SUDDEN WEAKNESS
History
Leroy is a 2.5-year-old West Indian boy brought to the A&E department by ambulance. He was in the children’s playground at the park apparently fit and well. He suddenly fell over whilst running to his mother and when he tried to get up, his right arm and leg were not moving and he was unable to speak. His speech has returned a little but is still far from normal and he is still not using his right side. He recognizes his parents but is a little drowsy. He is visibly upset. Nothing like this has ever happened before and his development to date has been normal. He is right-handed. Leroy was born at term following an uneventful pregnancy to healthy, unrelated parents. Apart from an orchidopexy at 13 months, he has no significant past medical history. He is on no regular medication and has no known allergies. He has two older sisters.
Examination
Leroy is a generally healthy Afro-Caribbean boy. His airway is patent and he is breathing without support. His oxygen saturation is 100 per cent in 15 L of oxygen via face mask. His pulse is 92 beats/min and regular with a capillary refill time of 2 s. His blood pressure is 80/54 mmHg. His temperature is 37C. Examination of the cardiovascular, respiratory and abdominal systems is normal. He is quite sleepy but knows his parents and responds appropriately to attempts to examine him. He has drooping of the right side of his mouth but his right eye closes when he cries. He will reach out with his left hand to his favourite toy when it is held to his left but ignores it if it is introduced from his right-hand side until it is almost in the midline. Tone, power, sensation and reflexes are normal on his left side. He has increased tone in his right arm and leg but power is weak. Reflexes are brisk throughout his right side. His plantar is flexor on the left but extensor on the right.
Questions
• What is the clinical diagnosis?
• List the possible causes
• What important and relevant screening test would he have had as a neonate?
• What investigation does he need?
• What are the key points in the acute and long-term management?
Medicine
Leroy has an acute right hemiplegia and hemianopia. He has an upper motor neurone VIIth nerve palsy as evidenced by eye closure despite weakness of the facial muscles. The clinical diagnosis is an acute stroke. Most childhood strokes are due to arterial occlusion (thrombotic or embolic), but venous thrombosis and haemorrhage do occur. The commonest cause of childhood stroke is sickle cell disease (SCD), affecting at least 10 per cent of such children. Many children with stroke have another medical condition.
Causes of acute stroke in childhood
• Haematological abnormalities – SCD, polycythaemia, leukaemia, disorders of coagulation
• Cardiac disease – congenital, especially cyanotic and acquired, e.g. endocarditis or Kawasaki disease. Usually embolic
• Infection – meningitis, local head and neck infections or bacteraemia
• Intracerebral vascular pathology – ruptured aneurysm, arteriovenous malformation
• Autoimmune disease – systemic lupus erythematosus, juvenile idiopathic arthritis, sarcoidosis
• Metabolic disease – homocystinuria, mitochondrial disorders
• Trauma
Sickle cell disease is now part of the neonatal screening programme. Previously all children from populations with a high incidence would be checked prior to any procedure that could precipitate a sickling crisis, such as surgery. In acute stroke it is crucial to distinguish between ischaemic and other categories because this alters the investigations and management. A brain MRI should be undertaken as soon as possible, but if it is not available within 48 hours, a CT scan is an acceptable initial alternative. Scanning should be undertaken urgently if there is a depressed level of consciousness at presentation or a deterioration. Further investigations will depend on the scan findings. For example, all children with arterial ischaemic stroke should have MRI angiography of the cervical and proximal intracranial arterial vasculature and cardiac echocardiography. They should also be investigated for an underlying prothrombotic tendency, such as protein C deficiency, as should those with venous thrombosis. A platelet count and clotting studies are needed in intracerebral haemorrhage. The acute medical management aims to minimize future disability. He needs regular neurological observations and monitoring of his vital signs with urgent scanning and admission to the paediatric intensive care unit if there is deterioration. If an arterial ischaemic stroke is confirmed and haemorrhage and SCD are excluded, aspirin is started. Anticoagulation should be considered in specific circumstances, such as cerebral venous sinus thrombosis.
The long-term management of stroke is a prime example of the benefits of a systematic, multidisciplinary approach to assess and monitor the medical, social, emotional and educational needs of the child. All children should have an assessment within 72 hours of admission and the professionals involved should have early liaison with their counterparts in the community to ensure smooth transition of care. A key worker from one of the teams should coordinate the package of care and act as a central point of contact for the family.
KEY POINTS
• MRI (or CT) scanning should be performed within 48 hours where there is a clinical diagnosis of childhood stroke.
• At least 10 per cent of patients with sickle cell disease will have a stroke during childhood.
• The management of childhood stroke should be multidisciplinary.
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