Arteriovenous fistula of superficial temporal vessels.

We present a case of patient with a rare vessel pathology - arteriovenous fistula (AVF) of superficial temporal artery and vein. The 56-year-old man was admitted to the Department of Neurology because of a headache in the right temporal region with concomitant buzzing sound in his right ear. The pain was present mainly in the evenings and was stronger when touching the temporal region. He denied having any head injury in the last few years. There were no signs of central nervous system involvement in the neurological examination. Within the right temporal area a subcutaneous mass with redness of the surrounding skin and with palpable and audible pulsatile thrill was observed. In computed tomography (CT) of the head no abnormalities were found. In duplex-Doppler ultrasound examination of carotid arteries the systolic blood velocity in the right external carotid artery was over two times higher than in the left one. Its flow profile was turbulent and low-resistant. In CT angiography (CTA) an AVF between superficial temporal artery and vein was revealed - it was located at the level of the right zygomatic arch. In both CTA and magnetic resonance angiography, no abnormal connections between extra- and intracranial vessels were found. The patient underwent surgery with good result - all the symptoms disappeared. AVFs of vessels of the scalp are rare and their aetiology is mainly traumatic or iatrogenic. By describing this case we wanted to draw attention to less frequent causes of headache.

[Long-term prognosis in young adults after a cerebral ischemic episode]. / Rokowanie odlegle u mlodych chorych z przebytym incydentem niedokrwienia mózgu.

To asses long term prognosis in young adults with cerebral ischaemia a follow up study was performed. Eighty-four patients aged 18 to 45 years with the diagnosis of the first-ever ischaemic stroke or TIA were followed up. Four of them (4.8%) died within the first four weeks of cerebral ischaemia onset. Information about all but one patient (98.75%) who survived the first episode of cerebral ischaemia was obtained. The follow up time lasted 4 months till 8 years (mean 52 months +/- 20 months). Three of the patients died during the follow up (all--vascular deaths), twenty one others experienced second cerebral ischaemia one month to 80 months after the first one. Among the patients with first-ever ischaemic stroke the calculated incidence of vascular death or recurrent stroke in the patients who survived the first episode was 5.6% per year, and after 24 months--10.9%. Twenty-eight day mortality rate was in that group 5.6%. Twenty-eight day mortality rate, was in our group similar to the data from the literature, but recurrent stroke and vascular deaths appeared in our material twice as often as in the literature. It could be the effect of lifestyle and socioenvironmental determinants.

[On the determination of brain death]. / W sprawie orzekania stanu smierci mózgu.

In a prospective study of 20 cases of brain-stem death, EEG and TCD studies were correlated with clinical findings. We suggest that TCD can be incorporated into institutional protocols as a rapid and useful technique for confirmation of brain death. Our observations underline the need for electrophysiological confirmation (evoked potentials) of brain-stem death in patients with primary infratentorial lesions.

[Detection of patent foramen ovale by transcranial Doppler ultrasonography]. / Wykrywanie droznego otworu owalnego w badaniu przezczaskowej ultrasonografii Dopplera.

This study compares the value of transcranial Doppler ultrasound (TCD) and transesophageal echocardiography (TEE) for detecting a patent foramen ovale (PFO). A total of 61 stroke patients under 65 years of age, were studied. A PFO was detected by TEE in 27 of 61 patients and these results were used as the gold standard. TCD confirmed the presence of PFO in 23 of the 27 patients and detected a PFO in a further 5 patients with normal TEE results. TCD had a sensitivity of 85% (23 of 27) and accuracy of 85% (52 of 61). The prevalence of PFO detected by both methods was 38%.

Metabolite Profile in Pyramidal Tracts after Ischemic Brain Stroke Assessed by 1H MRS. A Multicenter Study.

The magnitude of the motor deficit in patients with stroke depends not only on the size and location of the destroyed brain tissue, but also on axonal injury in the descending motor pathways which appears after stroke. After cerebral ischemia, there are no visible abnormalities in conventional MRI in the intact pyramidal tracts despite the process of neuronal destruction by Wallerian degeneration. Conventional MRI is not a sensitive test for Wallerian degeneration in the acute or subacute time period as it shows no changes within the first four weeks. Magnetic resonance spectroscopy (MRS) has been used for better quantification of the extent or severity of fibre damage by evaluating metabolite alterations in normal-appearing corticospinal and corticopontal tracts. This study assessed the role of 1H MRS in the detection of changes in cerebral metabolite levels in pyramidal tracts after cortical/ subcortical infarction and to compare metabolite alterations to clinical outcome (assessed by Barthel index, Scandinavian Stroke Scale). The study included 31 patients who had suffered an ischemic cortical/subcortical stroke involving the motor cortex or the descending fibers. Ratios of NAA/Cr, Cho/Cr, lip/Cr and Lac/Cr from internal capsules and cerebral peduncles were measured and compared with clinical status assessed by Barthel index and Scandinavian Stroke Scale (SSS). The ratio of NAA/Cr was significantly decreased (p<0.001) in the normal-appearing ipsilateral internal capsule in comparison with the control group. Cho/Cr and lac/Cr ratios were increased compared to the control group (p=0.019). Decrease of NAA/Cr ratio correlated with clinical status assessed by Barthel index and there was a correlation between clinical improvement (assessed by SSS) and lac/Cr ratio. Tissue metabolite concentrations distant from the infarcted region correlated with the clinical course and had predictive value. Proton MRS is very useful tool for evaluating major changes in metabolite levels in pyramidal tracts after brain stroke.

Early-onset Alzheimer's disease with a de novo mutation in the presenilin 1 gene.

A 32-year-old woman diagnosed with very rapidly progressing early-onset Alzheimer's disease (EOAD), age of onset 29 years, and S170F mutation in presenilin 1 gene (PSEN1) is presented. Neuroimaging conducted 2 years after the first symptoms was typical for the advanced stage of Alzheimer's disease (AD), showing cortical brain atrophy, particularly within hippocampus, frontal and temporal cortex. The unaffected parents of the proband are not carriers of the mutation. The paternity was confirmed by microsatellite typing, strongly suggesting de novo origin of S170F mutation. In silico modeling of S170F mutation impact on presenilin 1 (PS1) transmembrane structure indicates that the mutation considerably alters putative interactions of PS1 with other proteins within gamma-secretase complex.

Morphological changes in the peripheral nervous system in the case of congenital malformations of the spinal cord.

A 21-year-old oligophrenic man developed after upper respiratory tract infection, quadriplegia with sphincter and respiratory disturbances. Lumbar punction revealed subarachnoid bleeding and elevated cerebrospinal protein level. Guillain-Barré syndrome and subarachnoid hemorrhage were diagnosed. At autopsy intraspinal angioma (C2-D6) and diastematomyelia (D11-lumbar segments) were found. Beside, intraspinal hemorrhage was present. Morphological examination of posterior and anterior spinal roots as well as peripheral nerves was done. Spheroids, axonal degeneration and prominent loss of myelinated fibers were observed in the proximal parts of the spinal roots. Axonal degeneration of myelinated fibers and regenerated fibers were noted in the distal parts of spinal roots and in peripheral nerves. Abnormal, fetal-like vessels were present in the spinal roots. Two mechanisms of acute and chronic changes (transneuronal and Wallerian degeneration) are discussed.

[Myasthenic crisis during pregnancy: case report and literature survey]. / Przelom miasteniczny w czasie ciazy: opis przypadku i przeglad pismiennictwa.

We report the successful use of high-dose intravenous immunoglobulins during pregnancy in a young woman with myasthenic crisis. To our knowledge, immunoglobulins in the management of myasthenic crisis during pregnancy have not been previously described. It is emphasized that both the patient and her fetus tolerated the treatment well.