2017, Vol 17, No 3
Decompressive hemicraniectomy for treatment of malignant cerebral oedema after ischaemic stroke – a review of clinical trials
Aktualn Neurol 2017, 17 (3), p. 137–143
DOI: 10.15557/AN.2017.0014
ABSTRACT

Extensive ischaemic strokes resulting from middle cerebral artery occlusion may lead to massive cerebral oedema and cause an abrupt intracranial pressure increase. Life-threatening malignant cerebral oedema, which is found in 1–10% of patients with supratentorial stroke, usually develops from day two to day five after the onset of the symptoms of stroke. It frequently concerns younger patients with no prior history of ischaemic events. Clinical signs include consciousness disorders that accompany profound neurological deficits. Conventional conservative treatment is poorly effective. It seems therefore that decompressive hemicraniectomy may be a promising option. This article is a review of the most important randomised clinical trials and meta-analyses concerning this treatment method. The results indicate that hemicraniectomy in patients younger than 60 years of age is associated with lower mortality but many patients still require permanent or temporary care. The effects of surgical treatment in patients older than 60 years of age are worse. Hemicraniectomy does increase survival, but this effect is achieved at the cost of very severe disability in the vast majority of patients. The decision about hemicraniectomy should be made with caution after discussing the prognosis concerning survival and probable poor performance with the patient and his or her family. According to the current recommendations, there are no grounds for rendering patients ineligible for the procedure due to the dominant hemisphere involvement.

Keywords: ischaemic stroke, malignant cerebral oedema, decompressive hemicraniectomy
DOWNLOAD ARTICLE (PDF)
Duchenne muscular dystrophy: overview and future challenges
Aktualn Neurol 2017, 17 (3), p. 144–149
DOI: 10.15557/AN.2017.0015
ABSTRACT

Duchenne muscular dystrophy is a muscle disease caused by mutation in the gene that encodes the cytoskeletal protein dystrophin. It is inherited in an X-linked recessive fashion. A number of therapies are continuously being developed to slow down the progression of the disease and increase patients’ life expectancy. Steroid use in Duchenne muscular dystrophy is associated with a lower mortality rate (hazard ratio = 0.24; 95% CI = 0.07–0.91; p = 0.0351). Although recent studies have concluded that prolonged steroid use is associated with short stature and overweight, a meta-analysis of 12 studies has shown that steroids can increase strength, muscle function, and quality of life. Restoration of dystrophin gene expression is the basis of genetically engineered therapies. Potential therapies of this type include exon skipping, the use of recombinant adenoassociated virus which delivers mini-dystrophin, and surrogate gene transfer. In their development, the common challenges are associated with the size of gene product and the origin of dystrophin gene expression. Stem cells are promising for future therapy. Regardless of the challenges and controversies associated with stem cells, several clinical trials show an increase of muscle strength in patients who have received such therapies.

Keywords: Duchenne muscular dystrophy, therapy, genetic engineering, stem cells
DOWNLOAD ARTICLE (PDF)
Chosen aspects of cross-cultural adaptation of neuropsychological tests
Aktualn Neurol 2017, 17 (3), p. 150–157
DOI: 10.15557/AN.2017.0016
ABSTRACT

Neuropsychological test outcomes are influenced by education, literacy, ethnic origin and the culture background of the patient. One of the greatest neuropsychological challenges in the 21st century is to create a diagnostic tool that measures a pure cognitive process, regardless of the level of education or the national and cultural background of the patient. The paper is aimed at presenting selected problems associated with the adaptation of neuropsychological tests focusing on their feasibility in another culture. Culture-fairness of testing is associated with tasks used, their content and the testing context. In some non-Western cultures, psychometric testing is inadequate, as testing as such is atypical. Cognitive tests not only need normative data for each culture, but they also require cultural adaptation of tasks. Visuospatial tests are considered to be more “culture-fair” than verbal tasks, but some of the test results may be dependent on semantic knowledge. Likewise, the praxis tasks requiring performance or imitation of communicative gestures depends on their prior knowledge and use in a given culture. When adapting memory tests (especially tests of logical memory) for use in a new cultural context, particular emphasis should be put on the relevance of the material for the target population. As executive function tests engage more basic cognitive processes (such as language and visuospatial function, praxis and memory), their adaptation is associated with all of the elements outlined above. Also, since culture influences problemsolving strategies, some tasks may be irrelevant for a different culture due to the problem structure and not the task content. Creating culture-free cognitive measures, including screening tests, is very challenging. Further studies should focus on devising new tools adapted to specific cultural contexts with the proper understanding of the population at which the test is targeted.

Keywords: culturally-competent care, cross-cultural comparison, neuropsychological tests
DOWNLOAD ARTICLE (PDF)
Hypersexuality in Parkinson’s disease
Aktualn Neurol 2017, 17 (3), p. 158–161
DOI: 10.15557/AN.2017.0017
ABSTRACT

Hypersexuality is defined as a continuous, devastating and persistent need associated with human sexual life. It belongs to a group of complex behavioural disorders referred to as impulse control disorders. It is estimated that the incidence of hypersexuality in people with Parkinson’s disease ranges from 2% to 4%. In patients treated for Parkinson’s disease, hypersexuality is diagnosed more often than in the general population. The pathophysiology of hypersexuality in patients with Parkinson’s disease is not fully understood. It is suggested that the treatment of the underlying disease plays a significant role. In the literature, the majority of reports of hypersexuality cases have been linked to treatment with dopamine agonists, however, cases treated with levodopa, monoamine oxidase inhibitors or deep brain stimulation have also been reported. The risk factors of hypersexuality in patients with Parkinson’s disease include male gender, early onset of the disease and treatment with dopamine agonists. Little is known about the optimal management strategies for Parkinson’s disease patients with hypersexuality. Two long-term follow-up studies, although conducted in small groups, have indicated that discontinuation of dopamine agonists leads to full remission or clinically significant reduction of the symptoms of hypersexuality. Further studies are needed to determine how to successfully treat hypersexuality in patients with Parkinson’s disease.

Keywords: hypersexuality, Parkinson’s disease, risk factors, treatment
DOWNLOAD ARTICLE (PDF)
Ischaemic stroke in the artery of Percheron region mimicking nonconvulsive status epilepticus
Aktualn Neurol 2017, 17 (3), p. 162–166
DOI: 10.15557/AN.2017.0018
ABSTRACT

The occurrence of a cerebral circulation variant called the artery of Percheron is a rare phenomenon. In the case of its obstruction, the medial fragments of both thalami and sometimes the midbrain become ischaemic. Typical clinical signs include: consciousness and memory disorders, restricted vertical eyeball mobility and pupil reaction pathology. Aetiological factors are usually small vessel disease or cardiogenic embolism. The clinical signs of ischaemic stroke in the artery of Percheron region may mimic nonconvulsive status epilepticus. This article presents a patient with ischaemic stroke within both thalami and mesencephalon, initially diagnosed with and treated for status epilepticus. The exact knowledge of the signs produced by stroke in the artery of Percheron region offers chances for rapid diagnosis and implementation of proper treatment. The article illustrates difficulties associated with the diagnostic process and therapeutic decision-making in patients with atypical signs of stroke.

Keywords: stroke, nonconvulsive status epilepticus, artery of Percheron
DOWNLOAD ARTICLE (PDF)
The cognitive and emotional functioning of a female patient with a heritable form of the night eating syndrome – case description
Aktualn Neurol 2017, 17 (3), p. 167–171
DOI: 10.15557/AN.2017.0019
ABSTRACT

Background: In the Diagnostic and Statistical Manual of Mental Disorders (DSM-5) the night eating syndrome has been classified as one of other specified feeding or eating disorders. It is estimated that this syndrome affects about 1.5% of the population; the prevalence of its familial form is not known. Material and methods: The study applied the Pittsburgh Sleep Quality Index, the Mini-Addenbrooke’s Cognitive Examination, the Rey–Osterrieth Complex Figure Test, the Trail Making Test, the Auditory Verbal Learning Test, the Beck Depression Inventory, the State-Trait Anxiety Inventory and free diagnostic methods. Case description: The paper presents a case of a 35-year-old female patient hospitalised due to sleep disorders and episodes of night binge eating occurring for about 15 years. The performed examinations did not reveal any depressive, anxiety or positive symptoms nor symptoms indicating anorexia or bulimia. The neurological examination did not reveal any deviations from normality which was confirmed by the imaging examination of the head performed with a contrast agent, by the electroencephalographic examination and by biochemical examinations. The family history indicated the occurrence of similar symptoms in two family members. Results: The results of the psychological examination confirmed the lowering of sleep quality and the deterioration of cognitive functioning in the aspect of short-term verbal memory and of the ability to concentrate attention as well as the lowering of the level of semantic fluency in a narrow scope. Conclusions: Performing differential diagnostics resulted in diagnosing a familial form of the night eating syndrome. Considering the low prevalence of the discussed illness in the population, there is a necessity to deepen the research related to this form of the syndrome.

Keywords: night eating syndrome, cognitive functioning, emotional functioning
DOWNLOAD ARTICLE (PDF)