Clinical and electroencephalographic aspects of the Lennox syndrome on eighty subjects were studied.1) Age of onset: It most commonly occurs between one and six years (60 cases 76% in our series). The earliest onset in our group was six months.2) Clinical seizure pattern: All the cases exhibi ted both tonic spasms and atypical absence or either of the two. Twelve out of the eighty patients (15%) also exhibited an additional astatic seizure.3) Underlying disease: Twenty-six patients (32.6%) were idiopathic. The majority w ere found to have various underlying illnesses.4) Mental deficiency: Seventy patients (87.5%) were found to have mental deficiency.5) Electroencephalogram: Diffuse slow spike-waves were observed in all subj ects in interictal period. Desynchornization, rapid synchronization and recruiting rhythm were seen during ictal period. These findings indicate the diencephalon as the origin of pathophysiology.6) Longitudinal follow-up on each case revealed close relationship of this syndrome with infantile spasms. It also revealed that some changed to generalized epilepsy.7) Conclusion: The Lennox syndrome is a form of childho od epilepsy with a strong age dependency. It has a specific seizure pattern and EEG features. Mental deficiency is one of the cardinal features of this syndrome, the majoriy of the patients resisting therapy. Thus, this syndrome should be regarded as an independent clinical entity. Although there are multiple factors considered contributing to the formation of this syndrome, dysmaturity of the brain seems essential as one can see from the age distribution in this syndrome. We conclude, therefore, that the combination of immaturity of the brain and cerebral dysfunction plays the basic role in manifestation of this syndrome.
The thirty-eight patients with acute idiopathic polyneuritis who were hospitalized at Department of Pediatrics of Kyushu University from 1962 through 1968, were studied. Twenty-three of the 38 patients were male and other fifteen pat ients were female. Preceeding illnesses occurred in 21 patients: upper respiratory infections, 14, diarrhea, 5, mumps, one, and acute otitis media, one.
Two autopsy cases of infantile subacute necrotizing encephalopathy were reported. The first case was a 7 month old boy, who showed psychomotor retardation at the age of 4 months, developed infantile spasms with hypsarrhythmia, and died suddenly with unexplained fever and dyspnea. The postmortem examination of the brain revealed symmetrical necrosis of bilateral lenticular and caudate nuclei. Sharply demarcated and symmetrical foci of necrosis were noted in the tegmentum of the midbrain and the pons. Histologically, the lesions were characterized by spongy degeneration, cavity formation, and proliferation of small blood vessels, being consistent with Leigh's subacute necrotizing encephalomyelopathy. The mammillary body was intact, though a necrotizing lesion was found around the third ventricle.
A 17-year-old boy with peculiar movement of head, hands and trunk with rhythmic oscillation, 1to 2 per second forward and backward was reported. This tic-like movement said to have developed after he has suffered from an unknown severe febrile illness. On pneumoencephalogram striking enlargement of lateral ventricles and communicating cavum septi pellucidi were demonstrated. The boy made a remarkable recovery, following a right ventriculoauriculostomy with insertion of a Pudenz valve. Hence, the bobbing tremor might be considered to be nonspecific manifestations of hydrocephalus which presumably causes pressure upon basal ganglia or other extrapyramidal structures. Of clinical interest is an association with psychopathic tendency such as freque nt vagrancy, confabn tendency, collectomania and emotional instability which was not found in the previous cases. These personality changes might be considered from the same viewpoints as the bobbing tremor in which disturbance o f basal ganglia seems to play an important role. The bobble-head doll syndrome is a peculiar neuropathlogical disorder which seems to be one of the extrapyramidal disturbances, but this bobbing tremor is a unique one; the head bobbing movement disappears or at least markedly decreases, following surgical relief of hydrocephalus.
Long hour polygraphic recordings were repeatedly performed in 19 premature, 8 small for date and 3 full term infants. The recordings were made every week or two after delivery until the time of their calculated date of aerm gestation. No difference is presen t electroencephalographically among REM sleep, wakefulness and non-REM sleep before 32 weeks of conceptional age. EEG is characterized by burst-suppression pattern.1320c/s spindle like fast rhythm with 0.5-1.5 slow waves are characteristically seen in bursts. Difference between REM and non-REM sleep appears for the first time around 3 2-33 weeks. The latter is characterized by burst-suppression pattern (an immature pattern of trace alternant). After 34-35 weeks, REM sleep shows low voltage desynchronized pattern with some semirhythmic theta and alpha waves which increase with advancing age. Difference between moderately deep sleep and deep sleep appears for the first time around 36-37weeks. The former is characterized by increase of slow waves and their amplitudes (slow wave sleep pattern). The latter shows trace alternapt.13-20c/s spindle like fast rhythm decreases gradually to disappear around 40 weeks. After 36-37 weeks EEG shows gradual development with increase of activity in each stage. Burst-burst intervals of trace alternant decrease gradually with advancing age but their modal values are relatively constant throughout various conceptional ages.
Neonatal purulent meningitis (20 cases) of the 401 autopsied cases in the neonatal period were examined pathologically. Postmortem cerebral microangiography and Benzidin stain were done in 5 of those cases.1. In the cases of purulent meningitis, vasculitis, thrombosis and subarachnoid bleeding were found in the meninx, and cerebral edema, periventricular leukomalacia, ventriculitis and ependymitis were found in the cerebrum.2. The histo l ogical findings in the periventricular white matter and matrix, and the localization of the degeneration of nerve cells in the grey matter were similar to those caused by anoxic changes.3. The histological and microangiographical observations showed the feature of the cerebral circulatory disturbance, such as vasculitis and thrombosis in the meninx, and venous stasis, vascular tortuosity and unfilling blood vessels with contrast media in the cerebrum and meninx.4. The periventricular leukomalacia occured often in the neona tal purulent meningitis. The development of periventricular leukomalacia in the neonatal meningitis might be concerned to perinatal abnormalities, pulmonary complications or cerebral circulatory disturbance. The cerebral circulatory disturbance and edema might promote the local cerebral hypoxic state in the periventricular regions.
More than 30 cases had been reported under the diagnosis of congenital muscular dystrophy (CMD)in literature, but it is a debatable problem whether this is a clinical entity. By clinical and g enetical studies of 78 cases of CMD, visited to the pediatric department from 1959 to 1968, the author co ncluded this is one subtype of progressive muscular dystrophy. Clinical characteristics are as follows; proximal muscular weakness and hypotonia with facial muscle involvement, though floppy in neonatal period, joint contractures developed earlier, DTRs are diminished or absent, and mental retardation; moreover febrile or afebrile convulsion occurred with high incidence. Clinical course is slowly progress ive, but only few cases could walk. Laboratory data are compatible with those of Duchenne type PMD exce pt the low motor nerve conduction velocity and occasional idiopathic hypercholesterolemia. Mode of g enetic transmission was considered to be autosomal recessive.