The value of lung ultrasound in COVID-19 pneumonia, verified by high resolution computed tomography assessed by artificial intelligence.

BACKGROUND: Lung ultrasound (LUS) is an increasingly popular imaging method in clinical practice. It became particularly important during the COVID-19 pandemic due to its mobility and ease of use compared to high-resolution computed tomography (HRCT). The objective of this study was to assess the value of LUS in quantifying the degree of lung involvement and in discrimination of lesion types in the course of COVID-19 pneumonia as compared to HRCT analyzed by the artificial intelligence (AI). METHODS: This was a prospective observational study including adult patients hospitalized due to COVID-19 in whom initial HRCT and LUS were performed with an interval < 72 h. HRCT assessment was performed automatically by AI. We evaluated the correlations between the inflammation volume assessed both in LUS and HRCT, between LUS results and the HRCT structure of inflammation, and between LUS and the laboratory markers of inflammation. Additionally we compared the LUS results in subgroups depending on the respiratory failure throughout the hospitalization. RESULTS: Study group comprised 65 patients, median 63 years old. For both lungs, the median LUS score was 19 (IQR-interquartile range 11-24) and the median CT score was 22 (IQR 16-26). Strong correlations were found between LUS and CT scores (for both lungs r = 0.75), and between LUS score and percentage inflammation volume (PIV) (r = 0.69). The correlations remained significant, if weakened, for individual lung lobes. The correlations between LUS score and the value of the percentage consolidation volume (PCV) divided by percentage ground glass volume (PGV), were weak or not significant. We found significant correlation between LUS score and C-reactive protein (r = 0.55), and between LUS score and interleukin 6 (r = 0.39). LUS score was significantly higher in subgroups with more severe respiratory failure. CONCLUSIONS: LUS can be regarded as an accurate method to evaluate the extent of COVID-19 pneumonia and as a promising tool to estimate its clinical severity. Evaluation of LUS in the assessment of the structure of inflammation, requires further studies in the course of the disease. TRIAL REGISTRATION: The study has been preregistered 13 Aug 2020 on clinicaltrials.gov with the number NCT04513210.

Validation of the Polish version of Severe Respiratory Insufficiency Questionnaire.

INTRODUCTION: Improvement in the quality of life (QoL) is an essential outcome in patients with chronic respiratory failure (CRF). However, its reliable and comparative assessment is difficult in this highly heterogeneous group of patients. Severe Respiratory Insufficiency Questionnaire (SRI) has shown to have high psychometric properties to measure specific health-related QoL in patients with CRF due to different pathologies. OBJECTIVES: The aim of this study was to validate the Polish version of the SRI. PATIENTS AND METHODS: The Polish version of the SRI was created according to the procedure of translation and back­ translation of the original version. Patients with CRF treated with long­term oxygen therapy (LTOT) or home mechanical ventilation (HMV) were invited to the study. Polish SRI and 36­ Item Short Form Health Survey (SF­36) questionnaires were completed during 2 consecutive visits scheduled at a 2-4 week interval. The results were statistically tested for validity, viability, and reliability. The time and ability of completing, sociodemographic and clinical data were recorded. RESULTS: A total of 113 patients were enrolled. Seventy­ five participants (66%) completed the questionnaires without any assistance. A significant concurrent validity was confirmed by a correlation analysis between the SRI and the SF­36 scales. An exploratory factor analysis explained 69% of the variance of the questionnaire. High internal consistency was proved by the Cronbach α coefficient 0.951 for the Summary Scale. Repeatability was very high for all subscales (intraclass correlation coefficient, 0.871-0.915) and for the summary score (0.923, P <0.001). CONCLUSIONS: Our study demonstrated that the Polish version of the SRI is valid, reliable, and reproducible and may be used in research involving CRF.

KinesioTaping after botulinum toxin type A for cervical dystonia in adult patients.

INTRODUCTION: Studies explored physiotherapeutic approaches in cervical dystonia (CD) patients with or without botulinum toxin (BoNT) injections, however the results are varying. There are no clinical trials investigating the effects of kinesiology taping in CD patients. The objective of this study is to investigate the efficacy of kinesiology taping as an adjunct to the BoNT injections in patients with CD. METHODS: Twenty-five patients were enrolled to the study. Patients were randomly assigned to the experimental 1 (BoNT + KinesioTaping), experimental 2 (BoNT + ShamTaping) or control (BoNT) treatment. After 12 weeks they were moved to the next experimental group and finally every patient received all 3 proposed treatment options. The severity of CD was quantified with the Toronto Western Spasmodic Torticollis Rating Scale (TWSTRS) including Torticollis severity, Disability, and Pain scales. Quality of life was evaluated using Craniocervical dystonia questionnaire (CDQ4). RESULTS: In all treatment groups, there was a significant improvement in dystonia symptoms measured with TWSTRS (total score) after BoNT injection regardless of the allocation to the experimental treatment (p < .05). ANOVA analysis revealed no differences in any of the TWSTRS variables after the intervention. Quality of life was significantly improved after application of taping (p < .05, p = .03). CONCLUSIONS: Application of KinesioTaping after BoNT injection provided no additional effect on the severity of dystonia, although the quality of life was improved in patients with CD. Further research investigating the effect of KinesioTaping prior to BoNT injection is required.

Principles of patients selection and safety of repetitive transcranial magnetic stimulation - position statement of the Section of Biological Psychiatry of the Polish Psychiatric Association. / Zasady kwalifikacji pacjentów i zasady bezpieczenstwa stosowania terapii przezczaszkowa stymulacja magnetyczna seria bodzców ­ stanowisko Sekcji Biologicznej Polskiego Towarzystwa Psychiatrycznego.

Transcranial magnetic stimulation (TMS) is a method of noninvasive brain stimulation developed since the 1980s. Repetitive transcranial magnetic stimulation (rTMS) is one of the methods of noninvasive brain stimulation, which is increasingly used to treat psychiatric disorders. Recent years witnessed a dynamic growth in the number of sites offering therapy with rTMS and of the interest of patients in this method in Poland. This article presents the position statement of the working group of the Section of Biological Psychiatry of the Polish Psychiatric Association concerning the proper patients selection and safety of use of rTMS in the therapy of psychiatric conditions. Before starting to use rTMS, the involved personnel should undergo a period of training in one of the centers with relevant experience. Equipment dedicated to perform rTMS should be appropriately certified. The main therapeutic indication is depression, including drug-resistant patients. rTMS may also be used in obsessive-compulsive disorder, negative symptoms and auditory hallucinations in schizophrenia, nicotine addiction, cognitive and behavioral disturbances in Alzheimer's disease, and post-traumatic stress disorder. The strength of magnetic stimuli and the overall dosing of stimulation must be based on the recommendations of the International Federation of Clinical Neurophysiology. The main contraindications are the metal elements in the body, especially medical electronic devices near the stimulating coil, epilepsy, hearing loss, structural changes in the brain, which may be associated with epileptogenic foci, pharmacotherapy, which lowers the seizure threshold, and pregnancy. The main side effects are induction of epileptic seizure, syncope, pain and discomfort during stimulation, as well as induction of manic or hypomanic episodes. The respective management is described in the article.

Transcranial Magnetic Stimulation as a Diagnostic and Therapeutic Tool in Various Types of Dementia.

Dementia is recognized as a healthcare and social burden and remains challenging in terms of proper diagnosis and treatment. Transcranial magnetic stimulation (TMS) is a diagnostic and therapeutic tool in various neurological diseases that noninvasively investigates cortical excitability and connectivity and can induce brain plasticity. This article reviews findings on TMS in common dementia types as well as therapeutic results. Alzheimer's disease (AD) is characterized by increased cortical excitability and reduced cortical inhibition, especially as mediated by cholinergic neurons and as documented by impairment of short latency inhibition (SAI). In vascular dementia, excitability is also increased. SAI may have various outcomes, which probably reflects its frequent overlap with AD. Dementia with Lewy bodies (DLB) is associated with SAI decrease. Motor cortical excitability is usually normal, reflecting the lack of corticospinal tract involvement. DLB and other dementia types are also characterized by impairment of short interval intracortical inhibition. In frontotemporal dementia, cortical excitability is increased, but SAI is normal. Repetitive transcranial magnetic stimulation has the potential to improve cognitive function. It has been extensively studied in AD, showing promising results after multisite stimulation. TMS with electroencephalography recording opens new possibilities for improving diagnostic accuracy; however, more studies are needed to support the existing data.

Transcranial magnetic stimulation as a diagnostic and therapeutic tool in cerebral palsy.

Purpose: Cerebral palsy (CP) is one of the leading causes of child disability, which profoundly affects the lives of whole families and contributes to the burden of health care. Despite the extensive rehabilitative, surgical and other therapeutic efforts of an array of specialists, a significant proportion of patients remain severely disabled. Transcranial magnetic stimulation (TMS) is a non-invasive diagnostic tool in various diseases of the cerebral cortex and cortico-spinal tract (CST). Repetitive TMS (rTMS) is able to induce a long-lasting cerebral plasticity, which is associated with a therapeutic effect in a number of psychiatric and neurological diseases. This article reviews the diagnostic findings gained with TMS in CP as well as therapeutic trials performed with rTMS. Views: The absence of responses in the motor cortex in the first months of life, as revealed by TMS, may predict the development of CP in children at risk. In a proportion of children with the unilateral form of CP, TMS documents the pathological preservation of ipsilateral, cortico-spinal connections from the non-lesioned hemisphere, which is associated with poor outcome. rTMS seems to be a safe method with significant therapeutic potential in CP. The data published so far reveals an almost unanimously significant therapeutic benefit in motor performance over placebo. However, the studies conducted to date have almost without exception involved children with unilateral palsy, and have focused nearly exclusively on therapy for motor symptoms. Conclusions: Magnetic stimulation brings significant diagnostic and therapeutic effects in CP. However, more studies that go beyond the limits specified above are still awaited.

Impact of obstructive sleep apnea syndrome on peripheral nerves and the effect of continuous positive airway pressure: a prospective observational case series.

Purpose: Obstructive sleep apnea syndrome (OSAS) is suggested to have a strong association with peripheral neuropathy (PNP). However, knowledge about this relationship is still very limited. The aim of this prospective case series was to investigate the peripheral nerves in OSAS patients, along with related clinical symptoms, and to assess the effect of continuous positive airway pressure (CPAP) therapy. Methods: The nerves of upper and lower extremities of 25 patients with moderate to severe OSAS who complained of symptoms suggestive of peripheral neuropathy (PNP) were investigated electrophysiologically. The cross-sectional area (CSA) of the median nerve at the wrist and of the ulnar nerve in the epicondylar groove were assessed with ultrasound. Fifteen patients who showed abnormalities were then reassessed after 3 months of CPAP therapy, and again after an additional 6 months. Results: The most common findings were carpal tunnel syndrome (CTS) and ulnar neuropathy at the elbow (UNE). Surprisingly, CTS was seen in ultrasound twice as frequently as in electrophysiology. The main symptom was numbness in the upper and lower extremities. CPAP therapy reduced the CSA, improved the conduction at entrapment sites and alleviated the symptoms in some of the patients. Conclusions: CTS and UNE are the most frequent neuropathies in patients with OSAS. However, the clinical symptoms tend to be more generalized. CPAP therapy seems to be beneficial for both objective measurements and subjective symptoms.

Prediction of Recovery and Outcome Using Motor Evoked Potentials and Brain Derived Neurotrophic Factor in Subacute Stroke.

INTRODUCTION AND OBJECTIVES: Motor evoked potentials (MEPs) have been postulated to be useful in predicting recovery in patients with motor impairment. We aimed to investigate whether MEPs elicited by transcranial magnetic stimulation (TMS), serum brain derived neurotrophic factor (BDNF) and its genotype have prognostic value on stroke recovery in patients with hand paresis due to stroke. METHODS: This was an observational cohort study. Patients underwent TMS with MEPs from abductor digiti minimi evaluation between 2-14 (D0) and 30 days (D30) after stroke and their impact on motor function of the upper limb and general outcome was assessed after 3 months (D90). The presence of a BDNF gene polymorphism was determined and serum BDNF concentrations were measured at D0, D30 and D90. RESULTS: The presence of MEPs and their amplitude at rest and in effort significantly correlated with improvement of upper-limb paresis and general outcome after 3 months. Resting motor threshold did not have prognostic value. Central motor conduction time and MEP latency less consistently predicted stroke outcome or motor deficit improvement. Neither BDNF polymorphisms nor BDNF concentration at D0, D30 and D90 corresponded with the degree of paresis or the independence of patients 3 months after stroke. CONCLUSIONS: The presence of MEPs and their amplitude are useful predictors of upper-limb motor function recovery and general outcome after stroke. BDNF concentration and its genotype had no prognostic value. Further studies conducted on large cohorts are necessary to determine the usefulness of these methods in motor recovery and stroke outcome prediction.

Are there clinical and neurophysiologic predictive factors for a positive response to HF-rTMS in patients with treatment-resistant depression?

Better selection of patients with treatment-resistant depression for high-frequency repetitive transcranial magnetic stimulation (HF-rTMS) would make the procedure more efficient. The objective of this study was to search for clinical and neurophysiologic predictors of therapeutic response with a special focus on the bipolar population. Forty patients (30 bipolar) underwent 20 daily sessions of HF-rTMS. Clinical outcome measures included the 21-item Hamilton Depression Rating Scale, the Beck Depression Inventory, the Clinical Global Impression, and the Patient Global Impression. Neurophysiologic measurements included repeated estimation of the motor threshold and cortical silent period. Improvement was obtained in all psychometric scales, with no difference between unipolar and bipolar patients. Longer duration of the illness, higher number of prior hospitalizations, and more disturbed activity were associated with a worse response to rTMS, and somatic anxiety, sleep disorders, and health worries were positive predictors. In bipolar patients, longer disease duration and therapy with mirtazapine, mianserin, trazodone, hydroxyzine, and promethazine were associated with a worse response. Sleep disturbances, higher baseline motor threshold, and longer cortical silent period predicted a better response. In this study, we found several clinical and neurophysiologic predictors of better/worse responses to the standard HF-rTMS protocol. Our preliminary data need to be reproduced.

Repetitive transcranial magnetic stimulation for the treatment of cognitive impairment in frontotemporal dementia: an open-label pilot study.

BACKGROUND: Frontotemporal dementia (FTD) is one of the most frequent dementia types in patients under 65 years of age. Currently, no therapy can effectively improve the cognitive deficits associated with FTD. Repetitive transcranial magnetic stimulation (rTMS) is a noninvasive method of inducing brain plasticity with therapeutic potential in neurodegenerative diseases. The purpose of this study was to evaluate the effect of rTMS on cognitive, behavioral, and emotional function in FTD. METHODS: Nine patients (seven women, four men, mean age 61.7±10.1 years) with the behavioral variant of FTD, one with nonfluent/agrammatic variant primary progressive aphasia, and one with progressive nonfluent aphasia (subtypes of FTD) underwent 10 daily sessions of 10 Hz rTMS over the bilateral dorsolateral prefrontal cortex. Cognitive and behavioral assessments were administered before and after therapy. RESULTS: After rTMS, the Montreal Cognitive Assessment and letter and digit cancellation test scores, as well as reading time and error number in the Stroop test improved. The caregivers' impression of the daily functioning of patients improved in the Frontal Behavioral Inventory scores. These changes were not paralleled by an improvement of mood. CONCLUSION: The results indicate that rTMS may improve the cognitive performance of patients with FTD and warrant sham-controlled trials.

Association of peripheral neuropathy with sleep-related breathing disorders in myotonic dystrophies.

BACKGROUND: Myotonic dystrophy (DM) type 1 and type 2 are inherited diseases characterized by myotonia and myopathy. Additional symptoms include, among others, peripheral neuropathy and sleep-related breathing disorders (SRBDs). There is growing evidence for a complex association between DM1 and DM2, which was described in patients with diabetes mellitus and in the general population. In this study, we investigated whether there is an association between peripheral neuropathy and SRBDs also in the population of patients with DM. METHODS: The study included 16 patients with DM1 (mean age, 37.9±14.1 years; 20-69 years) and eight patients with DM2 (mean age, 47.6±14.1 years; 20-65 years), who underwent a sensory and motor nerve conduction study (NCS) and diagnostic screening for SRBDs. In both groups, the NCS parameters were correlated with respiratory parameters. RESULTS: In both groups, the amplitude of the ulnar sensory nerve action potential (SNAP) correlated with the mean arterial oxygen saturation (SaO2). In addition, in the DM2 group, the median SNAP correlated with the mean SaO2. In the DM1 group, the median SNAP and the distal motor latency (DML) of the ulnar nerve correlated with the apnea-hypopnea index, while the oxygen desaturation index correlated with the DML of the tibial nerve and with conduction velocity in the sural nerve. CONCLUSION: Our results indicate a complex association between neuropathy and SRBDs in DM1 and DM2. Axonal degeneration may contribute to nocturnal hypoxemia and vice versa. Neuropathy may contribute to muscle weakness, which in turn may cause respiratory events.

The influence of the repetitive transcranial magnetic stimulation on sleep quality in depression. / Wplyw przezczaszkowej stymulacji magnetycznej seria bodzców na jakosc snu chorych na depresje.

OBJECTIVES: Repetitive transcranial magnetic stimulation (rTMS) improves mood in depression. In this study we investigated whether the depression-related insomnia is modulated by this therapeutic method. METHODS: We examined 13 patients (mean age 50.6±13.9; 11 women) with bipolar or unipolar depression. During 20 consecutive days, excluding Saturdays and Sundays, they underwent 20 daily sessions of 10 Hz rTMS over the left dorsolateral prefrontal cortex (DLPFC). Outcome measurement included the Clinical Global Impression (CGI), the 21item Hamilton Depression Rating Scale (HDRS), the Athens Insomnia Scale (AIS) as well as sleep diary and actigraphy. RESULTS: After rTMS, the CGI and HDRS total score decreased significantly. Also, the insomnia-related items of HDRS improved. The AIS showed trend towards decrease. No significant changes were present in sleep diaries and actigraphy. CONCLUSIONS: The beneficial effect of rTMS on the mood in depression has been confirmed. The rest of the results suggest high frequency rTMS to the left DLPFC does not have strong effects on sleep quality in patients with depression. Additional interventions or modification of the rTMS protocol should be considered to improve insomnia in these patients.

Frequent difficulties in the treatment of restless legs syndrome - case report and literature review.

Restless legs syndrome (RLS) is one of the most common sleep disorders. The purpose of this paper is a case description of the patient suffering from RLS, concurrent with numerous clinical problems. In our patient, during long-term therapy with a dopamine agonist (ropinirole), the phenomenon of the augmentation, defined as an increase in the severity of the RLS symptoms, was observed. The quality of life of the patient was significantly deteriorated. Due to the augmentation of RLS symptoms the dopaminergic drug was gradually withdrawn, and the gabapentin as a second-line drug for the treatment of RLS was introduced. Because of the large increase of both insomnia and RLS symptoms during the reduction of ropinirole dose, clonazepam was temporarily introduced. In addition, in the neurological assessment of the distal parts of the lower limb sensory disturbances of vibration were found. The neurographic study confirmed axonal neuropathy of the sural nerves, which explained an incomplete response to dopaminergic medications. However, gabapentin treatment in the dose recommended in neuropathies was impossible due to bothersome side effects. Another important issue in the treatment of the patient were depressive symptoms and the fact that the majority of used antidepressants (mirtazapine, mianserin, tricyclic antidepressants) increase the severity of RLS. Among antidepressants recommended for the treatment of depression in patients with RLS (such as bupropion, moclobemide, reboxetine, tianeptine and agomelatine) only agomelatine exhibits promoting sleep properties. Because of the concomitant insomnia, this drug was applied in our patient.

[Cardiac abnormalities in patients with myotonic dystrophy--preliminary results]. / Zaburzenia kardiologiczne u pacjentów z dystrofia miotoniczna--doniesienie wstepne.

INTRODUCTION: Myotonic dystrophy (DM) is an inherited multisystem disorder associated with myotonia, progressive skeletal muscle weakness and atrophy, involvement of peripheral and central nervous system and sudden death likely due to atrioventricular block and/or ventricular arrhythmia. AIM OF THE STUDY: to assess the type and degree of cardiac and neurological involvement in patients (pts) with DM. MATERIALS AND METHODS: 10 pts (6 male), in mean age of 35 +/- 13 years, treated for DM type I (DM1)--7 pts and type II (DM2)--3 pts. All pts underwent a neurological examination including muscle strength assessment as well as cardiac diagnostics including: standard and 48-hour ambulatory electrocardiogram, echocardiographic examination, magnetic resonance imaging (MRI) of the heart and late potentials assessment. RESULTS: Muscle strength was moderately diminished (46-48 points in MRC sub score) in 3 pts with DM1 and mildly diminished (56-58 points in MRC sub score) in 2 pts with DM2. These patients showed clinical symptoms of myopathy. Cardiovascular examinations revealed: QRS duration above 110 ms in 5 pts, clinically significant supraventricular arrhythmia or atrioventricular block in 3 pts, focal myocardial fibrosis in 3 pts, asymmetric hypertrophy of inter-ventricular septum in 1 patient, presence of late potentials in 5 pts. We have not observed correlation between impaired muscle strength and cardiac abnormalities. However, most pronounced cardiac abnormalities were observed in 2 male DM1 patients with clinical symptoms of myopathy and lowest MRC score. At a mean follow up of 3.2 +/- 1.4 years none of the pts died. CONCLUSIONS: Cardiac involvement in pts with myotonic dystrophy is frequent and is characterized by phenotypic heterogeneity. Detection of cardiac abnormalities may require extensive diagnostics. The most important is the assessment of ECG. Cardiac and neurological abnormalities vary in intensity between patients without close relationship to each other.

Morphological changes of skeletal muscle in spinal and bulbar muscular atrophy (SBMA), Kennedy's disease: a case report.

Spinal and bulbar muscular atrophy (SBMA, Kennedy's disease) is an X-linked recessive disease affecting lower motor neurons. In the present case report, we describe morphological changes in a muscle biopsy obtained from a 62-year-old patient with gynecomastia and with the following neurological symptoms: dysphagia, dysarthria, wasting and fasciculation of the tongue, proximal weakness, fasciculations in the limb muscles, and an absence of all tendon reflexes. Neurogenic alternations were predominantly observed using light and electron microscopy. The angulated atrophic muscle fibers formed bundles. The numerous nuclei were pyknotic or pale, some of them were also ubiquitin positive; they were grouped inside so-called "nuclear sacks". At the ultrastructural level, atrophic muscle fibers revealed disruption and loss of sarcomeres, duplication of Z-line, and rod-like structures. The nuclei, often with irregular shapes, revealed varying degrees of chromatin condensation, from dispersed to highly condensed, like pyknotic nuclei. Occasionally electron-dense inclusions in the nuclei were found. Some myogenic features like hypertrophic muscle fibers and proliferation of connective tissue were also visible. The neurogenic and myogenic pathological changes suggested SBMA, which was confirmed with genetic analysis (trinucleotide CAG (glutamie)-repeat expansion in the androgen-receptor gene).

Negative influence of L-dopa on subjectively assessed sleep but not on nocturnal polysomnography in Parkinson's disease.

BACKGROUND: Sleep disorders are highly prevalent among patients with Parkinson's disease (PD). Chronic medication with L-dopa may be one of the factors that contributes to poor sleep quality. The aim of this study was to assess the effects of long term use of L-dopa on objective and subjective measures of sleep quality in PD patients. METHODS: Twenty-seven PD patients (mean age 62.5 ± 8.6 years, mean disease duration 7.3 ± 5.9 years, 11 females) underwent nocturnal polysomnography. Their sleep was rated subjectively using the Parkinson's disease sleep scale (PDSS), and their disease severity was assessed using the unified Parkinson's disease severity scale (UPDRS) standard questionnaire. Doses of L-dopa and other medications were correlated with parameters of sleep quality. The polysomnographic recordings were compared with those from 24 age- and gender-matched normal controls. RESULTS: The patients showed decreased total sleep time (TST) and sleep efficiency (SE), prolonged sleep onset and REM sleep latency and wake after sleep onset (WASO). Parts I-III of the UPDRS scores correlated with TST, SE and WASO but not with PDSS scores. L-dopa dosage and part IV of the UPDRS correlated with PDSS scores but not with polysomnographic parameters. CONCLUSIONS: Higher doses of chronically administered L-dopa correlated with lower sleep quality according to the subjective measures but not according to the polysomnographic parameters, which were related to the severity of PD symptoms. The low sleep quality according to the subjective measurements may result from complications of therapy at high doses of L-dopa.

Corticomotor excitability in drug-naive patients with Parkinson disease.

BACKGROUND AND PURPOSE: This study aimed to assess the indices of corticomotor excitability (CE) in drug-naive Parkinson disease (PD) patients and to investigate its relationship with asymmetry and severity of clinical symptoms. MATERIAL AND METHODS: Eleven (4 men) drug-naive PD patients (mean age: 53.1 ± 9.8 years) and 13 (7 men) healthy controls (mean age: 51.7 ± 4.2 years) were included. All PD patients were rated on the motor section of the Unified Parkinson's Disease Rating Scale (UPDRS) with measurement of the side-specific score separately for arms and legs. Resting motor threshold (RMT), central silent period (CSP), amplitude of motor evoked potential (MEP) and central motor conduction time (CMCT) evoked by a single pulse of the transcranial magnetic stimulation were recorded in all subjects from the left and right abductor digiti minimi (ADM) and extensor digitorum brevis (EDB). RESULTS: Parkinson disease patients showed higher MEP (1.8 ± 0.9 vs. 1.1 ± 0.8 mV, p < 0.05) and shorter CMCT (6.1 ± 0.9 vs. 7.4 ± 1.0 ms, p < 0.05) recorded from the ADM on the more affected side. CSP recorded from the more affected ADM was under the normal range in five and from the less affected ADM in four PD patients. For CSP recorded from the EDB, respective values are four for the more affected side and three for the less affected side. The rigidity from the more affected arm and leg correlated negatively with the respective CSP recorded from the ADM (r = -0.74, p < 0.01) and EDB (r = -0.68, p < 0.04). CONCLUSIONS: In the early stage of untreated PD the CE parameters are altered only on the more affected side. The shortening of CSP reflects the severity of rigidity on the more affected side.

Safety of nerve conduction studies in patients with implantable cardioverter-defibrillators.

OBJECTIVE: A patient with an implantable cardioverter-defibrillator (ICD) may suffer from neuromuscular disorders and may need to undergo a nerve conduction study (NCS). However, a NCS may be a source of electromagnetic interference (EMI). The aim of the present study was to investigate whether the interference from NCS used in a standardised test protocol affects ICD function. METHODS: Twenty patients (19 males; mean age of 59.8±9.9 years) with implantable ICDs (eight with integrated and 12 with true bipolar leads), treated with amiodarone and with symptoms suggesting neuropathy were included. NCS were conducted using repetitive stimulation with frequency of 2 Hz and single, rectangular pulses of intensity up to 100 mA. Stimulation was performed in standard sites including proximal sites in the arm. RESULTS: The impulses generated NCS were not detected by the ICD, irrespective of the site, rate or stimulus intensity. CONCLUSIONS: Standardised test protocol for an NCS is safe in patients with an ICD regardless of the leads type. SIGNIFICANCE: Current guidelines which limitate the NCS in patients with ICD may be the subject of revision.

[Cardiac, respiratory and sleep disorders in patients with myotonic dystrophy]. / Zagrozenia kardiologiczne i oddechowe oraz zaburzenia snu u pacjentów z dystrofia miotoniczna.

Myotonic dystrophy (MD) is a genetically determined disease with autosomal dominant mode of inheritance. Relatively recently, MD has been divided into two sub-types (MD1 and MD2). Clinical symptoms of MD1 result from the expansion of a (CTG)n trinucleotide of the gene coding for serine/threonine protein kinase and clinical symptoms in MD2 are associated with the expansion of (CCTG)n in I intron of the zinc-finger protein 9 (ZNF9). Myotonic dystrophies MD1 and MD2 are multisystem diseases with numerous symptoms and high interfamily variability, resulting from the fact that different organs are affected. Until now the mechanisms that lead to the damage of the central and peripheral nervous systems, heart muscle and endocrine system have not been fully understood. Symptoms that are characteristic of MD1 and MD2 are myotonic symptom, muscular weakness and muscular atrophy. In MD2, muscular weakness and muscular atrophy are expressed more significantly in proximal segments, which is a differentiating factor for patients with MD1 who have muscular weakness and muscular atrophy in distal segments. Apart from myotonia and symptoms of skeletal muscle damage, the disease affects smooth muscles, heart muscle and the central nervous system, causing cataract, endocrine disorders, cognitive dysfunctions, intellectual and personality disturbances as well as sleep disordered breathing with nocturnal hypoventilation, obstructive, central and mixed apneas and hypopneas. The symptoms of sleep disordered breathing is fatigue, reduced cognitive performance and excessive daytime sleepiness. The pathophysiology of the breathing disorders includes weakness of the respiratory muscles and disorder of the respiratory drive. Of some interest are the works in which authors evaluated the incidence and character of abnormalities in the peripheral and central nervous systems. It has been shown that the number of CTG-repeats in the same person with MD1 is not stable over time and may increase, which leads to disease progression and new clinical symptoms. Cardiologic disorders associated with myotonic dystrophy are common and are part of the clinical picture of the disease. The dominant pathology are conduction disturbances and cardiac arrhythmias. It is estimated that 40 to 80% of patients with MD1 have abnormalities in ECG, and rapid supra-ventricular and ventricular cardiac arrhythmias are the second common cause of death in patients with MD1. Unfortunately, most of these pathologies are asymptomatic until life-threatening conduction blocks and/or supra-ventricular tachyarrhythmias occur. Sometimes, prodromal symptoms such as collapsing, fainting or feeling of palpitation occur and they should always draw attention of the treating doctor of a patient with muscular dystrophy. This paper is aimed at characterizing some common cardiologic and sleep related respiratory disorders of patients with myotonic dystrophy which if not recognized in good time may lead to sudden death.