An unusual cause of stroke in young

Presenter :Dr. T. Ashok V ReddyPost graduate

BMCRIVICTORIA HOSPITAL

25 Yr old female From Banswadi area ,Bengaluru. Presented with complaints of sudden onset

loss of speech , weakness of right side upper and lower limbs and face from early morning after she woke up.

Presenting complaints and History

Early morning she woke up and found that she was unable to speak.

After 30 minutes ,she observed weakness of right upper and lower limbs and right face .

No history of seizures ,fever , headache, bladder and bowel disturbances.

Not a diabetic,not a hypertensive.No H/o abortions.

GENERAL PHYSICAL EXAMINATION- Pallor present VITALS : PR-90/min,regular in rhythm Peripheral pulses- Right side radial artery –Felt Left side brachial ,radial -Feeble Bilateral femoral ,popliteal ,dorsalis

pedis artery pulsations –Feeble No features suggestive of gangrene in limbs.

EXAMINATION

BP : Right upper limb -110/80 mm Hg Left upper limb and bilateral lower limbs –

not recordable Bruit heard over left carotid artery Respiratory rate -14/min Temp – 37.2celsius

Neurological examination- Motor aphasia present Right sided UMN facial nerve palsy

Right sided hemiplegia (Power 0/5), Right side hypotonia, Deep tendon reflexes –Right side 1+ Left side 2+ sensory system –normal cerebellar system could not be tested. Fundus examination -normal

CVS-S1 and S2 present No murmurs.RS and abdominal examination – normal

OTHER SYSTEMS

Hb-8.1g/dl MCV-61.5 Fl MCH-16.8 pg Platelet count-3 lakh/micro L

ESR-10mm at the end of 1hr Random blood sugar-105 mg/dL LFT –Albumin-3.5g/dL ALP-120 U/l

INVESTIGATIONS

T.Bilirubin-0.6mg/dL Globulin-4.3g/dL AST-21 U/L ALT-14 U/L Total protein-7.8g/dL

RENAL FUNCTION TESTS- Urea -15mg/dL Creatinine-0.6mg/dL

C reactive protein –positive HBs ag ,RA- negative VDRL,HIV –non reactive CT Brain – Normal

ANA profile –negative Serum homocysteine-10 micro mol/l Serum B12 levels-500pg/ml Sputum for AFB negative

ECG and ECHO Cardiography – normal Neck vessel doppler – Right side:Circumferential wall thickening of common carotid artery ,external carotid and internal carotid artery causing luminal narrowing. Left side: Circumferential wall thickening of

Common carotid ,internal and external carotid artery thickening causing near total occlusion.

Above features were suggestive of arteritis.

AORTO ARTERITIS Points in favour – young age , Feeble pulse in extremities except right upper limb.

Possible diagnosis

Tuberculosis Syphilis Takayasu disease Irradiation Rheumatic fever Ankylosing spondylitis

Causes of Aorto arteritis

1 Age at onset of disease<40 yrs. 2 Claudication of extemities. 3 Decreased brachial artery pulse. 4 BP difference >10 mmHg between arms. 5 Bruit over subclavian artery /aorta 6 Arteriogram abnormality .

If atleast 3 of above criteria are present ,diagnosis can be made with 90 % sensitivity and 97 % specificity .

ACR Criteria for diagnosis of Takayasu arteritis

Diagnosis of Takayasu arteritis was made. Steroids were started . Tab .Predisolone 40 mg per day . Tab.Aspirin 150 mg od

After 1 week ,power improved to 3/5 in right upper limb and to 4 /5 in lower limb.

No H/O claudication /fatigue /discomfort in limbs before the weakness developed.

No H/O fever,weight loss,myalgia,arthralgia.

No rise of imflammatory markers like ESR

.

Unusual features in this case

Described by Adams in 1827. In 1908 Takayasu described a wreath like

appearance of arterio venous anastamosis around optic papilla.

2 stages- Early active phase Chronic sclerotic phase.

Takayasu arteritis

Diminished /absent pulses in 84-96% Bruit over carotid/subclavian artery/aorta in

80-90% Hypertension in 33 -83% Retinopathy in 37 % Aortic regurgitation in 20-25% Pulmonary artery involvement in 14-100 % Neurological features like

headache,TIA,seizures in 10-20% 3.2 % present as ischemic stroke

Clinical features

Takayasu arteritis is rare disease which affects mainly women .

Manifestations range from asymptomatic disease to catastrophic neurological impairment.

The four most important complications are Takayasu retinopathy, secondary hypertension, aortic regurgitation, and aneurysm formation which have to be monitored intermittently at least once in 6 months.

Conclusion

Approximately half of those patients treated with steroids will respond, remaining patients respond to methotrexate; mycophenolate mofetil.

Treatment should aim to control disease activity and preserve vascular competence.

Fertility is not adversely affected and pregnancy does not appear to exacerbate the disease, although management of hypertension is essential.

References

Harrison’s text book of internal medicine,18th edition.

API Text book of medicine Monogram on vasculitis by

Dr.G.Narasimhulu. Gamboa R, et al. DNA sequencing of HLA-B

alleles in Mexican patients with Takayasu arteritis. Int J Cardiol 2000;75:S117–22.