Nonconvulsive Status Epilepticus Associated with Hashimoto's Encephalopathy / 대한임상신경생리학회지

Nonconvulsive status epilepticus usually presents with altered mentation without distinct manifestations of seizures. It may be related with various medical disorders. Hashimoto's encephalopathy is characterized by various neurological manifestations accompanied by high titers of anti-thyroid antibodies. Here, we report a patient with nonconvulsive status epilepticus caused by Hashimoto's encephalopathy who showed a dramatic response to steroids.

Palinopsia Preceding a Seizure in a Patient with Arteriovenous Malformation in the Occipito-Temporal Lobe / 대한간질학회지

Palinopsia means a visual image persisting for minutes to hours or reappearing episodically after the exciting stimulus has been removed. The anatomic correlation of palinopsia is not clear, whereas occipito-parietal and occipito-temporal lesions have been implicated. We describe a patient presenting palinopsia prior to motor seizures which was related to left posterior temporal lesion. A 36-year-old man had a generalized tonic clonic seizure after palinoptic positive visual afterimages. Brain MRI and Transfemoral cerebral angiography (TFCA) revealed an arteriovenous malformation of 1x1.5x2 cm3. We used oxcarbazepine for preventing recurrent seizures and planned gamma knife radiosurgery. Palinopsia is a very rare clinical manifestation as an aura of seizures. Palinopsia preceding clinical seizure as a localizing value, so that neuroimaging is mandatory in this clinical situation. This is the first reported case of palinopsia in Korea.

Palinopsia Preceding a Seizure in a Patient with Arteriovenous Malformation in the Occipito-Temporal Lobe

Palinopsia means a visual image persisting for minutes to hours or reappearing episodically after the exciting stimulus has been removed. The anatomic correlation of palinopsia is not clear, whereas occipito-parietal and occipito-temporal lesions have been implicated. We describe a patient presenting palinopsia prior to motor seizures which was related to left posterior temporal lesion. A 36-year-old man had a generalized tonic clonic seizure after palinoptic positive visual afterimages. Brain MRI and Transfemoral cerebral angiography (TFCA) revealed an arteriovenous malformation of 1x1.5x2 cm3. We used oxcarbazepine for preventing recurrent seizures and planned gamma knife radiosurgery. Palinopsia is a very rare clinical manifestation as an aura of seizures. Palinopsia preceding clinical seizure as a localizing value, so that neuroimaging is mandatory in this clinical situation. This is the first reported case of palinopsia in Korea.

Decreased Metabolism in the Cerebral Cortex in Early-Stage Huntington's Disease: A Possible Biomarker of Disease Progression?

BACKGROUND AND PURPOSE: Huntington's disease (HD) is an autosomal-dominant inherited neurodegenerative disorder. Genetic analysis of abnormal CAG expansion in the IT15 gene allows disease confirmation even in the preclinical stage. However, because there is no treatment to cure or delay the progression of this disease, monitoring of biological markers that predict progression is warranted. METHODS: FDG-PET was applied to 13 patients with genetically confirmed HD in the early stage of the disease. We recorded the initial and follow-up statuses of patients using the Independence Scale (IS) of the Unified Huntington's Disease Rating Scale. The progression rate (PR) was calculated as the annual change in the IS. The patients were divided into two groups with faster and slower progression, using the median value of the PR as the cut-off. FDG-PET data were analyzed using regions of interest, and compared among the two patient groups and 11 age- and sex-matched controls. RESULTS: The mean CAG repeat size in patients was 44.7. The CAG repeat length was inversely correlated with the age at onset as reported previously, but was not correlated with the clinical PR. Compared with normal controls, hypometabolism was observed even at very early stages of the disease in the bilateral frontal, temporal, and parietal cortices on FDG-PET. The decreases in metabolism in the bilateral frontal, parietal, and right temporal cortices were much greater in the faster-progression group than in the slower-progression group. CONCLUSIONS: A decrease in cortical glucose metabolism is suggested as a predictor for identifying a more rapid form of progression in patients with early-stage HD.