Barış Baslo

Muscle ultrasonography and electromyography correlation for evaluation of floppy infants

Floppiness in an infant may have a number of different etiologies from disorders of the brain to spinal cord lesions, neuropathies, neuromuscular junction disorders and myopathies. In this study we aimed to investigate the correlation of muscle ultrasonography (US) and electromyography (EMG) in the diagnosis of floppy infants. The study encompassed 41 floppy infants aged 2-24 months. The muscle US and EMG examinations were performed without awareness of the clinical diagnosis. The final diagnosis was established by molecular genetic tests or muscle/nerve biopsy. The neurogenic group consisted of 16 infants according to their US and EMG findings. Fifteen of them had spinal muscular atrophy proven by genetic analysis and one had polyneuropathy diagnosed by nerve biopsy. Six infants were in the myopathic group according to their muscle US and EMG results. All of them underwent muscle biopsy and microscopic examination revealed five congenital muscular dystrophy and one glycogen storage disease. In two infants the US and EMG data conflicted. Their biopsies were also insufficient for the diagnosis. Seventeen infants had normal US and EMG findings but pathologic cranial magnetic resonance imaging or metabolic/genetic tests. They were considered in the group of central hypotonia. Our results suggest a high concordance of US and EMG findings in the diagnostic work-up of neurogenic and myopathic disorders.

Large motor unit territories by scanning electromyography in patients with juvenile myoclonic epilepsy.

Juvenile myoclonic epilepsy is a genetically inherited disorder characterized by myoclonic jerks and generalized seizures. It has been proposed that patients with juvenile myoclonic epilepsy have larger motor units (MUs) than normals by MU number estimation and macro electromyography techniques. In this study, an experimental setup for scanning electromyography was built to investigate electrophysiologic cross-sections of the MU territories in 9 patients with juvenile myoclonic epilepsy, 3 patients with spinal muscular atrophy, and 10 healthy volunteers. Scanning electromyography was performed on the biceps brachii muscle. For each MU, three-dimensional maps of the MU territories were plotted. The length of MU cross-section and the maximum amplitude of each MU were measured from these maps and compared among the three groups of subjects. Like spinal muscular atrophy patients, patients with juvenile myoclonic epilepsy had significantly larger MU territories than normal controls.

Teaching NeuroImages: Swollen T1 nerve root in neurogenic thoracic outlet syndrome

A 17-year-old girl without any history of considerable trauma or previous disease was admitted with weakness and wasting in the right forearm and hand, more marked on the thenar eminence (figure). EMG revealed unrecordable medial antebrachial cutaneous sensory and low-amplitude median motor responses (recorded from thenar muscles) along with chronic neurogenic changes in the hand muscles. MRI showed a fusiform focal expansion of the right T1 root (figure). Neurogenic thoracic outlet syndrome (NTOS) was diagnosed and surgically decompressed.1

Classification of neuromuscular junction and tendon recordings of neuromuscular diseases by their spectrogram

In this study, the effect of spectrograms from neuromuscular junction and tendon records for normal, neurogenic and myopathic motor units being constructed via EMG Simulator v3.6 on the differential diagnosis were investigated. Multi-layer perceptron is chosen as classifier. If only the neuromuscular junction records are applied to the network, the performance is 73.33%. If only tendon records are applied to the input of network, the performance is 94.67%. When neuromuscular junction and tendon records are applied together to the network, the performance is 100%.

Determining phase duration of scanning EMG signals

There are more than one motor unit activities recorded simultaneously during scanning EMG recordings. It is not possible to determine the phase duration correctly by inspecting only one sweep. The other motor unit activities, which are out of interest, should be filtered for this purpose. In this study, a new method is developed by revealing the activity corridor of scanning EMG recordings, wavelet transform based noise reduction, autocorrelation function based signal cutting location detection, and alpha trim filtering are applied to the recorded signals for determining phase duration correctly. Data are recorded from normal people and neuromuscular diseased people. Dataset contains 20 scanning EMG recordings.

P440: Determining new features for diagnosis of neuromuscular diseases

Question: Individual motor unit action potential (MUAP) analysis and peak-ratio (PR) analysis are well known routine methods used to evaluate reinnervation of muscles in chronic partial denervation. The motor unit number estimation technique MUNIX may provide additional information on number of motor units and their average size (MUSIX). This study evaluated how these methods complement each other in a large muscle. Methods: MUAP, PR and MUNIX were prospectively studied in 15 biceps brachii (BB) muscles with MRC force 2-5 in patients with motor neuron disease (MND) and a disease duration of 7-108 months and in 8 BB muscles with MRC force 4-5 in 8 previous polio (PP) patients. MUNIX was performed in 9 healthy controls. Results: MUNIX showed large inter-subject variation both in MND (9-209) and PP (40-122). Compound motor action potential (CMAP) amplitude was strongly correlated with MUNIX and showed a similar inter-subject variation. MUSIX was rather constant at MUNIX values above 40, at values below 40 MUSIX was increased in 5 MND patients and one PP patient. In PP patients MUAP duration correlated with PR and with MUSIX, while in MND patients such correlations could not be found. MUNIX did not correlate with MUAP duration or PR in any patient group. MUAP duration was prolonged in 4/8 PP patients and in 14/15 MND patients and PR was decreased in 6/8 PP patients and in 12/14 MND patients. MUNIX was below 50 in 1/8 PP patients and in 7/15 MND patients. Abnormal MUNIX was not seen in any patient with normal MUAP duration or PR. MUAP duration was the only parameter that correlated with disease duration in MND patients. Conclusions: As expected MUAP duration and PR have the highest sensitivity in the biceps brachii muscle of patients with anterior horn cell disorders, emphasising that the strength of MUNIX may be monitoring of disease processes. The lack of correlations between different methods in MND may, in contrast to the correlations found in PP, reflect that MND patients are at different stages in the reinnervation process.

İdiyopatik Parkinson hastalığında motor ünite sayısı değişimi / Motor unit number estimation in Idiopathic Parkinson’s disease

Motor unit number estimation in Idiopathic Parkinson’s disease Objective: Motor neuron degeneration in parkinsonism is known to develop in Western Pacific (Guam) parkinsonism-amyotrophic lateral sclerosis complex, multisystem atrophy (MSA), postensefalitic parkinsonism, Creutzfeld-Jacob disease and dementia with parkinsonism linked to chromosome 17. Existence of motor neuron degeneration in these disorders brings up the question whether motor neuron degeneration develops in Idiopathic Parkinson Disease (IPD). Our study aims to answer this question. Methods: In this study, we estimated the motor unit counts in 24 patients diagnosed as IPD and 26 healthy control subjects with similar age (50-70) and gender, using threshold method. Results: Our study revealed the motor unit counts, done using threshold method, in IPD patients in thenar muscles were not different from the control group subjects. Conclusion: This result implied that there was not asymptomatic motor neuron degeneration in IPD, and IPD did not share the same physiological mechanism with other parkinsonism related disorders associated with motor neuron degeneration. However, since there are limited numbers of publications on this subject, to verify this conclusion, further studies using similar methods, with larger patient groups are needed.

Multiple anomalies with scoliosis – a case study

Discussion Long-standing overt ventriculomegaly in adults is a clinical entity characterized by chronic hydrocephalus. The onset of this condition occurs in infancy with a slow evolution and onset of clinical symptoms during adulthood. Limited case reports exists which describe this condition of severe hydrocephalus with the relatively spared neurological functioning and cognitive aspects. This case report demonstrates in vivo the level of adaptation to which human brain can reach under chronic mechanic stress conditions. Scoliosis may present with other anomalies. from 5th International Conference on Conservative Management of Spinal Deformities Athens, Greece. 3–5 April 2008

THO31 Decline of compound muscle action potentials and estimated motor unit numbers during Wallerian degeneration

Method: Forty three adult male Sprague-Dawley rat were divided 6 groups. In the Group 1 (distal study) and Group 2 (distal control), the nerve was transected and repaired on proximal part of the nerve, before the bifurcation. In the Group 3 (proximal study) and Group 4 (proximal control), the nerve was transected and repaired, 15 mm upper proximal part of the bifurcation. In the Group 5 (greft study) and Group 6 (greft control), a nerve segment 10 mm long was resected into the proximal part of the nerve and the resected segment was used as a nerve greft. Following sciatic nerve transection and surgical repair, rats received 50 mg/kg/day ALCAR or sham (equivalent volume of normal saline) for 14 days intraperitoneally. At the end of the 1 week, first, 2nd and 3th month, EMG records were done. Three months later, L4 and L5 dorsal root ganglia were harvested bilaterally and biopsies were taken from distal region of transected side of sciatic nerve. Also neuron and axon counts were done. Results: The nerve conduction velocities and amplitudes did not show any significant differences between all groups. However, the latencies of distal groups were found sicnificantly decreased. Also, neuronal loss ratios were found 9% in Group 1, 16% in Group 2, 10% in Group 3, 21% in Group 4, 30% in Group 5, 35% in Group 6 respectively. Conclusion: Better histopathological findings of ALCAR administred groups are supported that the positive effect of ALCAR on nerve regeneration. We could not showed any differences between all groups electrophisologically. It may depends that ALCAR is more effective on sensory nerves.