Fatal outcome of cervical myelopathy caused by fibrocartilaginous embolism. Rare cause of spinal vascular damage.

BACKGROUND AND PURPOSE: Fibrocartilaginous embolism is a rare cause of ischemic myelopathy. Authors report a case of a 39-year-old woman with progressive tetraparesis and severe autonomic dysfunction. Despite of the detailed examinations, the definite diagnosis was verified by autopsy. METHODS: The patient was admitted because of progressive pain and numbness of the upper extremities and tetraparesis. Hypotonic muscles of the lower extremities with mild tetraparesis were observed. Magnetic resonance imaging showed an intramedullary lesion at the level of the cervical V-VII vertebral. Patient's tetraparesis worsened gradually to plegia with urinary retention. Expansive, rapidly progressing multiple decubiti developed, which were resistant to therapy. In spite of the complex therapy, the patient died. RESULTS: No internal disease was found to explain the death by autopsy. Multiple subacute infarctions of the cervical myelon (involving the lateral columns as well) in the territory of the anterior spinal artery were verified by neuropathological examination. The occluded vessels were filled by a material containing cartilaginous cells, while signs of atherosclerosis or thrombosis were not present. CONCLUSION: Cartilaginous embolism of spinal arteries was diagnosed.

Epileptic Seizure Provoked by Bone Metastasis of Chronic Lymphoid Leukemia and Merkel Cell Carcinoma.

BACKGROUND: Merkel cell carcinoma (MCC) is a rare primary neuroendocrine cutaneous tumor, rarely metastasizing to the brain. Chronic lymphoid leukemia (CLL) is a disease predisposing to MCC. According to previous reports, headache and focal neurological deficits suggest disease progression to the brain. We present a patient with MCC whose seizure was not elicited by a cerebral metastasis, but by bone metastases compressing the brain. Case Presentation. A 62-year-old female patient had a history of CLL. A lesion with the appearance of an atheroma was removed from the right upper arm. Histology confirmed the diagnosis of MCC. She was admitted to the neurology department with her first GM seizure. The cranial MRI/MRA showed bone metastases in the right parietal and both frontal areas, compressing the brain. Flow cytometry of CSF did not reveal metastasis of MCC. CONCLUSIONS: The case history of the patient was unique even among the rare cases of MCC with neurological involvement. The seizure was not elicited by a cerebral metastasis, but by bone metastases compressing the brain. In addition to patient history, clinical presentation and radiological findings enabled a suspected diagnosis of skull metastasis of MCC compressing the brain, causing symptomatic epileptic seizures.

Anomalous equivalent potential temperature: an atmospheric feature predicting days with higher risk for fatal outcome in acute ischemic stroke-a preliminary study.

Acute stroke is a life-threatening condition. Fatal outcome is related to risk factors, some of these affected by climatic changes. Forecasting potentially harmful atmospheric processes may therefore be of practical importance in the acute care of stroke patients. We analyzed the history of all patients with acute ischemic stroke (N = 184) confirmed by neuroimaging including those who died (N = 35, 15 males) at our hospital department in the winter months of 2009. Patient data were anonymized, and the human meteorologists were only aware of patients' age, gender, and exact time of death. Of the meteorological parameters, equivalent potential temperature (EPT) has been chosen for analysis. EPT is generally used for forecasting thunderstorms, but in the case of synoptic scale airflow (10(6) m), it is suitable for characterizing the air mass inflowing from different regions. The behavior of measured EPT values was compared to the climatic (30 years) averages. We developed meteorological criteria for anomalous periods of EPT and tested if such periods are associated with higher rate of fatal outcome. The duration of anomalous and non-anomalous periods was nearly equal during the studied 3 months. Stroke onset distributed similarly between anomalous and non-anomalous days; however, of the 35 deaths, 27 occurred during anomalous periods: on average, 0.56 deaths occurred on anomalous days and 0.19 on non-anomalous days. Winter periods meeting the criteria of anomalous EPT may have a significant adverse human-meteorological impact on the outcome in acute ischemic stroke.

LADA type diabetes, celiac diasease, cerebellar ataxia and stiff person syndrome. A rare association of autoimmune disorders.

Celiac disease--in its typical form--is a chronic immune-mediated enteropathy with typical clinical symptoms that develops against gliadin content of cereal grains, and is often associated with other autoimmune diseases. In cases of atypical manifestation classic symptoms may be absent or mild, and extra-intestinal symptoms or associated syndromes dominate clinical picture. The authors present a longitudinal follow-up of such a case. A 63-years old woman was diagnosed with epilepsy at the age of 19, and with progressive limb ataxia at the age of 36, which was initially thought to be caused by cerebellar atrophy, later probably by stiff person syndrome. At the age 59, her diabetes mellitus manifested with type 2 diabetic phenotype, but based on GAD positivity later was reclassified as type 1 diabetes. Only the last check-up discovered the celiac disease, retrospectively explaining the entire disease course and neurological symptoms. By presenting this case, the authors would like to draw attention to the fact that one should think of the possibility of celiac disease when cerebellar ataxia, progressive neurological symptoms and diabetes are present at the same time. An early diagnosis may help to delay the progression of disease and help better treatment.

Dilemma of indication for thrombolysis in a patient with acute ischemic stroke treated with a novel oral anticoagulant.

Increasing age of the population is associated with a higher rate of cerebrovascular diseases, and every sixth stroke is the consequence of atrial fibrillation. In atrial fibrillation, vitamin K antagonists are routinely used to prevent cardioembolic strokes. Thrombolytic treatment recombinant tissue-type plasminogen activator (rt-PA) has established efficacy in acute ischemic stroke, but in anticoagulated patients, its use is contraindicated for those with an international normalized ratio of 1.7 or more. Recently, novel oral anticoagulants have become available. With conventional methods, however, it is difficult to assess the coagulation status of patients on these new treatments. We report the case of a patient treated with dabigatran who developed acute ischemic stroke and was considered for thrombolysis. Because of the prolonged thrombin time (TT), thrombolysis was not performed. Repeated coagulation tests 5 days after stroke, with unchanged anticoagulant (dabigatran) treatment, found 2-fold higher TT and activated partial thromboplastin time (APTT) compared with the values found in the acute stage. Routine coagulation tests (TT and APTT) do not reliably reflect the coagulation features of a patient; therefore, there is an urgent need to develop reliable biomarkers and an adequate guideline to help decision making regarding thrombolysis in those who develop their strokes while on one of these new oral anticoagulants.

Stroke prevention--a population screening day in district XII of Budapest.

Along with advances in the treatment of acute stroke, new efforts have been made to enhance efficiency of the prevention of cerebrovascular diseases. Population screening is a way to contact high-risk patients, and there is an increasing international and national experience with the procedure. However, efforts are associated with high costs, so an efficient method, complying with local features, should be selected from the various methods. A stroke prevention day was organized in Szent János Hospital, localized in district XII, and data were analyzed. Taking advantage of the potentials of a large hospital, a comprehensive risk assessment - within the capacity of health care workers - was performed. Program and contact information of the screening day was published in the local newspaper of the district. Data of 48 residents of the district were analyzed. In addition to neurologists, a radiologist, a cardiologist and an ophtalmologist, as well as health care workers were involved in the project. A data sheet was filled in for all participants, including known risk factors, BMI, blood pressure and serum cholesterol levels. All participants had duplex sonography of the cervical vessels, cardiac evaluation and ophtalmic examination. Data were analyzed anonymously, and - if participants approved - postcode and educational level were also recorded. Among the 48 individuals screened, 35 were female and 13 were male. Average age was 62.86 (+/- 8.57) years, and participants were typically of higher educational level. 5 individuals had no known risk factors, most of them had 2-3 risk factors, and multiple risk factors were not uncommon. Individuals with six and seven risk factors were also found. 20 of 27 patients with known hypertension had target blood pressure levels. By duplex sonography, 36 individuals had mild, 4 had significant atherosclerosis. There was no significant carotid stenosis or occlusion. Based on ophtalmic evaluation, 26 patients had signs of vascular disease (mainly hypertensive fundus changes). Cardiac evaluation detected 14 patients with cardiovascular risk. The high standard of primary care in the district was reflected by the fact that all the 6 high-risk individuals were already in the care of general practitioners (GP-s). One of the main conclusions from the evaluation of the data is that the press, family and local communities play a major role in recruiting people for a screening day. In order to increase efficiency and cost-effectiveness of the program, GP-s should also be involved in the planning process, because efficiency may be increased by pre-selecting high-risk individuals.

Role of diagnosis of dyslipidemia in primary and secondary vascular prevention in a neurology department.

Lipids have important functions in the human body, but high serum cholesterol level is an important risk factor for cardiovascular and cerebrovascular diseases. Prevention of stroke includes modifying risk factors, like dyslipidemias. Based on this theory, we examined in practice the possible role of a public care neurology and stroke department with a large patient turnover in vascular risk screening with regard to the diagnosis of hyperlipidemia. We reviewed all the medical records (irrespective of disease group;) of patients hospitalized in 2007 at Department of Neurclogy and Stroke Center of Szent János Hospital of the Municipality of the City of Budapest. Patients included in the study were classified into three groups: (1) those admitted with acute stroke; (2) those with a history of acute stroke, but without evidence of a novel cerebrovascular event; (3) no history and evidence of cerebrovascular disease during hospitalization. Our data show that 17.6% of patients was diagnosed with hyperlipidemia during hospital care, and another 18.5% was known to have elevated cholesterol levels. Altogether, 36.1% of the 1438 patients evaluated had hyper ipidemia. Known hypercholesterolemia was 18.4% in patierts admitted for acute stroke, 26.9% in patients formerly (but not currently) treated for cerebrovascular disease, and 13.6% in the third group. Newly diagnosed elevated cholesterol levels had highest rate (22.6%) in former stroke patierts (currently treated for other diseases); 20.4% in patients with acute stroke, and 13.2% in the third group. In the first two groups, the number of patients newly diagnosed with elevated serum cholesterol almost equaled to those with already known hypercholesterolemia. Based on our data, neurology departments have an important role in diagnosing hyperlipidemia and vascular prevention.