The usefulness of diffusion-weighted/fluid-attenuated inversion recovery imaging in the diagnostics and timing of lacunar and nonlacunar stroke.

INTRODUCTION: The DWI/FLAIR mismatch is a potential radiological marker for the timing of stroke onset. The aim of the study was to assess if the DWI/FLAIR mismatch can help to identify patients with both lacunar and nonlacunar acute ischemic stroke within 4.5 h of onset. METHODS: A retrospective study was performed in which the authors analysed data from 86 ischemic lacunar and nonlacunar stroke patients with a known time of symptom onset, imaged within the first 24 h from stroke onset (36 patients <4.5 h, 14 patients 4.5-6 h, 15 patients 6-12 h, and 21 patients 12-24 h). Patients underwent the admission CT and MR scan. The presence of lesions was assessed in correlation with the duration of the stroke. RESULTS: The time from stroke onset to neuroimaging was significantly shorter in patients with an ischemic lesion visible only in the DWI (mean 2.78 h, n = 24) as compared to patients with signs of ischemia also in other modalities (mean 8.6 h, n = 62) (p = 0.0001, Kruskal-Wallis ANOVA). The DWI/FLAIR mismatch was characterised by a global sensitivity of 58%, specificity 94%, PPV 87.5%, and NPV 76% in identifying patients in the 4.5 h thrombolysis time window. For lacunar strokes (n = 20), these parameters were as follows: sensitivity 50%, specificity 92.8%, PPV 75 %, and NPV 81.2%. CONCLUSIONS: The presence of acute ischemic lesions only in DWI can help to identify both lacunar and nonlacunar stroke patients who are in the 4.5 h time window for intravenous thrombolysis with high specificity.

MRI brain findings in ephedrone encephalopathy associated with manganese abuse: Single-center perspective.

BACKGROUND: Manganese (Mn) is a well-known toxic agent causing symptoms of parkinsonism in employees of certain branches of industry. Home production of a psychostimulant ephedrone (methcathinone), involving the use of potassium permanganate, became a new cause of intoxications in Poland. CASE REPORT: This article presents clinical symptoms, initial brain MRI findings and characteristics of changes observed in follow-up examinations in 4 patients with manganese intoxication associated with intravenous administration of ephedrone. All patients in our case series presented symptoms of parkinsonism. T1-WI MRI revealed high intensity signal in globi pallidi in all patients; hyperintense lesions in midbrain were observed in three patients, while lesions located in cerebellar hemispheres and pituitary gland in just one patient. The reduction of signal intensity in the affected brain structures was observed in follow-up studies, with no significant improvement in clinical symptoms. CONCLUSIONS: Brain MRI is helpful in the assessment of distribution as well as dynamics of changes in ephedrone encephalopathy. Regression of signal intensity changes visible in brain MRI is not associated with clinical condition improvement. Although brain MRI findings are not characteristic for ephedrone encephalopathy, they may contribute to diagnosing this condition.

[Encephalopathy caused by intravenous potassium permanganate used for illegal production of methcathinone (ephedrone) from medicines containing pseudoephedrine]. / Encefalopatia spowodowane dozylnym uzywaniem preparatów zawierajacych nadmanganian potasu stosowany jako reagent w produkcji metkatynonu (efedronu) z leków zawierajacych pseudoefedryne.

Encephalopathy caused by manganese compounds used for illicit production of ephedrone (methcathinone) is described. The onset of disease could be observed after some months of regular intravenous use of ephedrone contaminated with manganese. In clinical picture dominate neurological signs and symptoms, mainly extrapyramidal syndromes: parkinsonism, tremor, muscle distonia, pro- and retropulsion. Some other symptoms may be observed: hypophonia or dysarthria, gain disturbances, impairment of precise movement, and micrographia. In cranial NMR often appears bilaterally an increase of an intensity of T1 signal in globus pallidus and in some other brain structures. Elimination of manganese with the use of chelating therapy as well as symptomatic treatment, mainly with the antyparkinsonic drugs, seems to be ineffective.

Variants of cerebral arteries - anterior circulation.

Advances in imaging techniques allow for in vivo identification of abnormalities and normal variants of cerebral arteries. These arterial variations can be asymptomatic and uncomplicated although, some of them increase the risk of aneurysm formation, acute intracranial hemorrhage, play a vital role in neurosurgical planning or can be misidentified as serious pathology and medical errors. The goal of this publication is to discuss arterial anomalies of anterior cerebral circulation, their prevalence and demonstrate radiological images of some of those variants. In this article we will discuss variants of internal carotid artery, anterior cerebral artery, anterior communicating artery, middle cerebral artery, persistent stapedial artery and fenestration.

[Spinocerebellar ataxias type 1 and 2: comparison of clinical, electrophysiological and magnetic resonance evaluation]. / Ataksja rdzeniowo-mózdzkowa typu 1 i 2 - porównanie oceny klinicznej, elektrofizjologicznej i rezonansu magnetycznego.

BACKGROUND AND PURPOSE: Spinocerebellar ataxias type 1 (SCA1) and type 2 (SCA2) belong to neurodegenerative disorders of autosomal dominant inheritance, genetically and clinically heterogeneous, caused by the expansion of CAG trinucleotides. Trunk and limb ataxia, dysarthria, dysphagia, gaze palsy, sensory and motor axonal neuropathy are the dominant features in both entities. The aim of the study was to evaluate the differences between genotype and phenotype based on clinical and electrophysiological assessment of the visual, auditory pathways, and EEG alterations in comparison with the cerebellar and brain atrophy in MRI. MATERIAL AND METHODS: 44 patients with SCA1 and 24 cases with SCA2 confirmed molecularly were examined neurologically and using the International Cooperative Ataxia Rating Scale (ICARS). A correlation of clinical symptoms and signs, and CAG repeat numbers with EEG, visual (VEP) and brainstem auditory (BAEP) evoked potentials, and MRI alterations were evaluated. RESULTS: A statistically significant negative correlation between the age of disease onset and number of CAG repeats in both types of SCA was found. Examined patients with SCA2 were younger, with longer disease duration and more pronounced cerebellar and brain atrophy in MRI. We found a significant correlation between ICARS and CAG repeats in this group. The dysphagia, pyramidal tract involvement and depressive reaction were significantly frequent in SCA1 patients. However in SCA2 patients, the peripheral nerve damage and extrapyramidal signs were more prominent. The amplitude of P100 visual evoked potentials was significantly lower in SCA1 patients and negatively correlated with CAG repeats. CONCLUSIONS: These results provide further evidence for the phenotypic differences of genetically defined SCA1 and SCA2 patients, expressed by more frequent involvement of the pyramidal tract and depression reaction in SCA1, in contrast to peripheral nerve involvement and extrapyramidal signs in the clinical feature of SCA2 phenotype. Furthermore, atrophy of the brain and cerebellum revealed in MRI was more pronounced than electrophysiological functional alterations, especially in SCA2. The decreased amplitude of P100 VEP in SCA1 patients was the only electrophysiological parameter differentiating between both groups of patients.

[Progressive atrophy of the globus pallidus--case report]. / Postepujacy zanik galki bladej--opis przypadku.

The authors report a case of atrophy of the globus pallidus in a woman aged 25 years, diagnosed alive. The diagnosis was based to a large extent on MRI findings. Atrophy of the globus pallidus (AGP) is a rare disease, recognized mostly in neuropathological examination. Its etiopathogenesis has not been explained so far. Since no specific abnormalities have been detected in laboratory tests, the clinical diagnosis of AGP is only probable. However, AGP should be suspected if such extrapyramidal symptoms are present as dystonia, choreoathetosis, muscular rigidity, and characteristic localisation of lesions in MRI. At present only comprehensive symptomatic treatment is possible.

[Neural listeriosis presenting as leptomeningitis: a case report]. / Neurolisterioza przebiegajaca pod postacia ropnego zapalenia opon mózgowo-rdzeniowych--przypadek kliniczny.

The Listeria monocytogenes infection is rare and difficult to diagnosis. However, it is associated with a high mortality in adults. A case is presented of a 78-year-old woman with an immunological deficit following viral infection (herpes zoster).

[Clinical and genetic study of juvenile form of Huntington's disease]. / Badania kliniczne i genetyczne w mlodzienczej postaci choroby Huntingtona.

Huntington's disease (HD) is an autosomal dominant neurodegenerative disorder caused by high instability and extension of CAG sequences within the coding region of IT15 gene. It affects both sexes and age at onset of the disease may be different but usually occurs in midlife. The term Juvenile Huntington's disease is generally applied to 10% of the cases with onset before 20. We present clinical features and results of DNA analysis in 16 patients from 14 families aged 9 to 36. The age of onset was between 5 to 20 years; duration of the disease was from 2 to 16 years. In 10 cases the mutated gene was transmitted by the affected father; only in two cases by the mother. In all cases anticipation manifested by earlier onset of the disease in subsequent generations and expansion of CAG repeats was documented. The number of CAG repeats was between 50 and 92 (mean 67.3). Progressive mental deterioration, declining school performance, hyperactivity and emotional disturbances were the first symptoms of juvenile HD. Neuropsychological assessment showed mean IQ in Wechsler test 59.6 and Mini-Mental State Examination scores 22.8. Rigidity and bradykinesia were predominant features in the cases with juvenile onset, the remaining ones developed choreatic movements. Three persons had epileptic seizures; two (both females) revealed behaviour and psychiatric disturbances. Amplitudes of somatosensory evoked potentials, visual evoked potentials and brainstem auditory evoked potentials were markedly reduced. MRI of the brain showed atrophy of heads of the caudate nuclei, putamen and globus pallidus.

Training in rapid auditory processing ameliorates auditory comprehension in aphasic patients: a randomized controlled pilot study.

Experimental studies have often reported close associations between rapid auditory processing and language competency. The present study was aimed at improving auditory comprehension in aphasic patients following specific training in the perception of temporal order (TO) of events. We tested 18 aphasic patients showing both comprehension and TO perception deficits. Auditory comprehension was assessed by the Token Test, phonemic awareness and Voice-Onset-Time Test. The TO perception was assessed using auditory Temporal-Order-Threshold, defined as the shortest interval between two consecutive stimuli, necessary to report correctly their before-after relation. Aphasic patients participated in eight 45-minute sessions of either specific temporal training (TT, n=11) aimed to improve sequencing abilities, or control non-temporal training (NT, n=7) focussed on volume discrimination. The TT yielded improved TO perception; moreover, a transfer of improvement was observed from the time domain to the language domain, which was untrained during the training. The NT did not improve either the TO perception or comprehension in any language test. These results are in agreement with previous literature studies which proved ameliorated language competency following the TT in language-learning-impaired or dyslexic children. Our results indicated for the first time such benefits also in aphasic patients.

Correlations between cerebellar and brain volumes, cognitive impairments, ApoE levels, and APOE genotypes in patients with AD and MCI.

Due to the increasing incidence of Alzheimer's disease (AD), many studies have aimed to improve its diagnosis. Particular attention has been focused on measuring volumes of brain structures. Only few studies have investigated whether the cerebellar volume changes with the stage of dementia. It is controversial whether the serum apolipoprotein E (ApoE) level is an appropriate AD marker. This study was designed to clarify the significance of both cerebellar volume measurements and ApoE level measurements as markers of neurodegenerative changes. This study included 55 subjects with AD, 30 subjects with mild cognitive impairments (MCI), and a control group with 30 subjects. We measured the brain, cerebellum, and brain stem volumes with magnetic resonance imaging (MRI). We determined serum ApoE levels, APOE genotypes, and neuropsychological test scores. In the control group, we found that ApoE levels were significantly higher for subjects with the APOE 2/3 genotype than those with the 4/4 genotype. This finding may indicate that ApoE plays a protective role against AD development in subjects with the APOE 2/3 genotype. ApoE levels were not significantly different in patients with AD and MCI. No correlations were found between serum ApoE levels and Mini-Mental State Examination (MMSE) scores or the volumes of brain structures. This study could not confirm the appropriateness of the cerebellum volume as an early AD marker. Correlations were found between cerebellar volume, brain volume, and the MMSE scores.

Eight-year survival and continuation of therapy in a patient suffering from prostate cancer with metastases and pathological fractures of vertebrae.

Prostate cancer (PCa) is a major health problem and one of the main causes of cancer mortality in men [1]. In patients with PCa, bone metastases manifest in 100% of patients when the PSA level exceeds 100 ng/ml causing pains and posing a risk for pathological fractures [2]. We report a case of a 70-year-old male with PCa and pathological fractures of the vertebrae, in whom we observed long-term regression and an 8-year-survival while undergoing continuous therapy. As far as we know, this is the first reported case in literature with such an unexpected outcome.

Cognitive functioning in neurologically symptomatic and asymptomatic forms of Wilson's disease.

We sought to determine the pattern of cognitive deficits in patients with Wilson's disease (WD) with different type and degree of neurological involvement, and to interpret the findings in relation to the underlying pathology. A total of 67 WD patients were examined with a neuropsychological test battery assessing different aspects of cognitive processing. The patients were subdivided into three groups: neurologically asymptomatic, neurological with pure basal ganglia lesions, and neurological with more extensive pathology. The results were compared with 50 matched healthy controls. Patients with a neurological form of WD showed a mild but definitive impairment in all cognitive functions. In contrast, the neurologically asymptomatic patients showed no deficits when compared with normal controls. Multifocal pathology was associated with more severe cognitive deficits than selective basal ganglia lesions but did not contribute significantly to memory impairment. A range of cognitive functions, including frontal-executive ability, aspects of memory and visuospatial processing, are affected in the neurologically symptomatic WD patients. In contrast, no subliminal deficits were observed in the asymptomatic patients. The lesions of the basal ganglia seem to be of central importance in explaining the symptomatology.