153© Springer International Publishing AG, part of Springer Nature 2018R. Govindarajan, P. C. Bollu (eds.), Sleep Issues in Neuromuscular Disorders, https://doi.org/10.1007/978-3-319-73068-4

Table A.1 Segmental innervation of the spinal cord

Spinal cord segment Major muscles innervated

Muscle action

Deep tendon reflexes

C1, C2 Rectus lateralis, rectus capitis anterior, longus capitis, longus cervicis, sternocleidomastoid

Neck flexion

C3 Longus capitis, longus cervicis, trapezius Neck lateral flexion

C4 Diaphragm, trapezius, levator scapularis, scalenus anterior and medius

Shoulder elevation

C5 Deltoid, supraspinatus, infraspinatus, teres minor, biceps, scalenus anterior and medius

Shoulder abduction

Bicep reflex

C6 Serratus anterior, subscapularis, teres major, latissimus dorsi, pectoralis major, biceps, brachialis, brachioradialis, extensor carpi radialis longus, supinator, scalenus anterior, medius, and posterior

Elbow flexion and wrist extension

Brachioradialis reflex

C7 Serratus anterior, latissimus dorsi, pectoralis major and minor, triceps, pronator teres, extensor carpi radialis longus, flexor digitorum superficialis, flexor carpi radialis. Extensor carpi radialis brevis, extensor digitorum, extensor digiti minimi

Elbow extension and wrist flexion

Triceps reflex

C8 Pectoralis major and minor, triceps, flexor digitorum superficialis, flexor digitorum profundus, flexor pollicis longus, pronator quadratus, flexor carpi, abductor pollicis longus, extensor pollicis longus, extensor pollicis brevis, extensor indicis, abductor pollicis brevis, flexor pollicis brevis, opponens pollicis

Thumb extension

T1 Flexor digitorum profundus, intrinsic muscles of hand, flexor pollicis brevis, ppponens pollicis

Finger abduction and adduction

L1, L2 Psoas, Iliacus, sartorius, gracilis, pectineus, adductor longus, adductor brevis

Hip flexion

L3 Quadriceps, adductor longus, magnus, and brevis

Knee flexion Patellar tendon reflex

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154

Spinal cord segment Major muscles innervated

Muscle action

Deep tendon reflexes

L4 Tibialis, quadriceps, tensor fascia lata, adductor magnus, obturator externus, tibialis posterior

Ankle dorsiflexion

Patellar tendon reflex

L5 Extensor hallucis longus, extensor digitorum longus, gluteus medius and minimus, obturator internus, semimembranosus, popliteus

Toe extension

Tibialis posterior reflex

S1 Gastrocnemius, soleus, gluteus maximus, obturator internus, piriformis, biceps femoris, semitendinosus, popliteus, peroneus longus and brevis, extensor digitorum brevis

Ankle plantar flexion

Achilles reflex

S2 Biceps femoris, gastrocnemius, soleus, piriformis, flexor digitorum longus, flexor hallucis longus, intrinsic muscles of foot

Knee flexion

S3 Intrinsic muscles of foot, flexor hallucis brevis, flexor digitorum brevis, extensor digitorum brevis

Rectal sphincter tone

Table A.2 Anatomical localization and causes of lower motor neuron lesions

Localization Causes Signs and symptomsSpinal cordForamen magnum

Meningioma, chordoma, atlanto-axial subluxation

Spastic quadriparesis, neck pain and stiffness, facial numbness, ipsilateral Horner Syndrome

Anterior cord Anterior spinal artery infarction

Upper and lower motor paralysis, spinothalamic sensory loss, sphincter dysfunction

Central cord Syringomyelia Paraparesis, wasting and fasciculations in arms, sensory loss in shawl distribution

Posterior cord Extrinsic compression, vitamin B-12 deficiency, multiple sclerosis

Loss of proprioception and vibration, sensation of constricting bands, tingling, numbness

Conus medullaris

Intrinsic tumors, extrinsic compression

Lower sacral saddle sensory loss, sphincter disturbance, lower back and perineal pain, foot and ankle weakness

Cauda equina Extrinsic compression, spinal stenosis

Early sphincter dysfunction, paraparesis, sensory loss and weakness in multiple bilateral dermatomes

Anterior horn Amyotrophic lateral sclerosis (ALS), poliomyelitis

Progressive flaccid weakness, wasting, fasciculations

Nerve root/plexus

Mechanical injury, malignancy, external compression

Single limb, and/or radicular weakness, pain and paresthesias with indistinctly demarcated sensory loss, absent deep tendon reflexes, no muscular atrophy or fasciculations

Peripheral nerve Guillain–Barré syndrome (GBS), chronic inflammatory demyelinating polyneuropathy

Focal or distal severe weakness, prominent muscle atrophy, hyporeflexia, and fasciculations. Sharply demarcated sensory loss, pain and paresthesias in nerve distribution, and autonomic disturbances

Neuromuscular junction

Myasthenia gravis, Lambert–Eaton syndrome, botulism

Fluctuating weakness, diplopia

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155

Table A.3 Classification of deep tendon reflexes

ReflexesAssociated muscle group

Associated spinal dermatomal

Associated spinal nerve root

Site of stimulus for reflex

Conditions with abnormal reflexes

Biceps Bicep brachii C5 Musculo cutaneous nerve

Bicep brachii tendon at cubital fossa

Myopathy neuropathy, poliomyelitis, neuritis, hyperthyroidism

Brachioradialis Extensor carpi radialis

C6 Radial nerve

Brachioradialis tendon at radial styloid process in wrist

Myopathy neuropathy, poliomyelitis, neuritis, hyperthyroidism

Triceps Tricep brachii C7 Radial nerve

Tricep tendon at back of elbow joint

Myopathy neuropathy, poliomyelitis, neuritis, hyperthyroidism

Patellar Tibialis anterior, quadriceps

L4 Femoral nerve

Patellar tendon below the patella

Lumbar radiculopathy

Babinski Extensor hallucis longus

L4-5, S1-2 Tibial nerve Lateral side of sole of foot

Disc herniation, lumbar radiculopathy

Achilles Gastrocnemius, soleus

S1 Tibial nerve Achilles tendon in a dorsiflexed foot

Disc herniation, hypothyroidism, diabetes, neurosyphilis, alcoholism

Fig. A.1 PAP headgear along with a nasal mask. The elastic material of the headgear helps hold the mask in place and gives a good seal. The headgear comes in different sizes and shapes depending on the model. After a few months of usage, the headgear is usually replaced due to the wear and tear and loss of elasticity

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156

Fig. A.2 Actigraphy watch: This is worn on the wrist and helps to track the body movements and inactivity. Actigraphy recording is usually done for 2–4 weeks to monitor the sleep–wake patterns of patients. The inactivity recorded in the actigraphy watch is used as a surrogate for sleep in ana-lyzing the sleep–wake rhythms. Patients are encouraged to wear the equipment day and night as much as possible. Sometimes, patients are asked to record their sleep–wake rhythms on a separate diary while performing actigraphy recording

Fig. A.3 Tubing: Tubing connects the headgear to the PAP device and helps deliver the pressure generated at the PAP machine to the interface. The tubing shown below is quite simple and consists of a plastic hollow tube and a surrounding molded plastic with rubber rubber ends. Some modern tubing comes with a heating coil that helps prevent condensation of the humidity inside the tubing

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157

Fig. A.4 Portable monitor: This portable PSG equipment has audio and video recording along with EEG monitoring but may not have all the other channels that a conventional PSG recording equipment has. This equipment is typically used to obtain PSGs on patients that are admitted to the hospital who can’t obtain regular PSGs. The equipment is brought to the patient’s room and set up

Fig. A.5 Video recording of PSG: Video cameras (regular and night vision cameras) mounted on the ceiling (typically) will record the video during the PSG

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158

Fig. A.7 Wiring and electrodes

Fig. A.6 Audio recording: The microphone above the patient’s head (white device—in this picture) will record audio

Appendix

159

Fig. A.8 Home sleep study recording: The equipment is given or mailed to the patient along with the instructions to set it up

Fig. A.9 Headbox: This acts as an interface into which all the wiring from the recording electrodes go in. It is connected to the computer for data acquisition

Fig. A.10 A nasal mask interface. The soft silicone helps with the seal all along the area of contact and minimizes the air leak

Appendix

160

Fig. A.11 Skin prep gel used to clean the skin. Conductive electrode paste that is applied to the electrodes before setting them up on the scalp

Fig. A.12 Chest and abdominal belts that record thoraco-abdominal excursions during the PSG

Appendix

161

Sample CPAP Titration Protocol

Start with a CPAP pressure of 4 cm

Increase CPAP pressure by 1 cm of

H2O

If - 2 or more obstructive apneas- 3 or more Hypopneas- 5 or more RERAs- Greater than 3 min of loud snoring

If - 2 or more obstructive apneas- 3 or more Hypopneas- 5 or more RERAs- Greater than 3 min of loud snoring

Increase CPAP pressure by 1 cm of H2O till 30 min

or more sleep without respiratory events is

noted

Further exploration of PAP pressure can be considered after no clear respiratory events are noted.

At 15 cm of CPAP pressure, BiPAP can be considered for continued respiratory events.

Maximum CPAP pressure is 20 cm of H2O.

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162

Fig. A.13 Neuromuscular junction

Fig. A.14 Dermatomes of upper limb

C7

C6

C5

C4

C3

C8

T1

Appendix

163

Fig. A.15 Dermatomes of lower limb

Appendix

165© Springer International Publishing AG, part of Springer Nature 2018R. Govindarajan, P. C. Bollu (eds.), Sleep Issues in Neuromuscular Disorders, https://doi.org/10.1007/978-3-319-73068-4

AAASM Manual for the Scoring of Sleep and

Associated Events, 2Acid maltase deficiency, 64Actigraphy, 16, 17Adult nonobese diabetics, 98Aerophagia, 142Air swallowing, 142American Academy of Sleep Medicine

(AASM), 2, 10, 16, 17, 19, 104, 105Amyotrophic lateral sclerosis (ALS)

bulbar onset, 47diagnosis, 47frontotemporal dementia, 47, 48incidence, 46limb/spinal onset, 47monomelic, 48

Apnea-hypopnea index, 96Arterial oxygen content (PaO2), 9, 29, 130Arterial oxygen saturation (SaO2), 9, 11–13Average volume assured pressure support

(AVAPS), 124

BBecker’s muscular dystrophy, 64Benign epileptiform transients of sleep

(BETS), 6, 7Bi-level positive airway pressure

(BIPAP), 36, 37, 109, 124, 133Botulism

clinical diagnosis, 85clinical features, 85laboratory and electrodiagnostic

testing, 86treatment, 86types, 85

Botzinger complex, 25Breathing control, 25, 26

CCapillary endothelial cell hyperplasia, 97Capnography, 9, 53Charcot–Marie–Tooth (CMT) patients, 95Circadian rhythm disorders, 67, 68Claustrophobia, 143Common mode rejection ratio (CMRR), 3Continuous positive airway

pressure (CPAP), 3, 9, 15, 16, 18, 90, 96, 109, 121, 123, 124, 133, 139, 141, 142, 148, 149, 161

Contour PAP pillows, 148

DDaily sleep interference scale (DSIS), 95Daytime nap polysomnography (DPSG), 106Diabetic neuropathy (DPN), 98Diaphragm pacing, 55Diaphragm palsy, 96Diurnal tests, 105, 106Dorsal respiratory group (DRG), 25Duchenne muscular dystrophy (DMD),

62, 64–66, 108, 111

EElectrocardiography (EKG), 3, 6, 8,

11–16, 18, 84Electroencephalography (EEG)

abnormal pattern, 5, 6epileptiform-like patterns, 7Jack box, 5mimickers, 6normal pattern, 5procedure, 5sharp artifacts, 6sharp transients

Index

166

Electroencephalography (EEG) (cont.)BETS/SSS, 7hypnogogic hypersynchrony, 7K-complex, 6Mu rhythm, 6posterior slow waves of youth, 7POSTS, 6vertex sharp waves, 7wicket rhythms, 7

Electromyography (EMG), 7, 8Electrooculography (EOG),

2–4, 8, 15–17Emery-Dreifuss muscular

dystrophy, 64End-tidal CO2 monitoring, 15Epworth sleepiness scale (ESS) scoring,

87–89, 106Erasmus GBS respiratory insufficiency

score (EGRIS), 32, 33Excessive daytime sleepiness (EDS),

16, 66, 67, 87, 88Excitatory postsynaptic potentials (EPSP), 5Expiratory positive airway pressure, 123

FFascioscapulohumeral muscular

dystrophy (FSHD), 64, 65Finger pulse oximetry, 9, 17First-generation ventilators, 119Forced vital capacity (FVC),

28, 29, 31, 32, 35, 53, 55, 96, 133, 135

Forehead reflectance oximetry, 17Fourth-generation ventilators, 120

GGuillain-Barré syndrome, 23, 27, 31, 32,

34–36, 133, 135, 136

HHereditary sensory motor neuropathy, 96High-frequency filter, 3High-pass filter, see Low-frequency filterHirayama disease, 48Home mechanical ventilation (HMV), 111Home sleep testing (HST), 17, 159Home ventilation strategies, 134Hypercapnia, 28, 29, 37, 64, 65, 90,

102, 118, 122, 133, 135Hypersomnia, 66Hypnogogic hypersynchrony, 7

IInhibitory postsynaptic potentials (IPSP), 5Insomnia

motor neuron disorder, 52myopathies and muscular

dystrophies, 64, 65Inspiratory positive airway pressure, 122Insufficient sleep syndrome, 66International Classification of Sleep Disorders

(ICSD-3), 62, 64–67, 104International Ten-Twenty System, 4Internuclear ophthalmoplegia (INO), 77Invasive mechanical ventilation, 109–110

JJack box, 5

KK-complex, 6Kennedy’s disease, 46Kleine-Levin syndrome, 66

LLambert–Eaton myasthenic syndrome (LEMS)

causes, 82course of, 82, 83definition, 82diagnosis, 83, 84epidemiology, 82signs and symptoms, 83sleep issues, 89treatment

cholinesterase inhibitors, 84immunotherapy, 84tumor therapy, 84

Laser-assisted uvulopalatopharyngoplasty (LAUP), 10, 19

Late Onset Spinal Motor Neuronopathy (LOSMoN), 46

Limb-Girdle muscular dystrophy (LGMD), 64, 103, 113

Low-frequency filter, 3Low-pass filter, see High-frequency filter

MMaintenance of wakefulness test (MWT), 16Maximal expiratory pressure (MEP), 28, 29,

31, 32, 34–39Maximal inspiratory pressure (MIP), 28, 29,

31, 32, 34, 36–39, 135

Index

167

Mechanical ventilation (MV)CPAP, 121in GBS, 31–33

algorithm, 33, 35cardiovascular autonomic

dysfunction, 33EGRIS, 32endotracheal MV, 32motor weakness, 33MRC sum score, 32prolonged intubation, 35, 36respiratory failure risk, 32risk factors, 33, 34

myasthenia gravis, 36–38peak pressure, 122PEEP, 121plateau pressure, 122tidal volume and minute ventilation, 122

Medical Research Council (MRC) sum score, 32–34

MontageCPAP trial, 12intrathoracic pressure monitoring, 12multiple sleep latency test, 15REM sleep behavior disorder, 14seizures/suspected parasomnias, 13standard polysomnogram, 11

Motor neuron disordersALS

bulbar onset, 47diagnosis, 47frontotemporal dementia, 47, 48incidence, 46limb/spinal onset, 47monomelic, 48

chemo-sensitivity and respiratory control, 50

definition, 44diaphragm weakness, 50, 51PLS, 48PMA, 48SDB (see Sleep disordered breathing

(SDB))sleep diagnosis

nocturnal pulse oximetry, 53PSG, 53pulmonary function testing, 53sleep history and clinical

examination, 52, 53SNIP, 54

sleep disordersinsomnia, 52PLMS, 52RLS, 52

sleep fragmentation, 51sleep history and clinical examination, 53sleep management

comorbid conditions, 54diaphragm pacing, 55insomnia, 54NIV, 55RLS, 54sleep hygiene, 54tracheostomy, 55

sleep stages and respiration, 50SMA

clinical features, 44incidence, 44LOSMoN, 46SMA-LED, 46SPSMA, 46type 0, 45type 1, 45type 2, 45type 3, 45type 4, 45

spinal and bulbar muscular atrophy (X-linked), 46

upper airway resistance, 50Mu rhythm, 6Multifocal motor neuropathy with

conduction block (MMNCB), 96, 97

Multiple sleep latency test (MSLT), 8, 16, 90Muscle-specific tyrosine kinase (MuSK), 75,

76, 78, 79Muscular dystrophies, see Myopathies and

muscular dystrophiesMyasthenia gravis (MG)

causes, 76clinical diagnosis, 78, 79clinical features, 76course, 77definition, 75electrodiagnostic testing, 79epidemiology, 76laboratory testing, 79SDB, 87, 88symptoms

dysphagia, 77leg weakness, 77localized muscle atrophy, 78muscle strength and exhaustibility

measurement, 78myasthenic crisis, 78ocular symptoms, 77respiratory insufficiency, 78tongue weakness, 77

Index

168

Myasthenia gravis (MG) (cont.)treatment

AChEI, 79azathioprine, 80corticosteroids, 80cyclosporine A, 80intravenous immunoglobulin, 81mycophenolate mofetil, 81plasma exchange, 81tacrolimus (FK 506), 81thymectomy, 81

Myasthenic crisis (MC), 36, 38, 77, 78, 81Myopathies and muscular dystrophies

central hypersomnolence disorder, 66, 67circadian rhythm disorders, 67, 68insomnia, 64, 65parasomnias, 67SDB

anatomical airway obstruction, 64coexisting cardiac abnormalities, 64diaphragmatic weakness, 63, 64lung volume reduction, 63normal breathing pattern, 62pathophysiological mechanism, 62, 63prevalence, 62

sleep related movement disorders, 65, 66Myotonic dystrophy (MD), 62, 64–67, 113

NNasal mask interface, 110Negative-inspiratory force (NIF), 28, 31, 35,

37Neurally adjusted ventilatory assist (NAVA),

120Neuromechanical matching, 26, 38Neuromuscular junction (NMJ)

botulism (see Botulism)clinical features, 75definition, 73diagnosis of sleep issues, 89, 90LEMS (see Lambert–Eaton myasthenic

syndrome (LEMS))treatment, 82

motor end-plate with acetylcholine receptors, 74

myasthenia graviscauses, 76clinical diagnosis, 78clinical features, 76course of, 77definition, 75electrodiagnostic testing, 79epidemiology, 75, 76

laboratory testing, 79symptoms, 77, 78treatment, 79–81

sleep disorders, 86, 87treatment, 90

Neuromuscular respiratory failure, 30causes, 26, 30evaluation, 26–28history and examinations, 31laboratory assessment, 28–30MV (see Mechanical ventilation (MV))

Nocturnal hypoventilation, 51, 96, 103, 104, 106, 108, 132

Nocturnal oximetry, 133Nocturnal polysomnogram

(NPSG), 15, 106Nocturnal tests, 106–108Non-invasive positive pressure ventilation

(NIPPV), 108–110, 123, 125Non-invasive ventilation (NIV)

advantages, 118in ambulatory care, 124assisted coughing techniques, 135AVAPS, 124BIPAP, 36, 37, 109, 124BPAP, 133complications, 125–126, 135contraindications, 125–126, 134CPAP, 124, 133definition, 117, 122–123first-generation ventilators, 119fourth-generation ventilators, 120general principles, 119goals, 134indications, 124, 125initiation and management, 122, 123IPPV, 134NAVA, 120PEEP, 133physiological benefits, 117positive pressure ventilator, 120second-generation ventilators, 120SRBD, 108, 109third-generation ventilators, 120

OOrbicularis oris weakness, 77Obstructive sleep apnea (OSA), 9, 17,

50, 52, 62, 64, 84, 86, 87, 96, 97, 112, 121, 124, 139, 140, 142–144

Overlap syndrome, 51Overnight oximetry, 107–108

Index

169

PParasomnias, 67Parasternal intercostals, 24Pediatric NMD, 101

diaphragmatic weakness, 102respiratory physiology, 102sleep-associated nocturnal respiratory

disorders, 103SRBD (see Sleep-related breathing

disorders (SRBD))Pediatric polysomnography

active sleep, 18indeterminate sleep, 18physiological variables, 17quiet sleep, 18respiratory scoring, 18

Periodic limb movements (PLMS), 7, 52, 89Peripheral arterial tonometry (PAT)

calibration, 11cost, 10measurement, 10

Peripheral neuropathydiaphragm palsy, 96MMNCB, 96restless legs syndrome, 97, 98risk factors, 96sleep apnea

adult non-obese diabetics, 98CMT patients, 95compound muscle action potential, 96CPAP therapy, 97DPN, 98DSIS, 95sensory conduction velocity, 97severity, 96supramaximal stimulation, 97

spinocerebellar ataxia, 97Phrenic motor neurons, 25Pittsburgh sleep quality index (PQSI), 90Pneumography, 9Polysomnogram study (PSG), 7, 10, 11,

17–18, 53, 55Polysomnography, 5, 10, 11, 17, 97

actigraphy, 16, 17airflow monitoring, 8, 9bi-level titration, 15body positioning and snoring, 9children (see Pediatric polysomnography)CPAP titration, 15definition, 1digital polygraphs, 3EEG (see Electroencephalography)EKG, 8EMG, 7, 8

end-tidal CO2, 15EOG, 8evolution, 1, 2gastroesophageal studies, 10HST, 17indications, 10MSLT, 16MWT, 16NPSG, 15PAT

calibration, 11cost, 10montage (see Montage)

RBD, 16sleep laboratory/sleep center, 4sleep staging and scoring, 2, 3split study, 15SRBD, 106–107technical considerations, 3, 4

Positive airway pressure (PAP) therapybattery-powered PAP machines, 148claustrophobia, 143contour PAP pillows, 148device maintenance, 148during travel, 148education, 140gel/rubber cushion, 143humidification problems, 146–148machines, 140mask air-leak, 144mask interface, 143mask pressure, 144–145mouth air-leak, 144noise level, 145–146patient compliance, 149patient day-to-day challenges, 140pressure intolerance, 141, 142sleep apnea, 139, 140symptoms, 149tubing problem, 146

Positive end expiratory pressure (PEEP), 121

Positive occipital sharp transients (POSTS), 6Positive pressure ventilator, 120Posterior dominant rhythm/posterior

alpha rhythm, 5Posterior slow waves of youth, 7Pre-Botzinger complex, 25, 38Primary lateral sclerosis (PLS), 48Progressive muscular atrophy (PMA), see

Sporadic Lower motor neuron syndromes

Pseudoperiodic lateralized epileptiform discharges (PLEDs), 7

Index

170

RRapid eye movement (REM) sleep, 96Rapid repetitive nerve stimulation (RNS), 86REM behavior disorder (RBD), 16REM sleep without atonia (RSWA), 52, 67Renshaw cells, 25Resistance to ischemic conduction block

(RICB), 97Respiratory effort related arousals (RERAs),

8, 17Respiratory failure

diagnosis, 132laboratory assessment, 28 (see

Neuromuscular respiratory failure)NMD classification, 130, 131pathophysiology, 131, 132symptoms, 133

Respiratory function, neurophysiology, 130Respiratory inductive plethysmography (RIP), 9Respiratory magnetometers, 9Respiratory muscles

expiratory muscles, 24, 25inspiratory muscles, 23, 24

Restless legs syndrome (RLS), 52, 65, 66, 87–89, 97, 98

Retrotrapezoid nucleus/parafacial respiratory group (RTN/pFRG), 25

Riluzole, 48R-K scoring system, 5Rostral ventral respiratory group (rVRG), 25

SScalene muscles, 24Scapuloperoneal SMA (SPSMA), 46Second-generation ventilators, 119–120Seronegative MG, 76, 79Single-breath test, 90Sleep disordered breathing (SDB)

central sleep apnea, 52mixed sleep apnea, 52nocturnal hypoventilation, 51obstructive sleep apnea, 51

Sleep fragmentation, 51Sleep onset REM periods (SOREMPs, 67Sleep-associated nocturnal respiratory

disorders, 103Sleep-Disordered Breathing in Neuromuscular

Disease Questionnaire (SINQ-5), 89Sleep-related breathing disorders (SRBD), 112

adverse clinical effects, 110assessment, 105clinical history, 104, 105definition, 101diagnostic and clinical evaluation, 104

diurnal tests, 105, 106HMV, 111invasive mechanical ventilation, 109, 110nasal mask interface, 110NIPPV, 108, 109overnight oximetry, 107, 108polysomnography, 106–107predictors, 105prevalence, 101sleep-related central hypoventilation, 103symptoms, 103

SMA, see Spinal muscular atrophy (SMA)Small sharp spikes (SSS), 7Sniff nasal inspiratory pressure (SNIP), 54Spinal and bulbar muscular atrophy

(X-Linked), 46Spinal muscular atrophy (SMA), 112, 113

clinical features, 44incidence, 44Kennedy’s disease, 46LOSMoN, 46SMA-LED, 46SPSMA, 46type 0, 45type 1, 45type 2, 45type 3, 45type 4, 45

Spinocerebellar ataxia (SCA), 97SPSMA, see Scapuloperoneal SMA (SPSMA)Strain gauges, 9

TTENSILON test, 79Thermistors, 9Thermocouples, 9Third-generation ventilators, 120Thymomas, 76Thymus gland, 76Tracheostomy ventilation, 135Transdiaphragmatic pressure (Pdi), 29

VVertex sharp waves, 7Vital capacity, 27, 28, 32, 35, 36, 38, 39

WWatchPAT system, 10Wicket rhythms, 7Willis Ekbom disease, 88, 89Willis-Ekbom disease, see Restless legs

syndrome (RLS)

Index