Clinical characteristics of upper airway resistance syndrome.

Polysomnographic findings and clinical symptoms were investigated in 14 cases of upper airway resistance syndrome. The mean scores of the Epworth sleepiness scale and self-rating depression scale in eight cases were 13.5 and 38.6, respectively. The mean sleep latency of the multiple sleep latency test in four cases was 10.2 min. Seven cases were treated with continuous positive airway pressure (CPAP), and one with hormone replacement therapy. The most common symptom was daytime sleepiness. Five cases had hypertension. CPAP reduced increasing negative esophageal pressure (Pes) and frequency of EEG arousals, and improved hypertension in one case. Hormone replacement therapy ameliorated increasing negative Pes and clinical symptoms.

Preliminary report on teratogenic effects of zonisamide in the offspring of treated women with epilepsy.

PURPOSE: We wished to assess the risk of teratogenicity of zonisamide (ZNS) in humans. METHODS: Pregnant epileptic women treated with ZNS and their offspring were prospectively monitored from June 1989 to December 1994. The outcome of pregnancy and status of neonates were examined based on the same standardized protocol. RESULTS: Twenty-six offspring exposed to ZNS with or without other antiepileptic drugs (AEDs) were studied. Malformations were detected in 2 offspring (7.7%) exposed to ZNS polypharmacy. Anencephaly was detected in one case at 16 weeks of gestation (case 1, artificial abortion), and atrial septal defect was detected in another case at 37 weeks of gestation (case 2, delivery by cesarean section). Serum concentrations of ZNS during the first trimester of pregnancy were 6.1 micrograms/ml in case 1 and 6.3 micrograms/ml in case 2; in both cases, the levels were below the therapeutic concentration range of ZNS. CONCLUSIONS: Teratogenic effects of ZNS were not clearly defined from these results since malformations were detected in two polypharmacy cases but not in four monopharmacy cases. The present data do not indicate that the risk of ZNS teratogenicity is greater than that of other conventional AEDs. However, such risk cannot be neglected even at therapeutic dosages or concentrations of ZNS, especially in patients receiving poly-pharmacy.

Reflex epilepsy induced by calculation using a "Soroban," a Japanese traditional calculator.

We report three cases of reflex epilepsy with myoclonic jerks of the right arm and fingers precipitated by calculation using a Soroban. An EEG spike-wave complex with left central prevalence was induced. Various types of stimulation were used to induce epileptic discharges, and a simultaneous mental task requiring a high degree of concentration and complicated and delicate finger movements was necessary to induce the epileptic discharges. Comparison of our cases with previously reported reflex epilepsy induced by higher mental activity led to the assumption that the neural mechanism inducing seizures in our cases is similar to that of writing epilepsy. Valproate was effective in reducing epileptic discharges, and all patients became seizure-free.

REM sleep without muscle atonia (stage 1-REM) and its relation to delirious behavior during sleep in patients with degenerative diseases involving the brain stem.

Nocturnal sleep was examined in 12 patients with degenerative diseases involving the brain stem and in 2 patients with late cerebellar cortical atrophy (LCCA). A peculiar sleep state, characterized by the concomitant appearance of a low-voltage mixed frequency EEG, rapid eye movements (REMs) and tonic EMG in mental muscles, repeatedly appeared during nocturnal sleep in all of the 12 patients with degenerative diseases involving the brain stem and it was called stage 1-REM after Tachibana et al. In 8 of the 12 patients, delirious or oneiric behavior appeared during, or soon after, the episodes of stage 1-REM. Inner experiences reported by one of the subjects well corresponded to his behavior during the episode of stage 1-REM. Stage 1-REM was not observed during nocturnal sleep of the patients with LCCA. These results indicate that a degenerative lesion in the brain stem induced stage 1-REM and delirious behavior during nocturnal sleep through abolishing muscle atonia of REM sleep and causing dissociation of the functional components characterizing REM sleep.

Three epileptic patients suffering from periods of frequent complex partial seizures and diagnostic criteria for epileptic status.

Presented here are three epileptic patients suffering from periods of frequent complex partial seizures. The importance of the following two points for the diagnosis of epileptic status of complex partial seizure is stressed. The first is the importance of EEG examination for differentiation of such epileptic status from psychogenic attack and absence status. The second is the importance of disturbed consciousness lasting between seizures. Based on EEG and clinical findings in our three patients, it was concluded that two of the three patients suffered from epileptic status of complex partial seizure and that the condition in the remaining one patient could not be judged as epileptic status of complex partial seizure. Detailed clinical and electroencephalographic findings in previously reported seven patients suffering from status epilepticus of complex partial seizure were compared with those of our two patients. The seven cases out of nine patients had epileptiform EEG activities beginning with localized rhythmic activities. All of the nine patients showed marked motor manifestations during the complex partial seizure such as adversive movement and automatic behaviors. Such motor manifestations of complex partial seizure are very useful for confirming the initiation and termination of the seizure. When the frequency of complex partial seizure is markedly increased, it become difficult to detect the initiation and termination of each seizure, and the discontinuous form of status epilepticus of complex partial seizure passes into its continuous form.

Positive occipital sharp transients in the human sleep EEG.

The characteristics of positive occipital sharp transients (POSTs) in the human sleep EEG were studied, and their characteristics were compared with those of lambda waves appearing in the occipital EEG during the waking state. The following findings were obtained: 1) POSTs were observed in 57% of 189 subjects examined during sleep. The incidence of the subjects showing POSTs was highest in the group of subjects aged 21-30 years. 2) The incidence of the subjects showing POSTs tended to be high among the subjects with a high alpha wave index. 3) The wave form and topography of POSTs had striking similarities to those of lambda waves. 4) The frequency of POSTs was highest during the initial 30 minutes' period of NREM sleep after the sleep onset in both nocturnal and diurnal sleep. 5) The frequency and amplitude of POSTs were not modified by changing the background illumination in the recording room. 6) No significant relation was found between the frequency of POSTs and dream experience during NREM sleep.

Sialidosis type 2 in Japan. Clinical study in two siblings' cases and review of literature.

Clinical and biochemical findings in two siblings (24-year-old sister and 20-year-old brother), born to consanguineous parents, are described. Both showed progressive generalized myoclonus, macular cherry-red spots, moderate cerebellar ataxia, coarse facies, vertebral deformities, vacuolation of peripheral lymphocytes, bone marrow cells and epithelial cells of conjunctiva, but had normal intelligence. The excretion of sialyloligosaccharides in the urine of both was 3-5 times larger than in the controls. The activities of alpha-neuraminidase and beta-galactosidase in leukocytes and cultured skin fibroblasts were reduced. The clinical and biochemical findings were those of the newly described condition, sialidosis type 2. Review of all Japanese cases considered as sialidosis type 2 showed that the most characteristic clinical features as distinct from type 1, were coarse facies and bone deformities, especially of vertebral bodies.