PART 2 VII XII. Horner Syndrome

Transcription

1 PART 2 CN V VII V XII Horner Syndrome 1

2 Neuromuscular Disease Clinical presentations, signs and symptoms Lecture presented at the EAN Teaching Course in Burkinje Faso, Nov Wolfgang Grisold (1) Anna Grisold (2) Stefan Meng (3) (1) Ludwig Boltzmann Institute for Experimental und Clinical Traumatology Donaueschingenstraße 13 A-1200 Vienna, Austria (2) Department of Neurology, Allgemeines Krankenhaus, University of Vienna, Austria (3) Department of anatomy, University of Vienna, Austria 2

3 COI All authors: No COI to report. The study was not funded. 3

4 Support: EMG - Reindorfgasse, Vienna Neuroscience Winter meeting society, Vienna Austria Widdicombe Enterprises Ludwig Boltzmann Institute for Experimental und Clinical Traumatology, Vienna, Austria 4

5 Is it neuromuscular? Combinations? Case history Clinical presentation new concepts examples 5

6 Neuromuscular System (A common construct) (Extracranial parts of the Cranial nerves) Nerve Roots Plexus Peripheral nerves Neuromuscular junction Muscle Autonomic system 6

7 NCV/EMG/US Upper extremities Neuropathies? Neuropathies: How often? Median (CTS) 103,00 Ulnar 25,00 Radial 2,50 Cut. Femoral 10,00 Disc (> 50 yrs) Cervical (aged) 600,00 Lumbar (aged) 1079,00 PNP DM II: 6400 Pro Patient years Source: Callaghan, 7 Ann Arbor 2016, USA

8 Combinations of neuromuscular and central nervous system. Examples: MND/ALS Some hereditary neuropathies Myotonic dystrophy etc. 8

9 Case history Family history (tree) Diseases, medications, Exposition, trauma Onset, duration, acute, chronic progressive, fluctuating, episodic Symptoms: motor, sensory, autonomic movement, composite Acquired/genetic Before examining the patientcreate hypothesis 9

10 Time Acquired Acute episo Hereditary 10

11 Symptoms and signs Motor Sensory Composite function Autonomic Neuropathic Pain Distribution 11 11

12 Symptoms and signs Skin Skeletal system 12 12

13 Distribution of weakness, atrophy Strength (BMRC) Motor Time: Fluctuating, episodic Muscle volume Atrophy Hypertrophy Phenotype distribution: myopathy, polio, club foot 13 13

14 Winging 14

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16 Proximal weakness 16

17 Carcinomatose Neuromyopathy 17 EMG

18 Percussion myotonia 18

19 Muscle hypertrophy Examples Pseudohypertrophy Endocrinopathy Focal muscle hypertrophy Overuse infections Amyloid 19

20 Steroid myopathy 20

21 Movement Fasciculation Myokymia Rippling Stiffness, myotonia Percussion myotonia Cramps Tongue Ultrasound 21

22 Quality Sensory assessment Sensory Instruments Type of fiber lesion Tinel Hofmann sign Sclerotoma, myotoma, neurotoma 22

23 Instruments 23

24 B A: superficial peroneal nerve B. Infrapatellar nerve Tinel Hofmann: red circle A 24

25 Foot 25

26 Simple (but complex) functions: Test Composite function! Opening and closing buttons Recognition of objects Balance: Romberg Stance on one leg Can you put on your trousers standing freely one leg? Muscle: Hands up Standing up from squat Push ups 26

27 Dry mouth, dry eyes? Ask for Autonomic dysfunction Voiding Sexual dysfunction Orthostasis Gastrointestinal 27

28 Pain characteristics (stabbing, shooting, electric) Neuropathic Pain Distribution- cave Allodynia Sensory, motor involvement Elicited by : eg Tinel Hofmann 28

29 Neuropathic Pain conditions Acute episodic Painful neuropathy Small fiber Trauma causalgia Localized Surgery Erythromelalgia Phantom- pain PRURITUS 29

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31 Painful neuropathies Alcohol Metals Drugs (eg. CIPN) Immune-mediated, Vasculitis Hereditary ( eg Fabry) 31

32 Skin Haemangioma 32 Waterhouse F. syndrome

33 Thickening of nerves Greater auricular nerve (intraoperative) 33

34 Radiofibrosis syndrome 34

35 Hyperkeratosis in denervation 35

36 Skin Lymphoma 36

37 Hand Foot Syndrome 37

38 Skeletal system 38

39 Focal dysfunction/weakness 39

40 Distribution: Neuropathies and Radiculopathies 40

41 Further Ancilliary Tests NCV/EMG Imaging: ultrasound, MRI, CT Autonomic test Biopsy Laboratory Genetics 41

42 Nerve Ultrasound 42

43 Neuromuscular lesions CN Plexus lesions Mononeuropathies Polyneuropathies Neuromuscular transmission Muscle 43

44 Facial nerve: 3 different problems VII 44

45 Cases of tongue involvement 45

46 The Plexus Cervical Brachial Lumbar Sacral (Coccygeal) 46

47 Phrenic nerve palsy Local tumor affecting the cervical plexus 47

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49 Lumbar, sacral - Anatomy 49

50 Sacral Plexus Vascular 50

51 Mononeuropathy 51

52 CTS 52

53 Acute Swelling and median nerve dysesthesia 53

54 Acute Swelling and median nerve dysesthesia. Ultrasound : synovial cyst, yellow: median nerve. 54

55 Ulnar nerve lesion Median nerve: Thenar atrophy 55

56 SNUS 56

57 Chemotherapy paravasate, damage of cutaneous nerve 57

58 Radial nerve palsy after fracture 58

59 Ultrasound shows preserved continuity of the radial nerve 59

60 Diagnosis of nerve tumors 60

61 Hip operations Hip operations Femoral nerve Sciatic nerve (traction) Cutaneous nerve 61 From: Sunderland

62 Arrow points towards screw from the acetabulum in contact with the sciatic nerve 62

63 Nerve reconstruction Neuroplasticity: Brachial plexus surgery: Phrenic nerve and intercostal Contralateral C 7 transfer Hand transplant 63

64 M. Tibilialis posterior transfer 64

65 Polyneuropathy Distribution Statistics symmetric Asymmetric 65

66 Frequency Diabetes Toxic neuropathies HIV Immune mediated Infection 66 66

67 Hereditary Small fiber Painful neuropathies Neoplastic Immune mediated Paraneoplastic 67 67

68 Frequency of neuropathy Most frequent: Diabetes, cryptogenic and toxic. Inflammatory, genetic, etc less frequent. 68

69 Diabetes Symmetric Distal symmetric neuropathy Diabetic autonomic neuropathy Pseudotabic type CIDP like 69

70 Diabetes Asymmetric forms Multiplex Truncal neuropathy Diabetic amyotrophy 70

71 Diabetes Prediabetic neuropathy Insulin neuritis 71

72 Diabetes CN: III, (VII) Mononeuropathy: CTS Diabetic muscle infarct Diabetic cachexia 72

73 Chemotherapy induced neuropathy 73

74 Chemotherapy induced neuropathy 74

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77 Toxic neuropathies Alcohol Industrial agents Historical: Spanish toxic oil syndrome U boat oil TCE neuropathy 77

78 Infection HIV Leprosy CMV Herpes VII Mononeuropathy Motor involvement Myelopathy 78

79 HIV and the PNS ALS-variant syndrome Autonomic Toxic Drug treatment CIDP Cytomegalovirus DILS Drug-induced Guillain-Barré Mononeuritis multiplex Neuromyotonia Sensory motor polyneuropathy VII nerve palsy Mononeuropathy Brachial plexopathy 79

80 Hereditary neuropathies CMT Fabry Amyloid Porphyria 80

81 CMT detected in an asymptomatic patient. Left: foot deformity. 81

82 NMT MG (AchR pos) MusK LRP4 Seronegative LEMS Neuromyotonia Other NMT disorders 82 82

83 83 Ocular myasthenia

84 Triple furrow tongue 84

85 Lambert Eaton myasthenic syndrome Autoimmune presynaptic disorder 50 % paraneoplastic (SCLC) Electrophysiology: Increment Clinical: proximal weakness, facilitation of reflexes 85

86 Neuromyotonia 86

87 Muscle Distribution Onset/Course Acquired/genetic 87

88 Muscle Hereditary Inflammatory Endocrine Metabolic Deposition Toxic 88 88

89 Muscle Infection Systemic disorders Myotonia Critical illness Sarcopenia Cachexia 89 89

90 Hyperlordosis Shoulder girdle weakness Shortened achilles tendons 90

91 A quick test for myopathic weakness 91

92 Mitochondrial myopathy Distribution and CN Face Optomotor Bulbar FSH, OPD; Myotonia, mitochondrial myopathy NMT (MG),OPD, OPD, DM, IBM, Myotonia Proximal >> distal 92

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94 Myositis DM (25 %) (Anti-TiF1γ ) PM (10 %) Necrotizing myopathy (NAM) 94

95 Volar atrophy IBM 95

96 Axial myopathy 96

97 Toxic myopathies, Hyper CK emir Toxic myopathies: see: Hyper CK emia: EFNS guidelines on the diagnostic approach to pauci- or asymptomatic hyperckemiaeuropean Journal of Neurology 2010, 17: (download) 97

98 Motor Neurone Disease MND ALS Variations Genetic forms Kennedy syndrom 98

99 ALS Variants Genetic types 99

100 Flail arm syndrome Atrophy of shoulder girdle (flail arm) Thigh and leg muscles preserved 100

101 Sources: 101

102 Join the World Brain Day 2018! Check the WFN website for information. 102

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