Croatica 2011, 60 (1) ORIGINAL SCIENTIFIC PAPER 3
ABSTRACT - Iron deficiency anemia (IDA) is the most common nutritional problem worldwide. Owing to
the role of iron in brain energy metabolism, neurotransmitter function and myelin formation, IDA may lead
to behavioral, developmental and cognitive dysfunctions. Iron is an essential element and has an important
role in several metabolic and enzymatic processes including NADPH reductase activity and cytochrome
oxidase system. Our aim was to investigate the electrophysiological effects of IDA on the peripheral nervous
system, and to evaluate whether or not the possible electrophysiological abnormalities are reversible with
appropriate doses of iron therapy. Electrophysiological evaluations were performed in 52 patients with newly
diagnosed IDA and 30 age-matched healthy controls. Electrophysiological evaluations were repeated after
3 months of oral iron therapy. Normal electrophysiological findings were recorded in 38 (73.07%) patients,
while 4 (7.69%) patients had polyneuropathy (PNP) and 10 (19.24%) had carpal tunnel syndrome (CTS)
findings. Except for one patient with PNP and CTS findings each, the electrophysiological findings of all
patients were found to have returned to normal ranges after 3 months of oral iron therapy. Detection of iron
responsive neuropathic processes (PNP and CTS) in IDA patients suggested that IDA may cause peripheral
nervous system involvement. It is important to emphasize the examinations for IDA as an etiologic factor on
planning treatment for neuropathy patients. In cases where IDA is present, it would be beneficial to treat IDA
with iron before applying other treatment options for neuropathy.
CLINICAL REVIEWS 13
ABSTRACT – The aim was to determine prospectively the frequency and clinical characteristics of vertiginous
syndromes in a neurology outpatient clinic of a community hospital during one-year period with 6-
month follow up. The study was carried out at Department of Neurology, Varaždin General Hospital, in
Varaždin County, Croatia, with a catchment population of 180,000. Data were prospectively collected from
one neurologist’s practice during 12-month period with 6-month follow up (May 2007 – November 2008).
During the study period, 1670 patients were examined and 173 (10.3%) of them complained of vertiginous
symptoms (mean age 56, range 16-89 years; 113 (65.3%) females). Benign paroxysmal positional vertigo was
most common, recorded in 52 (30.1%) cases, followed by vestibular neuritis in 29 (16.8%) and other etiology
in 28 (16.2%) cases. Central forms of vertigo were present in 17 (9.8%) patients. The authors conclude that
vertigo of peripheral origin, i.e. benign paroxysmal positional vertigo, is the most common type of vertiginous
syndromes encountered in this secondary neurological practice. Since this diagnosis allows for rapid
and effective relief of patient symptoms, it is crucial that the neurologist is trained in recognizing and treating
this neuro-otologic entity.
21
ABSTRACT – Neurovascular diseases and stroke as their terminal stage currently are the third cause of mortality
and morbidity in the population and the leading cause of disability and hospitalization. That is the
reason for the great interest of thousands of experts in the research in all related fields, from medical and
social to economic aspects of stroke. Recent studies show that besides common stroke risk factors there is
strong association between stress, psychological stress in particular, and an increased stroke risk in terms of
maladaptation to stressful situations. Exposure to permanent, chronic stressors and suspended or delayed
physical reactions may break homeostatic mechanisms and power of adaptation and stress managing. After
physiological, psychological and behavioral response of the body to prolonged and chronic stress, destructive
power of stress reactions translates to the somatic level by changing the neurotransmitter, hormonal and
immune responses, modifying and impairing organ functions. Eventually, this leads to a “degenerative cascade”
and morphological changes, thus additionally elevating the risk of stroke.
CASE REPORTS 29
ABSTRACT – Familial idiopathic basal ganglia calcification is a neurodegenerative autosomal dominant
disorder of yet unknown gene locus. First manifestations of the disease start in the third to fifth decade. After
good health during childhood and young adulthood, the disease occurs with progressive movement disorders,
cognitive or behavior impairment and pyramidal failure. Neuroradiological visualization shows bilateral
calcifications of the basal ganglia and subcortical calcifications of the brain and cerebellum. Metabolic
course like hypo- and pseudohypoparathyroidism as well as neuroferritinosis was excluded. The disease is
progressive and does not respond to usual therapy for extrapyramidal disorders.
35
ABSTRACT – Restless legs syndrome, primary or secondary, is one of the most common movement disorders.
The most frequent causes of secondary restless legs syndrome are uremia, anemia, pregnancy, etc. A
case is presented of a 77-year-old male with very pronounced restlessness in the legs at night and positive
heredity for restless legs syndrome, resulting in continuous insomnia, poor quality of life and depression
with suicidal impulses. Examinations ordered by a neurologist verified distinct sideropenic anemia. Upon
consultation with a hematologist, substitution therapy with intravenous iron preparation was introduced
along with clonazepam, which resulted in complete regression of restless legs syndrome, with significant
improvement in sleep quality and thus in the patient’s quality of life. Although occurring at a high incidence,
this syndrome often remains unrecognized and untreated. Continuous education about the features of this
syndrome, its timely recognition, appropriate work-up algorithm and therapeutic guidelines are extremely
important to improve the quality of life of patients with restless legs syndrome.
ABSTRACT - Aim of the study: To present a case of successful microsurgical clipping of the aneurysm within
the fenestrated vertebrobasilar junction via far lateral transcondylar approach.
Background: Surgical treatment of low basilar aneurysm and vertebrobasilar (VB) junction remains one of the most challenging and most difficult procedures in neurosurgery. Saccular aneurysms of the VB junction are often associated with a fenestration of the basilar artery. Endovascular procedures are performed for the treatment of such aneurysms, but may be difficult in some cases. Case description: A case is presented of a 53-year-old patient with VB junction aneurysm that was microsurgically clipped successfully via far lateral transcondylar approach. The patient was discharged from the hospital fully recovered 10 days after the surgery. Conclusion: The far lateral approach adequately exposed the VB junction aneurysm for successful clipping of the aneurysm and was associated with no morbidity. It should be considered as a good alternative to endovascular treatment. In developing countries, the microsurgical procedure may be given preference because it is less expensive and does not require preoperative intra-arterial digital subtraction angiography or follow up. 53 55
Neurologia Croatica 2010, 59 (3-4) ORIGINAL SCIENTIFIC PAPER
ABSTRACT – Purpose: To assess whether retinal nerve fiber layer (RNFL) thickness value obtained with
optical coherence tomography (OCT) in ocular hypertension patients can predict glaucomatous changes in
visual field over a 2-year period.
Methods: This comparative analysis included 76 eyes: 36 ocular hypertension (OHT) and 40 healthy. The OCT parameter RNFL thickness and Octopus automated perimetry (SAP) were performed in ocular hypertension and healthy eyes at baseline and after 2 years. Results: In ocular hypertension group, 6 (16.6%) eyes developed SAP glaucomatous abnormalities in 2-year period. Comparison of RNFL thickness between OHT eyes with and without glaucomatous changes and healthy eyes showed statistically significant thinning of RNFL in the inferior quadrant at baseline in OHT eyes with SAP glaucomatous changes. Conclusion: Thinner OCT obtained inferior RNFL in OHT eyes is associated with future development of perimetric glaucoma changes. CLINICAL REVIEWS 127
ABSTRACT - Neuroprotection is a therapeutic procedure, which attempts to save brain tissue neurons from
irreversible injury. In the last two decades, many pre-clinical (experimental) studies have been done on animal
models, which have convincingly shown that different pharmacological and non-pharmacological interventions
may reduce ischemic brain injury by acting on elements of ischemic cascade or preventing reperfusion
injury. Even though we have been using the STAIR guidelines for pre-clinical and clinical testing of
drugs in neuroprotection for nearly ten years, clinical studies of neuroprotective drugs (in humans) have not
shown adequate neuroprotective effect in the treatment of acute ischemic stroke. Despite previous failures,
clinical studies in the field of neuroprotection are continuing. At this moment, we are waiting for the results
of ongoing studies. The most promising interventional or pharmacological agents are hypothermia, albumins,
lovastatin, magnesium and citicoline. It is believed that the future of neuroprotection will be based on
the usage of thrombolytic therapy along with new neuroprotective agents or procedures.
137
ABSTRACT - In September 2004, the Croatian Ministry of Health approved rt-PA for acute treatment of
ischemic stroke. For the next almost 5 years, only three centers in Croatia started this mode of treatment.
Since June 2008 started routine use of rt-PA in other neurological departments around Croatia. In our Neurological
Intensive Care Unit since May 2006 till the end of May 2008, 66 patients have been thrombolysed.
This kind of treatment is still applied to a relatively small group of patients due to at least: a quite narrow time
window for treatment, strictly inclusion/exclusion criteria, low information level of general practitioner and
emergency staff and fear od adverse effect. More frequent use of rt-PA begin since June 2008. thanks to continous
informing on general and medical population, training of neurologists from hospitals around Croatia
and finaly by expansion of the time window for tretament.
145
ABSTRACT – Wilson’s disease is a rare autosomal recessive disorder of copper transport resulting in copper
accumulation in the liver, brain and cornea. The incidence is estimated to be 1:30000-80000 in most populations.
The Wilson disease gene, now designated ATP7B, situated on 13th chromosome (13q14.3), was cloned
in 1993. The clinical phenotypes include hepatic, neurologic and psychiatric diseases, or a combination of
these. A combination of clinical features, various laboratory parameters and Kayser-Fleischer rings is necessary
for making the diagnosis. Mutation analysis may provide definitive diagnosis and is becoming part of
diagnostic algorithm, especially in patients with indeterminate clinical and biochemical features. Family
screening of first-degree relatives should be undertaken. Recognition of the disease at an early stage is crucial
because treatment prevents severe liver damage and/or lifelong neurologic disabilities. The aim of treatment
is reduction of tissue copper concentration either by enhancing its urinary excretion or by decreasing its
intestinal absorption. Treatment includes copper-chelators, such as penicillamine and trientine as well as
zinc salts. Liver transplantation is treatment option in cases of acute liver failure or treatment-resistant and
severe advanced liver disease. It is not indicated in patients with mainly neuropsychiatric presentation.
CASE REPORTS 155
ABSTRACT – Vertebral artery dissection (VAD) has only recently been recognized as a rare cause of transient
ischemic attack (TIA) or infarction in vertebrobasilar basin. Development of new diagnostic techniques
associated with lower risk for the patient has resulted in a higher rate of this diagnosis. Early diagnosis is even
more important knowing that cerebral infarction may ensue weeks after dissection. The symptoms of cerebellar
or brain stem infarction can be prevented or alleviated by early diagnosis and timely treatment. Development
of thrombi and potential thromboembolic events in vertebrobasilar basin can usually be prevented
by timely anticoagulant therapy. Experiences acquired to date suggest that cerebral infarction or TIA due to
VAD is generally found in younger persons, however, it does not mean that VAD cannot occur in individuals
older than 50. Presentation is made of a female patient with VAD and recurrent TIAs in vertebrobasilar circulation.
The aim is to remind the readers that VAD is by no means so very rare or harmless cause of TIA,
and to present appropriate diagnostic work-up and treatment in case of suspect VAD.
161
ABSTRACT – Posttraumatic ischemic stroke after minor head injury is a rare and unexpected event. There
are 65 cases of middle cerebral artery (MCA) occlusion described in the literature. Arterial dissection, cerebral
vasospasm and thrombosis have been postulated as the possible pathogenetic mechanism. A case is
presented of a 40-year-old man having sustained a minor head injury as a driver in a car accident. Twentyfour
hours after the accident, he became soporous and was admitted to intensive care unit, where magnetic
resonance imaging revealed mild brain edema on the left side with slight shift on the opposite side. He was
transferred to our institution. Control computed tomography (CT) scan revealed edema and shift progression.
Two days later, repeat CT scan showed further progression and the patient underwent decompression
craniotomy; the patient died in two days. This case report points to the need of due attention be paid
in any case of minor head injury where complications develop within 24 hours because a relatively mild head
injury may be a cause and co-factor in the etiology of fatal stroke.
ABSTRACT – Neurinoma is a benign tumor originating from Schwann cells of the myelin sheath of peripheral
nerves. The tumor is benign, encapsulated, not infiltrating the nerve, slowly growing, of homogeneous
structure, and symptoms produced by neurinoma may be misdiagnosed. In this report, successful operative
treatment of a large neurinoma of the superficial peroneal nerve as a very rare location in a 69-year-old female
patient is described. Operative treatment is successful if performed before the irreversible nerve lesions
have developed because the tumor itself does not infiltrate the nerve and can be extirpated in toto.
171 182 185
Neurologia Croatica 2010, 59 (1-2) CLINICAL REVIEWS 5
ABSTRACT – Due knowledge of the semiology of epileptic seizures and of the classification of the respective
type of epilepsy or epileptic syndrome is necessary to reach an accurate diagnosis and to choose optimal
antiepileptic drug. Following diagnostic guidelines, history, heterohistory and videopolygraphic data may
occasionally suffice to delineate the clinical picture, which will then serve to define quite precisely the
anatomic origin of epileptogenesis and localize the epileptic focus. A correctly made initial diagnosis will
reduce the time needed for patient work-up and enable faster choice and introduction of the most potent
antiepileptic or decision on a more radical mode of treatment, thus substantially decreasing the risk of developing
a pharmacoresistant form of disease. At the same time, identification of the clinical phenotype and its
anatomic correlate reduces the possibility of diagnostic error, which may result in extensive and unnecessary
diagnostic procedures and specialist examinations.
23
ABSTRACT – Surgical techniques of treating patients with pharmacoresistant epilepsy have changed considerably
in the last 25 years. Greatest success has been achieved in patients with magnetic resonance imaging
(MRI) positive temporal lobe epilepsy (TLE), especially in patients with hippocampal sclerosis, and more
recently in patients with extratemporal lesional epilepsy. This improvement is mostly due to large progress in
imaging technology, but also in neurophysiological methods and microsurgical techniques. Very important
is careful patient selection and preoperative evaluation via identification of the semiology of seizures, localization
of epileptogenic lesion, and ictal onset zone. In preoperative evaluation of patients with pharmacoresistant
epilepsy, modern diagnostic techniques can be divided into noninvasive and invasive. Noninvasive
methods include: (a) video-electroencephalography (EEG) recording (interictal and ictal); (b) preoperative
neuropsychological testing; (c) dedicated neuroradiological examination, i.e. 3T brain magnetic resonance
imaging (MRI), with specialized MRI techniques including morphometric MR analysis, MR spectroscopy,
functional MRI with MR tractography, and MR volumetry; (d) nuclear-medicine methods, i.e. single photon
emission computed tomography (SPECT) and positron emission tomography (PET); and (e) magnetoencephalography
(MEG). Invasive methods include: (a) Wada test (intracarotid amobarbital or etomidate testing);
(b) semi-invasive video-EEG monitoring, using sphenoidal or foramen ovale electrodes; and (c) invasive
video-EEG monitoring, using subdural strip and grid as well as depth electrodes. Invasive EEG monitoring,
where selection of the type of intracranial electrodes and their placement depends on the localization of
epileptogenic region, is used if MRI is nonlesional, if there is discrepancy between ictal or interictal EEG
recordings and brain MRI, between the seizure semiology and the imaging findings, and between the seizure
semiology and EEG recording. It is also used in case of dual pathology and polytope changes, when it is not
known which pathomorphological lesion is epileptogenic, and in case of positive brain MRI, when pathomorphological
lesion is localized near motor and eloquent cortex. The possible complications include intracranial
hemorrhage, cortical lesions and infection.
35
ABSTRACT – Epilepsy is the most common neurologic disorder, which can be efficiently treated by pharmacotherapy
in most patients. For full therapeutic success to achieve, the clinical phenotype or epileptic syndrome
should be strictly defined; optimal dosage of appropriate antiepileptic therapy should be introduced
as early as possible (or in the form of rational dual therapy as needed); and due care should be taken of the
specific populations such as women of reproductive age and the elderly, considering their physiologic characteristics,
comorbidities, altered mechanism of action of the antiepileptics used (pharmacokinetics and
pharmacodynamics); adjuvant therapy and possible interactions with antiepileptics. According to literature
data, there are 25%-40% of pharmacoresistant patients, in which a satisfactory control of seizures cannot be
achieved by the antiepileptic therapy available. These patients are candidates for preoperative examination
and neurosurgical treatment, i.e. classic neurosurgical resection or minimally invasive neurosurgical procedure
(implantation of vagus nerve stimulator).
CASE REPORTS
ABSTRACT – Lance-Adams syndrome, first described in 1963, is a condition characterized by development
of chronic post-hypoxic action myoclonus due to a temporary lack of or inadequate brain oxygen supply. A
coagulation disorder should be suspected when an unexplained hemorrhage occurs. Patients with Lance-
Adams syndrome show favorable response to sodium valproate and clonazepam. A case is presented of a
22-year-old man admitted to University Department of Neurology, Zagreb University Hospital Center, in
January 2008. Clinically, the patient presented with headache, nausea, vomiting, mild right hemiparesis and
truncal ataxia. Brain magnetic resonance revealed spontaneous atypical subdural hematoma in the region of
the right middle cerebellar peduncle, vermis and left cerebellar hemisphere. Laboratory findings revealed
coagulation factor XIII deficiency. Two weeks of admission, the patient developed spontaneous subdural
hematoma in the right frontoparietal region. In the meantime, clinically he also developed generalized action
myoclonus that was, according to our belief, a part of chronic post-hypoxic action myoclonus, i.e. Lance-
Adams syndrome (secondary to intracranial hemorrhage). Oral administration of sodium valproate and clonazepam, and replacement therapy for normalization of coagulation factor XIII concentration resulted in
complete regression of myoclonus.
67
ABSTRACT – Ritscher-Schinzel syndrome or Cranio-Cerebello-Cardiac (3C) syndrome is a rare autosomal
recessive disorder characterized by malformation of the posterior cranial fossa, congenital cardiac defect and
craniofacial dysmorphism. The syndrome was first described in 1987 by the example of two sisters born to
healthy parents. Criteria for the diagnosis of 3C syndrome were published in 2001 by Leonardi et al. Presentation
is made of a 37-year-old patient that was operated on for cleft palate soon after birth, then also for
ventricular septum defect. Since 1989, the patient has been treated for epilepsy with signs of psychomotor
retardation. In 2008, computed tomography revealed Dandy-Walker malformation. In March 2009, the patient
was hospitalized at University Department of Neurology, Zagreb University Hospital Center. Magnetic
resonance of the brain verified the malformation described above, with enlargement of the posterior cranial
fossa with a cystic growth and aplasia of the caudal part of the vermis, and expanded areas of gray matter
heterotopy bilaterally subependymally. Clinical findings included craniofacial dysmorphism, syndactyly of
the first and second right toes, flexion contractures of the second and third finger distal phalanges of both
hands, and thoracic segment scoliosis. Radiologic examinations showed hypoplasia of the first rib on the
right, and congenital block of the bodies and articular processes of C6-C7 vertebrae with sinistro-convex
scoliosis of the thoracic spine. Based on clinical, radiological and neuroradiological examinations, we believe
that our patient meets all the criteria for Ritscher-Schinzel syndrome. It is the first case described in Croatia
and the oldest patient reported in the literature.
73
ABSTRACT – Neurosyphilis develops in 10%-33% of untreated persons with early syphilis about 15-30 years
from the original infection with Treponema pallidum. The main signs of tertiary form of syphilis or general
paresis are dementia, myoclonic jerks, seizures, psychosis, dysarthria, action tremor and quadriparesis. We
reported on a 40-year-old patient that developed partial seizures with rare secondary generalization, while
headache and slow progressive dementia occurred several months later. Except for cognitive decline, there
were no other neurological signs of disease. Electroencephalography revealed slowing down of background
frequency and paroxysmal delta activity with element of bi- and triphasic activity typical for encephalopathies,
so extensive diagnostic evaluation was performed. Magnetic resonance imaging demonstrated cerebral
atrophy and disseminated T2 and FLAIR hyperintense lesions, especially of frontal and temporal localization.
Single photon emission computed tomography showed marked reduction of the cerebral blood flow.
Blood and cerebrospinal serologic testing confirmed the diagnosis of neurosyphilis.
83
ABSTRACT – A 35-year-old female had been medically treated since age 8 for generalized seizures, and
since age 19 for complex partial seizures, using carbamazepine (Tegretol®) ever since. After 4 years of carbamazepine
administration, she developed systemic lupus erythematosus (SLE) symptoms such as rash, collapse,
leukopenia, hypergammaglobulinemia and positive anti-nuclear factor (ANF). Clinical symptoms and
leukopenia normalized upon carbamazepine discontinuation and administration of chloroquine.
89
ABSTRACT – One percent of the general population are affected with epilepsy, while 25%-30% of epilepsy
patients are intractable to medications in spite of a recent increase in the number of new medications that are
available, and need neurosurgical operation. Vagus nerve stimulation (VNS) is a relatively new tool that has
been increasingly used in non-pharmacological management of pharmacoresistant epilepsies, reducing the
rate of epileptic seizures by 60%-70%. Very rare adverse events related to VNS included bronchoconstriction
in patients with a history of bronchial asthma associated with activation of the device. We present a patient
with bronchial asthma and pharmacoresistant epilepsy, in whom a VNS implanted at a stimulation of 0.75
mA was associated with activation of the device, which was found to temporally correlate with bronchoconstriction
as demonstrated by pulmonary function assessment.
95 97
Dana 23. travnja 2010. godine prvi puta u Hrvatskoj
i u široj regiji izvedena je u KBC-u Zagreb na
Rebru složena operacija 48-godišnje bolesnice s
teškim, farmakorezistentnim oblikom epilepsije,
koja se liječi od 7. godine života i u koje, usprkos
cijelom nizu antiepileptika, nije postignuta zadovoljavajuća
kontrola epileptičnih napadaja. Epileptični
napadaji su joj onemogućavali normalno
funkcioniranje u svim aspektima života. Bolesnica
je prosječno imala 20-tak epileptičnih napadaja na
dan, često u nizu; klinički fenotipski radilo se o
kompleksnim parcijalnim napadajima frontalnog
ishodišta (cingularna regija) obilježenim bizarnim
grimasama i verbalnim automatizmima te perseveracijama,
uz učestalu sekundarnu generalizaciju.
Standardno EEG monitoriranje skalp elektrodama
nije pokazivalo jasno područje mozga iz kojega
započinje epileptični napadaj. U planiranju i izvedbi
kirurškog zahvata, kao i u invazivnom elektroencefalografskom
monitoriranju koji je izveden
u kontinuitetu 4 dana ranije, a na inicijativu predstojnice
Klinike za neurologiju doc. dr. sc. Sanje
Hajnšek i predstojnika Klinike za neurokirurgiju
prof. dr. sc. Josipa Paladina, te uz veliku potporu
ravnatelja akademika Željka Reinera i pomoć prof.
dr. sc. Jörga Wellmera, predstojnika Centra za epilepsiju
Sveučilišne bolnice u Bochumu, sudjelovao
je tim liječnika neurologa (doc. dr. sc. Sanja
Hajnšek, doc. dr. sc. Željka Petelin, doc. dr. sc.
Zdravka Poljaković, dr. Sibila Nanković, dr. Vlatko
Šulentić), neurokirurga (prof. dr. sc. Josip Paladino,
mr. sc. dr. Goran Mrak, dr. Andrej Desnica),
biomedicinskih inženjera (doc. dr. sc. Velimir
Išgum, dipl. ing. Magdalena Krbot, dipl. ing. Ana
Branka Šefer, dipl. ing. Marija Mavar), neuroradiologa
(doc. dr. sc. Marko Radoš, dr. sc. Goran
Pavliša) te anesteziologa (mr. sc. dr. Tamara Murselović). U bolesnice je na visokorezolucijskom
MR mozga (3T) otkriven poremećaj razvoja
moždane kore, tzv. kortikalna displazija lokalizirana
u desnom frontalnom režnju. Odluka
za invazivno monitoriranje donešena je zbog planiranja
opsežnosti neurokirurške resekcije, tj. operacije
mozga. Budući da se u bolesnika u kojih se nađe promjena vidljiva na MR slikama ne može
reći koji je dio mozga odgovoran za nastanak
epileptičnog napadaja (tzv. epileptogena zona), jedini
način njenog otkrivanja i potpunog uklanjanja
je monitoriranje epileptične aktivnosti invazivnim
elektrodama (strip i grid elektrode). O opsežnosti uklanjanja čitave epileptogene zone ovisi u kojoj je
mjeri moguće smanjenje epileptičnih napadaja. U
bolesnika u kojih nije uklonjena čitava epileptogena
zona rezultati kirurškog liječenja pokazuju
značajno slabiju kontrolu epilepsije nakon operacije
nego u bolesnika u kojih je uklonjena čitava
epileptogena zona. Bolesnici su u prvom aktu implantirane
subduralne strip i grid elektrode u
područje same kortikalne displazije i oko nje,
frontalno desno. Dan nakon operacije
pristupilo se, uz pomoć kortikalnog stimulatora
Nicolet, kontinuiranom invazivnom elektroencefalografskom
monitoriranju temeljem kojeg se s
vrlo velikom preciznošću odredila zona početka
epileptičnog napadaja, te je uz pomoć posebnog
kompjutorskog programa napravljen plan
neurokirurške resekcije.
Kortikalni stimulator Nicolet sa 128-kanalnim pojačalom
je uređaj koji je zadnja inovacija u funkcionalnom
elektroencefalografskom monitoriranju.
Uz Centar za epilepsiju KBC-a Zagreb na Rebru,
uređaj takvog tipa posjeduju još samo dva
epileptološka centra u Europi (u Londonu i u Oslu).Treba napomenuti da se invazivnim monitoriranjem
može uz otkrivanje epileptogene zone
obaviti i detaljan prikaz moždane kartografi je (kortikalnih
centara za motoriku, govor, osjet). Uz
pomoć navedene metode se u bolesnika koji su
kandidati za resekciju tumora mozga ili kirurške
zahvate kod epilepsija umanjuje mogućnost oštećenja
kritičnih moždanih regija, što bi dovelo do
neprihvatljivog neurološkog deficita, a što je od
velike važnosti za poboljšanje kvalitete njihova
života.
Operacija uklanjanja patološke promjene, kortikalne
displazije te elektrodama otkrivene epileptogene
zone učinjena je nakon četiri dana intenzivnog
monitoriranja, a nakon inicijalnog postavljanja
subduralnih elektroda. U bolesnice je poslijeoperacijski
tijek nakon postavljanja elektroda, tijekom
četverodnevnog monitoriranja, kao i tijek nakon
resekcije kortikalne displazije protekao uspješno,
bez komplikacija. Poslijeoperacijski je bolesnica
potpuno bez epileptičnih napadaja, značajnog kognitivnog
poboljšanja, dobro se osjeća i nakon 40
godina upoznaje normalan život koji nikada prije
nije imala.
Učinjeni postupak invazivnog EEG monitoriranja
uz resekciju epileptogene zone nadopuna je postojećem
operacijskom programu epilepsija (lezionektomije,
selektivne amigdalohipokampektomije,
prednje temporalne resekcije, vagusne stimulacije
i dr.) koji se već godinama kontinuirano
primjenjuje suradnjom Referentnog centra Ministarstva
zdravstva Republike Hrvatske za epilepsiju,
Klinike za neurologiju, te Klinike za neurokirurgiju
Kliničkog bolničkog centra Zagreb u liječenju
farmakorezistentnih epilepsija.
Za Centar za epilepsiju Klinike za neurologiju:
Doc. dr. sc. Sanja Hajnšek
Doc. dr. sc. Željka Petelin
Dr. Sibila Nanković
Za Kliniku za neurokirurgiju:
Mr. sc. dr. Goran Mrak
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