Cvst 2

Interesting Case

Presented by Hong – Sarawoot

May 2010

ผู้��ป่�วยหญิงไทยอาย� 19 ป่� สิทธิ์�การร�กษา 30 บาท รพ.อ��น อาการสิ�าคั�ญิ : ตาเห"นภาพซ้�อน 5

ว�นก%อนมารพ.

History

- 1 สิ�ป่ดาห(ก%อนมารพ. ผู้��ป่�วยม)อาการป่วดศี)รษะท��วๆ ท�.งศี)รษะป่วดต�.อๆ ไม%เคัยเป่/นมาก%อนอาการป่วดศี)รษะอย�%ๆ ก"เป่/นขึ้1.นมาตอนท�างาน อาการเป่/นมากขึ้1.นเร��อยๆ ม)คัลื่��นไสิ�อาเจี)ยนต�.งแต%ว�นแรกท)�ป่วดศี)รษะเป่/นว�นลื่ะ 2-3 คัร�.ง pain score 9-10/10 (อาการเป่/นจีนมากท)�สิ�ดในว�นเด)ยว เร�มจีากpainscore 6-7)

- ขึ้ย�บต�ว ไอจีามแลื่�วป่วดมากขึ้1.น กลื่างคั�นนอนไม%หลื่�บ

History

- อาการเป่/นมากจีนนอนไม%หลื่�บ ไม%ม)ไขึ้� กนยาพาราเซ้ตามอลื่ 2 เม"ดท�ก 8 ชม.อาการไม%ด)ขึ้1.น

- 6 ว�นก%อนไป่ตรวจีท)� clinic ได�ยาฉี)ดแลื่ะยามากนอาการด)ขึ้1.น ไม%ทราบชนดยาอาการป่วดศี)รษะด)ขึ้1.น pain score เหลื่�อ 5 - 6

History

- 5 ว�นก%อนมารพ. เร�มม)ตาเหลื่% ร� �สิ1กเห"นภาพซ้�อนเวลื่ามองไกลื่ๆ ใกลื่�อ%านหน�งสิ�อได�ช�ดด) อาการเห"นไม%ม)ไขึ้� ไม%ม)หน�าเบ).ยว ไม%สิ�าลื่�ก ไม%ชา ไม%อ%อนแรง มองเห"นช�ดเจีนถ้�า ป่9ดตา 1ขึ้�าง ได�ยนป่กต ไม%ได�ยนเสิ)ยงในห� อาการป่วดศี)รษะย�งม)อย�% pain score ใกลื่�ๆเดม = 6-7

- อาการเห"นภาพซ้�อนเป่/นมากขึ้1.นจี1งมารพ.

History

Past History- ป่ฏิเสิธิ์แพ�ยา ม)ใช�ยาคั�มก�าเนดมา 1

ป่�ชนด 21 เม"ด- ม)บ�ตร 1คันแขึ้"งแรงด) ไม%เคัยม)

ป่ระว�ตแท�งบ�ตรมาก%อน- ไม%เคัยผู้%าต�ด- สิ�บบ�หร)�ว�นลื่ะ 1- 10 มวนมา 3 ป่�- ป่ฏิเสิธิ์ผู้��นแพ�แสิง หร�อป่วดขึ้�อ

ป่ฏิเสิธิ์โรคัเลื่�อดในคัรอบคัร�ว

Physical Examination

V/S T 37o C, P 97/min, RR 14/min, BP 136/99 mmHg

GA good consciousness, not pale, no jaundice, no pitting edema, no superficial vein dilatation

HEENT thyroid gland no enlargement

Respiratory system: normal chest movement, normal breath sound, no adventitious sound

CVS: apical impulse at 5 th ICS at Lt.MCL, no apical or parasternal heaving, no murmur

Abdomen soft, not tender, liver and spleen cannot be palpated

Neurological 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


CN V, VI, VII, VIII, XI, X, XI, XII

Stiff neck positive(terminal)

Cerebellar sign normal

Deep tendon reflex all 2+

Babinski ’s absent


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

Problem List

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

CN VI: Nerve Lesion vs. Nuclear Lesion Nerve Lesion

OphthalmoplegiaEsotropia

Nuclear LesionOphthalmoplegiaEsotropiaFacial WeaknessAltered LOC

General: diplopia

CN VI : ABDUCEN NERVE

CN VI:

Nerve Lesions:Meningeal tumorsPituitary AdenomaInflammation

- Increase intracranial pressure Nuclear Lesions:

Ischemia (pontine infarction)Central Demyelinating DisordersInflammation



CLINICAL Pontine lesion : LR palsy + facial palsy : horizontal diplopia

Petrous temporal bone : LR palsy + CN V palsy “ Gardenigo syndrome”

Increase intracranial pressure : false localizing sign

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

Investigation

CT Brain with contrast

CT brain with contrast: Empty delta sign

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

Progress Note

17/4/53 หลื่�งจีาก LP ม)ป่วดศี)รษะเลื่"กน�อย ไม%ม)ไขึ้�

EOM 100 100

0 + 100 100 + 100

100 100

Investigation

ANA speckle 1:160, Peripheral 1:160, Homogenous 1:160

ANA CSF homogenous 1:40 homogenous pattern

AntiHIV negative

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

Progress Note

20/4/53 หลื่�งจีาก LP ไม%ม)ป่วดศี)รษะ ไม%ม)ไขึ้�

EOM 100 100

40 + 100 100 + 100

100 100

Progress Note

20/4/53 ไม%ม)ป่วดศี)รษะ ไม%ม)ไขึ้� แขึ้นขึ้าแรงป่กตด)

EOM 100 100

60 + 100 100 + 100

100 100

MRI and MRV

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

Treatment

Enoxaparin 0.6 cc sc q 12 hr

Challenge warfarin(5)1x1-> warfarin(3)1x1 o hs

D/C

Conclusion

• Continue at least 6 mo Repeat MRV if no evidence thrombosis stop 2 wks for work up hypercoagulable state if hypercoagulable state present -> life long warfarin

• Protein C, Protein S level, antithrombin III level

• Lupus anticoagulant

• Anti beta2 glycoprotein IgG, IgM

• Anticardiolipin IgM, IgG

Warfarin

Warfarin Embryonopathy (ACC/AHA guideline vulvular heart disease 2006)

Incidence 4-10%, lower if dose < 5 mg/day

Midface hypoplasia, 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

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

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

Cerebral Venous Sinus Thrombosis

n engl j med 352;17 www.nejm.org april 28, 2005

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

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”

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

Hereditary Antithrombin deficiency

Protein C deficiency

Protein S deiciency

Factor V Leiden mutation

Prothrombin G20210A polymorphism

Homocysteinemia (rare)

Acquired Antiphospholipid syndrome

(APS)

Disseminated intravascular coagulopathy (DIC), chronic

Heparin induced thrombocytopenia/thrombosis (HIT/T)

Paroxysmal nocturnal hemoglobinuria (PNH)

Thrombophilic diseases

Precipitating

Head injury

Obstetrical delivery ;last trimester, after delivery 12/100,000

Oral contraceptive pills

Infection: Otitis and mastoiditis-> sigmoid and transverse sinuses

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

อุ�บั�ติ�การณ์�ความผิ�ดปกติ�ในผิ��ป�วยที่��ม�หลอุดเล�อุดด�าอุ�ดติ�น

Angchaisuksiri P, et al. Risk factors of venous thromboembolism in Thai patients. Int J Hematol

2007;86:397-402

อุ�บั�ติ�การณ์�ความผิ�ดปกติ�ในผิ��ป�วยที่��ม�หลอุดเล�อุดด�าอุ�ดติ�นTHAI Chinese Caucasia

n

Protein S deficiency 12.3% 8-33% 1-3%

Protein C deficiency 8.9% 4-19% 3-5%

Antithrombin deficiency 4.7% 4% 1%

Factor V Leiden 0% 0% 20%

Prothrombin mutation 0% 0% 6%

Elevated fibrinogen 33.3% - 3%

Elevated factor VIII 30.4% - 25%

Elevated factor XI 26.8% - 19%

Hyperhomocysteinemia 5% - 10-20%

Antiphospholipid antibodies 10% - 10%

Malignancy 19% 16-27% 9-25%


CONGENITAL

Factor V Leiden

- Hereditary resistance to activated protein C

- Factor V unable to degraded activated protein C

- Treatment : long term anticoagulant


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


Plasma level (%)

Preterm baby 30

Term baby 50

Adult 60-120

Heterozygosity 40-60

Homozygosity <10

Plasma protein C level


Clinical manifestation Homozygous (PC or PS < 10%) present in the early year of life neonatal purpura fulminans

Heterozygous (PC or PS 10-60%) thrombosis in teenage usual or unusual site

Neonatal purpura fulminans in a homozygous protein S deficiency patient


Acquired condition associated with low protein C

Increased utilization DIC Severe pre-eclampsia ARDS Postoperation IgG paraproteinemia

Decreased synthesis Hepatocellular disease Oral anticoagulants Vitamin k deficiency L-asparaginase treatment


Acquired condition associated with low free protein S

Increased C4BP pregnancy oral pill diabetes milletus inflammation: SLE, nephrosis, AIDS smoker

Decreased synthesis (PS or C4BP) preterm liver diseases vitamin K deficiency, cuamadin treatment


Prothrombin G20210A polymorphism

In 1996 discover a genetic defect G20210A of prothrombin gene

Elevated plasma prothrombin level

Increased thrombotic risk (2-6 times) due to enhanced thrombin generation


CONGENITAL

Hyperhomocysteinemia

- autosomal recessive

- endothelial damage leading to increased arteriosclerosis.

- >11 nmol/l increased risk of atherosclerosis

- >18 nmol/l increased risk of venous thrombosis

- both venous & arterial thrombosis

- synergistic risk factor with factor V Leiden

- treatment : vitaminB6 ,12 , folate


Homocysteinemia In 1969 Kilmer McCully suggested

homocysteinemia might be associated with premature coronary disease or stroke

Homocysteine is injurious to blood vessels Convert endothelium from antithrombotic to

prothromotic phenotype Smooth muscle proliferation

Both arterial and venous occlusion


ACQUIRED

Oral pills :

- Risk thrombotic stroke 9 times

- Depended on : age>35, duration, high estrogen, smoking ,DM ,HT , DLP,

1st 2 weeks after delivery

- Mechanism - intimal hyperplasia,

- increased blood viscosity

- decreased protein S,AT III,

- enhanced arterial hypertension


ACQUIRED

Pregnancy

- Decreased protein S,AT III level

- Venous stasis

- Increased factorVII ,VIII,fibrinogen level


ACQUIRED

Nephrotic syndrome

Related arterial &venous stroke

Mechanism - increased fibrinogen

- thrombocytosis

- enhanced platelet aggregrant

- decreased AT III ,protein S level

- DLP,steroid,diuretic used

Antiphospholipid syndrome:

“Vascular occlusion and antibodies

to anionic phospholipid.”


ANTIPHOSPHOLIPID SYNDROME

* Recurrent thromboembolism or pregnancy loss with antibodies

(antiphospholipid Ab, LA ,anti-B2 glycoprotein I Ab)

* Increased prevalence associated SLE, type of anticardiolipin Ab

In a series 1000 pateints the prevalence of feature of APS were :

- DVT 32%

- Thrombocytopenia 22%

- Livedo reticularis 20%

- Sroke 13%

- Superficial thrombophlebitis 9%

- Pulmonary embolism 9%

- Fetal loss 8%

- TIA 7%

Sixth CN palsy with other signs

Syndrome

Raymond’s syndrome: ipsilateral CN VI and contralateral paresis of the extremities

Millard-Gubler syndrome ipsilateral 6th and 7th CN palsy with contralateral hemiplegia

Foville’s syndrome: Millard-Gubler syndrome + lateral conjugate gaze palsy

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

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 )


Lying on petrous part of temporal bone with CN VOut of skull by carvernous sinus

Thank you for attention

Cvst 2

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