D57.213

Clinical Causes: Inheritance of sickle hemoglobin gene (HbS) from one parent combined with hemoglobin C gene (HbC) from the other Presence of abnormal hemoglobin that distorts red blood cell shape into a sickle or crescent form Vasculopathy caused by sickled cells obstructing blood flow in cerebral arteries Risk factors such as dehydration, infections, or physical stress which can exacerbate the blockage

Key Symptoms: Repeated episodes of neurological deficits such as weakness, numbness, or paralysis on one side of the body Sudden and severe headaches that may resemble migraines or migraines followed by neurological changes Transient ischemic attacks (TIAs) or mini-strokes Visual disturbances or loss of vision Difficulty speaking, understanding speech, or confusion Seizures in some cases Signs of increased intracranial pressure such as nausea or vomiting during acute episodes

Diagnosis Path: Blood tests to identify hemoglobin composition, including hemoglobin electrophoresis Complete blood count (CBC) to assess anemia levels Imaging studies such as MRI or CT scans of the head to detect areas of ischemia or infarction Transcranial Doppler ultrasound to evaluate blood flow in cerebral arteries Assessment of clinical history and neurological examination

Treatment Protocols: Hydroxyurea therapy to reduce the frequency of sickling episodes and vaso-occlusion Blood transfusions aimed at reducing the proportion of sickled cells and preventing stroke Antiplatelet agents or anticoagulants in certain cases to prevent clot formation Pain management during acute crises Regular neurological monitoring and screening to identify early signs of stroke or vascular events Lifestyle adjustments such as staying well-hydrated and avoiding extreme temperatures or physical stress Bone marrow transplant may be considered in rare cases and suited candidates