[Moyamoya disease as a rare cause of ischaemic stroke--case report]. / Choroba moyamoya jako rzadka przyczyna udaru niedokrwiennego mózgu--opis przypadku.

UNLABELLED: Moyamoya disease is a rare, progressive disease of the vessels diagnosed according to characteristic abnormalities of brain arteries in the angiography. The incidence of moyamoya disease in Europe is lower than in Asia and its clinical course in European population is probably different from Asiatic (older age of onset and rare incidence of hemorrhagic strokes). CASE REPORTS: Two young patients were diagnosed as moyamoya disease on the basis of clinical symptoms (ischaemic stroke) and results of brain vessels' angiography, which documented an occlusion of both internal carotid arteries above branching-off the ocular arteries in the first patient and stenosis of distal internal carotid arteries and proximal medial and anterior cerebral arteries in the second one. Both patients are under control of the Neurological Outpatient Department and their neurological state is stable. CONCLUSIONS: Despite that moyamoya disease is a rare cause of ischaemic stroke, it should be always considered as one of etiologic factors, especially in young patients.

[Transient cortical blindness--a complication after coronary angiography--case report]. / Przejsciowe zaburzenia widzenia korowe jako powiklanie po koronarografii--opis przypadku.

Transient cortical blindness is a very rare complication of cardiovascular and cerebral angiography procedures. We present a case of 63-years-old woman, who developed cortical blindness after coronary angiography. Computed tomography (CT) done immediately after blindness appearance showed bilateral hyperintensive areas (probably due to a leakage of contrast medium) in the occipital and parietal cerebral lobes. All visual symptoms disappeared during 48 hours and CT scan repeated after sight recovery did not show any focal lesions in the brain. A breakdown of the blood-brain barrier with direct contrast neurotoxicity seems to be the causal factor of neurological changes observed in our patient after coronary angiography.

[Moyamoya disease as a cause of ischemic cerebral stroke in young people]. / Choroba moyamoya jako przyczyna udaru niedokrwiennego mózgu w mlodym wieku.

35-year old patient was admitted to the Department of Neurology, Medical University of Bialystok because of paresis of his left upper limb, progressing over last 10 months and right facial nerve paresis, which started a month ago. During neurological examination he presented with right facial and arm paresis, dysarthric speech, obesity and hypertension. The patient was previously hospitalized in regional hospital, where a lumbar puncture was done revealing normal composition of cerebrospinal fluid. His brain CT revealed bilateral hypodensive areas in frontal and parietal regions of vasogenic character. Doppler ultrasound showed significant slowing of blood flow velocity in both internal carotid arteries. Brain angiography presented with very weak contrast filling of intracranial branches of carotid and vertebral arteries and showed stenosis at the terminal portion of the internal carotid arteries and at the proximal portion of the anterior and middle cerebral arteries. The patient had transthoracic and transesophageal echocardiography, Holter ECG, lab tests (routine lab tests plus coagulation system evaluation with C protein resistance test, anticardiolipne antibodies, antinuclear antibodies, anticytoplasmatic antibodies and thyroid hormones) checked--all tests were within normal range. Based on cerebral angiography and clinical symptoms, after excluding any other reasons of cerebral ischemia, the patient was diagnosed with moyamoya disease and arterial hypertension. The patient was treated pharmacologically with improvement--regression of face assymetry and dysarthria and diminishing of his right arm paresis. The authors of this paper pay attention to moyamoya disease as a rare reason of ischemic strokes in the young in our geographic region. They remind moyamoya disease diagnostic criteria, its etiology and treatment.

[Behcet's disease: a rare cause of multifocal lesions in the central nervous system]. / Choroba Behceta -- rzadka przyczyna zmian wieloogniskowych w osrodkowym ukladzie nerwowym.

Behcet's disease (BD) is a chronic multisystem inflammatory disease characterized mainly by recurrent episodes of orogenital aphthae and uveitis. In 10-30% of cases relapsing multifocal neurological symptoms occur. The authors present a 54-year-old female patient with the central nervous system manifestation of clinically diagnosed BD. Based on the reported case diagnostic (in particular differential diagnosis with multiple sclerosis) and therapeutic problems of BD are discussed.

[Adrenomyeloneuropathy: a late type of adrenoleukodystrophy linked to chromosome X]. / Adrenomieloneuropatia--pózna postac adrenoleukodystrofii sprzezonej z chromosomem X.

Adrenomyeloneuropathy is a late type of adrenoleukodystrophy. It is a hereditary disease linked to chromosome X and it is caused by abnormalities in the function of peroxisomes. Adrenomyeloneuropathy results from mutations in ABCD1 gene, that resides on chromosome Xq28 and encodes an integral peroxisomal membrane protein ALDP that belongs to the ATP-binding cassette-transporter family. The enzymatic defect concerns a transporter protein for acyl-CoA synthetase, taking part in beta-oxidation of very long chain fatty acids. This results in their accumulation in various organs. In the clinical picture spastic paresis of lower limbs, cerebellar ataxia, sensation and sphincteral disturbances predominate. This can lead to a misdiagnosis, especially shortly after the onset of symptoms, namely multiple sclerosis may be wrongly diagnosed. Coexisting endocrinological and quite often psychiatric disorders together with characteristic MRI findings facilitate the diagnosis. The diagnosis can be confirmed by a biochemical assay of very long chain fatty acids. We present a case of a 31-year-old man with adrenomyeloneuropathy. We based our diagnosis on a clinical picture and wide range of diagnostic procedures including: neuroradiologic findings, electrophysiologic, hormonal and biochemical tests, which are discussed in this article.

[Wearing off phenomenon presenting with features of paroxysmal abdominal pain]. / Napadowe bóle brzucha jako objaw reakcji "wyczerpania".

UNLABELLED: Degenerative process in Parkinson's disease affects substantia nigra and other central structures of an extrapyramidal system but it can also affect central and peripheral autonomic centres. One of the most frequent late complications in levodopa therapy is a wearing off phenomenon. We present a patient treated for Parkinson's disease in whom during the period of levodopa wearing off we observed a paroxysmal abdominalgia apart from other features of a typical movement disorders like: increasing rigidity, gait disturbances and tremor. Abdominalgia consisted of stomach cramps, with variable localization in epi-, meso- and hypogastrium. Rectal tenesmus was also present. The patient was treated with analgesics, spasmolytics and carminative drugs with no effect. Abdominal pains regressed after an intake of the next levodopa dose. The patient presented with other features of a gastrointestinal tract autonomic system dysfunction like: chronic constipation, preterm satiety resulting in food intake reduction and a decrease in body weight. There was no organic lesions of the gastrointestinal system that could explain such disturbances. Pharmacologic treatment modification (more frequent levodopa dosage, additional dopamine agonist) resulted in some improvement. CONCLUSION: It is possible that the abdominal pains could be a clinical manifestation of a digestive tract dyskinesias, occurring during