ABSTRACT
Although currently it is known that neuronal hyperexcitability is the mechanism underlying epilepsy, this condition was previously associated with spiritual factors. Epilepsy surgery is considered in patients in whom administration of anti-seizure drugs is infeasible. Functional mapping of the brain and intraoperative monitoring have improved safety of epilepsy surgery. A ketogenic diet is considered in patients in whom surgery is not adaptive. The American made-for-television movie ...First Do No Harm (1997) highlights the usefulness of the ketogenic diet for intractable epilepsy. Notably, ketogenic diet therapy was approved for public health insurance coverage in Japan in 2016.
Subject(s)
Diet, Ketogenic , Drug Resistant Epilepsy , Epilepsy , Humans , Drug Resistant Epilepsy/surgery , Epilepsy/drug therapy , Epilepsy/surgery , Ketone Bodies , Brain , Treatment OutcomeABSTRACT
A 49-year-old woman with intellectual disability and a food preference for fried chicken entered a nursing home. After nursing home diet, she developed episodic attacks of hyperammonemic encephalopathy. Her characteristic food preference and the negative results for brain and liver imaging studies suggested urea cycle disorder. A high plasma citrulline level on amino acid analysis and a genetic test for citrine gene confirmed a citrine deficiency (adult-onset type II citrullinemia). Although a low-carbohydrate diet was insufficient, a combination therapy of a low-carbohydrate diet and a medium-chain triglyceride (MCT) oil was effective. MCT oil may be a promising treatment option.
Subject(s)
Citrullinemia/drug therapy , Citrullinemia/etiology , Nursing Homes , Triglycerides/administration & dosage , Citrullinemia/diagnosis , Citrullinemia/genetics , Diagnosis, Differential , Female , Heterozygote , Humans , Middle Aged , Mitochondrial Membrane Transport Proteins/genetics , Mutation , Oils , Treatment Outcome , Triglycerides/chemistryABSTRACT
Moyamoya disease is a rare cerebrovascular disorder with unknown etiology and psychiatric symptoms occasionally manifest initially. Capgras syndrome is a unique neuropsychiatric symptom that is a delusional misidentification of a familiar person replaced by an identical imposter. We report the case of a 51-year-old woman with frontal lobe ischemic stroke caused by moyamoya disease, presenting with Capgras syndrome. Dysfunction of frontal areas may be attributable to development of Capgras syndrome.
