Decline of compound muscle action potentials and statistical MUNEs during Wallerian degeneration.

AIM OF THE STUDY: In two previous studies, we found that the compound muscle action potential (CMAP) amplitude loss was significantly higher than the loss of estimated motor unit numbers in the course of Wallerian degeneration (WD). In order to overcome some drawbacks of the method previously used, we performed a similar CMAP vs MUNE comparison by using the statistical motor unit number estimation (MUNE) method. PATIENTS AND METHODS: Initial electrophysiological studies on 6 patients were performed between 22 and 98 hours after the injuries; it was possible to make repeated examinations, four times in 1 nerve, twice in 1 nerve and three times in 4 nerves, before the eventual complete disappearance of the CMAPs. RESULTS: The transected/intact (T/I) side CMAP ratios declined steeply as WD evolved. They were significantly lower than the relatively stable MUNE ratios 48 hours after the injury. CONCLUSION: This study, performed with the use of statistical MUNE, strengthens our previous observation by the incremental method that might have some relevance to the pathophysiology of early WD. CMAP amplitude loss that is more than expected from the amount of axonal degeneration may indicate a considerable amount of inactive muscle fibers in the motor units innervated by the nerve fibers, which are undergoing degeneration but still retain their excitability. Although technical sources of error cannot be totally excluded, our findings could more likely be explained by the failing of neuromuscular synapses in an asynchronous order before complete unresponsiveness of the motor unit ensues.

Effect of socks on the assessment of vibration sensation.

Objective. To investigate the difference between the measurement of vibration sensation without and with socks. Material and Methods. Fifty healthy volunteers (H group) and 19 patients with diabetic polyneuropathy (PNP group) were included. The sites of measurement were the great toe (GT) and medial malleolus (MM). A standard 128 Hz tuning fork was used in the measurements. Results. Mean duration of vibration sensations without and with socks was as follows: in the H group, 19.4 ± 4.2 and 19.5 ± 4.2 s at GT and 15.1 ± 3.3 and 14.6 ± 3.3 s at MM; in the PNP group, 13.4 ± 3.8 and 12.7 ± 4.1 s at GT and 11.9 ± 3.8 and 11.7 ± 3.4 s at MM. No significant difference was found between the measurements without and with socks, except those found at the MM in group H (P = 0.02). This significant difference was further analyzed in terms of effect size which was concluded to be practically insignificant (Cohen's d < 0.2). Shorter mean vibration duration was measured at MM as compared to GT that could be explained by the damping effect. Conclusions. Wearing socks of moderate thickness does not have any important effect on the duration of vibration sensation. This might be considered as a reflection of the remarkable properties of vibration sensation.

Motor unit number estimation in transected peripheral nerves.

OBJECTIVES: To study motor unit number estimation (MUNE) in acutely transected peripheral nerves, and to retest our previous observation which had revealed a discordance between the loss of compound muscle action potential (CMAP) size and decrease in MUNE during Wallerian degeneration. METHODS: In eight patients with nine transected median or ulnar nerves, a total of 18 electrophysiological studies were performed before the complete nerve degeneration ensues. CMAP recordings and incremental MUNE studies were performed by stimulation of the nerves at the wrist level and recording from the appropriate hand muscles. The same studies repeated on the contralateral side. RESULTS: Injury side to intact side ratios of the MUNEs were significantly higher than the CMAP ratios. Mean step areas in MUNE studies were found to be lower on the transected sides after 72 hours post-injury. DISCUSSION: These findings support the existence of an electrophysiologically observable asynchrony in neuromuscular synapse dysfunction during Wallerian degeneration.

Cortical excitability in Duchenne muscular dystrophy.

OBJECTIVE: To investigate the probable cortical excitability changes in DMD by electrophysiological means. METHODS: Sixteen cases with DMD, 10 age-matched control children (CC) and 10 healthy adult volunteers (AC) were studied with a transcranial magnetic stimulation (TMS) test battery composed of central conduction time, cortical silent period and paired TMS paradigm. RESULTS: There were no significant differences between DMD and CC groups except for lower amplitude motor responses in DMD cases. These two groups showed a similar pattern of excitability with less short interval intracortical inhibitions and shorter silent period durations as compared to the AC subjects. CONCLUSIONS: The electrophysiological tests performed in our DMD patients did not reveal abnormalities caused particularly by the disorder. SIGNIFICANCE: TMS excitability studies performed in DMD boys may not provide findings other than those related to the developmental age.

Inflammatory/demyelinating central nervous system involvement in familial Mediterranean fever (FMF): coincidence or association?

BACKGROUND AND OBJECTIVE: Familial Mediterranean fever (FMF) is an inherited inflammatory disease characterized by recurrent febrile polyserositis. Central nervous system (CNS) involvement in FMF is uncommon, but recently cases with multiple sclerosis (MS) and FMF have been reported. Here we assess patients with both FMF and MS, in order to clarify any relationship between FMF and MS, and to evaluate disease characteristics. PATIENTS AND METHODS: Our MS database between 1986-2005 was screened retrospectively, and patients with both FMF and inflammatory/demyelinating CNS disease were evaluated among a total of 2800 patients including definite MS (n = 2268) and other demyelinating disorders. RESULTS: There were 12 patients with FMF, who developed a CNS disorder with multifocal white matter lesions. Median age at onset of FMF was 7 years, and median age at neurological onset was 26.8 years. Nine patients (including two siblings) had definite MS according to clinical and MRI findings, whereas 3 patients had atypical features suggesting other demyelinating disorders. Disease severity varied among the patients between very mild to a fatal course. All 8 patients evaluated for oligoclonal IgG bands in CSF were positive. CONCLUSION: The rate of FMF among our patients with definite MS is almost 4 times the expected prevalence in Turkey. Our series including a sibling pair concordant for FMF and MS may suggest that similar genetic susceptibility and environmental factors might be responsible, although coincidence still remains a possibility. A prospective study on a larger sample seems to be justified.

Paired transcranial magnetic stimulation in hemifacial spasm.

OBJECTIVES: The aim of this study was to investigate the changes in cortical excitability in patients with hemifacial spasm (HFS) in order to examine the physiological state of facial nerve nucleus. METHODS: Nineteen patients with HFS and 13 control subjects were examined at rest. The procedure was repeated during voluntary contraction in 6 of control subjects. By paired transcranial magnetic stimulation, conditioning and test motor evoked potentials (MEP) were recorded from bilateral orbicularis oris muscles at interstimulus intervals (ISI) of 20, 25, 30, 50, 75 and 100 ms. RESULTS: In control subjects at rest, ISI's of 20, 25, 30 ms evealed facilitation of the test MEP and inhibition developed at ISI's of 75 and 100 ms. Test MEP facilitation in the contracting controls and on the symptomatic sides of HFS patients was significantly less than the resting controls. Asymptomatic sides of HFS patients behaved similar to the corresponding symptomatic sides and to the contracting controls, although the values were not statistically significant when compared to the resting controls. CONCLUSION: The lessening of facilitation found in this study can be interpreted as an abnormal finding that reflects the changes in the excitability of facial motoneuronal system in patients with HFS. However it is difficult to exclude the possibility that this finding can occur due to the normal behavior of cortex during voluntary or involuntary contraction.

Disposable concentric needle electromyography electrodes artifact discharge mimicking positive sharp waves.

OBJECTIVES: To determine a new artifact discharge produced from the disposable concentric needle (DCN) electromyography (EMG) electrodes. METHODS: We have recorded the activity obtained after the first insertion of 41 DCN (37 mm) and 36 DCN (50 mm) in several muscles during the rest. The number of the patients was 77 (26 males, 51 female). RESULTS: We observed an artifact discharge (AD) resembling positive sharp waves (PSWs) in 31 patients of 77 insertions. The AD occurred with 18 of 41 insertions of DCN 37 mm and 13 of DCN 50 mm. The artifact resolved when the needle has been moved in the muscle or in the adipose subcutaneous tissue. CONCLUSION: This AD should be recognized when using DCN electrodes to avoid confusion with PSWs. Recognizing these artifacts during needle EMG is important to avoid false positive results.

F wave parameters and F tacheodispersion in mild diabetic neuropathy.

F tacheodispersion and some F wave parameters have previously been suggested to be useful in the detection of peripheral nerve involvement in diabetic patients with normal nerve conduction studies. In this study, ulnar and tibial F wave parameters of diabetic cases with normal motor and sensory nerve conduction studies (Group 1) were calculated, F tacheodispersion histograms were constructed and compared with the same parameters obtained from the normal controls (Control group) and diabetic cases with abnormal motor and sensory nerve conduction examinations (Group 2). There was a tendency towards lower conduction velocities in ulnar and tibial tacheodispersion histograms of Group 1 and Group 2, as compared to Control group. Most of the F wave values were significantly abnormal in Group 2 in comparison to other two groups, whereas statistically significant differences between Group 1 cases and Control group were found in minimum ulnar F wave conduction velocity (FCVmin), mean ulnar FCV (FCVmean), tibial FCVmin and tibial FCVmean values. Unlike other methods, F waves give information about the entire length of the motor nerve fiber. In mild neuropathies, in which nerve fibers are damaged uniformly, testing the whole length of a nerve with this method may be beneficial in detection of a mild conduction abnormality.

Neuromuscular consequences of prolonged hunger strike: an electrophysiological study.

OBJECTIVES: The purpose of this study was to determine the electrophysiological consequences of neuromuscular and central nervous system involvement in a group of patients presented with the neurological complications of a long-term hunger strike (HS). METHODS: Motor and sensory nerve conduction (NCV), F wave, somatosensory evoked potential (SEP) and motor evoked potential (MEP) studies were performed in 12 male and 3 female patients (mean age: 29.4) following HS. RESULTS: All patients whose weight loss was 11-31 (mean: 22.8) kg after 69-day HS, had neurological findings consistent with Wernicke's encephalopathy or Wernicke-Korsakoff syndrome. Abnormally prolonged latency and/or low amplitude sensory nerve action potentials were found in 7 patients. The amplitudes of compound muscle action potentials were significantly reduced in ulnar, median and tibial motor NCV studies as compared to the controls. F waves elicited by median nerve stimulation at wrist and muscle responses evoked by cervical and lumbar magnetic stimulation had significantly prolonged latencies. MEPs recorded from the lower extremities showed a slight prolongation in central conduction times. The cortical response latencies were prolonged in tibial SEPs. CONCLUSIONS: The most prominent finding in this patient group was the low amplitude of CMAPs elicited in motor NCV studies which was concluded to be resulted from the reversible muscular changes. The other electrophysiological findings suggested that peripheral nerves and long central nervous system pathways were also mildly involved.

Peroneal nerve palsy: the role of early electromyography.

Footdrop is rare in the newborn period. In this report, the clinical and electrophysiological features of a newborn whose peroneal neuropathy was detected in the delivery room is presented. Her first electrophysiological examination, at the 13th hour of life, revealed a peroneal nerve lesion. All findings elicited in this and subsequent examinations suggested an antenatal onset. Clinical recovery began within weeks of the birth and she was able to walk, with no residual neurological abnormality, by her first birthday. Early electrophysiological examination of a newborn with peripheral neuropathy may provide valuable information about the time of onset and pathophysiological features of the nerve lesion. In spite of the early electromyogram findings revealing severe nerve injury, a good prognosis is possible in newborns with peroneal neuropathy.

Concentric needle electrode for neuromuscular jitter analysis.

We used a concentric needle electrode (CNE) with 2 kHZ low-cut filter and a single fiber electrode (SFE) in the same subjects for neuromuscular jitter measurement in the extensor digitorum communis (EDC) and orbicularis oculi (OOc) muscles. At the same session, 20 jitter values were obtained from each subject with each electrode. For EDC (during voluntary contraction), mean jitter values with SFE and CNE were 23.4 +/- 8 micros and 23.3 +/- 8 micros in 10 normals; and 56.8 +/- 28 micros and 57.4 +/- 33 micros in 10 myasthenics. For OOc (during electrical stimulation), mean jitter values with SFE and CNE were 17.9 +/- 5 micros and 16.3 +/- 4 micros in 11 normal subjects, and 41.2 +/- 29 micros and 36.7 +/- 27 micros in 10 myasthenics. For both muscles, the numbers of individual abnormal jitter values with SFE and CNE were highly comparable. Both needles labeled the same patients as having "normal" or "abnormal" neuromuscular transmission. CNE may be an alternative to SFE in neuromuscular jitter analysis.

The use of botulinum toxin in localizing neuromyotonia to the terminal branches of the peripheral nerve.

In 2 patients with neuromyotonia, nerve blocks had no effect on the abnormal activity, while intramuscular injection of the botulinum toxin abolished the discharges in one and greatly diminished them in the other. Botulinum toxin thus helps to localize the origin of the neuromyotonic discharges to the terminal regions of the peripheral nerve in those cases where the more proximal portions cannot be held responsible.

Magnetic nerve root stimulation in two types of brachial plexus injury: segmental demyelination and axonal degeneration.

Magnetic cervical nerve root stimulation was performed in 9 patients with plexopathies secondary to suspension (SP) and in 12 cases with neurogenic thoracic outlet syndrome (NTOS). The findings were compared with those of the previously reported case groups: n-hexane polyneuropathy (HPNP), inflammatory demyelinating polyneuropathy (IDP), and motor neuron disease (MND). Muscle responses elicited by magnetic stimulation had very high rates of amplitude and area loss in the neck-axilla segments of the 6 SP patients. This, along with the other electrophysiological findings, suggested the presence of segmentally demyelinating plexus lesions. In NTOS patients, magnetic stimulation findings were not significantly different from those of the controls. Neck-axilla segment amplitude and are reduction rates in SP and IDP patients were significantly higher than those found in NTOS, HPNP, and MND groups, implying that magnetic nerve root stimulation may have a role in the demonstration of segmentally demyelinating lesions involving proximal nerve segments.

Electrophysiological findings in patients who received radiation therapy over the brachial plexus: a magnetic stimulation study.

Clinical and electrophysiological findings of 47 asymptomatic females who received radiation therapy (RT) over their brachial plexus region are presented and compared with 8 radiation-induced brachial plexopathy (RBP) and 4 neoplastic brachial plexopathy (NBP) patients. In the asymptomatic group, abnormal findings were more frequent in patients whose post-RT period was longer than 1 year. Flexor carpi radialis H reflex was delayed or absent in 19 patients (52%) in this subgroup of asymptomatic cases, as compared to only 2 (18%) of the patients with post-RT periods of less than 1 year. Magnetic cervical nerve root stimulation was performed in 16 asymptomatic cases, with the conclusion that there was no significant difference between the irradiated and non-irradiated sides with regard to latencies, amplitudes and areas of the muscle responses. In spite of this, muscle response amplitudes and areas on both sides were significantly lower than those obtained from healthy controls. It was postulated that this finding resulted from hypoexcitability to magnetic stimulation produced by slight nerve root damage. Any part of the brachial plexus could be affected in RBP and NBP patients. Myokymic discharges were found at a high rate (87.5%) in RBP group. Cervical magnetic nerve root stimulation may have a diagnostic value in these patients in localizing the nerve lesion over the brachial plexus.

Emery-Dreifuss muscular dystrophy with unusual features.

Two families with Emery-Dreifuss muscular dystrophy (EMD) are described. Several unusual features for EMD are emphasized. One of the patients had severe neuromuscular disability with inability to walk during early childhood. This patient also had mild bifacial paresis. His brothers had the typical slow progression of EMD. In some of the patients, muscle weakness distribution was more widespread than has usually been reported, with prominent involvement of finger extensors. It is suggested that there is a wide phenotypic spectrum in EMD. In both families, the disease segregated with markers spanning the EMD locus in Xq28.

Magnetic stimulation in hemifacial spasm and post-facial palsy synkinesis.

The facial nerve was stimulated trascranially with a magnetic stimulator in 14 normal controls, 14 hemifacial spasm patients, and 16 post-facial-palsy synkinesis patients. Magnetic stimulation in normal controls revealed muscle responses which had latencies with a mean value of 4.99 +/- 0.49 ms and amplitudes of 2.41 +/- 1.08 mV. In the same group, transosseal conduction time was calculated to be 1.20 +/- 0.13 ms. In the hemifacial spasm group, the amplitudes of the responses on the affected sides were lower as compared to the unaffected sides (mean values 1.78 vs. 2.41 mV, P = 0.01). Also, the threshold to magnetic stimulation was elevated on the affected sides. These findings are suggestive of the presence of a hypoexcitability to magnetic stimulation in the root entry zone. In the post-facial-palsy synkinesis patients, magnetic stimulation of the affected sides resulted in responses with long latencies and low amplitudes (mean latency 6.34 ms, mean amplitude 0.90 mV). In the recordings made with magnetic stimulation, the difference of the latencies between the two sides was larger as compared to those obtained by electrical stimulation. The transosseal conduction time was also remarkably prolonged on the affected side. These findings may suggest that magnetic stimulation can be an effective method of showing intracranially located lesions of the facial nerve.