A 15-year-old woman with a history of transient
dysarthria two years before, suddenly developed weakness of right upper extremity, right
facial palsy, and
dysarthria. She was admitted to our hospital on the third day. She had no
hypertension,
heart murmur and oedema. On neurological examination, she had mild right
hemiparesis including face muscles and mild
dysarthria. The right knee jerk was brisk with no Babinski's sign.
Ataxia and sensory disturbance were not present. T2-weighted MRI showed a hyperintensity at the posterior limb of the left internal capsule. Cerebral angiography was unremarkable. Ultracardiography and 24-hour electrocardiography were normal. Laboratory data revealed no inflammatory findings,
liver dysfunction,
hyperglycemia and
hyperlipidemia. Antinuclear and
anticardiolipin antibodies were negative. Prothrombin time was normal, but activated partial thromboplastin time was slightly prolonged (35.4 sec, normal 25.2-34.4).
Protein C,
protein S and
antithrombin III were normal.
Heparin cofactor II (HC II) activity was decreased (44%) with normal HC II
antigen (79%) and so she was diagnosed as
heparin cofactor II deficiency type II (
heparin cofactor II abnormality). Her father manifesting
thromboangitis obliterans also had low HC II activity with normal HC II
antigen. However, on her genetic analysis, we didn't detect any mutations in the coding region of HC II gene. Until now she has no recurrence of cerebrovascular attacks. On the basis of these results, we suspect that HC II deficiency was a possible risk factor of
cerebral infarction in this case because she was so young and had no general risk factors except for HC II. No
stroke associated with HC II deficiency type II has been reported up to date. This case is worth considering etiologies of juvenile
cerebral infarction.