Akinetic-rigid syndrome: An unusual presentation of hypoglycemic encephalopathy

2013 
Introduction: Hypoglycemia is an emergency. If untreated, it can result persistent neuronal injury of the central nervous system. The neurologic manifestations of hypoglycemia ranges from headache, seizure, different neuropsychiatric symptoms to stupurous, coma and stroke like symptoms. Akinetic­rigid syndrome, a severe form of extra pyramidal symptom is rarely described with hypoglycemic brain damage. The magnetic resonance imaging (MRI) findings are variable and reversible which ranges from normal study to diffusion restriction and white matter hyper intensities involving both cortical and sub cortical white matter as well as grey matter. Case Report: This case illustrates a 32­year­old male with history of intermittent hypoglycemia of unknown cause for three years duration presenting with prolonged akinetic mutism and extra pyramidal symptoms. His brain MRI revealed bilateral symmetrical white matter hyper intensities involving corona radiata, internal capsule and middle cerebellar peduncles with 18F­ fluorodeoxyglucose positron emission tomography with computed tomography (FDG PET CT) scan of brain showed hypometabolism involving bilateral parietal lobes, basal ganglia, thalamus and cerebellum, which reversed with correction of blood sugar within next four weeks. Conclusion: Hypoglycemic encephalopathy (HE) may develop reversible extra pyramidal syndromes. Clinico­neuro imaging correlation is not always possible. Besides routine evaluation for hypoglycemia, neuroimaging such as MRI of brain and 18F­ FDG PET CT scan may contribute to recognize the disease process and its outcome.
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