Fieke M. Cox

A 12-year follow-up in sporadic inclusion body myositis: an end stage with major disabilities

Sporadic inclusion body myositis is considered to be a slowly progressive myopathy. Long-term follow-up data are, however, not yet available. Follow-up data are important with a view to informing patients about their prognosis and selecting appropriate outcome measures for clinical trials. We performed a follow-up study of 64 patients with sporadic inclusion body myositis who participated in a national epidemiological study in the Netherlands. Case histories were recorded, and manual and quantitative muscle tests as well as laboratory tests were performed at baseline and 12 years (median) after the first out-patient visit. Date and cause of death were recorded for all deceased patients. Forty-six patients died during the follow-up period, two patients chose not to participate and one patient was lost to follow-up. The remaining 15 surviving patients had a mean disease duration of 20 years and were clinically evaluated at the second time point. The mean decline in strength was 3.5 and 5.4% per year according to the manual muscle testing and quantitative muscle testing, respectively. This decline was most pronounced in the lower legs, which were also the weakest extremities. Life expectancy was normal at 81 years, but activities of daily life were clearly restricted. At follow-up, all patients were found to be using a wheelchair, seven of them (47%) being completely wheelchair-bound. Disorders of the respiratory system were the most common cause of death. In three patients, euthanasia was requested and in another three, continuous deep sedation was applied. The fact that end-of-life care interventions were used in six patients (13%) reflects the severe disability and loss of quality of life at the end stage of this disease. Sporadic inclusion body myositis is a chronic progressive disorder, leading to major disabilities at the end stage of the disease due to extensive muscle weakness.

Magnetic resonance imaging of skeletal muscles in sporadic inclusion body myositis

OBJECTIVE To analyse whether MRI of upper and lower extremity muscles in a large patient group with sporadic IBM (sIBM) is of additional value in the diagnostic work-up of sIBM. METHODS Thirty-two sIBM patients were included. Magnetic resonance (MR) parameters evaluated in 68 muscles of upper and lower extremity were muscle atrophy, fatty infiltration and inflammation. These findings were correlated with disease duration, weakness and serum creatine kinase (sCK) levels. RESULTS Fatty infiltration was far more common than inflammation. Muscles most frequently infiltrated with fat were the flexor digitorum profundus (FDP), anterior muscles of the upper leg and all muscles of the lower leg, preferentially the medial part of the gastrocnemius. The rectus femoris was relatively spared compared with other quadriceps muscles as well as the adductors of the upper leg. Inflammation was common in general, but individually sparse, present in 78% of the patients with a median of two inflamed muscles per patient. A statistically significant correlation was found between the amount of fatty infiltration and disease severity, disease duration and sCK. CONCLUSION We provide a detailed description of the MRI in sIBM and show a distinct pattern of muscle involvement. Relatively severe affliction of the medial compartment of the gastrocnemius, combined with relative sparing of the rectus femoris or involvement of the FDP can be indicative of sIBM. MRI can contribute to the diagnosis in selected patients with clear clinical suspicion, but lacking the mandatory set of muscle biopsy features.

Detecting dysphagia in inclusion body myositis

Dysphagia is an important yet inconsistently recognized symptom of inclusion body myositis (IBM). It can be disabling and potentially life-threatening. We studied the prevalence and symptom-sign correlation of dysphagia. Fifty-seven IBM patients were interviewed using a standard questionnaire for dysphagia and 43 of these underwent swallowing videofluoroscopy (VFS). Symptoms of dysphagia were present in 37 of 57 patients (65%). Nevertheless, only 17 of these patients (46%) had previously and spontaneously complained about swallowing to their physicians. Both symptoms of impaired propulsion (IP) (59%) and aspiration-related symptoms (52%) were frequently mentioned. Swallowing abnormalities on VFS were present in 34 of 43 patients (79%) with IP of the bolus in 77% of this group. The reported feeling of IP was confirmed by VFS in 92% of these patients. Dysphagia in IBM is common but underreported by the vast majority of patients if not specifically asked for. In practice, two questions reliably predict the presence of IP on VFS: ‘Does food get stuck in your throat’ and ‘Do you have to swallow repeatedly in order to get rid of food’. These questions are an appropriate means in selecting IBM patients for further investigation through VFS and eventual treatment.

The heart in sporadic inclusion body myositis: a study in 51 patients

The purpose of this study was to explore the prevalence and nature of cardiac abnormalities in sporadic inclusion body myositis (sIBM). Fifty-one sIBM patients were cross-sectionally studied using history-taking, physical examination, measurements of serum creatine kinase activity, the MB fraction (CK-MB), cardiac troponin T (cTnT) and I (cTnI), a 12-lead electrocardiogram (ECG) and 2-dimensional echocardiography. Present cardiac history was abnormal in 12 (24%) out of 51 patients, 12 (24%) patients had abnormalities on ECG, mostly aspecific, and in 12 (24%) patients the echocardiograph showed abnormalities. Elevated CK-MB was present in 42 (82%) patients and 40 (78%) had an elevated cTnT in the absence of acute cardiac pathology. In contrast, in one patient (2%) cTnI was elevated. There was no apparent association between elevated biomarkers, ECG or echocardiographic abnormalities. The prevalence of cardiac abnormalities in sIBM does not seem to be higher than would be expected in these elderly patients. Elevated CK-MB and cTnT levels are common, in contrast to cTnI, but do not reflect cardiac pathology.

Standardized computer-based organized reporting of EEG: SCORE – Second version

Standardized terminology for computer-based assessment and reporting of EEG has been previously developed in Europe. The International Federation of Clinical Neurophysiology established a taskforce in 2013 to develop this further, and to reach international consensus. This work resulted in the second, revised version of SCORE (Standardized Computer-based Organized Reporting of EEG), which is presented in this paper. The revised terminology was implemented in a software package (SCORE EEG), which was tested in clinical practice on 12,160 EEG recordings. Standardized terms implemented in SCORE are used to report the features of clinical relevance, extracted while assessing the EEGs. Selection of the terms is context sensitive: initial choices determine the subsequently presented sets of additional choices. This process automatically generates a report and feeds these features into a database. In the end, the diagnostic significance is scored, using a standardized list of terms. SCORE has specific modules for scoring seizures (including seizure semiology and ictal EEG patterns), neonatal recordings (including features specific for this age group), and for Critical Care EEG Terminology. SCORE is a useful clinical tool, with potential impact on clinical care, quality assurance, data-sharing, research and education.

Timing of first event in inpatient long-term video-EEG monitoring for diagnostic purposes

BACKGROUND Long-term video-EEG monitoring (LTM) aims to record the habitual event and is a useful diagnostic tool for neurological paroxysmal clinical events. In our epilepsy monitoring unit (EMU) setting, admissions are usually planned to last up to five days. We ascertained time taken for the recording of a first event and determined correlations between different clinical characteristics and timings. METHODS We retrospectively reviewed diagnostic and classification LTM recording performed at a tertiary epilepsy centre. RESULTS Sixty-three recordings were reviewed. Most subjects (89%) had events at least once a week prior to admission. In 40 (63%) a habitual event was recorded, mostly (93%) within the first two days. No events were recorded on day four or five. A few characteristics were associated with a trend for events occurring earlier (events more than once a week vs less than once a week, motor symptoms compared with aura or dyscognitive events, and reduction of antiepileptic drugs versus no reduction). CONCLUSIONS Our finding suggests that, for diagnostic event recording in people with epilepsy or PNEA, a maximum recording time of three days is sufficient in two thirds of them, if event frequency is at least once a week. In the remaining third, prolonged recording up to five days did not result in capturing a clinical event. For these individuals, shorter admission could be planned, for example for 2days rather than 5days.

Value of video monitoring for nocturnal seizure detection in a residential setting

Following a sudden death at a residential care unit, the Dutch Health and Care Inspectorate advised intensification of the use of video monitoring (VM) at the unit. We assessed whether VM resulted in increased identification of seizures that required clinical intervention.

TREX1 mutations are not associated with sporadic inclusion body myositis

Background:  Sporadic inclusion body myositis (sIBM) is the most frequent acquired myopathy above the age of fifty. The exact mechanism causing this disease is not known, but immune‐mediated features are prominent and are probably to play a role in its pathogenesis. TREX1 gene mutations are associated with a large range of autoimmune diseases, such as systemic lupus erythematosus. We investigated whether mutations in the TREX1 gene were associated with sIBM.

What is a practical duration time for capturing psychogenic non-epileptic seizures by video-EEG monitoring?

We’ve read with great interest the article by Chen et al. on the practical duration time for capturing events by prolonged video electroencephalogram monitoring in the elderly [1]. One conclusion the authors made is it would be practical to monitor patients for only 24 h in case of suspected psychogenic non-epileptic seizures, as all recorded non-epileptic events in their study were captured during the first 24 h of video-EEG monitoring. The authors noted that others previously reported different findings, with a substantial proportion of subjects having a psychogenic non-epileptic seizure occurring after 24 h of monitoring. The authors hypothesize that this difference is caused by a different patient group (Chinese elderly versus Western adults) or way of monitoring (video-EEG versus ambulatory EEG). We disagree with the authors, as explained below. Chen et al. reported positive video-EEG monitoring (meaning an event captured during monitoring) in 48%. This number is lower than previously reported in literature, with positive recordings between 63 and 83%. This could be explained by a too short duration of monitoring in the study of Chen et al. Table 2 seems to confirm this, as it shows only 16 subjects (9% of their study population) were monitored longer than 24 h, of who 11 had an epileptic event during the monitoring, and no one had an psychogenic non-epileptic seizure (one ‘other’ event). Therefore, a maximum of 4 subjects only with psychogenic non-epileptic

Improving staff response to seizures on the epilepsy monitoring unit with online EEG seizure detection algorithms

OBJECTIVE User safety and the quality of diagnostics on the epilepsy monitoring unit (EMU) depend on reaction to seizures. Online seizure detection might improve this. While good sensitivity and specificity is reported, the added value above staff response is unclear. We ascertained the added value of two electroencephalograph (EEG) seizure detection algorithms in terms of additional detected seizures or faster detection time. METHODS EEG-video seizure recordings of people admitted to an EMU over one year were included, with a maximum of two seizures per subject. All recordings were retrospectively analyzed using Encevis EpiScan and BESA Epilepsy. Detection sensitivity and latency of the algorithms were compared to staff responses. False positive rates were estimated on 30 uninterrupted recordings (roughly 24 h per subject) of consecutive subjects admitted to the EMU. RESULTS EEG-video recordings used included 188 seizures. The response rate of staff was 67%, of Encevis 67%, and of BESA Epilepsy 65%. Of the 62 seizures missed by staff, 66% were recognized by Encevis and 39% by BESA Epilepsy. The median latency was 31 s (staff), 10 s (Encevis), and 14 s (BESA Epilepsy). After correcting for walking time from the observation room to the subject, both algorithms detected faster than staff in 65% of detected seizures. The full recordings included 617 h of EEG. Encevis had a median false positive rate of 4.9 per 24 h and BESA Epilepsy of 2.1 per 24 h. CONCLUSIONS EEG-video seizure detection algorithms may improve reaction to seizures by improving the total number of seizures detected and the speed of detection. The false positive rate is feasible for use in a clinical situation. Implementation of these algorithms might result in faster diagnostic testing and better observation during seizures.