眼外肌麻痹.pptVIP

眼外肌麻痹 Neuromuscular junction Myasthenia Gravis Botulism Muscle Thyroid Mitochondrial Kearns-Sayre Progressive External Ophthalmoplegia MNGIE Congenital Myopathy Centronuclear Myopathy: Infantile Multicore Hereditary myopathies Oculopharyngeal Muscular Dystrophy Oculopharyngodistal myopathy Inclusion body myopathy with joint contractures ophthalmoplegia Early-onset myopathy with external ophthalmoplegia Congenital hereditary ophthalmoplegias Orbital myositis Neuropathies Neuropathies （ III, IV VI ） Botulism(肉毒中毒) (pupils involved early) CANOMAD Diabetes (pupil sparing III) Miller-Fisher syndrome Hereditary Congenital Mass: Neoplasm; Aneurysm Superior oblique myokymia Other: Brainstem Sensory (± Ataxic) neuropathy with anti-GD1b (disialosyl) antibodies CANOMAD ChronicAtaxicNeuropathyOphthalmoplegiaM-proteinAgglutinationDisialosyl antibodies Epidemiology Male Female Onset age: 39 to 76 years Clinical Onset: Acute, Subacute or Chronic Sensory loss (100%) Distal Symmetric Large small fiber modalities Legs arms Paresthesias Tendon reflexes: Absent Strength Normal in 50% Weakness (50%): Mild; Distal Gait Ataxic (Sensory) + Romberg Tandem relatively spared Cranial nerves Ophthalmoplegia (50% to 90%) Trigeminal (50%): Perioral paresthesias Facial (25%) Bulbar (66%) Course Usually slowly progressive over months to years Variants: Acute, Relapsing or Stepwise progressive sensory neuropathy Antibodies IgM M-protein: Often; κ λ Cross reactivity: Other gangliosides containing disialosyl groups GD3; GD2; GT1b; GQ1b NOT GM1; GM3; GD1a Anti-Pr2 activity Cold agglutinins (50%) Bind to: Dorsal root ganglion cells; Nerve terminals Similar neuropathy after treatment with monoclonal anti-GD2 antibody IgG anti-GD1b antibody associations Acute sensory neuropathy Polyclonal antibody M-protein: 93%; κ or λ Other lab Sedimentation rate: High CSF: Moderately high protein in some patients MRI: Occasiona