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A hypoglycaemic 'Peter Pan': a paediatric disease in an adult patient?
Insights
A 56-year-old male with a history of seizures experienced resolution of his condition after treatment for hyperinsulinaemic hypoglycaemia caused by pancreatic nesidioblastosis. This case highlights the importance of blood glucose monitoring in seizure patients.
Area of Science:
- Endocrinology
- Neurology
- Pathology
Background:
- A 56-year-old male with cerebral palsy and intractable generalized tonic-clonic seizures (GTCS) presented with vomiting.
- His medical history included lifelong GTCS despite anticonvulsant therapy.
Purpose of the Study:
- To investigate the cause of seizures in a patient with previously uncontrolled epilepsy.
- To determine the link between metabolic derangements and neurological symptoms.
Main Methods:
- Clinical presentation and medical history review.
- Capillary blood glucose measurement during a seizure.
- Diagnostic investigations for hyperinsulinaemic hypoglycaemia.
- Management of nesidioblastosis and monitoring of glycaemic control.
Main Results:
- A capillary blood glucose of 1.6 mmol/L was recorded during a seizure.
- Investigations confirmed hyperinsulinaemic hypoglycaemia secondary to diffuse pancreatic nesidioblastosis.
- Seizure disorder resolved completely with consistent normoglycaemia achieved through nesidioblastosis management.
Conclusions:
- Unrecognised hyperinsulinaemic hypoglycaemia can manifest as intractable seizures.
- Diffuse pancreatic nesidioblastosis is a potential cause of hyperinsulinaemic hypoglycaemia in adults.
- Achieving normoglycaemia is crucial for resolving seizure disorders associated with hyperinsulinaemic hypoglycaemia.
Summary:
A 56-year-old male presented to hospital with vomiting and was admitted for management of suspected aspiration pneumonia. His medical history was significant for a diagnosis of cerebral palsy and intellectual disability and he had suffered regular generalised tonic-clonic seizures (GTCS) since birth, despite multimodal anticonvulsant treatment. During his admission, his capillary blood glucose was noted to be 1.6 mmol/L during a seizure. Subsequent investigations confirmed hyperinsulinaemic hypoglycaemia secondary to diffuse pancreatic nesidioblastosis. His seizure disorder completely resolved when management of nesidioblastosis achieved consistent normoglycaemia.
Learning Points:
All patients who suffer seizure should have a blood glucose measured. Unrecognised hypoglycaemia in a neonate or infant confers a high risk of subsequent neurological damage. Persistent hyperinsulinaemic hypoglycaemia (PHH) in adults is highly likely to be caused by insulinoma, but diffuse pancreatic hyperinsulinism, particularly after bariatric surgery, should also be considered. Medical therapy of endogenous hyperinsulinaemic hypoglycaemia is complex, requiring intensive monitoring.
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