Subacute Neurologic Decline in a 4 Year Old with Sickle Cell Disease: A Case Report

Case Diagnosis: Spastic quadriparesis due to strokes in sickle cell disease

Case Description or Program Description: A typically developing 4 year old child with sickle cell disease was not taking hydroxyurea or receiving serial transcranial doppler examinations. She was following a vegan diet. She presented with episodes of bilateral upper extremity shaking concerning for seizure. She had left upper extremity ataxia. CTA demonstrated right ICA thrombus and she was initiated on exchange transfusion. She progressed to have spastic quadriparesis, subclinical seizures, dysphasia, inability to communicate, and difficult to control paroxysmal sympathetic hyperactivity. Serial magnetic resonance images over two weeks ultimately showed extensive bilateral infarcts including the deep brain structures with relative sparing of the brainstem and cerebellum. She had steno-occlusive disease involving both internal carotid arteries with extensive collateral vessels.

Setting: Tertiary care pediatric hospital

Assessment/Results: Patient was treated with exchange transfusion, enoxaparin sodium, and levetiracetam. She required clonidine, clonazepam, gabapentin, and propranolol for control of paroxysmal sympathetic hyperactivity. Once stable, she spent two weeks in acute inpatient rehabilitation prior to transitioning to outpatient therapy. Two years later, she can sit independently, feed herself, and has over 100 words. She may be receiving a bone marrow transplant.

Discussion (relevance): Abnormal flow on transcranial doppler is a strong predictor of stroke risk in children with sickle cell disease therefore they should be regularly performed with transfusion treatment as needed for stroke prevention. Stroke in sickle cell disease can have serious neurologic consequences at a young age.

Conclusions: Families must be educated about the importance of preventative care in sickle cell disease. Rehabilitation physicians should be aware of the pathophysiology and complication of sickle cell disease as they may provide care for these patients after stoke, for pain management, or in the setting of bone marrow transplant.