We describe an 8-year old boy with pseudobulbar palsy and speech disturbance associated with fetal distress, mild birth asphyxia and probably intracranial hemorrhage. There is a remarkable dissociation between orofacial voluntary movements and emotional expression. MRI revealed cortical atrophy and subcortical gliosis of both opercula. The clinical and neuroradiological features correspond to bilateral opercular syndrome. We assume that a reduction of cerebral blood flow involving both opercula induced pseudobulbar palsy with severe expressive speech disturbance.

Asphyxia↗

PubMedDate not supplied

Non-radioactive DNA diagnosis for the fragile X syndrome in mentally retarded Japanese males.

A rapid screening test was developed to detect CGG repeat expansion of the FMR-1 gene causing the fragile X syndrome by a non-radioisotope PCR technique. A biotin-labeled primer was initially used and the biotin-labeled PCR product was detected by means of chemiluminescence. The normal PCR product of around 300 bp was not created in the abnormal FMR-1 gene sample with this method. Four positive samples were found among those from 226 mentally retarded males, but the CGG repeat expansion was shown on Southern blot analysis in only one sample. To eliminate false-positive samples, a hybridization method involving a biotin-labeled (CGG)s oligonucleotide was developed for the PCR product and the CGG repeat expansion could be detected. Finally, 256 mentally retarded males in Japan were examined and only 2 abnormal samples were detected. The prevalence of this abnormality was less than 1%, which is relatively lower than those reported previously.

Base Sequence↗

PubMedDate not supplied

Prognosis and MRI after ischemic stroke of the basal ganglia.

The clinical courses and long-term prognoses in 16 young patients with infarctions of the basal ganglia were evaluated and the recent magnetic resonance imaging findings in 9 of them were examined. Only 5 of 14 patients (35%) had motor sequelae, 4 had hemiparesis, and 1 had gait disturbance. Secondary dystonia occurred in 1 patient. Magnetic resonance imaging disclosed a circumscribed lesion in the basal ganglia, as reflected by T2 high- and T1 low-intensity signals, in all patients. The abnormal region on T2-weighted images usually was more extensive than that observed on T1-weighted ones. The hemiplegic patients each had an area of abnormal intensity in the internal capsule or corona radiata with relatively high signals on the T2- and proton-density-weighted images. Mild to moderate asymmetric atrophy of the midbrain on the side ipsilateral to the stroke lesion was observed in 8 of 9 patients. The mechanism involved may be remote transsynaptic neuronal death of the substantia nigra, as well as Wallerian degeneration of the pyramidal tract.

Adolescent↗

PubMedDate not supplied

Absence of ganglionated plexus in bullfrog gallbladder.

We first carried out microscopic observation of the intramural nerves of a bullfrog gallbladder which were fixed and stained with a solution of OsO4 and ZnI2. We then investigated if the responses of isolated frog gallbladder evoked by electrical stimulation are mediated through the intramural nerves. The following results were obtained: 1. The nerve plexus and the perivascular nerves were observed in the subserosal layer of the wall of the gallbladder. These nerves do not have a ganglia. That is to say, no ganglionated plexus or ganglia were observed in the subserosal layer of the wall of the gallbladder. 2. Electrical stimulation caused the gallbladders to contraction with rectangular pulses (50 volt, 40 Hz) of durations of 0.5, 1, 2, 3, 4 and 5 msec for a period of 10 sec. Three blockers of nerve mediated responses, atropine (1 x 10(-6) M), guanethidine (1 x 10(-6) M) and tetrodotoxin (3 x 10(-7) M), had no effect on the gallbladder contractions induced by stimulation with pulses as short as 0.5 msec or as long as 5 msec. These results suggest that the bullfrog gallbladder may not contain nerves related to movement. Thus, the contraction of the bullfrog gallbladder induced by electrical stimulation does not seem to be modulated by extrinsic nerve terminals distributed in the gallbladder wall.

Animals↗