Diagnostic value of combined magnetic resonance imaging examination of brachial plexus and electrophysiological studies in multifocal motor neuropathy.

BACKGROUND/AIM: Multifocal motor neuropathy (MMN) is an immune-mediated disorder characterised by slowly progressive asymetrical weakness of limbs without sensory loss. The objective of this study was to investigate the involvement of brachial plexus using combined cervical magnetic stimulation and magnetic resonance imaging (MRI) of plexus brachialis in patients with MMN. We payed special attention to the nerve roots forming nerves inervating weak muscles, but without detectable conduction block (CB) using conventional nerve conduction studies. METHODS: Nine patients with proven MMN were included in the study. In all of them MRI of the cervical spine and brachial plexus was performed using a Siemens Avanto 1.5 T unit, applying T1 and turbo spin-echo T1 sequence, axial turbo spin-echo T2 sequence and a coronal fat-saturated turbo spin-echo T2 sequence. RESULTS: In all the patients severe asymmetric distal weakness of muscles inervated by radial, ulnar, median and peroneal nerves was observed and the most striking presentation was bilateral wrist and finger drop. Three of them had additional proximal weakness of muscles inervated by axillar and femoral nerves. The majority of the patients had slightly increased cerebrospinal fluid (CSF) protein content. Six of the patients had positive serum polyclonal IgM anti-GM1 antibodies. Electromyoneurography (EMG) showed neurogenic changes, the most severe in distal muscles inervated by radial nerves. All the patients had persistent partial CBs outside the usual sites of nerve compression in radial, ulnar, median and peroneal nerves. In three of the patients cervical magnetic stimulation suggested proximal CBs between cervical root emergence and Erb's point (prolonged motor root conduction time). In all the patients T2-weighted MRI revealed increased signal intensity in at least one cervical root, truncus or fasciculus of brachial plexus. CONCLUSION: We found clinical correlation between muscle weakness, prolonged motor root conduction time and MRI abnormalities of the brachial plexus, which was of the greatest importance in the nerves without CB inervating weak muscles.

Gait in amyotrophic lateral sclerosis: Is gait pattern differently affected in spinal and bulbar onset of the disease during dual task walking?

Amyotrophic lateral sclerosis (ALS) is characterized by weakness, fatigue, loss of balance and coordination. The purpose of the study was to examine gait in ALS patients. Gait was compared in ALS with spinal and bulbar onset, while performing dual mental and motor tasks. Dual-task walking was performed by 27 ALS patients, 13 with spinal- and 14 with bulbar-onset disease. Twenty-nine healthy subjects were used as a control group. The subjects performed a basic, simple walking task, dual-motor task, dual-mental task, and combined motor and mental tasks. Results showed that dual-task paradigm has an effect on gait in ALS patients. Gait was differently affected in spinal and bulbar onset of ALS by some of the given tasks. Mental tasks had a larger effect than motor tasks in all gait parameters. In conclusion, both ALS forms have impaired gait in dual tasks. Simple walk in patients with spinal onset shows higher variability of certain gait parameters compared to bulbar-onset patients and controls. Differences in gait could also indicate postural instability and possible falls in complex walking situations.

Cardiologic predictors of sudden death in patients with myotonic dystrophy type 1.

The aim of this study was to analyze survival, causes of death and cardiologic predictors of sudden death in a large cohort of patients with myotonic dystrophy type 1 (DM1). The study was comprised of 171 adult DM1 patients hospitalized at the Neurology Clinic in a 20-year period. Severe electrocardiographic (ECG) abnormality included at least one of the following: rhythm other than sinus, PR interval of ≥240 ms, QRS complex duration of 120 ms or more, and second-degree or third-degree atrioventricular (AV) block. Survival data were analyzed by the Kaplan-Meier test, log-rank test and Cox regression analysis. During the mean follow-up period of 9.4±5.4 years, a pacemaker was implanted in 5.8% of DM1 patients and 14% of patients died. The mean age at death was 55.6±12.5 years. The most common causes of death in our cohort were sudden death (41.7%) and respiratory failure (29.2%). The presence of palpitations (hazard ratio [HR]=4.7, p<0.05) and increased systolic blood pressure (HR=9.8, p<0.05) were significant predictors of sudden death. Among ECG parameters, severe ECG abnormality (HR=4.7, p<0.05), right bundle branch block (RBBB; HR=3.9, p<0.05) and bifascicular block (HR=5.8, p<0.05) were significant predictors of sudden death.

Hypogonadism and erectile dysfunction in myotonic dystrophy type 1.

Myotonic dystrophy type 1 (DM1) is the most common form of muscular dystrophy in adults. It affects many organs and systems besides muscle. Aim of this study was to assess frequency of erectile dysfunction (ED) and hypogonadism, the correlation between them and the impact of ED on quality of life (QoL) in patients with DM1. A series of 25 men (aged from 22 to 58 years) with a diagnosis of DM1 was analyzed. Muscular Impairment Rating Scale (MIRS) was used to assess severity of muscular involvement. Erectile function was assessed using the short form of the International Index of Erectile Function test (IIEF-5). Levels of follicle stimulating hormone (FSH), luteinizing hormone (LH) and testosterone were assessed. All patients completed the Serbian version of the SF-36 questionnaire as a measure of health-related QoL. ED was present in 18 (72%) of patients. Seven (28%) patients were euogonadic, 16 (64%) had compensated hypogonadism and 2 (8%) had primary hypogonadism. ED was somewhat more common in patients with hypogonadism (78% vs. 57%). Mental composite score of SF-36 was lower in patients with ED (p<0.05). Our results showed that 72% of men with DM1 had ED and hypogonadism. Studies with larger number of subjects are needed to resolve cascade of events that lays behind ED in DM1. Development of therapeutic strategies may have positive impact on QoL. Substitutive therapy with androgens may be benefitial.

Is hyperlipidemia correlated with longer survival in patients with amyotrophic lateral sclerosis?

Amyotrophic lateral sclerosis (ALS) is the most serious form of degenerative motor neuron disease in adults, whose relentless course leads to death within 2-5 years, generally due to respiratory failure. Apart from the age and site of onset, no other factors have consistently demonstrated to be related to the ALS outcome. The aim of the study was to investigate the influence of fasting serum lipid levels (cholesterol and triglycerides) and the body mass index (BMI) at the time of diagnosis on survival in ALS patients. The study included 82 patients with ALS residing in the Belgrade area who were diagnosed with ALS over a time period of 4 years (2006-2009). Survival was assessed by the Kaplan-Meier method. In this retrospective study, 39 (47.56%) patients had normal values of lipids and 43 (52.43%) patients had hyperlipidemia. The mean survival time from the onset of symptoms for patients with normal lipidemia was 4.21 ± 0.5 years, while the mean survival time from the onset of symptoms for patients with hyperlipidemia was 5.0 ± 0.67 years (P = 0.36). We also did not register a significant difference in survival in relation to gender, the site or age of onset, even though we noticed a longer survival in patients with hyperlipidemia in all of the examined groups, especially in the group of younger patients, with the onset of the disease before the age of 45 years. If we take into account the fact that BMI is pathophysiologically associated with cholesterol and triglyceride serum levels, the results in our study complement each other showing that patients with a higher BMI, registered in 28.8% of the cases, do not live longer. Our findings show that hyperlipidemia, which we found in 52.43% of our ALS patients, at the time of diagnosis, is not related to significantly longer survival.