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Query: UMLS:C0085584 (encephalopathy)
 18,178 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

We reported a female infant with early myoclonic encephalopathy (EME). She was diagnosed on the basis of clinical and laboratory features including electroencephalographic and magnetic resonance image (MRI) findings. Frequent erratic myoclonic seizures appeared since 28 days after birth and EEG showed a typical suppression-burst pattern. We administered a high-dose pyridoxal phosphate, thyrotropin-releasing hormone analogue (TRH), and then ACTH, but could not control the seizures at all. With seizure types, we observed the change from erratic myoclonus to tonic spasms in series, with concomitant EEG change to hypsarhythmia at the age of 6 months. Cranial MRI revealed delayed myelination in the white matter but no brain malformation. We administered ACTH to her again and succeeded partially in the decrease of the seizure frequency, and significantly in the improvement of EEG findings. It is supposed that the responsiveness to ACTH treatment changed with age as the seizure patterns developed from erratic myoclonus to tonic spasm.
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PMID:[A longitudinal study of clinical and electroencephalographic findings in a female infant with early myoclonic encephalopathy]. 165 45

The authors investigated the possibility of a thyrotropin-releasing hormone-related mechanism in a 43-year-old Japanese woman with Hashimoto's encephalopathy who experienced three relapses closely associated with the menstrual cycle. Her symptoms began at ovulation, worsened during the luteal phase, and improved during the menstruation phase. No abnormalities were found by brain magnetic resonance imaging and cerebral angiography. Intravenous administration of thyrotropin-releasing hormone induced symptoms of myoclonus and tremor similar to those observed during an exacerbation. The intensity and duration of involuntary movements induced by thyrotropin-releasing hormone were dose-dependent. The patient's symptoms were controlled effectively by thyroxine replacement therapy. On the basis of these findings, thyrotropin-releasing hormone may have an important role in Hashimoto's encephalopathy.
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PMID:Case report: thyrotropin-releasing hormone-induced myoclonus and tremor in a patient with Hashimoto's encephalopathy. 748 24

A male infant with early infantile epileptic encephalopathy (EIEE) was reported. Tonic spasms in series appeared since 1 month after birth and EEG showed a typical suppression-burst pattern. The patient was treated with a high-dose pyridoxal phosphate and thyrotropin-releasing hormone (TRH), but seizures were not controlled. ACTH was administered and the seizures disappeared transiently. The seizures reappeared during tapering ACTH and apparent cerebral shrinkage followed the ACTH therapy. Then, the patient who evolved into West syndrome was treated with ketogenic diet. The seizures disappeared immediately and EEG findings were improved. It is suggested that the ketogenic diet should be tried early for the treatment of EIEE.
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PMID:[Effect of the ketogenic diet for West syndrome into which early infantile epileptic encephalopathy with suppression-burst was evolved]. 757 84