重症疾病性神经肌肉病(英文课件).pptVIP

Clinical Features Major feature is flaccid weakness Tendon reflexes depressed Ophthalmoplegia may be present Myalgias are uncommon Diagnostic criteria of CIM ● SNAP amplitudes 80% of the lower limit of normal ● Needle EMG with short-duration, low-amplitude MUPs with early or normal full recruitment, with or without fibrillation potentials ● Absence of a decremental response on repetitive nerve stimulation Diagnosis ● Muscle histopathologic findings of myopathy with myosin loss ● CMAP amplitudes 80% of the lower limit of normal in two or more nerves without conduction block ● Elevated serum creatine kinase (CK） ● Demonstration of muscle inexcitability *For a definite diagnosis of critical illness myopathy, patients should have all of the first five features. Nerve conduction studies Low-amplitude CMAPs Long duration CMAPs Normal SNAPs Phrenic nerve conduction normal latencies diaphragm CMAP amplitudes reduce Electrophysiologic Features EMG Fibrillation potentials and positive sharp Motor unit potentials low amplitude and short duration Electrical inexcitability by direct needle stimulation Features of the histopathology in thick filament myosin loss (Fig. 5) Electron microscopy reveals selective loss of thick (myosin) filaments (Fig. 6) Inflammatory changes are conspicuously absent Morphologic Features Figure. 5 Muscle histopathology in a critically ill patient with thick filament myosin loss. (original magnification, 100) (courtesy of Dr. Andrew Engel). Figure. 6 Electron microscopy of muscle in CIM. (original magnification, 44,000) (courtesy of Dr. Andrew Engel). CIP Direct needle stimulation of the muscle Electrical inexcitability in CIM There is a response in CIP (Fig. 7) Serum CK Muscle biopsy FIGURE. 7 Results of direct and indirect muscle stimulation. CMAPs from the anterior tibial muscles of a patient with critical illness polyneuropathy (left) and critical illness myopathy (right). Subtypes of CIM Muscle morph