Experience in the angiography for intracranial inflammatory processes is limited. Some cases with intracranial inflammation may begin with signs resembling a space taking lesion or with strokes or stroke-like episodes. In such cases much information will be provided by cerebral angiography as to the nature of the disease and its extent.
The arterial stenosis or obstruction due to intracranial inflammation has been considered to occur by three mechanisms; arteritis, mechanical compression by inflammatory exsudate and spasm.
Authors reported 2 children with angiographi cally demonstrated obstruction of main cerebral arteries. One is a year & 8 month-old boy who showed occlusion of the left middle cerebral artery with tuberculous meningitis. The other is a 2 month-old girl who showed an obstruction of the supraclinoid portion of the right internal carotid with a huge subdural empyema contral taterally. The autopsy disclosed the cause of the obstruction of the arteries, the arteritis in the first case, and the mechanical compression by packed empyema without any inflammatory changes of the vessels in the second.

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Electron microscopic observation was perfor med on biopsied muscles from two cases of Werdnig-Hoffmann disease.
The electron micrographs of degenerated muscles revealed various morphologic alterations consisted of: disarray and disappearance of myofilament; vacuolation of mitochondria and sarcoplasmic reticulum; irregularity and frag mentation of Zdisc; an increase in the width of the interfibrillary space; and focal dissociation of the basement membrane from the plasma mem brane. The degenerated nuclei with vacuolation and deep infoldings of nuclear membrane were found in atrophic muscle fibers.
The primary lesion of muscle degeneration could not be identified, due to an irregular ap pearance of above-mentioned histologic changes in our material obtained from severely affected children. Peripheral nerve fibers and muscle spindles among atrophic fibers showed no ultra structural change.
The satellite cells did not increase in number and contained only a few cell organelles showing a regressive alterations along with muscle fiber degeneration.

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Peripheral nerve involvement was reported in a case of Sjögren-Larsson syndrome. The patient was a 10 year-old boy with congenital ichthyosis, spastic paraplegia, and mental retardation. The motor nerve conduction velocity of his right ulnar nerve was reduced to 19.3m/sec, and CSF protein was increased to 195mg/dl. The sural nerve biopsy was performed, and the specimen was fixed in 1% osmic acid, and embedded in Epon.
Light microscopically, presence of myelinated fibers measuring 8 ki or less in diameter, “onion -bulb” formation with proliferation of Schwann cells and endoneural fibrosis were recognized. Electron microscopically, irregularity in size and shape of unmyelinated fibers and crenation of the axon in myelinated fibers were noted. Prolifera tion of the myelin sheath, its separation from the axon cylinder, and its circumferential cleavage were also noted. Collagen fibrils proliferated markedly around the nerve fibers.
It was not clear whether Sjogren-Larsson syndrome usually had such peripheral nerve in volvement, or this case incidentally carried it. Although this case was initially regarded as the transitional type of Refsum's disease, the serun level of phytanic acid was normal contrary to its higher value in that disease. Morphological findings of the peripheral nerve in this case were suggestive of its congenital hypoplasia rather than a degenerative process. It is conceivable that Sjogren-Larsson syndrome has extensive disorders of the ectodermal tissues including the peripheral nerve as well as the skin or the central nervous system.

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[in Japanese]

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A Korean female, delivered at full term by forceps, weighed 2, 650g. During pregnancy the mother hadhad a severe headache in the first trimester, but otherwise no particular symptoms. After birth the infant never cried, and moderate rigidity of the extxtremities, trismus, ocular fixa tion and absence of pupillary light reflex were noted. The papillae of the optic nerve were pale, but head circumference and plain skull films were normal. The patient developed fever, petechiae on the trunk and hepatosplenomegaly and expi red suddenly during PEG at 3 months of age. Laboratory studies revealed pleocytosis and markedly elevated protein (500-1, 400mg/dl) in the CSF, increased IgM fraction and dislocation of both hips. Just before death the PEG films showed remarkable periventricular calcificationsof the lateral ventricles, which were greatly dila ted, and thinning of the cortex.
Autopsy findings showed marked thinning of the cortex and severe meningoencephalitis with moderate calcification, interstitial pneumonia, hepatitis, pmn cell infiltration of the spleen etc.
Serologically and histopathologically, toxo plasmosis, cytomegalovirus and fungal infections were ruled out. An infectious agent was not identified but some virus, thought to be the cause of prenatal systemic generalized infection of the fetus was suspected to be the causative organism.

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A case of subacute sclerosing panencephalitis (SSPE) in a 10-year-old girl was reported since it had an interesting clinical course.
The patient had measles in April 1965. About 6 years later when she was 6 years and 10 months of age, she began to show symptoms of mental deterioration and urinary incontinence. Subsequently weakness of visual acuity, gait distur bance and myoclonic seizures appeared. Changes in EEG findings almost parallelled those of the clinical picture and finally, the EEG showed a typical pattern of periodic bursts of slow waves every 7 to 8 seconds. Titers of measles virus anti body in serum were 1: 1024 (NT) and 1: 128 (CF). Electrophoresis of spinal fluid samples re vealed high levels of γ-globulin.
The patient began to recover, though slightly, one and a half years after the onset of the dis ease: myoclonic seizures disappeared, gait disturbance was markedly improved and even ver bal communication became possible. Administra tion of amantadine hydrochloride (5mg/kg/day) was started in February 1973 a few days after signs of recovery were observed. There-fore, it is difficult to decide whether or not this drug acted effectively in this case. However, this condition did not last for long and deterioration started again two years after the onset of the disease. At present, 3 years after the onset the patient has the symptoms defined as II-C degree according to Freeman's classification.

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It is the purpose of the present study to examine the metabolism of intracerebral serotonin in order to investigate the pathogenesis of infantile spasms and Lennox syndrome which developed from infantile spasms. Results are summurized as follows.
1. 5HIAA concentration in the cerebrospinal fluid was 36.5±10.6ng/ml in patients with infan tile spasms (18 cases) and was 57.0±17.4 ng/ml in controls (25 cases) (P<0.01).
2. Increase of 5HIAA concentration in the CSF after Probenecid loading test (by Van Praag) in patients of infatile spasms was not signifi cant by different from that in controls.
3. Increase of 5HIAA concentration in the CSF after Probenecid with 5HTP loading test was 10.3±13.3ng/ml in patients of infantile spa sms and 64.9±46.3 ng/ml in controls (P<0.01).
4. Serum growth hormone and Cortisol con centration after 5HTP loading test increased less in patients with infantile spasms than in controls (P<0.05 and P<0.01 respectively).
We concluded that an alteration of serotonin metabolism may be due to hypoactivity of 1-aro matic amino acid decarboxylase.

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The report concerned a floppy male infant with severe mental retardation, facial involvement, and knee joint contructure. Based on the clinical and laboratory studies, including EMG and mus cle biopsy, the diagnosis of congenital muscular dystrophy (Fukuyama's cerebromuscular dystrophy)was made. The patient took a slowly progressive course, and died of pneumonia at the age of 6 years and 5 months.
The major pathological finding was diffuse cerebral and cerebellar micropolygyria with agy ria of bilateral temporal lobes. In addition, there was an abnormal connection of gray matter at the lower part of bilateral frontal lobes, a lesion presumably of fusing after hemispheric separa tion. Microscopically, the architecture of cerebral cortex was severely distorted with irregular arrangement of neurons and vascular prolifera tion. Anterior horn of spinal cord showed diminution in numbers and chromatolysis of neurons. Various skeletal muscles from different parts of the body nevertheless showed predominantly dys trophic changes rather than neurogenic atrophy.
The findings in the present case as well as increasing reports of the similar pathology in the same disease indirate clearly that this type of muscular dystrophy is a dysplastic disease of the central nervous system with dystrophic involve ment of muscles, although its pathogenetic cor relation remains unknown.

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