Life-threatening presentation of an acute cerebellar ischemic stroke secondary to a protein C deficiency

Acute cerebellar ischemic stroke is a rare disease in children. Typically, patients present with ataxia and cranial nerve palsy. Rarely, some patients show a severe intracranial hypertension syndrome with a life-threatening clinical presentation. We report a case of a 2-year-old male child who was admitted for deterioration of his consciousness level and vomiting. Cerebral imaging revealed a right cerebellar and brainstem infarction with an obstructive hydrocephalus and a tonsillar herniation. Angiography identified an occlusion of the right anterior inferior cerebellar artery. The child was referred to a neurosurgeon for the treatment of acute hydrocephalus and posterior fossa craniectomy. Etiological investigations revealed a protein C deficiency. Initially, the patient was maintained under fluid restriction and received mannitol several times as he had clinical and radiological signs of intracranial hypertension. He was also kept on mechanical ventilation and monitored. However, the evolution was complicated by the occurrence of a thrombosis of the vena cava and the renal vein.