Effect of liver transplantation on brain magnetic resonance imaging pathology in Wilson disease: a case report.

The authors present a case report of a 28-year-old patient with hepatic, but no neurological, signs of Wilson disease, with pathological changes in both the globi pallidi and caudate found with routine brain magnetic resonance imaging (MRI). The patient was recommended for liver transplantation by hepatologists, and during the two years of observation after liver transplantation, MRI brain abnormalities due to Wilson disease completely regressed. On the basis of this case, the authors present an argument for the prognostic significance of brain MRI in Wilson disease as well as current recommendations concerning liver transplantation in Wilson disease.

The effect of gender on brain MRI pathology in Wilson's disease.

Gender influence on the clinical manifestations of Wilson's Disease (WD) has been suggested; however, brain MRI pathology based on sexual dimorphism in WD has not yet been examined. The aim of this study was to analyse the effect of gender on brain MRI pathology according to the predominant form of WD. We retrospectively analysed the brain MR images of 204 newly diagnosed and untreated WD patients. The predominant form of the disease was neuropsychiatric (n = 105), hepatic (n = 67) or presymptomatic (n = 32). Overall, neuroimaging pathologies were found in 64.2 % WD patients. The clinical form analysis revealed significant gender-related differences. In the neuropsychiatric form, men presented with cerebellar atrophy and cortical brain atrophy more often than women (25/58 vs. 11/47; p < 0.05) and (23/58 vs. 12/47; p = 0.09), respectively. In contrast, women tended to present with globus pallidus lesions more often than men (25/47 vs. 20/58; p = 0.054). There were no gender differences observed in the hepatic form, but cortical brain atrophy presented more often in men than women (3/12 vs. 0/20; p < 0.05) in the presymptomatic form. According to our findings, there is a gender-dependent brain vulnerability to copper toxicity. We speculate that these differences are potentially related to an oestrogen protective effect and are due to differences in gender-related clinical forms.

[EEG value in cases of epileptic seizures in early phase of stroke]. / Wartosc badania EEG u pacjentów z napadami padaczkowymi we wczesnej fazie udaru mózgu.

The aim of our work was to assess the usefulness of EEG in patients with seizures in acute phase of stroke. EEG patterns of 54 patients with epileptic seizures at the onset of stroke were evaluated: 45 of ischaemic, 6 of haemorrhagic origin and 3 with lacunar stroke as confirmed by CT or MR examination. Out of 40 patients, who had a single or multiple seizures at the onset of stroke, EEG revealed focal slow waves in 90% and in 22.5% they were accompanied by interictal epileptiform discharges. None of those patients had recurrent seizures during the time of hospitalisation irrespectively of applying antiepileptic drugs. In the remaining 14 patients prolonged disturbances of consciousness were observed. EEG examination revealed simple partial status epilepticus (SE) in 10 and complex partial SE in 4 of them. In 11 of those patients EEG disclosed fragments of periodic lateralized epileptiform discharges (PLED) interrupted by seizure activity. The findings indicate that in patients with seizures in the acute phase of stroke EEG examination is very helpful in making the proper therapeutic decision by recognizing the SE. The introduction of antiepileptic drugs is generally not necessary in stroke patients with single seizures.

[PLED pattern and its clinical significance in stroke patients]. / Zmiany w eeg typu PLED i ich kliniczne znaczenie u chorych z udarem mózgu.

The pathophysiological connection between periodic lateralized epileptiform discharges (PLED) and epileptic seizures is still not clear. In the study clinical data and EEG findings were analysed in 22 patients aged 43-90 years with a history of stroke in whom EEG disclosed PLED. Eleven patients were studied in the acute phase of stroke and 11 were studied years after stroke when the diagnosis was established of poststroke epilepsy. In 2 patients in acute stroke group single epileptic seizures occurred and 5 had partial status epilepticus. In the group with poststroke epilepsy 4 had single seizures and 4 had epileptic status with partial epilepsy seizures. Thus, in 15 out of 22 patients PLEDs were noted after epileptic seizures. In all cases PLED appearance was connected with consciousness disturbances, lasting 1 to 17 days. In 6 cases PLED pattern was interrupted by seizure activity over one hemisphere, in 3 of them partial epileptic seizures were associated with it. In acute phase of stroke neuroimaging demonstrated the presence of fresh ischaemic foci, but in cases of poststroke epilepsy no such fresh foci were observed. These results suggest that PLED frequently can be associated with epilepsy, and in some patients it can be a bioelectrical manifestation of partial status epileptic.

Problems associated with the treatment of instability of the spine and the occipito-vertebral junction in rheumatoid arthritis.

This article discusses the clinical problems caused by the changes in the cervical spine accompanying rheumatoid arthritis.
The most commonly encountered pathology of the spine in the course of rheumatoid arthritis is C1-C2 instability, which can only be treated surgically. The development of new surgical techniques and operation methods has made it possible to obtain good outcomes. The clinical material described here includes 16 patients operated using new methods. The observation of these patients allows for the following conclusions:
- Fixation should involve only the C1-C3 segment, and is best accomplished by frame stabilization;
- The fixation method of choice is to achieve stability by using implants;
- The disease process of rheumatoid arthritis is progressive, which is changes in the cervical spine may serve as indicators of the further progress of changes, both at the point of surgery and in other parts of the cervical spine.

Hyperacute infarction: early CT findings.

The aim of our study is to determine how early and reliably ischemic brain infarcts can be detected on CT scanning. We report two cases of an early CT finding of acute ischemic infarcts where CT examination was obtained within the first 3 hours of onset of neurological symptoms. CT examination showed blurred outlines and decreased attenuation of the left lentiform nucleus, loss of the white-gray matter interface in the left insular cortex and left cortical global hypoattenuation with obliteration of left cortical sulci in one patient. In second patient CT showed the following: hyperdense left middle cerebral artery, loss of clear margins of left lentiform nucleus, subtle focal cortical hypodensity in the left fronto-parietal area with signs of mass effect in the form of ventricular compression. CT is still the first choice in the deferential diagnosis of acute stroke. Although older literature positions have suggested that CT was negative during the first 48 hours, modern CT technology can demonstrate positive findings even in the first 3 hours of onset. This is important in patients with acute stroke, as thrombolytic therapy when elected, has to be given within the first 3-6 hours after onset of symptoms. To determine how early and reliably ischemic brain infarcts can be detected on CT scanning, we report two cases of an early CT finding of acute ischemic infarcts where CT examination was obtained within the first 3 hours of onset of neurological symptoms.

[Lacunar strokes: clinical conditions vs. localization of lesions in neuroimaging studies]. / Udary zatokowate: stan kliniczny a lokalizacja ognisk w badaniach neuroobrazujacych.

Clinical records of 89 patients with lacunar stroke were reviewed, to evaluate the occurrence of neurological syndrome and to correlate the frequency of its signs with the localization of ischaemic lesions seen in neuroimaging examinations. "Pure" motor paresis (50%) and sensory-motor paresis (27%) were the most common clinical syndromes. The localization in putamen and corona radiata (37%), or in anterior part of the internal capsule and in the head of the caudate nucleus (35%) were the most frequent sites of localization. In the group of patients with lesions in anterior part of internal capsule or with lesions in putamen and corona radiata "pure" motor paresis was the most common clinical syndrome. Additionally, in the group of patients with lesions in putamen and corona radiata sensory motor paresis was also frequently observed (44%). During one year of follow-up 22 patients died. Dysarthria and disability (Barthel Index < 60) were the independent predictors of one year mortality. Non-neurological complications were the main cause of death.

[Improvement criteria after neuroleptic treatment and clinical and neuroradiological factors in schizophrenic psychoses. Preliminary studies]. / Kryteria poprawy po leczeniu neuroleptykami a niektóre parametry kliniczne i neuroradiologiczne w psychozach schizofrenicznych. Badania wstepne.

In 40 schizophrenic patients, various criteria of clinical improvement after neuroleptic treatment were compared in order to establish correlations between improvement after treatment and some clinical and MRI parameters. Three ways of evaluation of clinical improvement (CGI scale, PANSS index, percentage of improvement) correlated strongly with one another. Only the distribution of numbers of patients with different clinical improvement evaluated by the use of PANSS index was not statistically significant. Clinical improvement, evaluated with all three methods, significantly correlated with basal PANSS score as well as with the severity of positive symptoms and affective blunting, but not with the severity of schizophrenia negative symptoms. Only clinical improvement with the use of CGI demonstrated significantly better improvement in patients who had good previous response to neuroleptics. This particular method of clinical improvement evaluation, in contrast to other two methods, failed to reveal better response to neuroleptics among patients with no cortical atrophy found in MRI. Among patients with different improvement after treatment, evaluated with the use of all three methods, selected MRI parameters did not show significant differences with the exception of CGI improvement which correlated positively with the intensity of signal in T2-weighted image of gray matter in left medial frontal gyrus.

[Cortical atrophy in MRI, mental status and neuroleptic treatment effect in schizophrenia]. / Zaniki korowe stwierdzane w badaniu MR a obraz kliniczny i efekt leczenia neuroleptykami w schizofrenii.

MR imaging of the head was performed in forty schizophrenics (DSM-IV). Mental status was evaluated before and during 8-weeks of neuroleptic treatment. Cortical atrophy in frontal and temporal regions was found in 40% of subjects. They were older, had longer history of schizophrenia, were less active professionally and were more frequently hospitalized. Patients with and without cortical atrophy in MRI did not differ in the severity of schizophrenic psychopathology at baseline. During neuroleptic treatment negative schizophrenia symptoms were significantly better diminished in patients without cortical atrophy than in subjects with cortical atrophy in MRI; this regarded specially the severity of emotional blunting. Clinical improvement after 8-weeks of neuroleptic administration was less favorable in patients with cortical atrophy.

[Diagnostic value of oligoclonal band detection in cerebrospinal fluid using PhastSystem device]. / Wartosc diagnostyczna wykrywania pasm oligoklonalnych w plynie mózgowo-rdzeniowym z zastosowaniem aparatu PhastSystem.

The study confirming the presence of oligoclonal IgG in cerebrospinal fluid by isoelectric focusing (IEF) with PhastSystem equipment was carried out in 68 patients with clinically definite MS, in 23 with clinically probable MS and in 23 with other neurological diseases. Other indicators intrathecal synthesis of IgG were olso marked. According to the results it was confirmed that the most sensitive method of detection of intrathecal synthesis of immunoglobulins is finding of the oligoclonal IgG. Using the PhastSystem evidently shorties the diagnostic process. The sensivity of the method equals that of MRI and both have similar clinical value.

[Collateral circulation in patients with internal carotid artery occlusion depending on the localization of the ischemic stroke. Ultrasonographic study]. / Drogi krazenia obocznego u chorych z niedroznoscia tetnicy szyjnej wewnetrznej w zaleznosci od lokalizacji ogniska niedokrwiennego w mózgu. Badanie ultrasonograficzne.

The relationship between computerized tomography (CT) location of ischaemic strokes and the appearance of collateral circulation on transcranial Doppler ultrasonography (USG-D) was investigated in 30 patients with internal carotid artery occlusion. Evidence of collateral circulation via the anterior communicating artery was observed less frequently (88%) among patients with ischaemic lesions in the anterior and middle cerebral artery distribution, as well as the border zone area between these arteries, than in those with ischaemic strokes in the border zone area between posterior and middle cerebral arteries (100%). In patients in whom focal lesions were not found on CT, good collateral circulation via ophthalmic (100%), anterior communicating (100%) and posterior cerebral (84%) arteries was found on transcranial USG-D.