[A case of mitochondrial encephalomyopathy, lactic acidosis, and stroke-like episodes (MELAS) with treatment-resistant status epilepticus that was effectively treated with lamotrigine].

A 16-year-old woman with MELAS developed fever and myoclonic epilepsy which improved with conventional anti-epileptic drugs. Since seizures recurred one month after successful treatment, the doses of phenobarbital, clonazepan, and valproate were increased. However, there was no improvement and status epilepticus continued. The addition of lamotrigine resulted in a decreased frequency and good control of seizures. This case is important, showing satisfactory results from the addition of lamotrigine for treatment-resistant status epilepticus.

[Anti-aquaporin-4 antibody autoimmune syndrome with disturbance of consciousness, respiratory failure, and ophthalmoplegia associated with extensive brain stem involvement with intractable hiccup and nausea as an initial manifestation. A case report].

A 45-year-old female was positive for anti-aquaporin-4 antibody with disturbance of consciousness, respiratory failure, and ophthalmoplegia associated with extensive brain stem involvement with intractable hiccup and nausea as an initial manifestation. Her level of consciousness and state of respiration worsened approximately one month later. There was no abnormality in the cerebrospinal fluid examination. A lesion was found in the medullary tegmentum on brain MRI. The patient received steroid pulse therapy and her level of consciousness improved the next day. However, her state of respiration worsened, and she had extensively clinical involvement of the brain stem. Her symptoms gradually improved with intravenous administration of prednisolone and intravenous immunoglobulin therapy (IVIg). The patient had almost completely recovered, but she relapsed with cervical myelitis extending over 3 vertebral segments approximately 10 months later. She underwent steroid pulse therapy, oral prednisolone, and IVIg again and improved.

Quantitative electroencephalogram analysis in dementia with Lewy bodies and Alzheimer's disease.

Clinicopathophysiological differences between dementia with Lewy bodies (DLB) and Alzheimer's disease (AD) remain obscure. Our goals were to determine whether characteristic findings of electroencephalogram (EEG) power and coherence in DLB and a differential pathophysiological mechanism of quantitative EEG existed between DLB and AD. The group consisted of 15 patients with AD or DLB and 12 age-matched controls. Original EEG signals were recorded from 14 scalp electrodes positioned according to the International 10-20 System, using digitally linked earlobes as a reference. Although EEG power spectral analysis showed increasing EEG power density in patients with DLB in the delta and theta bands, such a difference did not exist in patients with AD. Compared with AD, the delta and theta band intrahemispheric coherence values in the fronto-temporo-central regions were higher in DLB. In the beta band, AD was lower than DLB in almost all temporo-centro-parieto-occipital regions. Comparing the mean power value between patients with/without donepezil treatment, there was a significantly lower EEG power density in the delta and theta bands in DLB subjects taking donepezil than in subjects not taking donepezil, whereas there was no significant difference in AD patients. These results suggest that cholinergic dysfunction is stronger in DLB than AD.