A clinical evaluation of brain growth is usually based on the serial measurement of head circu mference.In 1956, Mac Kinnon et al.devised a formula of calculating skull volume in adults. However, the study of the roentgenographic measurement of the skull volume has been repor ted by only a few authors.In this study the rela tionship between head circumference and skull volume was made.
In 173 normocephalic subjects (101 males and 72 females) in the range from 28 days to 14 years of age, both head circumference and skull volume was measured.Normal head circumference was determined on children below 6 years of age by the statistical data of the Ministry of Health and Welfare of Japan and on children over 6 years of age by the Nelhaus Standard.
The formula of the skull volume: V=0.51 (1/2×I×L×H+1/2×I×L×B)cm³. (Mac Kinnon et al.).L=true A-P diameter, H=true distance from vertex to the midpoint of the external audi tory meatus, B=true distance from bregma to the deepest part of the posterior fossa and I=widest transverse distance biparietally or bitem porally.
A significant correlation of headd circumferen ce with skull volume was found (r=0.93).The regression equation was Y=66.1 X-1, 820.Ther efore the head circumference reflected intracra nial volume accurately.The developmental curve of the skull volume was almostin accord with that of the head circumference.The skull volume in males were slightly larger than in females.It was concluded that the head circumference is one of the best index of brain growth in normal child ren.

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The skeletal muscle fibers can be divided into four types, type I, II A, II B and II C fibers by ATPase staining with preincubation at pH 9.4, 4.6 and 4.2; type II C fiber has been designated as an undifferentiated or precursor fiber observed dur ing the fetal in humans and at the fetal as well as neonatal stage in rats. Since the type II C fibers are frequently recognized in the diseased muscles of infants, we examined the biopsied muscles from three cases with Werdning Hoffmann dis ease at the ages of 11/26 and 8months respecti vely, and a 9month-old male infant with Fuku yama type congenital muscular dystrophy and a 10-month-old paralytic female with probable cervical cord injury at birth.
In Werdnig Hoffmann disease, the frequency of type II C fibers differed from case to case and from fascicle to fascicle, ranging from 8 to 65% of muscle fibers with the average of 20%. Nume rous type II C fibers were also observed in cases with congenital muscular dystrophy (26.3%) and probable spinal cord injury (8.8%).
Occasional type II C fibers seen in some muscle diseases including Duchenne muscular dystrophy, polymyositis etc. have been thought to be rege nerating or regerated fibers. However, those fi bers in the infantile neuromuscular diseases in this study may have been originated in a different manner because of a lack or paucity of regene rating process. The maturation arrest of muscle fibers has been proven experimentally after de nervation in rats and guinea pigs at the neonatal stage.Persistence of numerous myotubes and/or type II fibers were predominantly seen in those animals. Thus, the presence of numerous type II C fibers in various infantile neuromuscular disor-ders may reflect a delay or arrest in the muscle fiber type differentiation induced by a certain damage to muscle fibers, either neurogenic or myogenic, before or during muscle fiber type dif ferentiation.

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Computerized tomograms in six childhood cases of Moyamoya disease, with the ages from 1 year and 7 months to 10 years and 9 months, using EMI scanner 1, 000 with the 160×160 matrix were reported, and were compared with those in five normal children. One slice at the level of cerebral basal ganglia was selected, and macroscopic findings of polaroid photographs and EMI num bers based on print-out data of EMI scanner were investigated.The main CT scan abnormali ty in children with Moyamoya disease was thelow density area in the white matter.The loca tion and the extent of the lesion correlated well with the severity of clinical and neurological symptoms.Cerebral basal ganglia, which had been expected abnormal on CT because of the existence of Moyamoya vessels, revealed normal images in all cases, but EMI numbers showed abnormal values unilaterally in one case. Thus, EMI numbers were useful in order to detect the fine abnormalities, which seemed normal mac roscopically.

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Sturge-Weber disease is characterized by veri ous clinical symptoms such as facial nevus (port-wine stain), epilepsy, hemiparesis, mental retar dation and hemianopsia.
Plain skull roentgenography has been almost the only measure to disclose the intracranial le sion of this disease, because cerebral angiography and RI brain scanning were not so useful for this purpose.In this report, eight patients of Sturge Weber disease were studied by computed tomo graphy (CT) scan, and in six of them enhance-ment study was also performed to evaluate the role of this procedure in this disease.
According to these results, nonenhanced CT scan was far more useful than plain skull films for the detection of the secondary intracranial changes such as calcium deposits and cerebral atrophy.From the extent of calcification on this study, Sturge-Weber disease was classified into two groups: “localized”and“diffuse”types.
The most striking finding in this enhancement study was visualization of the choroid plexus with abnormal enhancement in five patients.This was interpreted as angiomatous changes in the choroid plexus as described by Falconer in his operative record (1960).
The leptomeningeal angiomatosis over the cal cified cortex has been a well known pathological finding.By means of enhancement study, diffuse enhancement over the hemisphere was observed in one case with localized calcification by nonen hanced CT scanning.A wide area adjacent to calcification was enhanced in two cases, and in three cases there was no detectable enhancement. Probably these findings indicate the different stages of this disease.These results led us to conclude that the area enhanced by contrast media seems to be diminished as the lesion of calcification is growing in size.It is also sugge sted that the early diagnosis would be possible by enhancement study even before any calcification is disclosed by nonenhanced CT scan, and that the extensively enhanced case of“localized” type would progress into“diffuse”type in our defini tion.
In conclusion, enhancement study would be indispensable not only for the detection of the precise intracranial lesion, but also for the eva luation of the pathological stages of Sturge Weber disease.

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CT brain scans were performed on approxima tely 1500 patients at the Matsudo clinic. Among them the apparently normal CT pictures were selected for quantitative measurements from 89 patients with convulsive seizures, headache or head trauma, but without any neurological ab normalities or any positive past or family history.
(1) Size and shape of the brain parenceyma
No remarkable changes in the transverse dia meter (b) with age were noted, but the longitudi nal diameter (a) gradually increasted up to the age of 2 years.
(2) Ventricular system
The ratio of the largest width of the anterior horn of the lateral ventricle to the transverse diameter of the brain (c/b) remained less than 30% in each age group, being some-what larger below the age of 1 year, low between 2 and 9 years and subsequently increasind with advancing age.The ratio of the lateral ventriclecaudate nucleus distance to transverse diameter of the brain fell between 9-11% in each age group.The width of the 3rd ventricle (e) was less than 5.7mm in each age group.
3) Subarachnoid space
In six children below the age of 1 year, the width was 1.9mm in one patient, 3.8mm in two patients and 5.7mm in three patients.In all sub jects, subarachnoid space with a constant width was noted only in the frontal temporal regions.
In only one achild between 1 and 2 years of age, the subarachnoid space had a width of 1.9mm while measurement was not possible in seven other patients.
In a ll subjects above the age of 2 years, mea surment was not possible.
4) Longitudinal These were distinctly noted below 1 year and above the age of 40 years.cerebral fissure and Sylvian fissure and sulci cerbri
These were distinctly noted below 1 year and above the age of 40 years.

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Two siblings with Cockayne's syndrome, 4 year and 2-year old girls, were reported.Moreo ver, clinical findings of 20 cases with this synd rome, including our two cases, in Japan were discussed.
Case P.O. (4-year) has been mentally retarded since 3 months, and exhibited cachectic dwar fism, pepper and salt type retinitis pigmentosa, photosensitivity, microcephaly, motor disturban ce and cerebellar signs.These signs were typical of Cockayne's syndrome.
Case S.O. (2ye ar), a younger sister of R.O., has been retarded in motor develoment since 8 months of age, and exhibited pepper and salt type retinitis pigmentosa, photosensitivity, microceph aly, mental difficiency and cerebellar signs.The re was no cachectic dwarfism, but short stature was present.These signs were not typical of Cockayne's syndrome.
In both c ases, computed tomography of the brain showed calcifications in the basal ganglia, and in R.O.the basal ganglia was enhancnd after infusion of contrast medium.This finding sugge sted that the blood-brain barrier was destroyed in the basal ganglia.
In both cases, nerve conduction velocities were determined in the right median nerve and the right peroneal nerve.Peripheral neuropathy was found in R.O., evidenced by markedly slow nerve conduction velocities.In S.O., nerve conduction velocities were at the lower limits of normal val ues or slightly slow.
The serum arylsulfatase-A activity in both cases was normal.
Clinical findings of Cockayne's syndrome in Japan were reviewed.The estimated age of onset varied from I month after birth to 3 years of age. Ten males and nine females were reported.In one case the sex of the patient was not referred.In the family history, consanguinity was found in 11 families of 17 affected families.There were 6 sib ling cases in 3 families.The results described above with regard to the family history are com patible with the autosomal recessive mode of inheritance.The clinical signs of 20 cases inclu ded mental deficiency in 19 cases, cachectic dwa rfism in 17 cases, photosensitivity in 16 cases, pepper and salt type retinitis pigmentosa in 15 cases, microcephaly in 16 cases and ankylosis in 16 cases.Other signs such as partial deafness, sunken eyes, optic atrophy and cerebellar signs etc.were observed in some more than half of cases.

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A 2 years old Japanese girl with a persistent primitive trigeminal artery (PTA) associated with aneurysms was reported.
She was born at Ap ril 21, 1973.Her gestational and neonatal history was unremarkable.She had been well untill July 9, 1975 when she had a first attack of convulsion. She was admitted to a local hospital but covulsive seizures continued about 6 nours with high fever in spite of treatment. When seizures were controlled, she was found to have right flaccid hemiplegia and aphasia.Seven days after the onset she was admitted to our hospital for further examination.
Neurological examination revealed that the right deep tendon reflexes were slightly dimini shed and Babinski sign was not elicited.The cerebrospinal fluid by lumber puncture was nor mal.EEG revealed a considerable slowing with decreased voltage in the left hemisphere. A left carotid angiography showed the presence of PTA and several aneurysms in the left internal carotid artery. PTA associated with aneurysm is rare, especially in children, and this is probably the first case report in Japan.

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A VA-year-old boy was admitted to our hospi tal because of frequent episodes of vomiting, mu scle weakness and heart failure which were usually initiated by febrile diseases including common cold, measles and viral pneumonia.Sli ght delay in motor and intellectual milestones was recognized;he learned to walk at the age of 18 month.On admission he was metally retarded (I.Q.:68), and of short and slender stature. Neu rologically fine finger tremor, generalized muscle hypotonia, weakness and atrophy, and weak ten don reflexes were noted.
The laboratory findings disclosed increased serum enzymes including CPK, GOT, GPT, nd LDH during the episodes.Remarkably elevated GOT isozyme, m-GOT, up to 115 (m/s=1.2) was thought to reflect mitochondrial jujury. Both serum as well as cerebrospinal fluid pyruvate and lactate were always elevated, more markedly during the episodes. An inhibitor to thiamine phosphateadenosine triphosphate phosphoryl transferase, an intramitochondrial entyme was detected in urine, suggesting a possibility of Leigh's encephalomyelopathy.Pyruvate dacarbo xylase activity in the muslce was low.
In the biopsied muscle, “ragged-red” fibers were demonstrated, characterized by accumula tion of a coarse material in red color on modified Gomori's trichrome staining.The number of mi tochondria was increased in hepatocytes.They showed an abnormal matrix and frequent loss of cristtae.
Electrocardiography revealed right ventricular hypertrophy and abnormal T and ST waves representing myocardial ischemia. A moderately dilated ventricular system in the brain was demonstrated by computed tomographic study. The awake electroencephalogram showed diffuse very slow activity in the all areas.
The presence of abnormal mitochondrial struc ture in the skeletal muscle and liver, and cardio myopathy suggetsts that the present case might have abnormal systemic mitochondrial metabo lism involving various organs.

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5year-old boy was admitted to our service with the chief complaints of the remittent fever (38-39°C), neck stiffness, vomiting and headache on Aug. 2, 1975. He was treated as having puru lent meningitis for over 2 months prior to admis sion to our service.His general condition was improved but findings of his cerebrospinal fluid remained unchanged. On Oct. 11, he had a clonic seizure of the right side of the body.EEG exami nation.brainscintigraphy and left carotid angio graphy revealed mass lesion in his left parietal lobe.Craniotomy was performed on his left pari etal region on Nov.4 (th). There was an abscess in the left parietal lobe and the capsule was re moved totally. The histological examination of the capsule presented a typical findings of tuber culous lesion. Subsequently the administration of Streptomycin.Isonicotinic acid hydrazide and Etanbutol and Rifampicin were instituted. For the increased intracranial pressure.removal of bone flap. the ventricular drainage and a ventri culoperitoneal shunt were done.The patient was discharged without neurological deficit except total blindess on May.21.1976. and maintained his health up to present (May.1978).
Tuberculous meningitis is very few nowadays and the cases of tuberculous meningitis with brain abscess formation seemed to be extremely rare and fatal in most of the cases.
A conservative therapy is made for tuberculous meningitis.But the mortality is high and the sequelae caused by the increased intracranial pressure is serious. If the ventricular drainge is made at the chronic stage.the mortality and the sequelae will decrease.
It is our impression that neurosurgical mana gement was essential for the diagnosis and the successful treatment in this case.

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Two patients with two different inherited dis eases, homocystinuria and Cockayne's syndrome, were found in one family.Their family tree had a dense consanguinity.The elder sister was a pa tient with homocystinuria which was due to cy stathionine synthetase deficiency and vitamine B₆ responsive.She had ectopia lentis, arachnodacty lia, osteoporosis and mental retardation. The younger sister was diagnosed as Cockayne's syndrome from her clinical signs of cachectic dwarfism, microcephaly, senile-like appearance, carious teeth, kyphosis, unsteady gait, intention tremor, mental retardation, retinal pigmentation, optic atrophy, photosensitive skin, cool extremi ties and decreased sweating and tearing.
EEG was normal and peripheral nerve conduc tion velocity was markedly reduced.The endo crinological studies revealed normal secretion of GH, TSH, PRL, LH and FSH, but much more PRL, LH and FSH were secreted during sleep in this patient than in controls.These findings indi cate the functional abnormalities of hypothala mus in this patient.Furtheremore, the platelet aggregation was markedly enhanced in the youn ger sister as in the elder sister with homocysti nuria.
A disorder in lipid metabolism has been sug gested but the basic defect still remains to be determined in Cockayne's syndrome.Both homo cystinuria and Cochayne's syndrome are inherit ed as autosomal recessive trait and considered different disease each other, but these diseases have similar abnormalities in ocular, skeletal, central nervous, cardiovascular and other system. It is very interesting whether two different inhe rited diseases appeared incidentally in the sib lings or common genetic defects exist in these two diseases.

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