61

Computed Tomographic Scans of Skeletal Muscles of Various Muscle Diseases Terutoshi Nakamigawa, MD, Hideo Shimoizumi, MD, Mariko Y Momoi, MD and Masayoshi Yanagisawa, MD Department of Pediatrics, Jichi Medical School, Tochigi

The application of computed tomographic scans to skeletal muscles deserves attention because of its nonin­vasiveness and objectivity. Some CT findings have already been reported about Duchenne muscular dystrophy, how­ever, not much has been known about muscle CT scans of other myopathies. Here we reported the characteristic muscle CT findings of varieties of muscle diseases, and its usefulness in the diagnosis and assessment of muscle in­volvement was discussed.

Cases and Methods Seventeen patients (3 to 17 years old) were studied. They included; 6 Duchenne muscular dystrophy (DMD), 4 Fukuyama congenital muscular dystrophy (FCMD), 1 Becker type muscular dystrophy, 1 nemaline myopathy, 1 Ullrich muscular dystrophy, 1 congenital muscle fiber type disproportion and 1 dermatomyositis. Scans were routinely obtained at several levels, and the findings at levels of mid-thigh and mid-calf were discussed in this study.

Results 1) In DMD, characteristic profiles of muscle involve­

ment were evident. In cases of around 10 years of ages, the most profound changes were in quadriceps muscles with unevenly decreased density. The earliest and most noticeable changes found in two patients aged 6 years old was the diffusely decreased density of the caput externum of gastrocnemius muscle, the pattern of which was differ­ent from that of other affected muscles. It seemed that the soleus was responsible for most of the pseudo-hypre­trophy of DMD.

2) All cases of FCMD showed most profound involve­ments of muscles in quality as well as in quantity. Even in the youngest case aged 3 years old, the majority of mus­cles had already been replaced by low density materials, the density of which was similar to that of fat tissue.

3) Non-dystrophic myopaties showed different pro­files of CT scans. In spite of the marked atrophy of all muscles, there could found no or little internal changes in the density and no obvious selectivity in the involvement.

Conclusions This study showed that CT scans of muscles revealed dif­ferent degenerative profiles of dystrophic and non­dystrophic muscles. Furthermore, changes in CT scan could be detected in early stages of the diseases. There­

.fore, CT scans should be of great use in diagnosis and management of the patients with various muscle disorders.

Key words: CT scan, muscle, my opa ties, muscle dystro­phy.

62

Positron Emission Tomographic Studies Using Pyruvate-l­llC on Mitochondrial Encephalomyopathy Momozou Toyoda, MD, Yukio Arai, MD, Norio Sakura­gawa, MD, Masaaki lio, MD and Hideki Horita, MD Division of Child Neurology, National Musashi Research Institute for Mental and Nervous Diseases, Kodaira, Tokyo (MT, Y A, NS); Department of Radiology, National Nakano Chest Hospital, Nakano, Tokyo (MI); Saitama Children's Medical Center, Iwatsuki, Saitama (HH)

Two patients with mitochondrial encephalomyopathy were subjected to studies on cerebral pyruvate meta­bolism involving positron emission tomography (PET) using pyruvate-l-llC.

Methods Pyruvate_l_llC was obtained by exchanging a carboxyl ring of pyruvate with HC02 using the pyruvate-ferre­doxine oxidase of Bacillus butyricus. PET scannings were started with the intravenous administration of pyruvate­l_llC and recorded with a Headtome-II (Shimadzu). Six images were taken, with a total of 36 minutes for the entire examination. PET images were compared with X­ray CT findings.

Subjects Case 1, a ll-year-old boy, with myoclonus epilepsy and ragged-red fibers in muscle biopsy specimens, was diag­nosed as having MERRF. Case 2, a 7-year-old girl, with mitochondrial encephalopathy, myopathy, lactic acidosis and stroke-like episodes, was diagnosed as having MELAS.

The PET images in case 1 showed high RI uptake in the cerebral cortex and basal ganglia heterogeneously, which was cleared slowly from these areas. A CT scan of this patient showed no outstanding changes. In~case 2, RI uptake was remarkably increased in areas corresponding to lower density areas seen on X-ray CT. As in case 1, the clearance of RI was very slow.

Discussion In normal brain tissues, pyruvate is mostly decarboxy­lated to acetyl-CoA and then transferred to the TCA-cycle. In this metabolic pathway, the HC of pyru­vate-l-IlC is rapidly cleared from the tissues through decarboxylation of pyruvate. Judging from the increased lactic acid levels in CSF or serum in these patients, the heterogenous RI uptake and slow clearance suggests that pyruvate metabolism may be impaired by the metabolical trapping of 11 C by lactic acids.

Since pyruvate metabolism may be affected in dis­orders involving mitochondrial enzyme abnormalities, the usefulness of PET studies with pyruvate-l-llC for deter­mining the distribution and activeness of the affected areas of the brain is suggested.

Key words: PET, pyruvate-1-11 C, mitochondrial encepha­lomyopathy.

Brain & Development, Vol 9, No 2, 1987 119

63

CT Studies on the Lower Extremities in Several Neuro­Muscular Diseases Masaaki Nishimura, MD Department of Pediatrics, National Sanatorium, Nagara Hospital, Gifu

O'Doherty et al introduced the use of computed tomo­graphy in neuro-muscular diseases. Previous reports stated that CT scan studies are valuable for monitoring the pro­gression of a disease and for detecting the density changes in individual muscle. I performed a CT scan study on several neuro-muscular diseases.

Computed tomography was performed with a CT IT 8600 Yokogawa on 56 patients. The mid thigh and mid­calf were the sites chosen. Of the 56 patients, 39 (includ­ing a female case) had Duchenne muscular dystrophy, 4 typical Fukuyama congenital muscular dystrophy, 4 aty­pical congenital muscular dystrophy, 2 suspected mali­gnant limb-girdle dystrophy, 2 myotonic dystrophy, one nemaline myopathy, 3 Kugelberg-Welander disease and one Werdning-Hoffmann disease.

Using the CT scans, average CT Hounsfield numbers and areas were established through incited computer tracing for the following muscles, quadriceps femoris, sartorius, gracilis, semimembranosus together with semitendineus, adductor and biceps femoris in the thigh, and anterior tibial, posterior tibial, extensor digitorum longus, peronei, soleus, and gastrocnemius muscles in the calf. The rela­tionships among individual disorders, age and the clinical stage according to Ueda were clarified.

In the Duchenne muscular dystrophy, the CT numbers of quadriceps, adductor, biceps and gastrocnemius mus­cles are already relatively low early on at stage 2. At this stage, the gracilis muscle is spared. With progression of the clinical stage, the CT number of each muscle decreased more slowly in the calf. The Fukuyama congenital muscu­lar dystrophy cases (8-13 years old) showed CT number values similar to that at stage 7 of DMD. The atypical FCMD cases showed CT numbers between those in FCMD and DMD.

The myotonic dystrophy cases showed severe involve­ment of the semimembranosus and semitendineus. The W-H and K-W cases showed characteristic sparing of the adductor muscle.

CT studies are valuable for monitoring the progression of a disease, and several neuro-muscular diseases show some characteristic findings on CT scanning. CT find­ings may facilitate a clinical diagnosis in several neuro­muscular diseases.

Key words: Neuro-muscular disease, muscle, CT scan.

120 Brain & Development, Vol 9, No 2,1987

64

Ultrasonographic Evaluation of the Gestational Develop­ment of Cerebral Sulci Toshiko Juri, MD, Shogo Kihira, MD, Toshihiko Yana­gawa, MD, Ryuzo Higuchi, MD, Eikichi Miyashiro, MD and Norihiko Tuda, MD Department of Pediatrics, Kainan City Hospital, Kainan, Wakayama (TJ); Department of Pediatrics, Wakayama Medical College, Wakayama (SK, TY, RH); Department of Pediatrics, Wakayama Rosai Hospital, Wakayama (EM, NT)

Ultrasonographic appearance of cerebral sulci of prema­ture infants may be useful for the judgment of gestational age. So, we investigated the correlation among appearance of cerebral sulci and post conseptial age, still more asym­metrical lateral ventricles.

Materials and Methods Fifty-eight neonates admitted to Newborn Intensive Care Unit at Wakayama Medical College between January 1985 and March 1986 were selected for study. Their gestational age ranged from 24 weeks to 40 weeks. They do not in­clude intracranial hemorrhage, anomaly, leukomalacia etc, affecting the appearance of cerebral sulci.

Electrical real time sector scanner with 5MHz trans­ducer (Yokokawa Medical corp. RT3,000) was used. Coronal views from anterior fontanel and sagital views from anterior and posterior fontanel were studied.

Results At about gestational age of 30 weeks, main sulci rapidly appeared in ultrasonograph.

First, callosal sulcus and sylvian fissure were observed at the gestational age of 24 weeks. Then, parietooccipital fissure, calcarine fissure and cingulate sulcus appeared. Next, central fissure, superior temporal sulcus and inferior frontal sulcus appeared. After the gestational age of 35 weeks or 36 weeks, many sulci appeared clearly.

Right cerebral sulci appeared one or two weeks earlier than the left side. In these cases, the left lateral ventricle was larger than the right in posterior body and occipital horn.

Conclusion Ultrasonographic studies of cerebral sulci are useful for the evaluation of the maturation and hemispheric asym­metry of neonatal cerebrum.

Key words: Ultrasonograph, cerebral sulcus, premature infant, gestational age.

65

Computed Tomography in Neuromuscular Diseases - Myopathic Diseases Kouzou Mutoh, MD, Yoshihiro Nakagawa, MD and Hiro­atsu Bojo, MD Division of Pediatric Neurology, Shizuoka Children's Hospital, Shizuoka

We examined the CT patterns in myopathic diseases to determine the validity of CT scanning in neuromuscular diseases.

Materials Twenty patients with myopathic diseases were studied by means of CT scanning. They consisted of 12 cases with Duchenne muscular dystrophy (DMD), 3 with Fukuyama type congenital muscular dystrophy, 2 with myotonic dystrophy, one with congenital muscular dystrophy, one with congenital myopathy and one with Becker muscular dystrophy. The patients with DMD were divided into 3 groups: DMD-A, 5 boys who could walk and didn't show Gowers' sign; DMD-B, 5 boys who could walk and showed Gowers' sign; and DMD-C, 2 who couldn't walk without support.

Methods The examination was performed with a GE 9,800 CT scanner. We routinely examined 8 slices: one through the neck one at the shoulder girdle, one at the upper arm, thre~ at the pelvis, one at the thigh level and one at the lower leg.

Results The CT scans of these 20 patients showed patterns speci­fic for the myopathic diseases. Pathologic changes seen on CT included intramuscular streaks (Stage 1), a patchy moth-eaten pattern (Stage 2), a confluent moth-eaten pat­tern (Stage 3) and diffuse low density (Stage 4). At stage 4, which is the end stage, the muscle contours were pre­served with little atrophy. This was in great contrast to the CT findings in neuropathic diseases. Changes were conspicuous in proximal muscles and anti-gravity muscles (gluteus maximus, quadriceps femoris, gastrocnemius and soleus muscles). The pathologic findings on CT increased in severity from Stage 1 to Stage 4, and were well corre­lated with the clinical findings. In DMD-A patients, stage 1 or 2 patterns were found in anti-gravity muscles. In DMD-B patients, these muscles showed stage 3 patterns. Stage 1 or 2 patterns appeared in iliopsoas and paraspinal muscles. In DMD-C patients, stage 3 or 4 patterns ap­peared diffusely, but were more prominent in proximal muscles and in the lower extremities.

Conclusion CT scanning in neuromuscular diseases is useful for (1) differentiation between neuropathic and myopathic diseases, (2) surveying the distribution of the affected muscles, and (3) follow-up of the disease. It may help to know the pathophysiology of neuromuscular diseases.

Key words: CT scan, neuromuscular disease, myopathic disease, Duchenne muscular dystrophy.

66

Subependymal and Intraventricular Hemorrhage in Low­Birth-Weight Infants: Ultrasonographic Study Shogo Kihira, MD, Toshiko Juri, MD, Toshihiko Yana­gawa, MD, Ryuzo Higuchi, MD and Michio Koike, MD Department of Pediatrics, Wakayama Medical College, Wakayama (SK, TY, RH, MK); Department of Pediatrics, Kihoku Branch Hospital, Wakayam Medical College, Wakayama (JT)

Subjects and Methods The 47 infants in this study weighed less than 2,500 g at birth, and their gestational age was within 37 weeks. Ultrasonographic scans were performed, using a 5 MHz sector transducer. Evaluations included serial observation of subependymal hemorrhage (SEH) and intraventricular hemorrhage (IVH), and assessment of ventricular size and shape, etc.

Risk factors for hemorrhage were statistically deter­mined in 26 infants under 1,500 g.

Results The incidence of SEH and/or IVH in a group of infants weighing less than 500 g were 100% (n = 2),500 to 999 g 78% (n = 11\ 1,000 to 1,499 g 50% (n = 1), 1,500 to 1,999 g 47% (n = 7), 2,000 to 2,499 g 14% (n = I). There were 7 cases with Papiles's grade III or IV hemorrhage. Six cases (weighing less than 1,000 g) out of 7 were dead. SEH occurred on the right side more frequently than on the left. When SEH occurred bilaterally, the d9minant site was invariably on the right.

Of 40 infants who underwent the 1st scan within 24 hours after birth, 24 had compressed lateral ventricles. Non-hemorrhagic infants with compressed ventricles showed good outcome on discharge except for one who died shortly after birth and one who presented unilateral ventriculomegaly, respectively.

Subependymal cysts were recognized in only 7 infants during the subacute period.

Of the surviving 37 infants, 10 showed enlarged ventri­cles on discharge. Ventricular enlargement was detected on the left side in a significantly higher incidence. It is unclear why such asymmetric modality exists in the evolution of SEH and in the dilation of the ventricle.

Statistically higher risk factors for hemorrhage were birth weight (p < 0.001), gestational age (p < 0.001), and pC02 on admission (p < 0.005).

Key words: Cranial ultrasonography, intracranial hemor­rhage, low-birth-weight infant.

Brain & Development, VoI9,No2, 1987 121

67

Digital Subtraction Angiography in Neonates and Children Yukuo Konishi, MD, Masanori Kuriyama, MD, Masakatsu Sudo, MD, Katsumi Hayakawa, MD, Yasushi Ishii, MD and Kaoru Konishi, MD Departments of Pediatrics (YK, MK, MS) and Radiology (KH, YI), Fukui Medical College, Matsuoka, Fukui; Department of Pediatrics, Fukui Hospital, Fukui (KK)

Abnormalities of the intracranial circulation in neonate and children are studied infrequently because of the dif­ficulty encountered on angiography and the subsequent morbidity. Digital subtraction angiography (DSA) is a relatively noninvasive method that may potentially pro­vide detailed images of intracranial vessels. We performed DSA 25 times in 23 pediatric patients including three neo­nates, and measured the cerebral circulation time (CCT).

Subjects and Methods Twenty-five patients (aged from two days after birth to twelve years old) were studied. Contrast material was in­jected into a peripheral vein in 21 patients and into an artery in the other 4. Amipaque (2 mg/kg) followed by a bolus injection of 4 ml of saline was given. We recorded the DSA images on videotape and analyzed them with a Nexus Image Processor and a Vax-llf750 computer. The region of interest (ROI) was set at the supraclinoid por­tion of the internal carotid and sinus confluence. Time­density curves were obtained for two ROls, and CCT was measured as the time from the carotid peak to the sinus peak.

Results Images of the diagnostic value were obtained in 16 pa­tients in whom the contrast material was injected into the carotid artery, superior vena cava or peripheral vein at the elbow. The images obtained in patients in whom the con­trast material was injected into the peripheral vein on hand was of no value. No adverse effects were seen when the total injection volume was limited to 4 ml/kg. CCT was measured in 14 patients. In 9 patients, no cerebro­vascular disease was disclosed; the mean CCT in this group was 4.3 ± 0.54 sec with a range of 3.73 to 5.06 sec. The prolongation of CCT was found in patients with su­perior sagittal sinus thrombosis, moyamoya disease and frontal hematoma. The mean CCT in this group was 6.1 ± 2.48 sec.

Conclusion DSA was useful for diagnosing cerebrovascular disease in neonate and children. In addition, measurement of CCT was very useful for quantitating the cerebral circulation in pediatric patients.

Key words: Digital subtraction angiography, cerebral circulation time, cerebrovascular disease.

122 Brain & Development, Vol 9, No 2,1987

68

Nuclear Magnetic Resonance Imaging in Two Children with Probable Multiple Sclerosis Hiroshi Inoda, MD, Masaharu Ochi, MD, Koh Hasegawa, MD, Shigeru lino, MD, Yoshihiro Takeuchi, MD, Hiroshi Yoshioka, MD and Tomoichi Kusunoki, MD Department of Pediatrics, Kyoto Prefectural University of Medicine, Kyoto

Two girls with probable multiple sclerosis (MS) underwent nuclear magnetic resonance (NMR) imaging, and the re­sults were compared with those obtained on common X­ray computed tomography (CT).

Case one: A seven-year-old girl visited our hospital with a complaint of double vision. Her ocular movement was abnormal. The left eye was deviated downward and its movement was limited to upward movement. Move­ment of the right eye to the left was limited. Paralysis of the extraocular muscles continued for about three months and then gradually improved without treatment. Three months after this episode, she presented cerebellar ataxia. She underwent CT and NMR imaging. CT scanning demonstrated an unclear low density lesion in the left cerebellar hemisphere. Spin echo (SE) imaging on NMR revealed multiple lesions as areas of increased signal inten­sity in the bilateral cerebellar hemispheres and occipital periventricular regions. Inversion recovery (IR) imaging showed, in the same regions, less clearly delineated lesions of decreased signal intensity.

Case two: A nine-year-old girl was admitted to our hos­pital with impairment of visual acuity. She was found to have optic atrophy and improved with prednisone. There­after she repeatedly suffered from paresthesia and paraly­sis of the extremities, in addition to impaired vision, for 4 years. At 10 years old, she underwent CT and NMR imag­ing. CT scanning demonstrated an unclear low density area adjacent to the occipital horn of the left lateral ventricle. NMR imaging revealed another lesion in the right occipital lobe.

In both cases, MS was suspected from the clinical course and the NMR imaging findings. NMR imaging demonstrated more extensive abnormalities than common X-ray CT did. SE images demonstrated abnormal regions as areas of increased signal density. IR images also showed abnormal areas of decreased signal density, but did not visualize lesions as well as SE imaging did. The SE techni­que of signal acquisition appeared to be superior to the IR technique in its ability to resolve abnormalities in patients with MS.

Key words: Multiple sclerosis, children, NMR imaging.

69

Magnetic Resonance Imaging in Tuberous Sclerosis Seijun Nakajima, MD, Ryosuke Murata, MD, Osamu Matsuoka, MD, Sung-8oo Lee, ~D, Hideji Hattori, MD, Hisashi Kawawaki, MD, Michinaga Nakamura, MD, Gen Isshiki, MD and Tomochika Kato, MD. Department of Pediatrics, Osaka City University Medical School, Osaka (SN, RM, OM, SL, HH, KH, MN, GI); De­partment of Pediatrics, PL Hospital, Osaka (TK).

The characteristic cerebral lesions in tuberous sclerosis comprise numerous sclerotic patches which consist of an overgrowth of astrocytes and bizzare giant cells. Calcium is often deposited in such a lesion and is visualized on computed tomography (CT). Magnetic resonance imaging (MRI) is more sensitive than CT for the detection of certain tumors and demyelinating diseases. We compared cranial CT and MRI in patients with tuberous sclerosis.

Subjects and Methods The subjects comprised 5 boys and 1 girl, ranging from 1 to 16 years of age. MRI was performed with a 0.5 tesla super-conduction magnet (Picker-International Co.). In all patients, the images were obtained in the transverse plane, and in several also in the coronal plane. Two MRI pulse sequences were used; inversion recovery (IR: repetition time, 2,100 msec; inversion time, 600 msec) and long spin echo (SE: repetition time, 1 ,800 msec; echo time, 120 msec). CT examinations were performed with a Soma­tom 2 or DR3 scanner.

Results 1) Findings of calcification on MRI were not detected

but periventricular nodules were detected on MRI as areas of high intensity in IR images and areas of slightly low in­tensity in long SE images, the same as the intensity of white matter.

2) Small T 1 and T 2-weighting lesions were detected in the subcortical white matter in 5 patients on MRI, one of which was demonstrated as an area of low density on CT. These lesions may reflect areas of demyelination.

3) There may be a relationship between the mental state and white matter lesions. The majority of mental re­tarded patients exhibited white matter lesions on MRI.

Conclusion Although MRI does not demonstrate calcification, we would like to emphasize that it is much more useful for obtaining information on the white matter lesions in tube­rous sclerosis.

Key words: Magnetic resonance imaging, MRI, CT, tube­rous sclerosis.

70

A 5-Year-Old Girl with Syringomyelia Diagnosed by Magnetic Resonance Imaging Hirofumi A otani, MD, Hiromi Katsura, MD, Tsunekazu Yamano, MD and Morimi Shimada, MD Department of Pediatrics, Shiga University of Medical Science, Ohtsu, Shiga

A case of a 5-year-old girl with syringomyelia and the Arnold-Chiari malformation (type I) was presented. Her symptoms were atypical for syringomyelia, and the final diagnosis was made on the basis of magnetic resonance imaging (MRI) findings.

At 3 years of age, the patient was noted to have paraly­sis and hypesthesia of the right lower leg. Two years later, paralysis of the left abducens nerve appeared suddenly, and then she was referred to our hospital. She had no history of dysfunction of defecation or voiding. On admission, she showed limitation of movement of the left ankle joint and mild claudication. Left knee jerks and the left abdominal reflex were absent, and the left anal reflex was also decreased. Although the sense of touch was intact, the sense of pain and vibration in left lower leg and that of temperature in the left instep were diminished. No sensory abnormalities were noted in the chest or upper extremities. As to the cranial nerves, in add"ition to the left abducens nerve palsy, the absence of the left corneal reflex and right deviation of the uvula on phonation were noticed.

The auditory brainstem response and peripheral nerve conduction velocity were within normal ranges. However, electromyography showed a neurogenic pattern of atro­phy in the left lower leg. Since these clinical features suggested a spinal lesion in this case, MRI examination was performed, which disclosed a remarkabie syrinx of the spinal cord at C2-Th 12. A syrinx was not observed in the medulla oblongata. Metrizamide CT of the spinal cord and myelography also showed that a cerebellar tonsil pro­truded to the posterior portion of C 1. Thus, this patient was diagnosed as having syringomyelia with the Arnold­Chiari malformation (type I), although the typical symp­tom of "forme suspension" was not seen in this case.

At 7 years of age, she underwent suboccipital craniec­tomy, CI-2 laminectomy and cerebellar tonsilectomy to prevent progression of the syrinx.

A diagnosis of syringomyelia has previously been made mainly on the basis of myelographic findings. However, this study showed that MRI examination seems to be more useful for making a diagnosis of syringomyelia.

Key words: Syringomyelia, magnetic resonance imaging, Arnold-Chiari malformation.

Brain & Development, Vol 9, No 2, 1987 123

71

Ultrasound Examination in the Detection, Diagnosis and Management of Fetal and Neonatal Intracranial Abnor­malities Yushiro Yamashita, MD, Toyojiro Matsuishi, MD, Yoichi­ro Yamaguchi, MD, Etsuo Otaki, MD, Takamoto Matsu­naga, MD, Junji Ishimatsu, MD and Teiji Hamada, MD Departments of Pediatrics (YY, YY, EO, TM) and Obste­trics (TM, JI, TH), Kurume University, Kurume, Fukuoka

With the advances in and increased availability of ultra­sonography, prenatal diagnosis of an intracranial abnorma­lity is now enabled. Here we report our evaluation of the ultrasonographic detection during pregnancy of intra­cranial abnormalities in 6 patients, with evaluation of the prenatal diagnosis, management and prognosis.

Method Between March 1984 and November 1985,840 high risk pregnant women were admitted to Kurume University Hospital. Prenatal ultrasound scans were performed after 15 weeks of gestation at 1 month intervals. 239 neonates were admitted to the neonatal intensive care unit and echoencephalography was performed repeatedly after birth. A total of 6 neonates were diagnosed as having an intracranial abnormality detected by ultrasound examina­tion during the prenatal period.

Results and Discussion The 6 patients with intracranial abnormality involved hy­drocephalus wi~ neural tube defect (3), Dandy-Walker syndrome (1), holoprosencephaly (1) and arteriovenous malformation (A VM) of Galen (1). An accurate diagnosis was made prenatally in all except for the .case of A VM of Galen, in which the diagnosis was confirmed only post­natally. This correct noninvasive diagnosis of A VM of Galen establishes the efficacy of both two-dimensional echoencephalography and of echo cardiography, combined with pulsed Doppler observation. There have been no re­ports with respect to Doppler echocardiography on cere­bral A VM. Our case of A VM of Galen revealed significant stolen diastolic Doppler signal on the aortic arch, which suggests a presence of cerebral A-V fistula. In the 4 pa­tients with hydrocephalus (the 3 patients with neural tube defect and the one with Dandy-Walker syndrome), V-P shunt operation was performed shortly after birth. The prognosis was very poor in 3 patients, with holoprosence­phaly 0), AVM of Galen 0), and with hydrocephalus with neural tube defect (1). The prognosis is good in the patient with Dandy-Walker syndrome at 1 year and 6 months of age with now normal psychomoter develop­ment.

Key words: Intracranial abnormality, ultrasonography, fetal diagnosis, management.

124 Brain & Development, Vol 9,No 2, 1987

72

Cerebral Blood Flow Patterns in Infancy: Developmental Study and its Clinical Application Nagako Murakami, MD, Satoshi Sanada, MD, Hideo Ino­ue, MD, Kuniaki Iyoda, MD and Shunsuke Ohtahara, MD Department of Developmental Neuroscience and Child Neurology, Okayama University Medical School, Oka­yama

Blood flow in the anterior cerebral artery (ACA) and intracranial pressure (ICP) were measured in order to assess the cerebral hemodynamics in neonates and infants.

A continuous mode Doppler flowmeter, Parks model 909, and a pulsed mode Doppler flowmeter, EME model TC 2-64, were used for the measurement of cerebral blood flow (CBF), and a San-Ei anterior fontanel pressure monitor for the measurement of ICP.

1) Normative data: The anterior cerebral blood flow was measured 202 times in normal control infants, from 15 minutes after birth to 11 months of age, and the pulsatility index (PI), i.e., systolic flow-diastolic flow / systolic flow, was calculated.

The changing PI pattern during the first 24 hours of life was similar in all cases. The mean PI value at 15 min after birth was 0.60 ± 0.01. Then the PI value gradually increased and reached the highest value of 0.85 ± 0.04 at 2-3 hours after birth, followed by a gradual decrease until 24 hours after birth, when it was 0.56 ± 0.03. It was stable from 24 hours to 11 months of age.

On comparison of the PI values obtained with the two apparatuses, a Parks model 909 and a EME model TC 2-64, no differences were observed between the 10 normal infants for which measurements were made with both apparatuses.

2) Investigation of cases with the West syndrome: PI, ICP and blood pressure were measured in 4 cases, before, during and after synthetic ACTH therapy. In all cases, an increase in ICP and a decrease in PI were induced by hy­pertension under ACTH treatment. There was a cross correlation between systolic blood pressure and PI. PI decreased when the systolic blood pressure increased above 120-150 mmHg. This decrease in PI may indicate disturbance of the autoregulation of CBF. Assessment of PI must be a useful indicator for ACTH treatment and may provide clues for elucidation of the mechanism of its side effect.

Key words: West syndrome, cerebral blood flow, pulsa­tiUty index, intracranial pressure, ACTH.

73

Relationship between Early Posture-Movement Abnorma­lities and CT Findings Shigetaka Imamura, MD, Kazuko Sakuma, MD, Seiichi Hirano, PhD and Norihiro Sato, PhD Department of Pediatrics, Hokkaido Prefectural Institute for Physically Disabled Children, Sapporo, Hokkaido

The relationship between abnormal posture and move­ments (P-M) seen in children before 6 months of age and the CT findings was investigated.

Subjects and Methods Children who I} visited our clinic before six months of age, 2} underwent CT before one year of age, and 3} were followed-up for observation of their development for at least one year, were included in this study (total, 146 cases). They were classified into (l) cerebral palsy, (2) severe or moderate CCD (cerebral coordination disturb­ance; Vojta), (3) light or very light CCD, and (4) normal groups on the basis of the characteristics of their P-M at the firs visit, and their CT findings, history, prognosis and treatment were investigated.

Conclusion I} Children with CP showed severe and fixed P-M

abnormalities such as opisthotonus, fixed asymmetry, dys­tonic attacks and a decerebrate posture from the severe neonatal period. The perinatal history was frequently ab­normal. They all developed CPo CT frequently (33/38, 64%) showed brain lesions (diffuse abnormalities such as cerebral edema and encephalomalachia or focal lesions such as localized hypodensity areas and calcification). Severe SFC (a large amount of cerebral fluid collection with suspected cortical atrophy) and moderate SFC (a wide SFC area seen on CT remaining in the temporal re­gion, with no cortical atrophy) were also found in 13/48 (27%) cases. Treatment from an early age should be con­tinued without fail.

2} Severe and moderate CCD are transient conditions. Severe or moderate SFC was seen in 19/35,55% of the cases, and cerebral lesions in 5 (14%) cases. Their clinical pictures, CNS abnormalities and CT findings were not fixed. Some of them (5/35, 14%) remained in fixed condi­tions (CP) or borderline abnormalities such as MBD, LD and clumsy child remained. The other luckier children (22/35, 63%) become normal. Vojta's treatment and/or other rehabilitation techniques should be tried.

3} The light and very light CCD cases showed subtle and transient abnormal P-M at an early age. Their CNS function was abnormal at that time and this may be confirmed by CT. But these abnormalities were transient and the patients became normal within a short period. Following-up of their development is desirable.

4} CT abnormalities seen at an early age occasionally faded to become normalized or changed into softer ones along with development. CT should be performed as early as possible.

Key words: Cerebral coordination disturbance (CCD), cerebral palsY, posture and movement, CT.

74

Real-Time Ultrasonography in Pediatric Neurosurgery Noriaki Kojima, MD, Akihiro Ijichi, MD, Takayuki Shira­kuni, MD, Norihiko Tamaki, MD and Satoshi Matsumoto, MD Department of Neurosurgery, Kobe University School of Medicine, Kobe, Hyogo

Fifty-five infants with intracranial lesions were evaluated by means of cranial sonography. The instruments used were two real-time scanners (Aloka SSD-208 and SSD-850) with 3.5 and 5.0 MHz transducers, respectively. The sonographic machines generated real-time images in coronal, sagittal or axial planes. The patients were divided into the following three groups according to the scanning methods.

I) Cranial ultrasound scans using the anterior fontanel­le as the acoustic window (41 cases): we studied subdural effusion, hydrocephalus, intraventricular hemorrhage (IVH), porencephalus and periventricular leukomalacia.

2} Intraoperative examinations, applying the transdu­cer to the dura or to the scalp over the pre-existing cranio­tomy defects (9 cases): intraoperative sonography allowed the identification of brain tumors, brain abcesses, arteriovenous malformations, isolated fourth ventricle and holoprosencephaly.

3) Postoperative sonography through surgical calvarial defects (5 cases): this group included grave patients with brain tumors.

Transfontanelle sonography of the infants provided useful information as to the features of the ventricular system, congenital cerebral anomalies and IVH. Repeated follow-up sonography was possible until closure of the anterior fontanelle. Generally it was difficult to obtain clear images of subdural fluid collection in the sagittal plane, although dilatation of the interhemispheric fissure seen in the anterior coronal section through the frontal lobes suggested this abnormality. Intraoperative scans through the dura allowed accurate identification and localization of the lesions and determination of the rela­tionship to the surrounding normal structures. Precise aspiration or cortical incision may facilitate better func­tional recovery in these young patients. Sonography through surgical calvarial defects was valuable in the follow-up management and for emergent procedures in grave patients.

Key words: Ultrasonography, pediatric intracranial le­sions, anterior fontanelle, intraoperative scan, calvarial defect.

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75

Cranial Ultrasonography of Premature Infants Part II. Mental and Motor Development Tohru Ikuse, MD, Ren Hasegawa, MD, Hiroo Matsuda, MD, Haruo Ichihashi, MD and Takashi Nakamura, MD Department of Pediatrics, School of Medicine, Kyorin University, Tokyo (TI, RH, HM, HI); Division of Neo­natology, Hachiooji Children Hospital, Tokyo (TN)

Cranial ultrasonography (cranial echo) was performed on low birth weight infants as a screening for intracranial lesions, and the findings were evaluated for any correla­tion with the neurological prognosis.

Subjects and Methods Involved in this study were a total of 76 preterm high-risk infants having low birth weights, less than 2,000 g, who were admitted to the NICU of the Metropolitan Hachiohji Children's Hospital during the period from June 1982 through May 1985 and underwent cranial echo within a week after birth, initially, and at I-week intervals there­after, and who, after being discharged from the hospital, could be followed up to a corrected age of 6 months or above (from 6 months to 3 years and 2 months, 1 year and 5 months on the average) for evaluation of develop­mental status according to Vojta's method and Enjoji's developmental scale for infants.

Results Abnormalities were noted in cranial echo in 21 of the entire 76 cases, i.e. grade I bleeding (Papile's classifica­tion) in 8 cases, grade II bleeding in 2 'cases, grade III bleeding in 1 case and uni- or bilateral ventricular dilata­tion without concomitant bleeding in 10 cases. The post-discharge assessment of development revealed ab­normalities of psychomotor development in 7 (33.3%) of the 21 cases showing abnormalities in cranial echo; more particularly in 1 case 02.5%) classified as having grade I bleeding, 1 case (50%) having grade II bleeding, 1 case (100%) having grade III bleeding and in 4 cases (40%) showing ventricular dilatation without concomitant bleed­ing, as against 7 (12.7%) of the remaining 55 cases exhibit­ing normal cranial echo. There was no difference between the group of normal cranial echo and the group of grade I intracranial bleeding in the incidence of abnormalities of psychomotor development, with a higher incidence value being noted for the groups with echographic evidence of ventricular dilatation or of grade II or III intracranial bleeding. Further studies in pursuit of the correlation be­tween various risk factors and the assessment of develop­ment in preterm infants are indicated.

Key words: Low birth weight infants, intracranial bleed­ing, cranial ultrasonography.

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MRI of the Intracranial Malformations Noriko Okamoto, MD, Hideo Shimoizumi, MD, Mariko Y Momoi, MD, Yoshifumi Yamamoto, MD, Osamu Tanaka, MD and Masayoshi Yanagisawa, MD Departments of Pediatrics (NO, HS, MYM, YY, MY) and Radiology (OT), Jichi Medical School, Minamikawachi, Tochigi

Magnetic resonance imaging (MRI) was applied for investi­gating intracranial malformations of 9 children. And its usefulness in evaluating detailed anatomical abnormalities of intracranial structures was discussed.

Cases and Methods Cases investigated included; 4 with agenesis of corpus cal­losum, 1 with lissencephaly, 1 with pachygyria, 1 with hydranencephaly, and 2 with arachnoid cyst. MRI studies were done by using Toshiba MRT22A equipped with resis­tive magnet which was operated at 0.22 Tesla. Inversion­recovery (IR) and spin-echo (SE) pulse sequences were used.

Results 1) Saggital images clearly visualized corpus callosum,

and complete agenesis was detected in 3, and partial agenesis in 1. Associated malformations were visualized as well.

2) In lissencephaly, saggital and horizontal sections revealed smooth surface of the cerebrum and atrophic white matter. In pachygyria, coarse gyral pattern was observed.

3) In hydranencephaly, saggital sections revealed detailed structural defects. Intact brainstem, cere bellum and the remnant of the occipital lobe were visualized.

4) Although arachnoid cysts were easily visualized by CT scan, the detailed anatomical relations to other struc­tures were best visualized by saggital images of MRI.

Conclusions 1) This study showed that MRI could give us detailed

informations of intracranial structural abnormalities. The advantages of MRI in sagittal images over CT scan should be emphasized.

2) The diagnosis of callosal defect was best achieved in saggital images by IR pulse sequences.

3) Detailed informations of anatomical localizations of abnormal structures such as arachnoid cyst by MRI should be especially of great help for the possible surgical pro­cedures for these anomalies.

Key words: MRI, intracranial anomalies, arachnoid cyst, agenesis of corpus callosum.

77

Brain CT Scans and Clinical Study in Very-Low-Birth­Weight Infants, Including Eight Cases of Cerebellar Poren­cephaly Hideaki Shime, MD, Hiroshi Suzuki, MD, Hiroyoshi Koi­de, MD and Toru Miyaji, MD Department of Pediatrics, Saitama Medical School, Sai­tama

Fifty-nine brain CT scans taken in very-Iow-birth-weight infants (:E;;; 1,500 g) during the past three years were stu­died retrospectively. Eight cases of cerebellar porencepha­ly were found, though this had never been reported in premature infants, despite the fact that many intracranial disorders following asphyxia and respiratory and cardio­vascular diseases in such infants had been detected.

Subjects and Methods Eighty-nine cases of very-Iow-birth-weight infants were ad­mitted to our premature nursery during the period from Jan. 1, 1982 to Dec. 31, 1984. We obtained brain CT scans in 59 of them, and studied them retrospectively.

Results a) Normal CT in 25 cases, b) enlargement of the extra­cerebral space in 17, c) megacisterna magna in four, d) unilateral ventriculomegaly in six, e) hydrocephalus in seven, f) cerebral porencephaly in two, g) brain stem atrophy in seven, and h) low density area in the posterior fossa in eight, were observed.

The clinical courses of patients a) to f) above were al­most similar to those previously reported.

g) Brain stem atrophy was found on CT scans in seven cases. Five of them developed infantile spasms later. This suggests that one of the main sites of lesions in infantile spasms is the tegmentum of the brain stem.

h) Low density area in the posterior fossa was found on CT in eight cases. Three of them showed cerebellar defective lesions on metrizamide CT or RI cisternography. Four of them showed no defective lesion in the posterior fossa on ultrasonography at the early neonatal stage. These lesions in the posterior fossa are believed to be cere­bellar porencephaly, which occurred after birth. Seven cases of cerebellar porencephaly, except for one with SFD, had respiratory and cardiovascular diseases, such as neonatal asphyxia, RDS, PDA, and/or apnea. The cerebral lesions such as intracranial hemorrhage, hydrocephalus and cerebral porencephaly, which had been observed in all cases of cerebellar porencephaly, finally result­ed in cerebral palsy , mental retardation and infantile spasms.

Conclusions Cerebellar porencephaly may occur in very-low-birth­weight infants. Thus, careful observation of them by ultrasonography is strongly recommended.

Key words: Cerebellar porencephaly, CT, premature in­fants, neurological outcomes.

78

Computed Tomographic Study of Term Infants with Neonatal Neurological Symptoms Masayo Horie, MD, Mutsuo Ishii, MD, Shinji Kishi, MD, Kimio Nishio, MD, Yoshimi Hokazono, MD, Kazuhisa Inukai, MD, Hideyuki Kito, MD, Kunishige Yoshizawa, MD, Jiro Ogawa, MD and Kenji Yokochi, MD Department of PediatriCS, Seirei-Hamamatsu Hospital (MH, MI, SK, KN, YH, KI, HK, KY, JO); Department of PediatriCS, Seirei-Mikatabara Hospital (KY), Hamamatsu, Shizuoka

The computed tomographic (CT) findings in term infants with neonatal neurological symptoms were studied. The subjects were 140 term infants. Ninety-five had a good prognosis, and were followed longer than two years. The other forty-five had developmental abnormalities, and were followed at least six months. CT scans were obtained with a GE CT /T 8800 scanner using the high resolution mode.

As to the CT findings with a good prognosis, the ventri­cles were compressed and falx images were common in the early neonatal period. Periventricular low density areas in the anterior and posterior horns were noticed. The basal ganglia and the thalamus were homogeneous in density, as the cerebellum was.

As to the neonatal CT scans with developmental ab­normalities, 19 infants had abnormal low density areas in the cerebral hemisphere and either the basal ganglia or the thalamus, 4 in the basal ganglia and/or the thalamus, and six inthe cerebral hemisphere. Nine showed no abnormal density areas on CT scans. Seven infants had focal low or high density areas. In the early neonatal period, abnormal low density areas were obscure or sometimes absent. In the later neonatal period, they became wider and more distinct. Thereafter, they became focal or sometimes disappeared. For predicting the outcome, CT scans ob­tained in the later neonatal period were thought to be useful.

Key words: Computed tomography, asphyxia, term­infants.

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MR Imagings of Degenerative Diseases of CNS in Children Hideo· Shimoizumi, MD, Masutomo Miyao, MD, Ritsuko Sawa, MD, Mariko Y Momoi, MD, Yoshifumi Yamamoto, MD, Osamu Tanaka, MD and Masayoshi Yanagisawa, MD Departments of Pediatrics (HS, MM, RS, MYM, YY, MY) and Radiology (aT), Jichi Medical School, Tochigi

The diagnosis of degenerative diseases of CNS mostly depends on clinical features and findings at autopsy. In this study, we investigated the MRI patterns of varieties of degenerative diseases of CNS of pediatric ages, analysed the specificity of each changes in MRI, and the usefulness of MRI were discussed.

Cases and Methods Ten cases with varieties of degenerative diseases were studied. They included; 1 case with Wilson disease, 2 with Tay-Sachs disease, 3 with leukodystrophy, 3 with mito­chondrial encephalomyopathy, and 1 with Fukuyama type congenital muscular dystrophy. MR imaging was ob­tained by Toshiba MRT 22A imager using a resistive magnet operated at 0.22T.

Results 1) Wilson disease; extremely low intensity areas (long T 1) were visualized in bilateral frontal lobes and left temporal lobe by Inversion-recovery (IR) and spin-echo (SE) scans. Changes in bilateral thalami and lentiform nuclei were demonstrated as low intensity areas (long TI) by IR scans, and as high intensity areas (long T2) by SE scans. 2) Tay­Sachs disease; MRI of 2 cases were strikingly similar. The grey matter was demonstrated as linear high intensity areas by IR scans, and the white matter was shown as low intensity areas by IR scans and high intensity areas by SE scans. 3) Leukodystrophy; changes of the white matter in 3 cases were striking. Focal low intensity areas in temporal lobes were considered to be reflecting cavity formations due to the massive loss of the white matter. 4) Mitochondrial encephalomyopathy; Leigh's encephalo­pathy showed long T1 areas by IR scans in bilateral thala­mi, basal ganglia and white matter, while long T2 areas by SE scans in white matter. Other cases also showed ab­normal white matter or basal ganglia. 5) Congenital

-muscular dystrophy; Bilateral periventricular areas were shown as long T2 areas by SE scans.

Conclusions MRI was shown to be far superior to CT scans in the visu­alizations of pathological changes of CNS, especially of the white matter. Furthermore, certain MRI changes were specific to each disease, which suggested the possible usefulness of MRI in the diagnosis of each disease.

Key words: MRI, CNS degenerative disease, Wilson dis­ease, Tay-8achs disease, mitochondrial encephalomyopa­thy.

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80

A Brain Ultrasonographic Follow-Up Study of the Ventri­cular Width in Low-Birth-Weight Infants Sadayuki Yano, MD, Koh Ichihashi, MD, Toshihiro Kura­matsu, MD, Masutomo Miyao, MD and Masayoshi Yanagi­sawa, MD Department of Pediatrics, Jichi Medical School, Minami­kawachi, Tochigi

Ultrasonography has been widely accepted as a reliable and noninvasive technique for diagnosing infantile intra­cranial abnormalities. Although the normal range of ventricular width of infants was established in our pre­vious paper, that of low-birth-weight infants has not been reported. We used real-time ultrasonography to measure the ventricular width in low-birth-weight infants on dif­ferent postnatal ages in whom there was no evidence of asphyxia, intracranial hemorrhage or serious respiratory distress.

Patients and Methods Brain ultrasonographic examinations were performed on 52 low-birth-weight infants at the corrected ages of 1, 3, 6, 9, 12 and 15-18 months. All infants were scanned using a mechanical sector scanner (Aloka, SSD-720) with a 5 MHz or 3 MHz probe. The width of the lateral ventri­cle (LV) and the third ventricle (III) were measured on the lateral ventricular body plane (S-2) and the third ventricle plane (C-2) respectively.

Results The mean values of LV-width were 1.9,2.8,4.1,2.8,3.5 and 3.7 mm respectively. The maximum width was 7 mm (6,9, 12 month-old infants). The mean values of III-width were 2.1, 2.8, 3.1, 3.3, 3.5 and 3.5 mm respectively. The maximum width was 6 mm (6-, 9-, l2-month-old infants).

Conclusion The width of both ventricles of l-month-old group were smaller than that of the others, and LV-width of 6-month­old group showed the greatest value which was greater than that of normal birth weight infants statistically.

Key words: Brain ultrasonography, low-birth-weight in­fants, L V-width, III-width.

81

Characteristics of the Proton Relaxation Times in Cerebral Diseases in Children Michio Masumura, MD, Akihiro Ishikawa, MD, Takayuki Shirakuni, MD, Satoshi Matsumoto, MD, Soichi Kodama, MD and Makiko Sugiura, PhD Department of Neurosurgery, Shin-suma Hospital, Kobe, Hyogo (,11M, AI); Departments of Neurosurgery (TS, SM) and Pediatrics (SK), Kobe University School of Medicine, Kobe, Hyogo; Kobe Women's College of Pharmacy, Kobe, Hyogo (MS)

Subjects and Methods By means of VISTA-MR (0.15 Tesla, resistive magnet; Picker International Co.), the proton relaxation times (spin-lattice relaxation time (T d and spin-spin relaxation time (T2)) of pediatric intracerebral lesions were mea­sured. As the control group, 43 children, 4 adolescents and 6 adults were used. The disease group included cases of congenital malformation, cerebritis, intracerebral hemorrhage, brain tumors and head injury. In some cases, the changes in relaxation times were followed up with the time course.

Results 1) Compared with that of T 1 values, the dispersion of T 2 values was great, and it was revealed that the values varied according to the differences between the right and left cerebral hemispheres and the individual slices. 2) Aberra­tions of body motion also had a great effect on the Tl and T2 values. 3) It was also confirmed that the measurement errors were great in cases having small brains such as in­fants. However, although there were various restrictions, the Tl and T2 values obtained were considered to be good enough for relative comparison if the T 1 and T 2 values for normal regions were within the error range of the con­trol group. In inflammation, demyelinating diseases and cerebral contusion, the prolongation of both Tl and T2 was observed from the very early stages. In inflamma­tion and mild contusion, the prolonged T 1 and T 2 became normalized again in several months.

In demyelinating diseases T 1 and T 2 of particularly infant cases were found to be greatly prolonged compared with normal T 1 and T 2 of age-matched controls. In cere­bral hemorrhage, both Tl and T2 were shortened at an acute stage and then became prolonged gradually, but in the blood-coagulated region both T 1 and T 2 showed a tendency of shortening.

Conclusion The Tl and T2 values obtained by MRI have various limit­ations, but they can be used as an index in the observation of the progress of cerebral diseases in children.

Key words: MRI, proton relaxation time.

82

Computed Tomography (CT) of Skeletal Muscle in Neuro­muscular Disorders-Comparison of CT Findings in Skele­tal Muscle with Histological Findings in Muscle Obtained on Surgical Biopsying-Hideo Nagao, MD, Takehiko Morimoto, MD, Mitsugi Takahashi, MD, Shinji Habara, MD, Hironao Nagai, MD and Hiroshi Matsuda, MD Department of Pediatrics, Ehime University School of Medicine, Ehime

We investigated whether or not CT of skeletal muscle is of value for the diagnosis of neuromuscular disorders, through comparison of CT findings in skeletal muscle with histological findings in muscle obtained on surgical bio­psying, and the results were as follows.

I) A slice of the thigh including the quadriceps femo­ralis, biceps femoralis and adductor muscles might be the most useful for the diagnosis, because the low density area (LDA) seen on CT in Duchenne muscular dystrophy were seen in these muscles from the earliest stage, and in the muscles of all the 14 cases aged over 3 years old.

2) The LDA seen on CT of the pelvic girdle and lower leg might be useful for the differential diagnosis be­tween Fukuyama type congenital muscular dystrophy, Werdnig-Hoffmann disease and floppy infants with mental deficiency.

3) There were clearly histological lesions even in cases in which LDA were not seen on CT of skeletal muscle. Muscle exhibiting remarkable LDA shows high prolifera­tion of connective tissue and accumulation of fat, but contains no muscle fibers. Therefore, the most suitable site for a biopsy is in muscle which exhibits mild LDA on CT.

Key words: Computed tomography, neuromuscular dis­order, floppy infant, muscular dystrophy.

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