Successful therapy with bevacizumab in a case of hereditary hemorrhagic telangiectasia.

Hereditary hemorrhagic telangiectasia (HHT), genetic disorder manifested by uncontrolled multisystem angiogenesis with epistaxis, gastrointestinal bleeding, iron-deficiency anaemia, and arteriovenous malformations (AVM) is often related with increased levels of vascular endothelial growth factor (VEGF). Bevacizumab, a VEGF inhibitor, reduces epistaxis, telangiectasias, and iron-deficiency anaemia. A case of a female patient with HHT and chronic gastrointestinal bleeding is presented. The patient required iron supplementation and multiple blood transfusions. Bevacizumab brought marked symptomatic improvement and allowed for transfusion-independence. It is intended to apply for approval of the indications for bevacizumab administration in HHT as the 'orphan drug'.

Variability of the blink reflex in patients with migraine.

BACKGROUND AND PURPOSE: Sensitization of brainstem trigeminal nuclei and activation of the trigeminovascular system are thought to play an important role in migraine. The blink reflex has become a valuable tool for investigating trigeminal nerve function. The aim of the study was to assess the differences in electrophysiological examinations of the trigeminal nerve (blink reflex) in a group of patients with migraine in comparison with a healthy control group. MATERIAL AND METHODS: The examination was conducted among 58 patients. Patients were diagnosed in the Polyclinic or hospitalized in the Department of Neurology of Warsaw Medical University in Bielanski Hospital. The study group included 29 patients suffering from migraine (diagnosed according to the International Classification of Headache Disorders, 2nd edition) and 29 patients without headaches served as controls. All patients underwent neurological examination and magnetic resonance imaging to identify organic disorders. The blink reflex was tested among all patients in accordance with electrophysiological laboratory standards. RESULTS: The latency of the R1 response was significantly shorter among patients with migraine. The latency of R2 and R2' responses was similar in patients and controls. A significant inverse correlation was observed between latency of R2 and R2' responses and frequency of migraine attacks. CONCLUSIONS: The inverse correlation between the frequency of attacks and the latency of R2 and R2' responses of the blink reflex confirms the abnormal eaxcitability induced by the high frequency of migraine attacks.

[Effect of postinfarct left ventricular dysfunction on late occurrence of mitral regurgitation--two year experience]. / Wplyw pozawalowego uszkodzenia lewej komory na wystepowanie póznej niedomykalnosci mitralnej--obserwacja 2-letnia.

The aim of this study was to evaluate factors that might influence on the occurrence of mitral regurgitation (MR) in patients (pts) following myocardial infarction (MI) after fibrinolysis--two year experience. The study group comprised 118 pts: 40 women and 78 men (mean age: 58 +/- 9 years) following MI, who underwent Doppler echocardiography which revealed no MR 3 weeks after the MI. The second echocardiographic examination was performed after 2 years. We evaluated the following parameters: presence and stage of MR, left ventricular end-diastolic diameter (LVDD), left atrial diameter (LA), end-diastolic volume (EDV), wall motion score index (WMSI), asynergic area (AA) and ejection fraction (EF). Results after 2 years were as follows: 43 pts (36%) presented without MR, 45 (38%) MR I degree, 27 (23%) MR II degree, 3 (3%) MR III degree. Echocardiographic parameter comparison after 2 years demonstrated the following changes: LVDD increase from 5.0 +/- 0.5 cm to 5.3 +/- 4.6 cm (p < 0.005), LA increase from 3.7 +/- 0.4 cm to 4.3 +/- 0.4 cm (p < 0.00001), EDV increase from 130 +/- 29 ml to 147 +/- 39 ml (p < 0.005), WMSI increase from 1.37 +/- 0.23 to 1.45 +/- 0.21 (p < 0.05), AA increase from 23.5 +/- 10.1% to 27.8 +/- 7.9% (p < 0.005) and significant EF decrease 50.4 +/- 7.9% to 46.9 +/- 7.1% (p < 0.005). These results demonstrate that the occurrence of MR 2 years after the MI is caused by left ventricular remodelling, as well as segmental and global function deterioration.

Effect of treatment with 21-aminosteroid U-74389G and glucocorticoid steroid methylprednisolone on somatosensory evoked potentials in rat spinal cord during mild compression.

The purpose of this investigation was to compare the effects of treatment with glucocorticoid steroid methylprednisolone (MP) and the 21-aminosteroid U-74389G on the conduction of somatosensory evoked potentials (SEPs) during experimental spinal cord compression. Forty-five adult male Wistar rats were anesthetized and a laminectomy performed at the Th9-Th10 level. Animals with the same SEP patterns prior to and after laminectomy were randomly allocated to one of three groups (15 rats in each). A 14.8-g weight was applied to the dural surface of the spinal cord for 60 min. The SEPs were continually recorded during compression. The rats received a single intravenous bolus dose of three different agents two minutes after the start of compression. Animals in the first group received 0.5 ml of 0.9% NaCl, the second group received 30 mg/kg methylprednisolone and the third group received 3 mg/kg U-74389G. Following drug infusion the time period required for the SEPs to be completely suppressed was assessed. If the SEPs were not fully suppressed, the amplitude of the most stable and significant component of the SEPs was measured. The time taken to complete the SEPs suppression was significantly shorter in the control group (p < 0.001, Wilcoxon) than in the groups with either MP or U-74389G. However, the time taken to achieve full suppression was not significantly different between the MP and U-74389G groups. The proportional reduction of amplitude N1P1 was significantly different between the control and MP groups as well as between the control and U-74389G groups. The proportional reduction of amplitude N1P1 was not significant between the MP and the U-74389G groups. The present data indicate that both the glucocorticoid steroid MP and the 21-aminosteroid U-74389G protect spinal cord function to a similar extent during mild compression.

[Prophylactic treatment of migraine]. / Zapobiegawcze leczenie migreny.

Migraine belongs to the most frequent idiopathic headaches affecting 5-10% of the population. The knowledge of migraine pathogenesis is as yet insufficient for complete elucidation of its causes. There is no effective drug for all patients which could interrupt the attack or prevent its development. The methods of migraine prevention could be divided into pharmacological and non-pharmacological. Among the drugs used for attack prevention drugs are mentioned known and used since a long time and drugs less commonly used such as riboflavin, sulpiride, alpha-adrenolytic agents. Own experiences are presented with the use of iprasochrom for migraine prevention. Besides pharmacological methods also non-pharmacological methods are discussed which are applied for attack prevention.

[Pathophysiology and treatment of spinal cord medulla injuries]. / Patofizjologia i leczenie urazów rdzenia.

In view of the ever increasing incidence of spinal cord injuries and their very high socioeconomic costs studies are conducted for reduction of their consequences. In recent years considerable advances have been achieved in their treatment. The contribution of various mechanisms damaging spinal cord is known presently rather well, with isolation of two groups of causes: one is the primary spinal cord injury as a result of direct force acting on it during trauma, the other is secondary damage caused by vascular changes following trauma, free radicals, calcium distribution changes, participation of opioid receptors and inflammatory process. For counteracting these mechanisms in secondary cord damage treatment with drugs is justified. Among the drugs the main role is played by steroids--both glucocorticoids and non-glucocorticoids /lazaroids or aminosteroids/. Other drugs include calcium channel blockers, opioid receptor antagonists, serotonin antagonists, cyclo-oxygenase inhibitors, osmotically active drugs, antioxidants, NMDA receptor antagonists. Besides drugs hypervolaemia, haemodilution and hypothermia are tried. Proper diagnostic procedures and effective treatment of cord injury consequences depend on the knowledge of the mechanisms of later consequences of cord injury, this enables achieving of ever more effective treatment results.

[Clinical applications of late negative evoked potentials--contingent negative variation (CNV)]. / Zastosowanie kliniczne póznego warunkowego potencjalu wywolanego--(PWPW)--contingent negative variation (CNV).

Contingent negative variation /CNV/ is a slow negative potential described first in 1964 by Walter et al. It is a correlate of cerebral activity in the frontal lobes connected with expectation of stimulus and frontal cortex preparation for the stimulus to come. CNV develops in the time between the warning signal /S1/ and the commanding signal /S2/. CNV contains two main components connected directly with brain function: the first one, so called early component, is connected with the process of orientation or warning /it is called also orientation wave/, the second one /late component/ is connected with the preparation for movement /expectation wave or preparatory wave/. The clinical application of CNV is for the evaluation of the correlation of potential changes with changes in cognitive functions occurring in various diseases. Numerous studies reported recently have confirmed the applicability of CNV on the diagnosis of dementia, Parkinson's disease, epilepsy, schizophrenia, anxiety states, chronic pains, including migraine.

[Correlation between results of transcranial doppler ultrasonography and SPECT in the brain of patients with ischemic cerebral stroke]. / Korelacja wyników badan przezczaszkowej ultrasonografii dopplerowskiej i SPECT-u mózgu u chorych z niedokrwiennym uszkodzeniem mózgu.

Single photon emission computerized tomography /SPECT/ and positron emission tomography /PET/ are used presently for the study of the cerebral blood flow /CBF/. The cost of these procedures limits the possibility of their use and makes them available mainly in large and rich clinical centres. Transcranial Doppler USG has no such drawbacks. The purpose of the present study was analysis of a possible correlation between marker cumulation /in SPECT/ in the vascularization area of the middle cerebral artery /MCA/ and the parameters Vs, Vm, Vd, PI, RI, R of the PUSG-G examination of these arteries in 50 patients aged 48-79 years treated for ischaemic cerebral episodes. Brain SPECT examination was done with Apex-SP-6-HR gamma camera assessing the distribution of 99mTc HMPAO marker in the vascularization area of both MCA. PUSG-D examination was done with Tc-2-64 unit. In both MCA the systolic Vs, the mean velocity Vm, the diastolic velocity Vd, the Gosling pulsation index PI, the Purcelot resistance index RI and the velocity amplitude R were measured. The following conclusions have been reached: 1/ in patients with ischaemic cerebral episodes a significant correlation was found between cerebral SPECT findings and the PUSG-D parameters; 2/ reduced perfusion of the cerebral tissue was correlated with lower values of Vs, Vm, Vd, and R and with higher values of PI and RI; 3/ slight disturbances of perfusion found in SPECT were not reflected in changed PUSG-D parameters; 4/ the results justify the use of PUSG-D for indirect assessment of blood flow in the MCA.

[Use of transcranial doppler ultrasonography in patients with vascular dementia]. / Zastosowanie przezczaszkowej ultrasonografii dopplerowskiej u chorych z otepieniem naczyniowym.

The aim of the study was the evaluation of the clinical usefulness of transcranial Doppler ultrasonography /TDU/ in patients with vascular dementia. The study was carried out in a group of 69 patients divided into two groups: group i of 32 patients aged 45-78 years, mean age 58.4 years with two or more ischaemic foci on TDU and dementia symptoms. The cases in this group fulfilled the criteria of dementia according to DSM-IV, ICD 10, MMS scale and Hachinski ischaemic scale. In TDU the following parameters were analysed: maximal velocity /Vmax/, minimal velocity /Vd/, mean velocity /Vmean/ of blood flow in the middle cerebral arteries, pulsation index /PI/ and resistance index /RI/. In group II the patients were divided into three subgroups according to ICD-10 criteria and localization of ischaemic foci: with cortical, subcortical and mixed lesions. The following conclusion have been drawn: 1/ the parameters of TDU could serve for differentiation of patients with vascular dementia; 2/ the PI and RI parameters in TDU are most valuable for diagnosis establishing; 3/ the transcranial Doppler ultrasonography is insufficient for differential diagnosis of dementia.

[Use of modified lateral nucleus pulposus herniation classification in low back pain]. / Zastosowanie zmodyfikowanej klasyfikacji przepuklin bocznych jadra miazdzystego krazka miedzykregowego w zespolach bólowych dolnego odcinka kregoslupa.

Nucleus pulposus herniation is one of frequent causes in spinal pains. The necessity of undertaking of appropriate treatment, often surgical, requires early establishing of precise diagnosis. The traditional classification of herniations into central, centrolateral and lateral seems to be insufficiently precise, especially from the surgical point of view. For that purpose, the authors, following the classification of Volle et al., applied in their analysis the new classification into central and lateral nucleus pulposus herniae and discerning among the latter ones four types with strictly defined localisation: mediolateral, lateral with recess involvement, intervertebral foramen hernia, outside intervertebral foramen hernia. Seventy-two cases were analysed. Hernia was diagnosed by means of CT. Particular attention was given to the most external hernias, that is into and outside the intervertebral foramen. The more accurate method for the assessment of hernias and bone changes was in CT the Reconstruction in sagittal and frontal planes and also in oblique projections. The authors believe that the diagnosis of lateral nucleus pulposus hernias in low spinal area, particularly those with herniation into and outside the intervertebral foramen should be based on CT in appropriate reconstructions.

[Correlation between electronystagmographic findings and results of transcranial Doppler ultrasonography of vertebrobasilar arterial system in patients with vertigo]. / Korelacje wyników badan elektronystagmograficznych i przezczaszkowej ultrasonografii dopplerowskiej tetnic ukladu kregowo-podstawnego u chorych z zawrotami glowy.

The purpose of the study was the determination of correlations between the results of electronystagmographic (ENG) investigations and the blood flow velocity in the vertebrobasilar arterial system measured by Doppler ultrasonography in patients with vertigo. The studied material comprised 68 patients (39 women and 29 men) aged 34-68 years (mean 52.4 years). ENG findings included unilateral hyporeflexia in 25 cases (37.7%), bilateral hyporeflexia in 15 (22.0%) and unilateral canal failure in 8 (11.9%). Doppler USG with neck rotation test showed normal flow velocity in 15 cases (22.0%) and pathological velocity in the remaining 53 cases (78.0%). These results showed that bilateral hyporeflexia is found usually in bilateral failure of vertebral arteries and unilateral hyporeflexia and unilateral canal failure were found with unilateral vertebral artery failure on the same side.

Electrophysiological investigations of the mild spinal cord injury in rats.

Somatosensory evoked potentials (SEPs) waveform recorded from the normal and injured spinal cord in rats were analyzed. A mild compression spinal cord injury was performed in two experimental models. The first model was used to assess the disappearance and the second one the return of the electrophysiological spinal cord function after injury. In the first experimental model typical changes in waveforms morphology from normal to isoelectric line during spinal cord compression were investigated. In the second model an isoelectric line was achieved by more severe but a shorter time of compression and the typical changes in SEPs morphology after decompression were described. Our evidence suggests that the spinal cord function disappears gradually during mild compression and can return early after decompression. The amplitude after decompression first improved and then gradually deteriorated which is probably caused by secondary insult. Experimental models are both effective and simple and may be used to evaluate the effect of treatment of spinal cord injuries.

[The evaluation of the usefulness of electronystagmography studies in Doppler's intracranial ultrasonography and brain stem evoked potentials in patients with vertigo]. / Ocena przydatnosci badan elektronystagmograficznych przezczaszkowej ultrasonografii dopplerowskiej i sluchowych potencjalów wywolanych z pnia mózgu u chorych z zawrotami glowy.

The aim of the work was the evaluation of correlations between the electronystagmographic results examinations and Doppler's intracranial ultrasonography and brainstem auditory evoked potentials in patients with vertigo. The were 68 patients tested (39 women and 29 men) aged 34-68 years. The obtained results showed for the correlations between the electronystagmographic results examinations and Doppler's intracranial ultrasonography and brainstem auditory evoked potentials.

[Embolization of AVM's of thoracic spinal cord with histoacryl glue]. / Embolizacja naczyniaków odcinka piersiowego rdzenia kregowego za pomoca kleju histoakrylowego.

The authors present two cases of inoperable AVM's of thoracic spinal cord successfully treated by embolization with histoacryl glue (B. Brown Melsungen AG). The glue used for embolization is characterized by instant polymerization when comes in contact with blood. A mass of polymer is visible on X-ray thanks to contrast medium Lipiodol and metallic powder Tungsten that are added to glue and injected together with the latter. In the first case, a female who presented with paraparesis and walked only with assistance embolization performed in one session resulted in neurological improvement enabling independent walking at follow-up 2 yrs later. In the second case of a young male not walking for severe paraparesis embolization performed in three sessions resulted in significant neurological recovery. 10 days after the first session the patient became ambulatory. 6 months after treatment he presented with mild paraparesis and was still ambulatory and leading independent life. In each case embolization was performed after balloon occlusion test during which the function of spinal cord was monitored by somatosensory evoked potentials and neurological assessment.

[The physiopathology of acute spinal cord injury and a hope for a successful treatment]. / Patofizjologia urazu rdzenia kregowego i nadzieje na skuteczne leczenie.

The acute phase of spinal cord injury includes primary and secondary pathological patterns. Primary patterns include the effects of contusion, laceration, stretch of neural tissue and direct vascular trauma. These changes are irreversible. Secondary patterns include posttraumatic ischemic changes, loss of energy metabolism, oedema, release of cytotoxic substances such as free radicals, and electrolyte changes such as an increase in intracellular calcium ions. These changes may be reversible. This determines treatment strategies. Free-radical scavengers, opioid receptor antagonists include TRH and its analogues, calcium channel blockers, volume expander, osmotic diuretics, hypothermia, antioxidants, cycloxygenase inhibitors, serotonin antagonists and NMDA receptor antagonists were tested in experimental models during the last 4 years. The successful treatment should break the feedback loops and trails of secondary injury cascade in many places so combined treatment connected with many elements and surgery decompression is necessary.

[Somatosensory evoked potentials in the assessment of preserved function of long spinal cord pathways versus decisions of operation]. / Somatosensoryczne potencjaly wywolane w ocenie zachowania czynnosci dlugich dróg rdzeniowych a decyzja operacyjna.

In the light of several presented clinical cases the authors point out the usefulness of somatosensory evoked potentials for the objective determination of the continuity of long pathways in the spinal cord, which in correlation with the clinical findings and results of imaging methods helps in making the decision of surgical intervention. The non-invasiveness of this method makes possible repeated safe control examinations for assessment of the dynamic of lesions in the cord and objective assessment of the neurological condition after operations on the cord.

[Diagnosis of the upper thoracic outlet syndrome]. / Diagnostyka zespolu górnego otworu klatki piersiowej.

Basing on a review of literature and own experience the authors present the aetiology and various diagnostic methods of thoracic outlet syndrome (t.o.s.), especially electrodiagnostic and Doppler studies. Provocative tests like Wright, Adson or hyperabduction manouvers are discussed and their role in modern diagnosis of t.o.s. is presented.

Elevated plasma atrial natriuretic peptide concentrations in hypertensive patients with acute myocardial infarction.

BACKGROUND: Atrial natriuretic peptide (ANP) has been extensively studied in cardiovascular disorders in recent years. Particular attention has been paid to the role of ANP in the pathogenesis of hypertension and congestive heart failure and in the neurohormonal response to myocardial infarction. However, no published data are available on the significance of ANP in hypertensive patients with acute myocardial infarction. METHODS: We studied the relationship between plasma ANP concentration and systolic blood pressure, diastolic blood pressure, left atrial dimension, left ventricular diastolic dimension and left ventricular mass index in patients with essential hypertension in the acute phase of myocardial infarction. Plasma ANP concentrations were determined at 0, 4, 8, 16, 24, 48 and 72 h after admission in 40 patients with a first myocardial infarction (18 hypertensive patients, group 1; 22 normotensive patients, group 2). Left atrial dimension and left ventricular diastolic dimension were assessed echocardiographically within the first 48 h of acute myocardial infarction. RESULTS: Maximum and mean plasma ANP values at 0, 4, 8, 16, 24, 48, and 72 h as well as mean ANP concentrations within the 72 h period were higher in group 1 than in group 2 (P < 0.001). Plasma ANP concentration, left atrial dimension (r = 0.59) and left ventricular diastolic dimension (r = 0.56) were positively correlated in both groups. CONCLUSION: Acute myocardial infarction is characterized by a more pronounced rise in plasma ANP concentration in hypertensive patients than in those without a history of hypertension. Plasma ANP concentration correlates with left heart chamber sizes.

[The effect of the neck rotation test on auditory evoked brainstem potentials in healthy subjects]. / Wplyw testu skretu szyi na sluchowe potencjaly wywolane z pnia mózgu u osób zdrowych.

The purpose of the study was to assess the effect of neck rotation test on the pattern of brainstem auditory evoked potentials in healthy subjects. The study was carried out on 40 healthy subjects aged 19-26 years using a Neuromatic 2000 C device of Dantec, by the generally accepted method. The records were taken with the patient in lying position with head in neutral position and during head rotation through 60 degrees to the right and then to the left side. A click stimulus was fed into the tested ear, its frequency was 10 Hz and it was repeated 2000 times. The filters used were 100 Hz-2 kHz. The records obtained during neck rotation showed that the latency times of various waves were slightly prolonged on the side of the rotation (but were still within normal limits), while on the contralateral side they were similar to those in normal position. Moreover, neck rotation to the right or to the left side caused a bilateral slight shortening of the latency time (in absolute values) between waves I-III, at the expense of slight prolongation of this time between waves III-V which was still within the variability range of the normal values.