World Association of Sleep Medicine (WASM) 2016 standards for recording and scoring leg movements in polysomnograms developed by a joint task force from the International and the European Restless Legs Syndrome Study Groups (IRLSSG and EURLSSG).

This report presents the results of the work by a joint task force of the International and European Restless Legs Syndrome Study Groups and World Association of Sleep Medicine that revised and updated the current standards for recording and scoring leg movements (LM) in polysomnographic recordings (PSG). First, the background of the decisions made and the explanations of the new rules are reported and then specific standard rules are presented for recording, detecting, scoring and reporting LM activity in PSG. Each standard rule has been classified with a level of evidence. At the end of the paper, Appendix 1 provides algorithms to aid implementation of these new standards in software tools. There are two main changes introduced by these new rules: 1) Candidate LM (CLM), are any monolateral LM 0.5-10 s long or bilateral LM 0.5-15 s long; 2) periodic LM (PLM) are now defined by runs of at least four consecutive CLM with an intermovement interval ≥10 and ≤ 90 s without any CLM preceded by an interval <10 s interrupting the PLM series. There are also new options defining CLM associated with respiratory events. The PLM rate may now first be determined for all CLM not excluding any related to respiration (providing a consistent number across studies regardless of the rules used to define association with respiration) and, subsequently, the PLM rate should also be calculated without considering the respiratory related events. Finally, special considerations for pediatric studies are provided. The expert visual scoringof LM has only been altered by the new standards to require accepting all LM > 0.5 s regardless of duration, otherwise the technician scores the LM as for the old standards. There is a new criterion for the morphology of LM that applies only to computerized LM detection to better match expert visual detection. Available automatic scoring programs will incorporate all the new rules so that the new standards should reduce technician burden for scoring PLMS.

Overview of sleep & sleep disorders.

Sleep is defined on the basis of behavioural and physiological criteria dividing it into two states: non rapid eye movement (NREM) sleep which is subdivided into three stages (N1, N2, N3); and rapid eye movement (REM) sleep characterized by rapid eye movements, muscle atonia and desynchronized EEG. Circadian rhythm of sleep-wakefulness is controlled by the master clock located in the suprachiasmatic nuclei of the hypothalamus. The neuroanatomical substrates of the NREM sleep are located principally in the ventrolateral preoptic nucleus of the hypothalamus and those of REM sleep are located in pons. A variety of significant physiological changes occur in all body systems and organs during sleep as a result of functional alterations in the autonomic and somatic nervous systems. The international classification of sleep disorders (ICSD, ed 2) lists eight categories of sleep disorders along with appendix A and appendix B. The four major sleep complaints include excessive daytime sleepiness, insomnia, abnormal movements or behaviour during sleep and inability to sleep at the desired time. The most important step in assessing a patient with a sleep complaint is obtaining a detailed history including family and previous histories, medical, psychiatric, neurological, drug, alcohol and substance abuse disorders. Some important laboratory tests for investigating sleep disorders consist of an overnight polysomnography, multiple sleep latency and maintenance of wakefulness tests as well as actigraphy. General physicians should have a basic knowledge of the salient clinical features of common sleep disorders, such as insomnia, obstructive sleep apnoea syndrome, narcolepsy-cataplexy syndrome, circadian rhythm sleep disorders (e.g., jet leg, shift work disorder, etc.) and parasomnias (e.g., partial arousal disorders, REM behaviour disorder, etc.) and these are briefly described in this chapter. The principle of treatment of sleep disorders is first to find cause of the sleep disturbance and vigorously treat the co-morbid conditions causing the sleep disturbance. If a satisfactory treatment is not available for the primary condition or does not resolve the problem, the treatment should be directed at the specific sleep disturbance. Most sleep disorders, once diagnosed, can be managed with limited consultations. The treatment of primary sleep disorders, however, is best handled by a sleep specialist. An overview of sleep and sleep disorders viz., Basic science; international classification and approach; and phenomenology of common sleep disorders are presented.

Diagnosis and treatment of sleep disorders caused by co-morbid disease.

Sleep disorders, both insomnia and hypersomnia, are commonly associated with various co-morbid conditions, including general medical and neurologic disorders, psychiatric illnesses, and secondary or symptomatic restless legs syndrome/periodic limb movements in sleep. Diagnosis of the co-morbidity is the first step in treatment, followed by an assessment of the sleep disturbance. This begins with a complete history and physical examination, followed by laboratory testing such as polysomnography, multiple sleep latency testing, and actigraphy. The treatment of sleep disorders caused by co-morbid conditions is discussed under seven categories, including the use of general measures (e.g., sleep hygiene, encouraging patients to develop good sleep habits, medication), treatment of the co-morbid condition, treatment of insomnia, treatment of excessive daytime somnolence, treatment of parasomnias, treatment of sleep-wake schedule disorders, and treatment of secondary or symptomatic restless legs syndrome.

The short exercise test is normal in proximal myotonic myopathy.

OBJECTIVES: Proximal myotonic myopathy (PROMM) is a multisystem disorder that may mimic myotonic dystrophy (MD). Previously we demonstrated that the 60 s exercise test was normal in two siblings with PROMM. The test enabled distinction of PROMM from MD, as there is a well documented immediate post-exercise compound muscle action potential (CMAP) amplitude decline in MD. METHODS: We now performed exercise testing using several exercise durations in 8 PROMM patients from 6 kinships, and one MD patient, extending our previous observations. Repetitive stimulation and needle electromyography findings were also recorded. RESULTS: The 10 (n = 8), 30 (n = 5), and 60 (n = 5) s, and the 5 min (n = 1) exercise tests were normal in all PROMM patients. Specifically, the maximum post-exercise CMAP amplitude decline was 8%. In contrast, the MD patient had CMAP amplitude declines of 48% (10 s exercise test) and 26% (30 s exercise test). The distribution of repetitive stimulation and motor unit duration abnormalities were variable and less diagnostically useful. CONCLUSIONS: The 10, 30, and 60 s exercise tests help distinguish PROMM from MD. As the 10 s exercise test is rapid and easily tolerated, we recommend this test for clinical testing.

Posttraumatic delayed sleep phase syndrome.

Circadian rhythm sleep disorders may occur after traumatic brain injury. We describe a 48-year-old man who presented with sleep onset insomnia and cognitive dysfunction after a car accident. A diagnosis of delayed sleep phase syndrome (DSPS) was confirmed by sleep logs and actigraphy, which revealed sleep onset in the early morning hours and awakening around noon.

Comorbidities associated with overactive bladder.

Overactive bladder (OAB), defined as symptoms of frequency, urgency, and urge incontinence, that occur singly or in any combination in the absence of local pathologic or metabolic factors, is a highly prevalent disorder with an unknown etiology. Few risk factors for OAB have been elucidated through epidemiologic studies, and even less is known about the contribution of OAB to other morbidities. An overview is provided of the impact of OAB on other problems now known to coexist with OAB including falls and fractures, urinary tract and skin infections, sleep disturbances, and depression.

Trapezius CMAP amplitude asymmetry in accessory neuropathy.

In accessory neuropathy electrodiagnosis, upper trapezius compound muscle action potential (CMAP) latencies and amplitudes are commonly measured. The few prior reports describing middle and lower trapezius recording have traditionally emphasized latency value determination. The utility of amplitude measurement with middle and lower trapezius recording has not, to our knowledge, been previously described in individual patients with accessory neuropathy. We report three patients (A-C) who developed unilateral accessory neuropathy following surgical procedures. Accessory nerve conduction studies were performed with surface recording over the upper, middle, and lower trapezius muscles. Latency values were normal except for a prolonged lower trapezius latency value in patient B. Side-side trapezius amplitude comparisons revealed striking asymmetries from all three recording sites in patients A and B (71-95% CMAP amplitude decrements) and in the lower trapezius recording of patient C. Middle and lower trapezius side-side CMAP amplitude comparisons may increase the sensitivity of accessory neuropathy electrodiagnosis.

Median and ulnar palm-wrist studies.

OBJECTIVES: Routine carpal tunnel electrodiagnosis frequently includes median (MPW) and ulnar (UPW) palm-wrist mixed nerve conduction latency determinations over 8 cm. Despite widespread use, normative palmar latency difference (PLD) and UPW values, and the relative utility of onset latency (OL) or peak latency (PL) measurements are controversial. The current study was conducted to determine normative values for these parameters. METHODS: MPW and UPW studies were performed unilaterally in 33 normal controls. The PLD-OL and PLD-PL were calculated. The mean, range, standard deviation, and upper limits of normal were determined. 74 hands (50 patients) with both clinical and electrophysiologic median neuropathy were also studied. RESULTS: The abnormal MPW and UPW cut-offs were both 1.8 ms (OL), and 2.3 ms (PL). The abnormal PLD cut-offs were 0.5 ms (OL and PL). Using either OL or PL, PLD parameters were similar within controls, and also within CTS patients. Using either OL or PL, UPW parameters were similar between controls and CTS patients. CONCLUSIONS: An abnormal PLD cut-off of 0.5 is recommended. This is slightly higher than some prior recommendations, however it should minimize the likelihood of false positive studies. Onset and peak latency measurements are likely to have similar clinical utility.

Cervical root stimulation at C5/6 excites C8/T1 roots and minimizes pneumothorax risk.

Needle electrical cervical root stimulation may be performed lateral to the C5/C6 or C7/T1 spinous process interspaces. Pneumothorax has been reported following C7/T1 root stimulation. We evaluated the efficacy of a modified C5/C6 stimulation technique in exciting C8/T1 roots in 15 normal subjects and 36 patients with motor neuron disease (204 procedures). No instances of a 50% or greater amplitude decline occurred. C5/C6 interspace stimulation, therefore, may be used to excite C8/T1 roots while minimizing pneumothorax risk.