Multimodal Evoked Potentials as Potential Biomarkers of Disease Activity in Patients With Clinically Isolated Syndrome.

OBJECTIVE: There is an ongoing search for markers useful in monitoring and predicting disease activity at the early stage of multiple sclerosis (MS). The goals of this study were to prospectively evaluate the changes in parameters of multimodal evoked potentials (EP) and cognition within a 3-year follow-up period in patients with clinically isolated syndrome (CIS), and to assess the prognostic value of baseline findings with regard to the disease outcomes. METHODS: In 29 patients (20 women, nine men, mean age 31.1) multimodal (visual, brainstem auditory, somatosensory, event-related) EP and neuropsychological tests (NT) were performed at baseline (T0) and after 1 (T1) and 3 (T3) years. Their results were compared longitudinally between baseline, T1, and T3. Baseline results confirmed conversion of CIS into multiple sclerosis (MS) and disability level at T1 and T3 using multiple comparisons and a logistic regression model. RESULTS: Apart from mean N13/P16 SEP (somatosensory evoked potentials) amplitude (lower at T1 and T3 than at baseline (T0 1.02 ± 0.37 µV, T1 0.90 ± 0.26 µV, T3 0.74 ± 0.32 µV, p < 0.05 for both comparisons), no significant changes of EP or NT parameters were found in longitudinal assessment. Baseline P300 Pz latency was longer for the patients with MS than for those with CIS at T1 (352.69 vs. 325.56 ms). No predictive value was shown for any of the analyzed baseline variables with regard to conversion from CIS into MS. SIGNIFICANCE: Baseline ERP abnormalities were associated with their short-term conversion into MS. ERP are worth considering in multimodal EP evaluation at the early stage of MS.

Multiple fusiform cerebral aneurysms detected after atrial myxoma resection: a report of two cases.

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Cognitive performance, fatigue and event-related potentials in patients with clinically isolated syndrome.

OBJECTIVES: Cognitive impairment and fatigue are regarded as important aspects of multiple sclerosis. The aim of this study was to evaluate cognitive performance, the level of fatigue and parameters of event-related potentials (ERP) in patients diagnosed with clinically isolated syndrome (CIS). PATIENTS AND METHODS: The study comprised 44 patients with CIS and 45 healthy controls. Cognitive performance was assessed using the Brief Repeatable Battery of Neuropsychological Tests (BRBNT), fatigue - using the Fatigue Severity Scale (FSS) and Modified Fatigue Impact Scale (MFIS). Auditory ERP were performed and the parameters of N200 and P300 components were analyzed. Neuropsychological and electrophysiological measures were referred to clinical and radiological features of the disease activity. RESULTS: Forty five% of patients failed in at least one test from BRBNT, mainly within the domains of memory and attention. In 18% of patients FSS corresponded with moderate or severe fatigue. The mean latency of N200 and P300 was significantly longer and amplitude of P300 was lower in those patients with CIS than in the controls. Significant correlations were found between the results of MFIS and tests evaluating verbal memory and attention, as well as between N200 latency and results of tests for verbal memory. CONCLUSIONS: Cognitive performance and fatigue deserve attention from the earliest clinical stage of MS. Abnormalities of event-related potentials in CIS suggest early impact of the disease on functional neural networks.

Event-related potentials and cognitive performance in multiple sclerosis patients with fatigue.

The aim of this study was to evaluate event-related potentials (ERP) and cognition in multiple sclerosis (MS) patients with regard to fatigue and disease-related variables. The study comprised 86 MS patients and 40 controls. Fatigue was assessed using the Fatigue Severity Scale (FSS/FSS-5) and the Modified Fatigue Impact Scale (MFIS/MFISmod). N200 and P300 components of auditory ERP were analyzed. Cognition was evaluated by means of Brief Repeatable Battery of Neuropsychological Tests (BRBNT). The results of ERP and BRBNT were compared between non-fatigued, moderately and severely fatigued MS patients and controls. P300 latency was significantly longer in the whole MS group and in the fatigued patients than in the controls. A positive correlation was found between P300 latency and MFIS/MFISmod results, independent from age and MS-related variables. The fatigued patients scored less than non-fatigued ones in tests evaluating memory, visuomotor abilities and attention. Results of these tests correlated significantly with fatigue measures, independently from MS-related variables. Fatigue in MS patients showed significant relationships with impairment within the memory and attention domains. Parameters of auditory ERP, as electrophysiological biomarkers of cognitive performance, were not independently linked to fatigue.

Acute hyperkinetic syndrome due to ephedrone abuse.

OBJECTIVES: A new form of manganese poisoning is related to the intravenous use of self-prepared methcathinone hydrochloride (ephedrone). Manganese encephalopathy typically manifests as a levodopa-resistant parkinsonism. MAIN POINTS: A 32-year-old drug-addicted man with acute gait disturbances after the ephedrone injections was presented. Choreic movements, severe postural instability, and "cock-walk" gait were observed. Magnetic resonance imaging T1 images showed high signal intensity of white matter in the basal ganglia and pituitary gland, and T2 images showed decreased signal mostly of globus pallidus with decreased N-acetylaspartate and choline levels in MR spectroscopy. CONCLUSIONS: The unusual pattern of MR imaging may explain the unusual clinical symptoms with dominant hyperkinetic syndrome.

Magnetic resonance spectroscopy findings as related to fatigue and cognitive performance in multiple sclerosis patients with mild disability.

BACKGROUND: The origin of fatigue in multiple sclerosis (MS) remains unclear. Magnetic resonance spectroscopy (MRS) provides an insight into metabolic properties of the brain. OBJECTIVES: The aim of the study was to evaluate brain MRS measurements in MS patients, with regard to fatigue and cognition. METHODS: The study comprised 32 MS patients and 43 controls. Fatigue was assessed using the Fatigue Severity Scale (FSS) and the Modified Fatigue Impact Scale (MFIS) and cognition - using the Brief Repeatable Battery of Neuropsychological Tests (parts of BRBNT). MRS voxels were placed in the parietal white matter (PWM) and the posterior cingulate gyrus (PCG); N-acetyl-aspartate (NAA), choline (Cho) and myoinositol (mI) to creatine (Cr) ratios were determined. Relationships were searched between MRS measurements and fatigue as well as BRBNT results. RESULTS: MS patients in comparison with controls showed decreased NAA/Cr and increased mI/Cr ratios in PCG and PWM, respectively. No significant relationships between MRS parameters and fatigue measures, BRBNT results or MS-related variables were found. CONCLUSIONS: The decrease of NAA and increase of mI within white and gray matters in MS patients do not show a significant relationship with cognitive performance or fatigue.

[Acute idiopathic longitudinally extensive transverse myelitis with peripheral axonal motor neuropathy]. / Ostre idiopatyczne podluzne zapalenie rdzenia kregowego z towarzyszaca aksonalna neuropatia ruchowa.

Acute longitudinally extensive transverse myelitis (LETM) is a rare disease of autoimmune, connective tissue or infectious etiology (bacterial or viral) in which spinal cord lesion extends vertically over three or more vertebral segments. The most common cause of LETM is neuromyelitis optica (Devic's syndrome). It's typically clinically characterized by a tetraparesis or paraparesis of the lower extremities, superficial and deep sensation disturbances and sphincter dysfunction. Differential diagnosis is complex and based on immunological, serological, biochemical tests. Authors present 37-year-old healthy woman with sudden onset of flaccid tetraplegia preceded by upper limbs paresthesia. MRI examination of cervical and thoracic part of the spinal cord revealed extensive, multiple hyperintensive lesions extending from C3 up toTh9. In electroneurography examination coexistent severe peripheral axonal motor neuropathy was diagnosed. Despite performed profound tests the etiology of LETM in our patient remained unknown.

[Ballism as a rare form of hyperkinetic movement disorder]. / Ruchy balistyczne jako rzadka postac pozapiramidowych zaburzen ruchowych.

Hemiballism is a very rare movement disorderthat presents with involuntary unilateral flinging movements of the limbs. The movements are often violent and have wide amplitudes of motion. Hemichorea and hemiballism share pathophysiology and etiology, and commonly coexist in the same patient. Hemiballism was recognised as almost pathognomonic of a lesion in the contralateral subthalamic nucleus (body of Luys). Cerebrovascular diseases remain the most common etiology of ballistic movements. Interestingly, nowadays nonketotic hyperglycemiain elderly patients with diabetes mellitus is considered to be the second most common reported cause. Other more rare causes of hemiballism include encephalitis, cerebral toxoplasmosis (AIDS), multiple sclerosis, brain tumor (primary, metastatic) and metabolic disturbances. Prognosis is favorable for most patients with spontaneous resolution of clinical symptoms and dopamine receptor blocking drugs are highly effective.

Neuropsychological testing and event-related potentials in the assessment of cognitive performance in the patients with multiple sclerosis--a pilot study.

AIMS: The aim of the study was to evaluate cognitive impairment in multiple sclerosis (MS) patients using neuropsychological testing (NT) and auditory event-related potentials (ERPs) with reference to clinical variables, with an attempt to re-assess NT and ERP results after a year. METHODS: The study comprised 21 patients with MS. ERPs results were compared to age-matched controls. Correlations were searched among ERPs and NT results, duration of MS and disability. NT and ERPs were repeated after a year and their results were compared with the initial ones. RESULTS: In NT, 90-100% of patients showed impaired memory and attention. Latencies of ERPs were prolonged in patients compared with controls. NT results correlated with clinical variables and N2 parameters. Results of NT, but not ERPs, improved after a year. CONCLUSIONS: MS patients present with moderate cognitive impairment and ERP abnormalities, with dysfunction of subcortical-prefrontal circuit as their possible background. NT are more useful than ERP in monitoring cognitive performance in MS patients.

Mild cognitive impairment and event-related potentials in patients with cerebral atrophy and leukoaraiosis.

OBJECTIVE: The influence of cerebral atrophy and leukoaraiosis (LA) on the degree and profile of cognitive impairment remains unclear. DESIGN: The aim of the study was to assess neuropsychological features of cognitive performance and parameters of event-related potentials (ERP) in subjects with generalised cerebral atrophy and LA. SETTING: Department of Neurology, University of Medicine. PATIENTS AND PARTICIPANTS: Forty-two patients with LA and/or cerebral atrophy and twenty controls. MEASUREMENTS AND RESULTS: Neuropsychological testing (NT) included Mini Mental State Examination (MMSE), Auditory Verbal Learning Test (AVLT) and Trail Making Test (TMT). Auditory ERPs were performed and parameters of the N2 and P3 components were compared in the patients and controls. Relationships were analysed between radiological indices of atrophy and LA, and NT and ERP results. Results of NT suggested generalised mild cognitive impairment in all the patients. P3 and N2 latencies were longer in the patients than in controls, especially in the LA subgroup. Correlations were found for indices of atrophy, AVLT and ERP parameters. There was a predominant influence of age upon ERP parameters and radiological indices. CONCLUSIONS: Cerebral atrophy and LA result in deficits in memory and attention. NT and ERP may be used as complementary methods in the assessment of cognitive impairment in patients with cerebral atrophy and LA.

[Epilepsy in the elderly people]. / Padaczka u osób w podeszlym wieku.

Recently an increasing number of cases of epilepsy revealed after 60 years of age have been noted. Epilepsy in the elderly may cause some diagnostic and therapeutic difficulties, it constitutes social problems as well. The authors present current epidemiological data, discuss pathogenesis of seizures, clinical course and prognosis, rules of differential diagnosis and treatment, highlighting specific features of the disease in this age group.

[Tau-pathies: neurodegenerative diseases with pathology involving tau protein]. / Taupatie--choroby zwyrodnieniowe osrodkowego ukladu nerwowego zwiazane z patologia bialka tau.

Microtubule-associated protein tau (MAP-tau) plays an important role in stabilizing neuronal structure, in axonal transport, regulating cellular division, and apoptotic processes. In recent years the structure and functions of MAP-tau, as well as its physiological changes and pathology, have been extensively studied. Degenerative diseases of the central nervous system having MAP-tau involved in their pathogenesis are called tau-pathies. This group includes Alzheimer's disease, frontotemporal dementia, progressive supranuclear palsy, and corticobasal degeneration. The authors present the clinical pictures of these diseases (with the dominant features of dementia), and current possibilities in their diagnosis and treatment. MAP-tau as a marker for neurodegenerative process is already used in the diagnosis of tau-pathies. Further studies on its pathology may also give rise to some new therapeutic strategies.

Epilepsy in middle-aged and elderly people: a three-year observation.

An analysis of the medical documentation and investigation of 130 cases of epilepsy diagnosed in a group of people over 50 years of age (average: 65.4 years) revealed that the most common type of seizure in the group studied was partial (66.2%), followed by seizures with secondary generalization (33.8%). Epilepsy was caused by cerebrovascular disease (50.8%) considerably more often in patients over 74 years of age, craniocerebral trauma in patients addicted to alcohol (13.1%), especially those under 65 years of age, primary or metastatic neoplastic disease (10.7%), and others. The authors wish to draw attention to the leukoaraiosis factor, which might be the proepileptogenic cause of epilepsy recognized in the group of patients over 74 years of age (56.5%) and is much more frequent in this group than in the group of patients under 65 years of age (1.6%). Moreover, some drugs, such as L-dopa and Baclofen, might have been related to the epileptic seizures. In 29 patients (22.3%) the definite cause of late-onset epilepsy was unknown. The authors suggest in such cases, both follow-up tomographic examination and careful clinical examinations. In the study group of patients with initially unknown seizure etiology, some diseases, such as cerebral tumor or colon and pancreatic neoplasm, were diagnosed during follow-up examination. These processes were revealed several months after the first epileptic seizure.