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Biomedical subjects

J Kulczycki

Publications and source records attributed to J Kulczycki.

At least 91 records · Page 5Linked to original sources
PubMedDate not supplied

[Progressive supranuclear palsy, diagnostic problems in the light of own case].

A case is reported in which the neurological findings and disease course meet fully the criteria of the possible progressive supranuclear palsy. The authors discuss the principles of clinical diagnosis of the syndrome, characteristic oculomotor disturbances and parkinsonian syndrome especially pronounced in this disease in the muscles of the nape, trunk and upper extremities. The main features of neuropathological changes are described fibrillary degeneration and atrophy of neurons in midbrain and pons, with accumulation of tau protein in the neurons and astrocytes in the areas involved by the process.

Astrocytes↗
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[Correlation of clinical symptomatology with the results of brain imaging in cases of leukoaraiosis].

The aim of the study was establishing of correlations between the age of patients and their present and past diseases on one hand and the presence and intensity of leukoaraiosis (LA) on the other hand. The studied material comprised 91 patients hospitalized between Jan 1 1995 and June 15 1996 in whom features of LA were found on CT or MRI examination. Women and patients aged over 70 years prevailed in the group. Clinical analysis was based on neurological findings and routine diagnostic investigations. The intensity of LA lesions was assessed by means of a four grade scoring system known in literature. The analysis showed that higher grades of LA were more frequent on older patients: 14 had grade IV LA and 80 or more years of age. The greatest number of the patients had grade III LA and most of them were in the age range 70-80 years. Arterial hypertension was present in 64% of the patients, and more frequently it was associated with LA in younger patients. Stroke and myocardial infarction were given by 31% an 33% of the patients respectively in the history. Several had more than one stroke. Diabetes was found in 21 patients. Ischaemic brain episodes were the most cause of hospitalization.

Adult↗
PubMedDate not supplied

[Prospects of measles and subacute sclerosing panencephalitis (SSPE) elimination in Poland].

Following the introduction of vaccinations against measles in 1975 supplemented in recent years with booster vaccinations at the age of 6 years the epidemiological situation with respect to measles and SSPE has been gradually improving, particularly recently. In the paper discussion is presented on the question in what degree the present epidemiological situation of measles, the epidemiological supervision and vaccinations against measles in Poland meet the operative aim of measles and, consequently, SSPE elimination, as recommended by the WHO Regional Bureau. Attention is called to incomplete reliability of measles diagnosis based on clinical manifestations, economic difficulties in conducting serological investigations (detection of IgM antibodies to measles) necessary for measles confirmation, and shortcomings in vaccination organization.

Child↗
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[A sporadic case of Creutzfeldt-Jakob disease with peculiar neuropathological lesions].

The authors report a case of Creutzfeldt-Jakob disease in a man aged 60 years from a family without a history of similar disease. The disease extended over 11 months. In the clinical picture initially equilibrium disturbances and dementia with psychotic symptoms predominated, EEG pattern was not typical of CJD. Neuropathological examination revealed extensive spongiform lesions in the cortex of all cerebral lobes, in striatum and substantia nigra, moreover a considerable number of kuru plaques was found in cerebellar cortex. The authors consider that the case meets the criteria accepted for the sporadic form of CJD but believe that the final differentiation from the Gerstmann-Streussler-Scheinker syndrome should be based on genetic studies.

Cerebellum↗
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[Neurological status improvement in central pontine myelinolysis in alcohol-dependent patient].

The authors report a case of central pontine myelinolysis in a woman aged 30 years addicted to alcohol since at least 30 years. In the diagnosis the principal role was played by MRI. Attention is called to the disproportion between massive brainstem lesion and relatively low extent of neurological symptoms and signs. The patient had a several-weeks alcohol detoxication treatment and general supportive treatment. During that tine the neurological signs regressed nearly completely which suggests considerable reversibility or compensation of brainstem lesions in this syndrome.

Alcoholism↗
PubMedDate not supplied

[Sequence of clinical changes and evolution of MR images in a case of chronic SSPE].

Slow course case of subacute sclerosing panencephalitis is reported. The onset was at the age of 16 years and death followed after 5 years. He was observed during nine successive hospitalizations and the authors describe the changing clinical symptomatology parallelly with successive EEG records and brain MR imaging evolution. Successive phases of the disease were associated with hyperintense changes in T2-weighted.images, and were situated initially in occipital lobes, and involved later on periventricular areas and finally the whole white matter of both cerebral hemispheres. Subcortical brain atrophy developed parallelly.

Adolescent↗
PubMedDate not supplied

[The usefulness of MRI of the central nervous system in certain diseases of blood and hemopoietic system with neurological symptoms].

The results are presented of MRI studies of the brain and spinal cord in patients with neurological symptoms in the course of certain diseases of the haemopoietic system (leukaemias, lymphomas). The analysis showed that MR images, although not specific, can be very useful for the evaluation of the intensity and topography of haematological lesions in the central nervous system. However, the diagnosis of these processes is not easy. In the differential diagnosis focal lesions of vasogenic origin, infections and radiation-induced lesions should be considered, and in cases of lymphoma--metastases are a possibility.

Adult↗
PubMedDate not supplied

[Juvenile form of Huntington's disease--diagnostic problems].

Variability of clinical manifestation is an important feature of Huntington's disease (HD). It is due to the high instability of CAG sequences within a coding region of IT15 gene. We present five pedigrees in which apart from the adult form of HD the juvenile form of the disease affected some of the patients--as a result of genetic anticipation. Molecular analysis confirmed the well known fact that anticipation, which manifests itself by earlier onset of the disease in the subsequent generations, is strongly correlated with the degree of amplification of (CAG)n repeats in IT15 gene. An interesting feature of the presented data is the fact, that expansion of CAG repeats occurred not only at the paternal but also at the maternal transmission of the mutation. Some children in the presented HD pedigrees presented other neurological disturbances which could be suspected of HD; a molecular analysis revealing normal number of CAG repeats, enabled us to avoid misdiagnosis. The presented data provide evidence that clinical diagnosis of HD, particularly in cases with not very characteristic clinical picture--is not possible without DNA analysis--even in the families undoubtfully affected with the disease.

Adolescent↗
PubMedDate not supplied

[Subacute sclerosing panencephalitis (SSPE) in Poland in 1996-1999. Phase VII of epidemiologic studies].

In the seventh phase of epidemiological studies of SSPE data were gathered on patients who developed the disease in 1996-1999. In this time period the diagnosis was confirmed in only 10 cases (4 in 1996, 4 in 1997, 0 in 1998 and 2 in 1999). This is a significant reduction of incidence in relation to the preceding stages, in particular to the years 1993-1995 in which 49 new cases were still reported. This is not ruling out the possibility that at the beginning of the years 2,000 cases would be diagnosed in which the first symptoms developed several or more months earlier.

Adolescent↗
PubMedDate not supplied

[Familial Alzheimer's disease connected with mutation in presenilin gene 1 (P117L)].

We describe a Polish family with Alzheimer's disease in some of its members. Two sisters were observed and examined--also neuropathologically in the Institute of Psychiatry and Neurology in Warsaw. The disease onset was in our patients at 32 and 33 years. The first symptoms were memory loss and disorientation. Later on myoclonus and extrapyramidal stiffness were noted in both cases. Neurovisualizing examinations performed in both sisters showed diffuse brain atrophy. The symptoms increased rapidly and in short time (several months) the patients became mute and bedbound. They died at age 35 and 37 years. We were informed that the father of the patients suffered from very similar illness and died at age of 37 years and their older brother had the some symptoms and died at the age of 28 years. Post-mortem brain examination disclosed in the both hospitalized cases diffuse atrophy of the cerebral hemispheres, particularly severe in the temporal lobes. Microscopically senile plaques of various types were found in the cortex. The density of the plaques was very high but Alzheimer's fibrillary degeneration was found occasionally only. The amyloid burden in cortex of the examined brains, estimated as the measure of parenchymal amyloidosis beta, was two to six-fold higher in most areas compared with changes in sporadic AD and Down-syndrome cases. DNA was isolated from blood and tissue of both cases and from blood of their 8 children as well. In both patients mutation in presenilin 1 (PS1) gene of Prol 117 Leu was found and it was discovered that 4 persons of their progeniture were carriers of this mutation. The described mutation causes one of the earliest so far reported onset and death in FAD kindreds. Presenilin isolated from both cases and transfected into cultures of murine neuroblastoma and human kidneys provoked production of beta amyloid with increased A-beta 42/40 ratio.

Adult↗
PubMedDate not supplied

[Considerations of biopsy in neurological diagnosis].

The evolution is described in the views of neurologists on the indications to brain biopsy in clinical practice. In the 1960 and 1970s the main indication were diffuse progressing processes in brain associated with dementia. Presently, this biopsy is done mainly in cases of focal lesions of unclear aetiology. Technical advances in neurovisual examinations and the development of stereotactic methods sparing the patients open presently extensive possibilities of neuropathological diagnosis of lesions, even those situated deeply in the brain. The ethical and legal principles of the use of biopsies in neurological practice have not changed over years.

Alzheimer Disease↗
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[Subacute sclerosing panencephalitis (SSPE) in women during pregnancy].

Four women with SSPE during pregnancy were observed. The influence of pregnancy on the course of illness was in all patients unfavourable: the disease exacerbated, what could be explained by physiological immunosuppression connected with gestation. Delivery (in three cases by sectio caesarea) had no beneficial influence on the fatal course of SSPE. In two cases intrauterine necrosis of foetus took place in the 8th and 20th week from the beginning of SSPE. In other two cases healthy children were born. One of them does not exhibit any neurological abnormalities during the first 5 years of life.

Adult↗
PubMedDate not supplied

[Oligoclonal bands of four IgG subfractions and IgM and IgA in the serum and cerebrospinal fluid of friends with multiple sclerosis].

Using the methods of isoelectric focusing and immunoblotting the CSF and serum immunoglobulins were analysed in 47 patients with multiple sclerosis and 57 patients with other neurological diseases. The oligoclonal bands were found in the IgG fraction in 42 multiple sclerosis (ms) patients and 13 controls. The percent proportion of oligoclonal bands in various IgG subfractions was similar in both groups and was: 66% and 61% respectively for IgG1, 22% and 22% for IgG3, 12% and 17% for IgG2. In no case was IgG4 found. Additionally sporadic oligoclonality of IgA and IgM in CSF was found. Positive results were obtained in 2 and 2 cases with ms, and in one control case (only IgA), among controls the oligoclonal bands were found most frequently in neuroinfections (38.5%) and polyneuropathy (20.5%).

Humans↗
PubMedDate not supplied

[Comparison of the results of the treatment of patients with SSPE using various immunomodulating preparations].

The authors analysed the results of controlled treatment by three methods of patients in the 1st or 2nd phase of SSPE in a randomized group. The groups received: I. Propionibacterium granulosum KP-45 (interferon inducer) + isoprinosine, II--TFX (thymus extract) + isoprinosine, III--only isoprinosine. In all groups the treatment was continued during 6 months. The analysis of the clinical results of these methods failed to demonstrate a statistically significant superiority of any of these methods (among others, due to small number of cases) but an evident statistical tendency was revealed suggesting a better effectiveness of combined treatment (immunostimulator + isoprinosine) in relation to treatment with isoprinosine only. The final evaluation of the results requires further observations of these patients.

Adjuvants, Immunologic↗