Laryngeal stridor associated with congenital hindbrain malformation such as Arnold-Chiari malformation, myelomeningocele and hydrocephalus has been reported and recognized in otolaryngological and pediatric literatures, but scanty in neurosurgery. The authors treated a posterior fossa tumor of a child with laryngeal stridor which disappeared after operation. Surgery disclosed caudal displacement of the medulla compressed by a cystic tumor. Although uncertain in detail, the pathogenesis of laryngeal stridor in such posterior fossa lesions is assumed to be the traction of the vagus nerve exerted by a caudally displaced medulla at the craniovertebral junction. The authors reviewed the literatures and discussed the pathogenesis of laryngeal stridor in association with congenital malformations and a tumor in the posterior fossa. Treatment of the acute respiratory distress secondary to laryngeal stridor was also mentioned.
Though it is widely known that anatomically there exists a communicating channel between the carotid and vertebral arterial systems, this occipito-vertebral anastomosis can be hardly visualized by the conventional angiography and little has been reported about the angiographical demonstration of the anastomosis. Two cases were presented in which the anastomosis was visualized by carotid and vertebral angiographies. The first case is a 15-year-old female and clinical diagnosis was a teratoma of the temporalregion on the left side. In this case the carotid angiography of the right side incidentally resulted in visualizing the external carotid system selectively and the occipito-vertebral anastomosis was demonstrated in the angiogram. The second case was also a 15-year-old female and was suffering from noncommunicating hydrocephalus of unknown origin. The left vertebral angiography by percutaneous procedure, revealed that basilar artery was occluded just proximal to its bifurcation and at the same time the external carotid system on the left side was also visualized through the anastomosis. Significance of the anastomosing channel was discussed and it was suggested that the anastomosis could be a potential collateral channel which would function when the cerebral circulation was disturbed by intracranial pathological processes.
Changes in the basic wave patterns of EEG were investigated on 349 healthy children under 4 years of age. The basic waves of EEG, which obtained from the right occipital area, were divided into 5 or 10 bands according to frequency ranges by means of a conventional or specially prepared automatic analyzer, respectively. Results were as follows: 1) δ waves, which dominated in energy percent in newborn, continuously decreased thereafter. 2) θ wave increased rapidly and reached the peak of their energy percent at 7 months of age. The peak remained as such until 3 years of age, and rapidly decreased in the energy percent thereafter 3) α waves increased linearly with advance of age particularly during the ages of 3 to 6. 4) The energy percent of β1 and β2 waves was observed to be very low in all of these ages. 5) The θ waves surpassed δ at 7 months of age and α did θ during 4 to 5 yeares of age.
Three typical cases of Tay-Sachs disease were reported. Membranous cytoplasmic bodies were demonstrated electron microscopically in myenteric plexus ganglia of appendix surgically removed in one case. Reviewing Japanese literature, it was found that 46 cases of Tay-Sachs disease had been reported since 1900 till 1969. On 49 cases, including 3 personal cases here reported, all Japanese in nationality, some clinicogenetical aspects were analysed. The following is a summary of the results. 1) Sex ratio: Male: female=1: 1.13 2) Consanguinity in family history positive in 19 among 41 families (46.3%) 3) Fraternal recurrence rate=23.4% 4) Age of onset: most commonly between 6 to 8 months of age 5) Cherry-red spot: 48/49 (98%) 6) Convulsions: 15/21 (70%)
Two cases of the acetonemic vomiting associated with epilepsy were studied with the systemic in travenous administration of small dose (1mg/kg) of thiopental sodium. The following three EEG findings were carefully assessed following immediately the administration of thiopental sodium: (1) the induction and the distribution of the beta rhythms; (2) the behavior of the bilateral spike & wave discharges, present in the baseline EEG; (3) the appearence of focal elements. One case responded with a prompt induction of symmetrical beta rhythms, depression in the occurrence of the bilateral spike & wave discharges, and no focal features. Another case responded with a almost absent beta rhythms diffusely, persistence of the bilateral spike & wave discharges, and no focal features. The former may be classified according to Lombroso's discription as having primary centrencephalictype (Primary Bilateral Synchrony), and the latter as having cortico-centrencephalic type.
The patient was a girl whose parents were consanguineous. She showed developmental retardation with progressive dysfunction of the motor system soon after birth, and died at 3 years and 10 months of age. The major clinical findings at 2 years and 11 months of age were loss of hearing and speech, hypotonia of skeletal muscle, absence of tendon reflexes, loss of pain sensation, and optic atrophy. The EEG showed spike and wave, and PEG showed slight dilatation of the lateral ventricles. The pathological examination revealed numerous spheroids in tegumentum of the brain stem and posterior horn of the spinal cord, and abundant pale eosinophilic bodies in the cerebral cortex, basalganglia, thalamus and hypothalamus. Large histiocytes were noted in the spleen and lymphnodes, and Kupffer's cells in the liver were also hypertrophic. These cells had eosinophilic and PAS-positive granular materials in the cytoplasm.