Krzysztof Słotwiński

Parry–Romberg syndrome: clinical, electrophysiological and neuroimaging correlations

Parry–Romberg syndrome (PRS) is a rare disorder, described in the nineteenth century by Caleb Parry and Moritz Romberg, characterized by acquired and slowly progressive atrophy of one side of the face. The pathogenesis of PRS is still unclear. Immune-mediated processes are thought to be a basic factor in PRS etiology, but autonomic nervous system might also be impaired. A case of PRS in a 26-year-old woman with coexisting disturbances in the lower left limb is presented. The multimodal electrophysiological studies were done, including electroencephalography, visual, brain auditory, somatosensory and trigeminal somatosensory evoked potentials, blink reflex, standard neurographic and electromyographic examinations, quantitative sensory tests and autonomic tests. Neuroimaging studies consisted of brain MR, single voxel proton MR spectroscopy, diffusion tensor imaging with fiber tractography. Based on multimodal electrophysiological and neuroimaging studies, it was concluded that the impairment in PRS is multisystemic, i.e., motor, sensory, and autonomic. A cortical origin of the symptoms is possible.

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.

The possible meaning of fractional anisotropy measurement of the cervical spinal cord in correct diagnosis of amyotrophic lateral sclerosis

Diagnosis of amyotrophic lateral sclerosis (ALS) is based on clinical criteria and electrophysiological tests (electromyography, and transcranial magnetic stimulation). In the search for ALS biomarkers, the role of imaging procedures is currently emphasized, especially modern MR techniques. MR procedures were performed on 15 ALS patients and a sex- and age-matched control group. The MR examinations were performed with a 1.5-T MR unit, and the protocol consisted of sagittal T1-weighed images, sagittal and axial T2-weighed images, and sagittal T2-weighed FAT SAT images followed by an axial diffusion tensor imaging (DTI) sequence of the cervical spinal cord. FA values in individual segments of the cervical spinal cord were decreased in the ALS group in comparison with the control group. After comparing FA values for anterior, posterior, and lateral corticospinal columns, the greatest difference was observed between the C2 and C5 segments. Spinal cord assessment with the use of FA measurements allows for confirmation of the motor pathways lesion in ALS patients. The method, together with clinical criteria, could be helpful in ALS diagnosis, assessment of clinical course, or even the effects of new drugs. The results also confirmed the theory of the generalized character of ALS.

Profile of autonomic dysfunctions in patients with primary brain tumor and possible autoimmunity

OBJECTIVE Cerebral lesion due to different neurological conditions could be complicated by autonomic dysfunction, reported in the literature as a sympathetic hyperactivity. The mechanisms of dysautonomia still remains partial. The aim of the study was to assess the profile of autonomic dysfunction in patient with primary brain tumors, with attempt to estimate the additional factors in pathogenesis of dysautonomia. MATERIAL AND METHODS Neurological examinations, the Lows autonomic disorder questionnaire, electrophysiological autonomic tests (Heart Rate Variability test at rest and during deep breathing, spectral analysis of R-R intervals, sympathetic skin response test), studies of peripheral nerves, blood sampling collection for antibodies were done in 33 patients with recognized primary brain tumors. RESULTS The averaged Lows Questionnaire score in the patients group was significantly higher than in the controls, systolic blood pressure was increased, heart rate tended to be higher without significance, but heart rate variability was severe low, LF/HF ratio also tended to be higher in the patients group. In SSR test the amplitude of responses from hand and foot was significantly lower without changes in their latencies. We found changes in the electrophysiological tests of peripheral nerves, and positive anti-neural antibodies in 5 patients. CONCLUSIONS The results of the study indicated to the sympathetic nervous system hyperactivity in patients with primary brain tumors. Local brain lesion with high intracranial pressure, additional peripheral nerve damage probably in the course of autoimmunity, and direct influence of autoimmunity to the central part of autonomic nervous system are possible in the pathogenesis of dysautonomia.

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

ObjectiveThe influence of cerebral atrophy and leukoaraiosis (LA) on the degree and profile of cognitive impairment remains unclear.DesignThe 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.SettingDepartment of Neurology, University of Medicine.Patients and participantsForty-two patients with LA and/or cerebral atrophy and twenty controls.Measurements and resultsNeuropsychological 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.ConclusionsCerebral 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.

Is peripheral paraneoplastic neurological syndrome possible in primary brain tumors

Systemic malignant diseases cause the induction of autoimmunity, for example, paraneoplastic syndromes. There are no proofs of paraneoplastic syndromes in primary brain tumors. The aim of the study was to evaluate the involvement of the peripheral nervous system, together with an assessment of onconeuronal and antineural antibodies as indicators of humoral immune response against nervous system in patients with primary brain tumors.

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.

Clinical and nutritional correlations in Parkinson's disease: Preliminary report.

BACKGROUND Parkinsons disease (PD) is one of the most common neurodegenerative disorders. Malnutrition is an essential problem in the late stage of PD. Lowering of body mass is seen in 30% of patients, and malnutrition or the risk of malnutrition in 24% and 60%, respectively. OBJECTIVES The aim of the study was an analysis of the relationships between the parameters of the nutritional stage and the advancement of clinical symptoms in PD patients. MATERIAL AND METHODS A total of 40 patients (18 men, 22 women) with PD were analyzed, mean age: 70.7 years. In all patients, structured anamnesis, Nutrition Risk Screening (NRS 2002), body mass index (BMI), morphology, and basic biochemical tests were conducted. Skin fold thickness was also measured. The results were referred to the score of different scales. RESULTS The study showed a lot of correlations between the severity of PD, mostly motor symptoms in the Unified Parkinsons Disease Rating Scale (UPDRS), and parameters characterizing the patients nutritional status. We revealed a correlation between malnutrition and PD duration, and l-DOPA frequency intake. The global score of parts I, II and III of UPDRS were correlated with the nutritional status. The results confirmed the existence of more severe smell change and taste impairment in the late stage of PD, with more pronounced malnutrition. CONCLUSIONS The duration of PD, motor and non-motor PD symptoms, and the frequency of l-DOPA intake closely correlate with the nutritional status. Understanding of the multifactorial interdependence might be useful in the estimation of the algorithm for monitoring the nutritional status of PD patients and taking early nutritional intervention.

Cognitive impairment, event-related potentialsand immunological status in patientswith systemic lupus erythematosus

BACKGROUND Cognitive impairment (CI) is a frequent problem in lupus patients, regardless of their overt neuropsychiatric (NP) involvement. OBJECTIVES The aim of our study was to test cognitive abilities in systemic lupus erythematosus (SLE) patients by means of neuropsychological testing and event-related potentials (ERPs), and to search for their cognitive abilities correlations with a wide range of auto-antibodies. MATERIAL AND METHODS A total of 37 SLE patients were subjected to a battery of neuropsychological tests, recommended by the American College of Rheumatology (ACR), and to ERPs. They were also tested for a wide range of auto-antibodies (anti-cardiolipin (aCL), anti-β2-glycoprotein I (anti-β2-GPI), lupus anticoagulant, anti-dsDNA, anti-nucleosome, anti-ribosomal P (anti-Rib-P), anti-ganglioside, anti-Ro/SS-A, and anti-La/SS-B. RESULTS Cognitive impairment was found in 35% of patients, mostly with NP SLE (NPSLE), and was associated with higher disease activity, measured by the SLE Disease Activity Index (SLEDAI), and with a longer duration of central nervous system (CNS) involvement. There were no differences in the immunological status between CI patients and those without cognitive decline, but some antibodies were correlated with worse results in certain neuropsychological tests (anti-dsDNA and worse results of Rey Complex Figure Test - RCFTc for copying and RCFTr for recall, and of verbal fluency test (VFT); aCL IgG and worse results in Digit Span (DS) and in RCFTc). Event-related potentials showed prolonged N200 and P300 latencies in SLE patients in comparison to controls, but no differences were found between SLE and NPSLE patients. Mean P300 latency was significantly longer in patients without anti-nucleosome antibodies. CONCLUSIONS Event-related potentials can be used as a complementary tool in assessing CI in SLE patients. The immunological status of patients with CI did not differ from that of patients without cognitive problems.

Symptoms of degeneration of the pyramidal tracts in conventional magnetic resonance imaging and diffusion tensor imaging in a young woman with primary lateral sclerosis

Primary lateral sclerosis (PLS) is one of the forms of motor neuron disease (MND), affecting only upper motor neurons. The diagnosis of PLS should be made on different diagnostic criteria, for example, Pringle or Gordon, but it is usually a diagnosis of exclusion. There are no characteristic findings in standard laboratory and electrophysiological assessment. We present details of a 31-year-old woman who had suffered from progressive paraparesis with right-side predominance. Conventional MRI of brain and spinal cord and diffusion tensor imaging (DTI) studies showed Hyperintense lesions in the upper part of the cervical spinal cord at the level C1 in lateral funicules, in the medulla oblongata at the pyramidal decussation and in the midbrain. Brain DTI revealed changes along the corticospinal tracts on fractional anisotropy (FA) maps. MRI of the thoracic spinal cord showed in T2-weighted images hyperintensive regions in the course of the lateral corticospinal tracts. This aided in PLS recognition.