Creutzfeldt-Jakob disease mimicking Lewy body dementia - a case report. / Choroba Creutzfeldta-Jakoba o poczatkowym przebiegu nasladujacym otepienie z cialami Lewy'ego ­ opis przypadku.

Lewy body dementia and Creutzfeldt-Jakob disease are recognized on the basis of certain diagnostic criteria. However, common symptoms such as: dementia, extrapyramidal syndrome, psychotic disorders may cause difficulty to make the correct diagnosis especially in the early stage of the disease. Each of these diseases may have atypical onset. The further course and the appearance of other symptoms indicate a proper diagnosis. Electroencephalogram and examination of 14-3-3 proteins in cerebrospinal fluid are helpful in the differential diagnosis. We present a case of a 66-year-old patient initially suspected of Lewy body dementia. On admission, psychomotor retardation, dysarthria, upper extremities dysmetria, extrapyramidal tension in the upper limbs, lower extremities ataxia, slow gait and unstable Romberg test were present. Mini-Mental State Examination (MMSE) score was 24/30. On neuropsychological assessment early stage of dementia was diagnosed. Anxiety-depressive symptoms were observed with periodic visual-auditory components. After less than 3 weeks there was a deterioration of neurological state. Dysarthria and lower limbs ataxia were increased, ataxia of the trunk appeared and psychomotor retardation got worse. There was significant progression of cognitive impairment, therefore complete neuropsychological examination was impossible to perform. MMSE score was 12/30 (12 points less than three months earlier). The course of the disease and additional tests results confirmed the diagnosis of sporadic Creutzfeldt-Jakob disease.

Radiological Evaluation of Strategic Structures in Patients with Mild Cognitive Impairment and Early Alzheimer's Disease.

BACKGROUND: The aim of the study was to evaluate the diagnostic value of two measurement techniques in patients with cognitive impairment - automated volumetry of the hippocampus, entorhinal cortex, parahippocampal gyrus, posterior cingulate gyrus, cortex of the temporal lobes and corpus callosum, and fractional anisotropy (FA) index measurement of the corpus callosum using diffusion tensor imaging. MATERIAL/METHODS: A total number of 96 patients underwent magnetic resonance imaging study of the brain - 33 healthy controls (HC), 33 patients with diagnosed mild cognitive impairment (MCI) and 30 patients with Alzheimer's disease (AD) in early stage. The severity of the dementia was evaluated with neuropsychological test battery. The volumetric measurements were performed automatically using FreeSurfer imaging software. The measurements of FA index were performed manually using ROI (region of interest) tool. RESULTS: The volumetric measurement of the temporal lobe cortex had the highest correct classification rate (68.7%), whereas the lowest was achieved with FA index measurement of the corpus callosum (51%). The highest sensitivity and specificity in discriminating between the patients with MCI vs. early AD was achieved with the volumetric measurement of the corpus callosum - the values were 73% and 71%, respectively, and the correct classification rate was 72%. The highest sensitivity and specificity in discriminating between HC and the patients with early AD was achieved with the volumetric measurement of the entorhinal cortex - the values were 94% and 100%, respectively, and the correct classification rate was 97%. The highest sensitivity and specificity in discriminating between HC and the patients with MCI was achieved with the volumetric measurement of the temporal lobe cortex - the values were 90% and 93%, respectively, and the correct classification rate was 92%. CONCLUSIONS: The diagnostic value varied depending on the measurement technique. The volumetric measurement of the atrophy proved to be the best imaging biomarker, which allowed the distinction between the groups of patients. The volumetric assessment of the corpus callosum proved to be a useful tool in discriminating between the patients with MCI vs. early AD.

Posterior reversible encephalopathy syndrome with neurological sequelae - a case report.

Posterior reversible encephalopathy syndrome (PRES) is a set of neurological symptoms including impaired consciousness, cognitive disorders, seizures, blurred vision, dizziness and headache. The symptoms are closely related to the location of pathological changes in the brain; bilateral occipitto-parietal region is most often affected. Both focal neurological symptoms and encephalopathy are usually transient. We present the case of 64-year-old woman with PRES. She suffered from many internal diseases and was admitted to the hospital due to impaired consciousness, speech disorders, balance disorders. Significant neurological deterioration was observed within several days from the onset. Differential diagnosis included stroke and viral neuroinfection (cytosis 23 cells /ul, protein level of 93 mg/dl); magnetic resonance examination revealed lesions typical for posterior reversible encephalopathy.After 6 weeks of hospitalization patient condition improved, but did not restore to the premorbid state. The patient was discharged with generalized cognitive impairment.

Combined use of biochemical and volumetric biomarkers to assess the risk of conversion of mild cognitive impairment to Alzheimer's disease.

INTRODUCTION: The aim of our study was to evaluate the usefulness of several biomarkers in predicting the conversion of mild cognitive impairment (MCI) to Alzheimer's disease (AD): ß-amyloid and tau proteins in cerebrospinal fluid and the volumetric evaluation of brain structures including the hippocampus in magnetic resonance imaging (MRI). MATERIAL AND METHODS: MRI of the brain with the volumetric assessment of hippocampus, entorhinal cortex, posterior cingulate gyrus, parahippocampal gyrus, superior, medial and inferior temporal gyri was performed in 40 patients diagnosed with mild cognitive impairment. Each patient had a lumbar puncture to evaluate ß-amyloid and tau protein (total and phosphorylated) levels in the cerebrospinal fluid. The observation period was 2 years. RESULTS: Amongst 40 patients with MCI, 9 (22.5%) converted to AD within 2 years of observation. Discriminant analysis was conducted and sensitivity for MCI conversion to AD on the basis of volumetric measurements was 88.9% and specificity 90.3%; on the basis of ß-amyloid and total tau, sensitivity was 77.8% and specificity 83.9%. The combined use of the results of volumetric measurements with the results of proteins in the cerebrospinal fluid did not increase the sensitivity (88.9%) but increased specificity to 96.8% and the percentage of correct classification to 95%.

Predicting the conversion of mild cognitive impairment to Alzheimer's disease based on the volumetric measurements of the selected brain structures in magnetic resonance imaging.

INTRODUCTION: Mild cognitive impairment (MCI) is defined as abnormal cognitive state, but does not meet the criteria for the diagnosis of dementia. According to the new guidelines Alzheimer's disease (AD) involves not only dementia's phase but also predementia phase which is asymptomatic and pathological process in the brain is already present. For this reason it is very important to determine the suitability of markers which should be positive before onset of the first symptoms. One of these biomarkers is a structural magnetic resonance imaging with hippocampal volumetric assessment. The aim of this study was to investigate the usefulness of structural brain magnetic resonance imaging with volumetric assessment of the hippocampus and entorhinal cortex, posterior cingulate gyrus, parahippocampal gyrus, temporal gyri: superior, medial and inferior, to predict the conversion of MCI to AD. MATERIAL AND METHODS: Magnetic resonance imaging of brain was performed at the baseline visit in 101 patients diagnosed with MCI. Clinic follow-ups were scheduled after 6.12 and 24 months. RESULTS: Amongst 101 patients with MCI, 17 (16.8%) converted into AD within two years of observation. All measured volumes were lower in converters than non-converters. Discriminant analysis was conducted and sensitivity for MCI conversion to AD was 64.7%, specificity 96.4%. 91% of patients were correctly classified (converter or non-converter). CONCLUSIONS: Volumetric measurements may help clinicians to predict MCI conversion to AD but due to low sensitivity it cannot be use separately. The study group requires further observation.

Shoulder joint tuberculosis.

BACKGROUND: Despite the fact that joint tuberculosis is one of the most common forms of extrapulmonary tuberculosis, it is a disease entity that is very rare in Poland (less than 100 cases a year in the last 10 years). The symptoms are non-specific, and thus the disease is rarely taken into account in preliminary differential diagnosis. CASE REPORT: A 68-year-old female patient was admitted to the Internal Diseases Clinic due to oedema and pain of the right shoulder joint. The pain has been increasing for about 8 months. Physical examination revealed increased circumference and elevated temperature of the right shoulder joint. Limb function was retained. The full range of radiological and laboratory diagnostic examinations was performed, including the biopsy of the affected tissue which revealed the presence of Mycobacterium tuberculosis in the bacterial culture. Clinical improvement was obtained after introduction of TB drugs. CONCLUSIONS: Radiological diagnostic methods (X-ray, CT scans, MRI scans) provide high precision monitoring of articular lesions. However, the decisive diagnosis requires additional laboratory tests as well as histopathological and bacteriological assays.

Brainstem ischemic stroke without permanent sequelae during the course of spontaneous internal carotid artery dissection - case report.

BACKGROUND: Internal carotid artery dissection (ICAD) is a frequent cause of a stroke in young patients. Risk factors which can lead to dissection include neck injury and diseases of the inner wall of the artery. Common symptoms in ICAD are cervical pain and headache, Horner's syndrome, paralysis of the cranial nerves and subsequently cerebral and retinal ischemia. MR angiography in TOF technique and brain MRI in T1- and T2-weighted images, FLAIR and DWI sequences are the method of choice in patients with ICAD but contrast-enhanced multislice computed tomography remains the fastest and the most available diagnostic method. CASE REPORT: A 39-year old woman, previously healthy, presented to the Hospital Emergency Department because of increasing neck pain on the right side and difficulty in swallowing. The neurological examination revealed: drooping of the right eyelid with narrow palpebral fissure, dysarthria, anisocoria (narrower pupil on the right side), unilateral hypoesthesia on the left side, weak palatal and pharyngeal reflexes on both sides, paresthesia within the left half of the body. Seven days before, the patient felt a sudden, severe neck pain radiating to the temporal apophysis. CT angiography revealed a defect in contrast filling within the left internal carotid artery and right vertebral artery. MRI of the head with MR angiography showed internal carotid artery dissection on the left side and dissection of the right vertebral artery and no ischemic changes within the brain. CONCLUSIONS: CT and MR angiography are methods characterized by high sensitivity in detecting dissection of the cervical arteries.