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THAISE AGRA TEIXEIRA.  GENERALIDADES THAISE AGRA TEIXEIRA.

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Presentation on theme: "THAISE AGRA TEIXEIRA.  GENERALIDADES THAISE AGRA TEIXEIRA."— Presentation transcript:

1 THAISE AGRA TEIXEIRA

2  GENERALIDADES THAISE AGRA TEIXEIRA

3  QUADRO CLINICO  FRAQUEZA MUSCULAR FLUTUANTE  FADIGABILIDADE  MUSCULATURA ESQUELETICA ESTRIADA THAISE AGRA TEIXEIRA

4  QUADRO CLINICO  ENVOLVIMENTO MUSCULAR ▪ MUSCULOS OCULARES ▪ MEMBROS ▪ EXTENSORES DO PESCOÇO ▪ TRONCO THAISE AGRA TEIXEIRA

5  CLASSIFICAÇÃO  IDADE ▪ NEONATAL – 10-15% sintomatico ▪ ADULTO  ANTICORPOS ANTI-AchR ▪ POSITIVO ▪ 85% GENERALIZADA ▪ 50-60% OCULAR ▪ NEGATIVO-10-20% ▪ ANTICORPOS ANTI- MUSK(muscle specific protein kinase)

6  CLASSIFICAÇÃO THAISE AGRA TEIXEIRA

7  CLASSIFICAÇÃO  ETIOLOGICA ▪ AUTOIMUNE ADQUIRIDA ▪ NEONATAL TRANSITORIA ▪ MEDICAMENTOSA ▪ CONGENITA THAISE AGRA TEIXEIRA

8  CLASSIFICAÇÃO  ETIOLOGICA ▪ MEDICAMENTOSA

9 THAISE AGRA TEIXEIRA

10 Schema of normal neuromuscular junction. Thanvi B R, Lo T C N Postgrad Med J 2004;80: Copyright © The Fellowship of Postgraduate Medicine. All rights reserved.

11 Schema of neuromuscular junction in myasthenia gravis (note: widened synaptic cleft, reduced number of acetylcholine receptors, and simplification of postsynaptic membrane). Thanvi B R, Lo T C N Postgrad Med J 2004;80: Copyright © The Fellowship of Postgraduate Medicine. All rights reserved.

12  FATORES QUE PIORAM QUADRO CLINICO  CALOR  ESTRESSE  INFECÇÃO  HIPERTIREOIDISMO  GRAVIDEZ  ALTERAÇÕES K/Ca THAISE AGRA TEIXEIRA

13  Box 1: Characteristic clinical features of myasthenia gravis  Ocular muscle weakness is usually the initial presentation and may be the only feature throught the course in about 10% of patients. The ptosis (and diplopia) is exacerbated by the prolonged upward gaze toward a fixed target for one minute.  In most cases, weakness progresses from ocular muscles to involve other muscles in a craniocaudal direction. The weakness of intercostal muscles and diaphragm leads to dyspnoea on exertion or at rest. The orthopnoea with rapid resolution on sitting up and diaphragmatic paradox are important clinical signs of neuromuscular breathlessness.

14  Box 1: Characteristic clinical features of myasthenia gravis  Ocular muscle weakness is usually the initial presentation and may be the only feature throught the course in about 10% of patients. The ptosis (and diplopia) is exacerbated by the prolonged upward gaze toward a fixed target for one minute.  In most cases, weakness progresses from ocular muscles to involve other muscles in a craniocaudal direction. The weakness of intercostal muscles and diaphragm leads to dyspnoea on exertion or at rest. The orthopnoea with rapid resolution on sitting up and diaphragmatic paradox are important clinical signs of neuromuscular breathlessness.

15  Box 1: Characteristic clinical features of myasthenia gravis  Deep tendon reflexes are intact or may be brisk.  There are no objective sensory deficits.  In severe cases, respiratory failure may ensue, needing intubation and mechanical ventilation.  Symptoms may fluctuate and there may be remissions of variable periods, particularly at early stages.

16  DIAGNOSTICO LABORATORIAL  Edrophonium (Tensilon test ▪ Easy to administer, no need for expensive equipment. Limitations: false positives and false negatives, occasional serious side effects, for example, hypotension and arrhythmias THAISE AGRA TEIXEIRA

17  DIAGNOSTICO LABORATORIAL  Ice test ▪ Nearly 80% sensitive and highly specific to diagnose myasthenic ptosis, no need for cardiac monitoring, can be done in an office setting. Not commonly used, applicable only when ptosis is present THAISE AGRA TEIXEIRA

18  DIAGNOSTICO LABORATORIAL  AChR antibody in serum ▪ Nearly 80%–85% sensitive in generalised and 60%–70% in ocular myasthenia gravis, highly specific, non-invasive, now widely available. May be the diagnostic “gold standard”. Titres do not always correspond with the severity of myasthenia gravis THAISE AGRA TEIXEIRA

19  DIAGNOSTICO LABORATORIAL  Repetitive nerve stimulation ▪ Sensitivity around 75%. Uncomfortable to patient, not specific. Not reliable if the limb is cold, or patient on acetylcholinesterase inhibitors THAISE AGRA TEIXEIRA

20 A typical recording of compound muscle action potentials with repetitive nerve stimulation at low frequency in a patient with myasthenia gravis. Note the gradual decline in the amplitude of the compound muscle action potential with slight improvement after the fifth or sixth potential. THAISE AGRA TEIXEIRA

21  DIAGNOSTICO LABORATORIAL  Single fibre electromyography ▪ Most sensitive test. Needs costly equipment, not specific THAISE AGRA TEIXEIRA

22 Single-fiber electromyography showing so-called jitter phenomenon (second action potential wave group). THAISE AGRA TEIXEIRA

23  DIAGNOSTICO LABORATORIAL  Anti-MuSK antibodies ▪ Found in a subset of seronegative myasthenia gravis. Only recently described; not widely available yet THAISE AGRA TEIXEIRA

24  DIAGNOSTICO LABORATORIAL  Computed tomography/MRI of chest ▪ To diagnose associated thymic tumours. Non-invasive. Greater yield in patients >40 years of age. May be used post-thymectomy to look for residual thymic tissue in patients who deteriorate unexpectedly THAISE AGRA TEIXEIRA

25  DIAGNOSTICO LABORATORIAL  Computed tomography/MRI of chest ▪ To diagnose associated thymic tumours. Non-invasive. Greater yield in patients >40 years of age. May be used post-thymectomy to look for residual thymic tissue in patients who deteriorate unexpectedly THAISE AGRA TEIXEIRA

26 CT scan of chest showing an anterior mediastinal mass (thymoma) in a patient with myasthenia gravis. THAISE AGRA TEIXEIRA

27 Cogan sign. Patient changes gaze from downward position (A) to primary position (B). Both lids are seen to overshoot in twitch (B) before gaining their initial ptotic position (D). In this case, Cogan sign is seen more obviously on right, whereas left lid is more ptotic. THAISE AGRA TEIXEIRA

28 Increasing left ptosis developing upon sustained upward gaze in patient with myasthenia gravis (A through F). Note limited elevation of left eye, denoting superior rectus palsy (A). A initially, C after around 20 seconds, F after 1 minute. THAISE AGRA TEIXEIRA

29  TRATAMENTO  ANTICOLINESTERASICOS  IMUNOSSUPRESSORES ▪ CORTICOIDES SISTEMICOS ▪ AZATOPRINA ▪ CICLOSPORINA ▪ METOTREXATO ▪ CICLOFOSFAMINA  TIMECTOMIA  PLASMAFERESE  IMUNOGLOBULINA THAISE AGRA TEIXEIRA

30  TRATAMENTO  ANTICOLINESTERASICOS THAISE AGRA TEIXEIRA Efeitos dos anticolinesterásicos Bradicardia Hipotensão Secreções excessivas Broncoconstrição Sufocamento Hipermotilidade gastrintestrinal Redução da pressão intra-ocular Miose neostigmine piridostigmine edofrônio

31  TRATAMENTO  IMUNOSSUPRESSÃO ▪ CORTICOIDES SISTEMICOS ▪ AZATIOPRINA ▪ CICLOSPORINA ▪ METOTREXATO ▪ CICLOFOSFAMINA THAISE AGRA TEIXEIRA

32  TRATAMENTO  TIMECTOMIA THAISE AGRA TEIXEIRA

33  TRATAMENTO  PLASMAFERESE  IMUNOGLOBULINA THAISE AGRA TEIXEIRA

34 Figure. (A) A 61-year-old woman with marked generalized myasthenia gravis, 2 days after plasma exchange treatment, with only minimal spontaneous ptosis at baseline. Toyka K V Neurology 2006;67: ©2006 by Lippincott Williams & Wilkins

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