16. Two pelvic bones (left and right) articulating with the axial skeleton via the sacrum. The pelvic bones are each made up of three bones: Ilium ishium pubis These bones are fused by puberty to form the pelvic bone. The pelvic girdle can only move as a unit (L and R shoulder girdles can move independent of each other
17. Hip flexors iliopsoas(iliacus, psoas major and minor) pectineus rectus femoris sartorius gracilis tensor fasciae latae rectus femoris Hip extensors gluteus maximus biceps femoris semitendinosus semimembranosus
18. Hip (thigh) abductors gluteus medius gluteus minimus tensor fasciae latae Hip (thigh) adductors adductor brevis adductor longus adductor magnus gracilis pectineus
19. Hip Internal Rotators Gluteus medius Gluteus minimus semimembranosis semitendinosis Hip External Rotators Adductor magnus Adductor longus External Rotators Gluteus maximus Gluteus medius Sartorius Biceps femoris
20. Proximal limb girdle syndromes Most common pattern of muscle weakness Predominantly the proximal muscles of legs and arms Distal muscles are also involved in much lesser extent. Neck flexors and extensors also frequently involved. Seen in most hereditary and acquired myopathies
21. MRC scale expanded version 5 normal power 5- equivocal, barely detectable weakness 4+ definite but slight weakness 4 able to move against gravity and some resistance 4- capable of minimal resistance 3+ capable of transient resistance collapse abruptly 3 active movement against gravity 3- able to move against gravity but not throuh full range 2 able to move with gravity eliminated 1 trace contraction 0 no contraction
23. Diagnosis based on pattern of inheritance Autosaomal dominant FSHD LGMD type-1 Autosomal recessive LGMD Metabolic myopathy X-linked Duchenne Becker Emery Dreifuss
24. Temporal evolution Duchenne muscular dystrophy usually identified by 3yrs of age Most FSHD and LGMD begins in adolescence or later Dermatositis occurs in children and adults Polymyositis rarely occurs in children but in any decade in adult life Inclusion body myositis occurs most commonly in te elderly. Myopaties with acute or sub acute progression- dermatomyositis and polymyositis Chronic slow progression over years- muscular dystrophies Non progressive weakness – congenital myopaties
35. Facioscapulohumeral dystrophy Onset is between 7-27 years Initially face is involved, in particular zygomaticus , orbicularisoculi & orbicularisoris Masseter ,temporalis , extraocular & pharyngeal muscles are spared
36. Scapular fixators –latissimusdorsi, lower trapezius ,rhomboids & serratus anterior are involved Deltoid is spared Biceps weakness >triceps weakness Forearm muscles are normal POPEYE EFFECT
37. Polyhill sign six "hills" (1) enlarged infraspinatus muscle (2) superior angle of scapula (3) prominence of acromio-clavicular joint due to wasting of trapezius muscle, (4) prominence of infero-lateral part of the deltoid muscle (5) some preserved bulk in the middle of the wasted biceps brachii (6) prominent brachioradialis muscles
38. Inflammatory myopaties polymyositis, dermatomyositis - Proximal > distal. Ocular and facial muscles almost never affected. Symmetrical involvement. IBM - Proximal, distal and quadriceps, asymmetrical involvement.
39. Distribution of weakness Scapuloperoneal- scapuloperonealdystrophy, emery dreifuss dystrophy, LGMD 1B, LGMD-2A, sarcolycanopathies, LGMD-2I, acid maltase deficiency. Facioscapular- FSHD Distal arm proximal leg weakness involving quadriceps – inclusion body myositis. In addition, weakness is assymetrical. Ptosis and facial weakness with out ophthalmoplegia- FSHD and myotonic dystrophy.
56. Myopathies with myoglobinuria Familial Sarcoglycanopaties hypokalemic periodic paralysis Metabolic myopaties Duchenne and becker Inflammatory myopaties Acquired causes Drugs and toxins heat stroke NMS Trauma infections
57.
58. 60% of infants with SMA type I are floppy babies at birth. Prolonged cyanosis may be noted at delivery.
59. In some instances, the disease can cause fulminant weakness in the first few days of life. Such severe weakness and early bulbar dysfunction -> mean survival of 5.9 months.
71. DD of increased CK Myopaties Dystropinopaties(carrier state) drug induced Inflammatory myopaties Metabolic myopaties Muscular dystrophies Nonneuromuscular Hypothyroidism, HYpoparatyroidism Malignant Hyperthermia Medications Race, sex Motor neuron disease ALS Post polio syndrome Neuropathies BS & CIDP
72. Muscle biopsy Tissue can be obtained by either open or closed procedure Muscles that are severely weak (MRC grade 3 or less) should not be biopsied. Muscles recently studied by needle EMG should be avoided; artifacts created by needle insertion. Biopsy should be taken from muscles having grade 4 strength Muscle of choice- upper limb –deltoid, lower limb- vastuslateralis.
73.
74. In dermatomyositis - perifascicular atrophy & expression of immune attack complex