TRH therapy is effective for spinocerebellar degeneration, improving clinical conditions such as dysarthria and gait disturbance. Herein, we describe a 24-year old woman diagnosed with dentatorubral-pallidoluysian atrophy (DRPLA) by gene analysis. TRH therapy was administered because she showed involuntary movements, which were unresponsive to several anti-convulsants including valproic acid (VPA) and clonazepam (CZP). Her activities of daily living improved with TRH therapy, but 1 year after, it was interrupted for reduction of efficacy and appearance of hallucinations. The effects on her involuntary movements are also documented.

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When drug therapy with paroxetine was started in a 76 year-old man with senile depression, tremor of both upper limbs and episodic disturbance of consciousness accompanying impaired dexterity occurred. On EEG, generalized 2-3Hz spike-slow wave complexes centered in the frontal region were observed corresponding to disturbance of consciousness. After paroxetine was discontinued, treatment with antiepileptic drugs resulted in regression of disturbance of consciousness and abnormal EEG, and these symptoms did not recur even after the antiepileptic drugs were tapered and discontinued. In this patient, spike-wave stupor appeared for the first time after the start of paroxetine treatment, suggesting drug-induced seizure. This is the first report of spike-wave stupor related to selective serotonin reuptake inhibitors (SSRI) in Japan and worldwide. When disturbance of consciousness occurs during treatment with SSRI, the possibility of seizures has to be examined. Furthermore, especially in patients predicted to have low drug resistance, caution has to be exercised in the administration of SSRI.

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We report a case of frontal lobe epilepsy where the patient developed complex partial status epilepticus (CPSE). The patient was a 36-year-old man who experienced a partial seizure consisting of loss of consciousness with tonic posture of the limbs at the age of 14 years. No more seizures were observed after that until the age of 36 years, when two more similar seizures occurred. His EEG showed frequent spikes in the left frontal region. Thirteen days after the seizures, he suddenly felt very anxious and was not able to sleep. On the following day, he showed a blank look and slow responses to any stimuli, together with persistent abnormal behaviors such as wandering aimlessly. His EEG showed continuous slow waves over the bilateral frontal area. His SPECT showed hyperperfusion in the left orbitofrontal region. On the basis of those clinical symptoms and the results of the EEG and SPECT, he was diagnosed with CPSE of the frontal lobe region. The hyperperfusion on the SPECT resolved after two months of CPSE. This suggests that ictal SPECT is very useful in diagnosing CPSE and localizing epileptogenic zones.

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