Susac's syndrome: clinical course and epidemiology in a Central European population.

Objective: Susac's syndrome is characterized by inflammation and occlusion of pre-capillary arterioles with the clinical triad of branch retinal artery occlusion (BRAO), encephalopathy and hearing loss. No epidemiological data are available for the disease.

Methods: All neurology departments in Austria were addressed to report adult patients who were on immunosuppressive treatment for a diagnosis of Susac's syndrome between 1 August 2010 and 1 August 2015.

Orbital tomography: Molecular band maps, momentum maps and the imaging of real space orbitals of adsorbed molecules.

The frontier orbitals of molecules are the prime determinants of their chemical, optical and electronic properties. Arguably, the most direct method of addressing the (filled) frontier orbitals is ultra-violet photoemission spectroscopy (UPS). Although UPS is a mature technique from the early 1970s on, the angular distribution of the photoemitted electrons was thought to be too complex to be analysed .

Multimodality MRI and MRA for decision making in minor stroke: a case with internal carotid and distal middle cerebral artery occlusion.

We report the case of a 65-year-old man who presented with mild, rapidly improving stroke symptoms. Acute magnetic resonance imaging disclosed no diffusion abnormalities but a tandem internal carotid artery/distal middle cerebral artery occlusion associated with a large corresponding deficit on perfusion imaging. In addition, there was a cross-flow to the middle cerebral artery via the anterior communicating artery.

Phenotypes of the N88S Berardinelli-Seip congenital lipodystrophy 2 mutation.

Recently, two missense mutations (N88S, S90L) in the Berardinelli-Seip congenital lipodystrophy gene have been identified in autosomal dominant distal hereditary motor neuropathy and Silver syndrome. We report the phenotypic consequences of the N88S mutation in 90 patients of 1 large Austrian family and two unrelated German families. Variation in the clinical and electrophysiological phenotype enabled us to distinguish six subtypes.

Phenotypic and genotypic heterogeneity in hereditary motor neuronopathy type V: a clinical, electrophysiological and genetic study.

We report on a large four-generation Austrian family with autosomal dominant distal hereditary motor neuronopathy type V (distal HMN V). Forty-seven at-risk family members, of whom 21 were definitely affected, underwent detailed clinical, electrophysiological and genetic studies. The age at onset was in the second decade of life in most affected individuals, but clinical presentation was rather variable.

Diffusion-weighted imaging with navigated interleaved echo-planar imaging and a conventional gradient system.

Purpose: To demonstrate the technical feasibility and precision of a navigated diffusion-weighted (DW) MR imaging method with interleaved echo-planar imaging and test its diagnostic sensitivity for detection of ischemic stroke.

Materials And Methods: Apparent diffusion coefficient (ADC) measurements were performed in phantoms, and six healthy adult volunteers were examined to determine intrasubject (precision) and intersubject (reference range) variations in absolute ADC and relative ADC (rADC) measurements. DW imaging maps and lesion rADC values were also obtained in 34 consecutive stroke patients to evaluate the sensitivity and reliability of DW-interleaved echo-planar imaging for detection of ischemic brain damage.

[Attitude of patients with multiple sclerosis to illness information].

The timing and extent of information which should be given to sufferers from multiple sclerosis (MS) are usually discussed without knowing the patients' opinion. In a pilot study we therefore retrospectively asked 50 patients with relapsing MS (mean age 34 +/- 9 years) for their feelings before and after having been informed about suffering from this disease and in respect to the timing of this information by means of an anonymous questionnaire. The response rate was 86%.

Risk factors for microangiopathy-related cerebral damage in the Austrian stroke prevention study.

Microangiopathy-related cerebral damage (MARCD) represents a common incidental MRI observation in the elderly. The risk factors of such findings are widely unknown. We therefore performed MRI in 349 randomly selected volunteers (ages 50 to 70 years) without neuropsychiatric disease, and evaluated the association of MARCD with conventional and recently suggested cerebrovascular risk factors such as apolipoprotein E genotypes, plasma concentrations of essential antioxidants and anticardiolipin antibody titres.

Pyogenic infectious spondylitis: clinical, laboratory and MRI features.

Pyogenic infectious spondylitis (PIS) is an uncommon but serious inflammatory disorder of the discovertebral junction with frequent involvement of neural structures including the spinal cord. We report a series of 41 patients (age range 21-75 years, mean age 59 years) with primary PIS confirmed by signal abnormality of the intervertebral disk and adjacent vertebral bodies on magnetic resonance imaging. The prevailing clinical symptom was focal back pain aggravated by percussion in 90% of patients.

[Prevalence and risk factors in the population of Graz (Austrian Stroke Prevention Study)].

The Austrian Stroke Prevention Study recruited 1960 randomly selected subjects aged 50 to 75 years during a 3-year period of enrollment. The response rate of the study was 32.4%.

Estrogen replacement therapy in older women: a neuropsychological and brain MRI study.

Objective: To determine if postmenopausal women receiving estrogen perform better on demanding cognitive tests than women without estrogen replacement and if this beneficial effect on cognition is caused by estrogen-related prevention of silent ischemic brain damage.

Design: Cross-sectional study comparing postmenopausal estrogen users and non-users.

Setting: Austrian Stroke Prevention Study.

The relation of cerebral magnetic resonance signal hyperintensities to Alzheimer's disease.

To further elucidate the relation of cerebral magnetic resonance signal hyperintensities to Alzheimer's disease (AD) we performed a case-control comparison between 30 consecutive patients with probable AD (age range 49-76, mean 65 years) and 60 asymptomatic volunteers matched for age, sex, and major cerebrovascular risk factors. We used a 1.5T magnet and determined the extent of morphologic abnormalities both by visual grading and measurement.

Superficial siderosis of the central nervous system: MRI findings and clinical significance.

We reviewed a 7-year series of brain MRI examinations to determine the frequency and clinical significance of superficial siderosis of the central nervous system (SS). SS was defined by widespread bilateral signal loss at the surfaces of the cerebral or cerebellar hemispheres, the brain stem and the spinal cord on T2-weighted images. Clinical data comprised a neurological examination of identified patients and a review of their case notes.

Magnetic resonance imaging correlates of transient cerebral ischemic attacks.

Background And Purpose: MRI of patients with a transient ischemic attack (TIA) may provide more detailed morphological insights than CT. We therefore studied the frequency and type of TIA-related infarcts shown by MRI, examined the utility of intravenous contrast material, and searched for potential predictors of infarct occurrence.

Methods: We performed 1.

MR of cerebral abnormalities concomitant with primary intracerebral hematomas.

Purpose: To determine whether arteriolar vessel wall degeneration in primary intracerebral hematomas might be associated with ischemic brain lesions and clinically silent (apparently intracerebral) previous hemorrhages.

Methods: The MR images of 120 consecutive patients (mean age, 60 years; age range, 22 to 84 years) with their first stroke caused by a primary intracerebral hematoma were reviewed retrospectively for coexisting ischemic damage and previous bleeds.

Results: Early confluent to confluent white matter hyperintensities, lacunes, or infarction were present in 83 (69%) of the patients, and 39 (33%) had had previous hemorrhages consisting of microbleeds or old hematomas.

Magnetic resonance imaging and spectroscopy of progressive cerebral involvement in Kearns Sayre Syndrome.

Kearns Sayre Syndrome (KSS) belongs to the group of so called 'mitochondrial encephalopathies'. Magnetic resonance imaging (MRI) and magnetic resonance spectroscopy (MRS) may have the potential to noninvasively detect and monitor disease specific cerebral involvement, as we wish to demonstrate in a patient whom we have followed for 3.5 years.

[Magnetic resonance tomography of the brain in amyotrophic lateral sclerosis].

We compared the magnetic resonance imaging results of 15 patients suffering from amyotrophic lateral sclerosis (ALS) with those of 30 age-matched controls to search for disease specific cerebral abnormalities. Symmetric hyperintensity along the corticospinal tract on the proton density spin-echo sequence was exclusively found in 4 ALS patients. It was associated with younger age, rapid disease progression and evolution of symptoms starting in the lower extremities.

Brain MRI findings and cognitive impairment in patients undergoing chronic hemodialysis treatment.

Although both morphologic cerebral damage and cognitive dysfunction are known to occur in patients on chronic hemodialysis (CHD) their extent and possible relation have been rarely studied. We therefore performed magnetic resonance imaging of the brain and neuropsychological testing in 30 consecutive CHD patients (mean age 58 years; range 37-69) and in an equal number of asymptomatic volunteers matched for age, sex and major cerebrovascular risk factors. Twenty-four (80%) of the CHD patients were demented according to the criteria of the Diagnostic and Statistical Manual of Mental Disorders IIIR and their mean scores on the Mini Mental State Examination (22.

Magnetic resonance imaging cerebral abnormalities and neuropsychologic test performance in elderly hypertensive subjects. A case-control study.

Objective: To search for a morphologic basis of cognitive impairment possibly associated with arterial hypertension using magnetic resonance imaging and a demanding neuropsychologic test battery.

Design: Case-control comparison with age, length of education, presence of diabetes, and presence of cardiac disease as matching criteria.

Setting: Austrian Stroke Prevention Study.

Magnetic resonance imaging and spectroscopy findings after focal status epilepticus.

The etiology of cerebral abnormalities after focal status epilepticus (SE) is unknown. Possible causes include hypoxia and the excessive release of excitatory amino acids. Magnetic resonance imaging (MRI) of a 21-year-old patient with "cryptogenic" continuous motor seizures showed swelling and signal hyperintensity of the contralateral parietotemporal cortex, the thalamus, and the ipsilateral cerebellum on T2-weighted images.

Anticardiolipin antibodies in normal subjects. Neuropsychological correlates and MRI findings.

Background And Purpose: Our aim was to assess the association of elevated anticardiolipin antibody (aCL) titers with silent brain damage and cognitive functioning in middle-aged and elderly normal subjects.

Methods: We determined the IgM and IgG aCL titers from 233 randomly selected clinically normal participants of a population-based stroke prevention study (age range, 44 to 82 years). aCL titers were categorized into negative (0 to 10 U/L), low positive (10 to 20 U/L), and moderately high positive (> 20 U/L).

White matter lesions on magnetic resonance imaging in a healthy elderly population: Correlations to vascular risk factors and carotid atherosclerosis.

We performed magnetic resonance imaging (MRI) and extensive risk factor evaluation on 196 randomly selected, clinically asymptomatic participants of the Austrian Stroke Prevention Study to determine the clinical correlates of MRI white matter lesions (WML) in normals. WML were present in 103 (52.6%) individuals.