Usefulness and yield of routine electroencephalogram: a retrospective study.

BACKGROUND: The electroencephalogram (EEG) is a common diagnostic tool used to investigate patients for various indications including seizure disorders. AIMS: To investigate factors that predict the presence of epileptiform abnormalities on EEG and review the common indications for ordering an EEG. METHODS: We retrospectively reviewed all routine adult EEG performed in a hospital over a 6-month period. Data collated included patient demographics, clinical indication for EEG, setting in which EEG was performed, activation procedures utilised, history of epilepsy, and whether the patient was on antiepileptic medication. Our primary objective was to evaluate the factors that were predictive of an EEG with epileptiform abnormalities. RESULTS: Two hundred and thirty-nine routine EEG were included with indications, including first seizure (25.9%), known epilepsy (25.1%), cognitive change (15.9%), syncope (15.0%), movement disorder (6.7%), psychogenic non-epileptic events (5.4%), unresponsiveness/intensive care unit (4.6%) and psychiatric presentation (1.3%). Most (48.1%) EEG were normal; 8.9% of the EEG demonstrated epileptiform abnormalities. Using multivariate logistic regression, three variables proved significant in predicting an EEG with epileptiform abnormalities. Any seizure as an indication (first seizure or seizure in known epileptic), increasing patient age, and EEG conducted in an inpatient setting and within 48 h of seizure event were all statistically more likely to yield epileptiform abnormalities on EEG. CONCLUSIONS: Our findings suggest that careful selection of patients based on appropriate indications for EEG referral would likely improve the yield of an EEG. Depending on the indication, a normal EEG result can be of similar usefulness to an abnormal EEG demonstrating epileptiform abnormalities.

Assessment of small sensory fiber function in myotonic dystrophy type 1.

BACKGROUND: Myotonic dystrophy type 1 (DM1) is a multisystem disorder affecting the peripheral nervous system. However, studies evaluating somatic small fiber sensory nerve function, which may contribute to pain in DM1, are lacking. METHODS: Using quantitative sensory testing of the hand and foot, we evaluated Aδ and C-fiber function. Of 20 adult DM1 patients recruited, 16 were analyzed. Their results were compared with those of 32 age- and sex-matched controls. RESULTS: No DM1 patient had diabetes mellitus or clinical evidence of small fiber neuropathy. In DM1, hand (P < .01) and foot (P = 0.02) warm detection thresholds were higher than those of controls. Cool detection thresholds were lower in the foot (P < .001). CONCLUSIONS: Subclinical small sensory fiber dysfunction occurs in DM1 patients without large fiber neuropathy. Further research with other modalities is required to characterize these disturbances as disease modifying therapies are developed.

Physiological differences in sarcolemmal excitability between human muscles.

INTRODUCTION: The sarcolemmal resting membrane potential (RMP) affects muscle excitability, contractility, and force generation. However, there are limited In vivo data on the normal RMP of the human sarcolemma between muscles. We hypothesize that the in vivo RMP may differ between human muscles with different physiological roles. METHODS: Muscle velocity recovery cycles were recorded from a proximal antigravity muscle, the rectus femoris, and compared with paired recordings from a distal non-antigravity muscle, the tibialis anterior, in 34 normal individuals. RESULTS: Significant differences in muscle relative refractory period (3.55 millseconds vs 3.87 milliseconds, P = .002), early supernormality (14.22% vs 10.50%, P < .0001), and late supernormality (5.43% vs 3.50%, P < .0001) were observed. DISCUSSION: The results strongly suggest a less negative RMP in tibialis anterior vs rectus femoris and attest to intermuscle differences in normal excitability and physiology. This novel finding employing an in vivo methodology highlights the need for muscle-specific normative data in future studies.

Sarcolemmal excitability changes in normal human aging.

INTRODUCTION: The exact mechanisms underlying the loss of skeletal muscle bulk and power with normal human aging are not well established. Recording of muscle velocity recovery cycles (MVRCs) is an in-vivo neurophysiologic technique we employed to assess the impact of age on sarcolemmal excitability. METHODS: MVRC recordings were obtained from tibialis anterior (n = 74) and rectus femoris (n = 32) muscles in 74 healthy subjects (18-84 years, median age 35 years, interquartile range 29-55 years). RESULTS: Increasing age was linearly associated with longer muscle relative refractory period (MRRP) and reduced early supernormality (ESN) in both tibialis anterior (MRRP: r2 = 0.38, P < 0.001; ESN: r2 = 0.33, P < 0.001) and rectus femoris (MRRP: r2 = 0.30, P = 0.002; ESN: r2 = 0.19, P = 0.01) muscles. DISCUSSION: The results are consistent with progressive depolarization of the resting sarcolemmal potential with normal aging. This may be an important mechanism in explaining age-related muscle decline. Muscle Nerve 57: 981-988, 2018.

Sarcolemmal excitability in the myotonic dystrophies.

INTRODUCTION: Chloride conductance disturbances contribute to sarcolemmal dysfunction in myotonic dystrophy type 1 (DM1) and type 2 (DM2). Studies using muscle velocity recovery cycles (MVRCs) suggest Na+ /K+ -adenosine triphosphatase activation becomes defective in advanced DM1. We used MVRCs to investigate muscle excitability in DM1 and DM2. METHODS: MVRCs were measured for patients with mild (n = 8) and advanced (n = 11) DM1, DM2 (n = 4), and normal controls (n = 30). RESULTS: Residual supernormality after multiple conditioning stimuli was increased in DM2 and advanced DM1. Advanced DM1 was distinguished by increases in muscle relative refractory period (MRRP) and reduced early supernormality as well as peak amplitude decrements for the first and last responses in train during repetitive stimulation. DISCUSSION: Prolongation of the MRRP indicates that depolarization of the resting muscle membrane potential occurs in advanced DM1, with possible implications for future therapeutic approaches. Muscle Nerve 57: 595-602, 2018.

Serum electrolyte concentrations and skeletal muscle excitability in vivo.

OBJECTIVE: Multi-fibre muscle velocity recovery cycle (MVRC) assessment is a well-tolerated method of evaluating sarcolemmal excitability in vivo that shows promise as a research tool and biomarker. MVRC parameters correlate with venous electrolyte concentrations in myopathies. We sought to determine the nature of any such relationships in individuals without muscle disease. METHODS: Tibialis anterior MVRCs were recorded and electrolyte concentrations measured from two groups of healthy volunteers. After studying a single measure cohort (n = 65, one recording/person), we studied a repeated measures cohort (n = 4, eight recordings/person) to better study intra-individual relationships using repeated measures correlation (rmcorr). RESULTS: In the single measure cohort, no significant correlations were present between MVRC parameters and electrolyte levels after accounting for age. In the repeated measures cohort, the relative refractory period (P < 0.01) and stimulus frequency measures (P < 0.01) correlated positively with potassium levels. Multiple late supernormality group measures correlated negatively with bicarbonate levels (P < 0.01). CONCLUSIONS: MVRC measures that vary with the resting muscle membrane potential correlate with venous potassium concentrations, as in myopathies. Late supernormality measures correlate with bicarbonate levels. SIGNIFICANCE: Determination of serum electrolyte levels may inform the interpretation of MVRC study results if variation in concentrations is anticipated to be significant.

Neuropathy in sporadic inclusion body myositis: A multi-modality neurophysiological study.

OBJECTIVE: Sporadic inclusion body myositis (sIBM) has been associated with neuropathy. This study employs nerve excitability studies to re-examine this association and attempt to understand underlying pathophysiological mechanisms. METHODS: Twenty patients with sIBM underwent median nerve motor and sensory excitability studies, clinical assessments, conventional nerve conduction testing (NCS) and quantitative thermal threshold studies. These results were compared to established normal controls, or results from a normal cohort of older control individuals. RESULTS: Seven sIBM patients (35%) demonstrated abnormalities in conventional NCS, with ten patients (50%) demonstrating abnormalities in thermal thresholds. Median nerve motor and sensory excitability differed significantly in sIBM patients when compared to normal controls. None of these neurophysiological markers correlated significantly with clinical markers of sIBM severity. CONCLUSION: A concurrent neuropathy exists in a significant proportion of sIBM patients, with nerve excitability studies revealing changes possibly consistent with axolemmal depolarization or concurrent neuronal adaptation to myopathy. Neuropathy in sIBM does not correlate with muscle disease severity and may reflect a differing tissue response to a common pathogenic factor. SIGNIFICANCE: This study affirms the presence of a concurrent neuropathy in a large proportion of sIBM patients that appears independent of the severity of myopathy.

Sarcolemmal depolarization in sporadic inclusion body myositis assessed with muscle velocity recovery cycles.

OBJECTIVE: To study patients with sporadic inclusion body myositis (sIBM) with muscle velocity recovery cycles (MVRC) to assess muscle membrane excitability, pathophysiological mechanisms and potential biomarkers of this disorder. METHODS: MVRC were recorded from 20 individuals with sIBM from tibialis anterior (TA) and rectus femoris (RF) muscles. Excitability parameters were compared with MVRC data obtained from 22 normal controls >50 years. RESULTS: Muscle relative refractory period was prolonged in both TA (6.4 ms vs 4.4 ms, P < 0.001) and RF (7.1 ms vs 3.9 ms, P < 0.001) of sIBM affected muscle when compared to controls. Early supernormality was reduced in both TA (3.6% vs 8.8% P = 0.001) and in RF (mean 5.4% vs 13% P < 0.001). Late supernormality was only decreased significantly in sIBM affected TA (1.8% vs 3.6% P = 0.001) but not in RF. No consistent correlations between MVRC parameters and clinical markers of sIBM disease severity were found. CONCLUSION: The resting sarcolemmal muscle membrane potential of sIBM muscle is depolarized relative to that of normal controls, which may be related to intramuscular amyloid deposition in sIBM. SIGNIFICANCE: Sarcolemmal depolarization may play a role in muscle dysfunction and weakness observed in sIBM patients.