1. Interesting Case Presented by Hong – Sarawoot May 2010

3. สิทธิ์การรักษา 30บาท รพ.อื่น

8. มีบุตร 1คนแข็งแรงดี ไม่เคยมีประวัติแท้งบุตรมาก่อน

9. ไม่เคยผ่าตัด

10. สูบบุหรี่วันละ 1- 10 มวนมา 3 ปี

12. CVS: apical impulse at 5 th ICS at Lt.MCL, no apical or parasternal heaving, no murmur Abdomensoft, not tender, liver and spleen cannot be palpated Neurological Examination: Physical Examination

13. Physical Examination Neurological Examination: Normal motor tone, motor power grade V all extremities, no sensory deficit, Esotropia, VA 20/20 both eyes, pupil 3 mmBRTL, normal visual field EOM 100 100 0 + 100 100 + 100 100 100 Fundoscopic exam: papilledema both disc

14. Physical Examination CN V, VI, VII, VIII, XI, X, XI, XII Stiff neck positive(terminal) Cerebellar sign normal Deep tendon reflex all 2+ Babinski ’s absent

15. 1. Suspected increase intracranial pressure with isolated sixth cranial nerve palsy Rt. for 5 days Problem List

16. 1. Venous Stroke 2. Brain parenchyma and Meninges: Chronic meningitis, Brain tumor with complication 3. Hydrocephalous(obstructive or any cause) 4. Idiopathic intracranial hypertension 5. Subarachnoid hemorrhage(less likely) DDx

18. Ophthalmoplegia

19. Esotropia

20. Nuclear Lesion

21. Ophthalmoplegia

22. Esotropia

23. Facial Weakness

24. Altered LOC

26. Meningeal tumors

27. Pituitary Adenoma

29. Ischemia (pontine infarction)

30. Central Demyelinating Disorders

32. Investigation(16/4/53) CBC: Hb 14.3 g/dl, Hct 45.1%,WBC 13400/cumm3, N 60.8%, L 27.9%, Plt 279,000, MCV 84 fl PT 11.3 sec, aPTT 22.3 sec BUN 17 mg/dl,Cr 0.8 mg/dl, Ba 141 mmo/lL, K 4.1 mmol/L, Cl 106 mmol/L, CO2 26 mmol/L

33. Investigation CT Brain with contrast

34. CT brain with contrast: Empty delta sign

35. Investigation Lumbar puncture: OP 50 cmH2O, CP 30 cmH2O CSF WC 0, RC 0, Glucose 55 mg/dl, Protein 22.5 mg/dl CSF gram stain not found, AFB not found, no encapsulated yeast, CryptoAg negative, VDRL negative BS 84 mg/dl AntiHIV negative

36. Progress Note 17/4/53 หลังจาก LP มีปวดศีรษะเล็กน้อย ไม่มีไข้ EOM 100 100 0 + 100 100 + 100 100 100

37. Investigation ANA speckle 1:160, Peripheral 1:160, Homogenous 1:160 ANA CSF homogenous 1:40 homogenous pattern AntiHIV negative

38. Investigation Lumbar puncture(19/04/53) OP 34, CP 17 CSF RC 25/cumm3, WBC 0, protein 19.6 mg/dl, glucose 51 mg/dl, BS 68 mg/dl

39. Progress Note 20/4/53 หลังจาก LP ไม่มีปวดศีรษะ ไม่มีไข้ EOM 100 100 40 + 100 100 + 100 100 100

40. Progress Note 20/4/53 ไม่มีปวดศีรษะ ไม่มีไข้ แขนขาแรงปกติดี EOM 100 100 60 + 100 100 + 100 100 100

41. MRI and MRV

44. MRI and MRV Diffuse venous sinus and cortical vein thrombosis. Suspected thrombosis in Lt. upper jugular vein is also present No evidence infarction or hemorrhage

45. Treatment Enoxaparin 0.6 cc sc q 12 hr Challenge warfarin(5)1x1-> warfarin(3)1x1 ohs D/C

47. Protein C, Protein S level, antithrombin III level

48. Lupus anticoagulant

49. Anti beta2 glycoprotein IgG, IgM

51. WarfarinEmbryonopathy(ACC/AHA guideline vulvular heart disease 2006) Incidence 4-10%, lower if dose < 5 mg/day Midfacehypoplasia, stippled chondral calcification,scoliosis, short proximal limbs, and short phalanges Risk at 6-12 wks of pregnancy Safe if first 6 wks and 2nd and 3rd trimester

52. Warfarin(ACC/AHA guideline vulvular heart disease 2006) During labor and delivery cause bleeding in the fetus and fetal cerebral hemorrhage Increase incidence of spontaneous abortion, prematurity, stillbirth Change to heparin several weeks before delivery

53. During Pregnancy Change to UFH or LMWH during 6-12 wks Stop and change to Heparin during 2-3 wks After 36 wks discuss risk UFH in low risk for fetus but increase risk of infection, osteoporosis, HIT

54. Cerebral Venous Sinus Thrombosis nenglj med 352;17 www.nejm.orgapril 28, 2005

56. Thrombosis of the Cerebral Veins and Sinuses Most affect young adult and children, about 75% are women Incidence 3-4 cases per million, and up to 7 per million in children >80% of all patients ,good neurologic outcome

57. Thrombosis of the Cerebral Veins and Sinuses Pathogenesis venus occlusion -> intracranial hypertension -> Enlarged, swollen veins, edema, ischemic neuronal damage, and petechial hemorrhages Impaired absorption of CSF 1/5 of intracranial hypertension “ No Neuro Sign”

58. Clinical Manifestation Severe headache gradually over a couple day (may a split second on set) Rare unilateral hemispheric symptoms: hemiparesis, aphasia Coma and seizure Paralysis eye movement

63. Protein C deficiency

64. Protein S deiciency

65. Factor V Leiden mutation

66. Prothrombin G20210A polymorphism

68. Disseminated intravascular coagulopathy (DIC), chronic

69. Heparin induced thrombocytopenia/thrombosis (HIT/T)

71. HYPERCOAGULABLE STATES CONGENITAL Factor V Leiden mutation(APC resistance) 40-60% Prothrombin Gene Mutatuion 18-20% Protein C deficiency 5-10% Protein S deficiency 5-10% Antithrombin III deficeincy 3% Dysfibrinogenemia 1% Hyperhomocystinemia

72. อุบัติการณ์ความผิดปกติในผู้ป่วยที่มีหลอดเลือดดำอุดตัน Angchaisuksiri P, et al. Risk factors of venous thromboembolism in Thai patients. Int J Hematol 2007;86:397-402

73. อุบัติการณ์ความผิดปกติในผู้ป่วยที่มีหลอดเลือดดำอุดตัน

75. HYPERCOAGULABLE STATES CONGENITAL Factor V Leiden - Hereditary resistance to activated protein C - Factor V unable to degraded activated protein C - Treatment : long term anticoagulant

76. HYPERCOAGULABLE STATES CONGENITAL Protein C deficiency - decreased protein C (activated by thrombin) decreased activated protein C to degrade factor V , VIII. Protein S deficiency - co-factor for protein C

77. HYPERCOAGULABLE STATES Plasma protein C level

79. present in the early year of life

80. neonatal purpurafulminans

81. Heterozygous (PC or PS 10-60%)

82. thrombosis in teenage

85. DIC

86. Severe pre-eclampsia

87. ARDS

88. Postoperation

89. IgGparaproteinemia

90. Decreased synthesis

91. Hepatocellular disease

92. Oral anticoagulants

93. Vitamin k deficiency

95. pregnancy

96. oral pill

97. diabetes milletus

98. inflammation: SLE, nephrosis, AIDS

99. smoker

100. Decreased synthesis (PS or C4BP)

101. preterm

102. liver diseases

104. Elevated plasma prothrombin level

107. Homocysteine is injurious to blood vessels

108. Convert endothelium from antithrombotic to prothromotic phenotype

109. Smooth muscle proliferation

112. Venous stasis

114. Antiphospholipid syndrome: “Vascular occlusion and antibodies to anionic phospholipid.” HYPERCOAGULABLE STATES

115. ANTIPHOSPHOLIPID SYNDROME * Recurrent thromboembolism or pregnancy loss with antibodies (antiphospholipidAb, LA ,anti-B2 glycoprotein I Ab) * Increased prevalence associated SLE, type of anticardiolipinAb In a series 1000 pateints the prevalence of feature of APS were : - DVT 32% - Thrombocytopenia 22% - Livedoreticularis 20% - Sroke13% - Superficial thrombophlebitis9% - Pulmonary embolism 9% - Fetal loss 8% - TIA 7%

116. Sixth CN palsy with other signs

117. Syndrome Raymond’s syndrome: ipsilateral CN VI and contralateral paresis of the extremities Millard-Gubler syndrome ipsilateral 6th and 7th CN palsy with contralateralhemiplegia Foville’s syndrome: Millard-Gubler syndrome + lateral conjugate gaze palsy

119. Syndrome Gradenigo’s syndrome: inflammation of tip of the temporal bone : CN V, VI, greater petrosal nerve -> unilateral paralysis of lateral rectus, pain (CN V), excessive lacrimation

121. Syndrome Duane’s syndrome: widening of the palpebral fissure on abduction and narrowing on adduction Gerhardt’s syndrome: bilateral abducens palsy Möbius syndrome: paralysis of extraocular muscles, especially abducens, with paresis of facial muscle Tolosa-Hunt syndrome: recurrent unilateral pain in retro-orbital region with palsy of the extraocular muscles (3rd, 4th, 6th , V1, V2 )

124. CN VI : ABDUCEN NERVE Lying on petrous part of temporal bone with CN V Out of skull by carvernous sinus

125. Thank you for attention