39

UItrasonographic Imaging of the Middle Cere­bral Artery and Adjacent Structures Shogo Kihira, MD, Sachiko Kusumoto , MD, Toshihiro Uehara, MD and Toshiko Juri, MD Department of Pediatrics, Wakayama Medical College, Wakayama

Recently, real-time ultrasonography has become an essential procedure for evaluation of brain disorders in infancy. Many authors reported ana tomical con­sidera tion s as to ultrasound images. However, there has been no detailed report yet abou t the imaging of brain arteries, despite that they are detec table by this technique. We investigated the ultrasound imaging of the middle cerebral artery (MCA) and adjacent struc­tures, i. e. the Sylvian fissure and in sula .

Materials and Methods 31 cases (18 males and 13 females) part icipated in this study. The age at examination ra nged from 25 weeks postconceptually to 10 months after birth. We performed routine and additional scans for each case, using a 5 MHz electronic sector scanner (RT-model 3000, Yokogawa Medical System) . OUf routine scans were modified according to Rumack's method. The additional scanning included 4 sagit tal (through the insu la) and 2 axial planes (through the circle of Willis and through the frontal horns, respectively).

R esults Routine coronal scans revealed the opercularization (cover ing of the insula by gyri) with increasing post­conceptual age (PCA). Insular gyrus formation became notable toward 40 weeks PCA , a "3"-shaped image being finally presented in coronal scans through the third ventricle. A continuous echogenecity which cor­responded to the horizontal portion of the MCA was detected well in coronal scans through the frontal horns. The peripheral portion (insula and opercular) of the MCA showed pulsations or, occasionally , short striae, which were superimposed on the image of the insula and Sylvian fissure.

Sagittal scans through the insula demonstrated the developmental change, that led t o a conspicuous "zebra" pattern after 35 weeks PCA . Pulsatile branches of the MCA were also seen scattered throughout the insu la, although a continuous echogenecity (implying one major branch of the MCA) was observed invari­ab ly along the lower margin of the insula.

I n axial scans through the circle of Willis, the horizontal portion of the MCA was detected bilateral­Iy. I n ax ial scans through the frontal horns, the insula and the superimposed pulsations of the MCA (in­sular portion) were identified contra laterally to the transducer.

Conclusion Ultrasonographic detection of the MCA was perform­ed in serial scanning planes. Developmental changes of the adjacen t structures were also visualized .

Key words: Ultrasound, middle cerebral artery, insula, Sylvian fissure.

40

Neuron Specific Enolase and S-IOO Protein in the Cerebrospinal Fluid of Patients with Neuro­logical Disorders Mituo Maehara, MD, Naoki Mizutani, MD and Kazu­yoshi Watanabe, MD Department of Pediatrics, Toyota Hospital, Toyo ta, Aichi (MM) ; Department of Pediatrics, Nagoya Univer­sity School of Medicine, Nagoya, Aichi (NM, KW)

The neuron specific enolase (NSE) and S-IOO protein levels in cerebrospin al i1uid (CSF) were determ ined in 46 children with neurological disorders and 14 control subjects.

The normal values for NSE and S-lOO protein were 3.6 ± 1.5 ng/ml and 0.27 ± 0.12 ng/ ml , respectively.

The NS E and S-IOO protein leve ls were elevated in patients with asep tic meningitis , acute transverse myelopa thy, intracra nial hemorrhage and acute en­cephalopathy , but were normal in patients with epilepsy, SSPE and polyneuropathy.

The e levat ion of NSE and S-IOO protein in CSF might be correlated with the degree of acute damage to neurons and glial cells. The simultaneous elevation of NSE and S-lOO protein was considered to be more prognostic of neurological sequelae, compared to the elevation of either one of these proteins alone.

Clinical measurement of the NSE and S-IOO pro­tein levels may be of value in neurological disorders as an index of tissue damage within the nervous system.

Key words: Neuron specific enolase, S-100 pro tein.

Brain & D evelopment, Vol 8, No 2, 1986 127

41

Neuron-Specific Enolase (NSE) in Serum and Cerebrospinal Fluid in Neurological Diseases in Childhood Hiroshi Takahashi. MD. Hisakuni Yoshida. MD. Shoko Urata. MD. Masafumi Kaneko. MD. Kennichiro Kane­ko. MD and Chikaya Ohtsuka. MD Department of Pediatrics. Juntendo University Ura­yasu Hospital. Urayasu. Chiba

Enolase is a dimeric enzyme with three subunit types. a. (i and 'Y . which give rise to five isozymes. aa, a(i, (i(i , at' and 'Y'Y. These show different organ distributions. Neurons specifically contain 'Y enolase , which has been called neuron-specific enolase (NSE). NSE has been said to be a useful marker for staging and monitoring the response to therapy in patients with small cell lung cancer and neuroblastomas.

I n the present study , we report the NSE levels in serum and cerebrospinal fl uid from patients with various neurological diseases.

Materials and Methods Serum samples were obtained from 44 patients with neurological diseases and 41 control patients. Cere­brospinal fluid (CSF) samples were obtained from 19 patients with neurological diseases and 26 control patients, respectively.

NSE is serum and CSF was measured by means of a double antibody radioimmunoassay.

Results The mean NSE leve l in serum from the 41 control patients was 11.6 ± 3.4 ng/ml. The NS E level tended to dec rease with increasing age. There was a signifi­cant difference in serum NSE levels between the under-5 -year-old grou p and the over-6-year-old one (p < 0.05).

The mean NSE level in CSF from the 26 control patients was 6.6 ± 2.6 ng/ml.

In the cases with uncontrolled epilepsy , co ntro lled epilepsy and mental retardation without any comp lica­tion, the mean NSE levels in serum were 17.7 ± 6.7 ng/ml (n = 11), 13.3 ± 4.1 ng/m! (n ~ 15) and 11.1 ±

2.7 ng/ml (n = 13), respectively. Significant differ­ences were found in serum NSE levels between un­controlled epilepsy and controlled epilepsy (p < 0.1), uncontrolled epilepsy and mental retardation (p < 0.005), and uncontrolled epilepsy and normal con­trols (p < 0.001). Whereas, no significant differences were found between norm al controls and mental re­tardation, or normal controls and controlled epilepsy.

High CSF NSE levels were seen in the cases with cerebral palsy due to anoxic encephalopathy (20.4 ngj ml) and meningoencephalitis (13.0 ng/ml). Extremely CSF high NSE levels were seen in the cases with acute infantile hemiplegia (183.6 , 149.6 ng/ml) and chronic subdural effusion (34 .8 ng/ml) in the acute phase; whereas with convalescence, the CSF NSE levels de­creased with clinical improvement.

Conclusion The origin of the high NSE levels of serum and CSF is presumably neurons, because neurons specifically contain NSE.

128 Brain & Development, Vol 8, No 2, 1986

Neuro n-Specific Enolase (NSE) might be a useful marker of neuronal damage.

Key words: Neurol/-specific enolase (NSE). epilepsy. cerebrovascular disease.

42

Normal CT in Children Sumimasa Yamashita , MD. Mana Kurihara, MD, Syota Miyake, MD, Michiko Hayashi. MD and Hiroko lwa­mOlO. MD Division of Pediatric Neurology, Kanagawa Children's Medical Center, Yokohama. Kanagawa

There have only been a few studies o n au thnrized norm al va lues on CT in infants and children . In our hospital, 4,300 CT scans of infants and children were made during the period of 1980 to 1983. In order to determine the norm al values on CT in infancy and children , we, a radiologist and pediatric neurologists , selected and analyzed 114 CT samples as tentatively normal films.

Methods The samples analyzed satisfied the fo llowing criteria ; the cases had neither epilepsy nor mental retardation, had not just had convulsions, and had no past history of encephalitis, encephalopathy or meningitis. The cases investigated had a birth history of a full term pregnancy and a birth weight of more than 2,000 g without asphyxia. A Toshiba TCT-60A scanner was used. All measurements were made froll1 the picture on the film. The portions measured were as follows: LV , maximum width of each lateral ventricle; IF . maximum width of the interhemispheric fi ssure ; SS, maximum width of the subdural space ; SF . width of the Sylvian fissure; C, maximum distance between the anterior horns; D, distance between the head of the caud ate nuclei in the lateral ventricles; III , width of the third ventricle; A , maximum transverse distance betw een the internal laminae at the same level; G, maximum width of the basal cistern; H, maximum transverse distance between the internal laminae at the same level ; LH , maximum width of the lateral horn ; P, longitudinal wid th of the pons; and IV, wid th of the fourth ventricle.

Results and Conclusion The indices which were useful for recogniz ing cerebral atrophy were CIA, D/A and G/ H, and D/ A being the best index. In all CT samples for children over 7 years old , D/ A was below 0.1. A subdural space of below 4 mm was found in about half the 1-year-old infants, and no subdural space was found over 2 years old . The normal width of the third ventricle was under 6 mm in children over 7 years of age. The fourth ventricle was under 7 mm at any age.

Key words: Normal CT. index, cerebral atrophy.

43

Computed Tomography in Epileptic Children, Especially Investigation of the Temporal Horn Mana Kurihara, MD, Sumimasa Yamashita, MD, Syota Miyake, MD, Michiko Hayashi, MD and Hiroko Iwa­moto, MD Division of Pediatric Neurology , Kanagawa Children's Medical Center, Yokohama, Kanagawa

The aim of this study was to ana lyze the changes seen on cranial computed tomography (CT) in epileptic children , especially in the area of the temporal horn.

Materials and Methods The subjects were 242 epileptic children, ex cluding ones with encephalitis, brain tumors. neurocutaneous syndromes and so on. The control subjects were 195 children without any neurological symptoms. CT scans were performed with a TCT-60A whole body scanner , and assessed using 14 check points ex cluding the temporal horn. Next. the areas of the temporal horns and adjoining hemispheres were examined with a Muto-Tablet-Desetizer.

R esults I . In epileptic children , CT abnormalities were found

in 46%, and the percentage being the highest for West syndrome (92%).

2 . The temporal horn ratio «area of temporal horn/ area of ipsilateral hemisphere) x 100) of the con­trols was greater in younger child ren.

3. The temporal horn rat io of all the epileptic chil­dren was greater than in the contro ls.

4 . The temporal horn ratio of the ep ileptic children with normal CT scans was greater than in the con­trols.

5. Enlargement of the temporal horn was seen in 2 of the controls (1%) and in 44% of the epileptic children with normal CT scans (35 %) (p < 0.01).

6. The frequency of enlargement of the temporal horn was not predominant in any particular epi­leptic type, but all the West syndrome cases had a large temporal horn .

7 . In epileptic children with normal CT scans whose temporal horns were enlarged , behav ioral dis­turbance (p < 0.05) and intractable seizu res (N .S.) were frequent. All 6 cases showing behavioral disturbance were boys and 5 had enlarged temporal horns (bilateral; 1 case, left side ; 1 case, and right Side; 3 cases).

Discussion CT scan abnormalities of the epileptic child ren of this series were seen in 46%. The frequency of enlarged temporal horn in epileptic children was greater than in the controls. In epileptic children with normal CT scans and enlarged temporal horns, behavioral disturb­ance and intractable seizures were frequent.

Key words: CT scan, epilepsy, temporal horn, be­havioral disturbance.

44

Positron Emission Tomography (PET) in De­myelinating Diseases Momozo Toyoda , MD, Tetsuo Matsusaka , MD , Sui Sone, MD, Norio Sakuragawa, MD, Hideaki Kudoh , MD, Masataka Arima, MD and Masaaki Iio, MD Department of Child Neurology, National Musashi Research Institute for Mental and Nervous Diseases, Kodaira, Tokyo (MT, TM, SS, NS); Division of Mental, Nervous and Birth Defect Research, National Center for Nervous, Mental and Muscular Disorders, Kodaira, Tokyo (HK, MA); Department of Radiology, National Nakano Chest Hospital, Nakano, Tokyo (MI)

Dynamic PET with "CO, was performed in patients with demyelinating diseases in order to examine the circu lation and " CO, metabolism in the central nerv­ous sys tem.

The subjects were as follows; two cases of adreno­leukodystrophy (ALD), one case of Krabbe's disease and one case of multiple sclerosis (MS). Using a cy­eloton, "CO, was prepared through bombardment of a 14N, gas target with a photon beam. Each tomo­graphic scan was started 10 seconds before inhala­tion of "CO, and recorded with a Headtome-Il (Shimadzu). The tota l examination time and about 5.5 minu tes and 16 images were obtained. One image took 20 second s to obtain.

In early images on "CO, dynamic PET, lower RI uptake areas were seen which corresponded to atrophic or low density areas seen on CT scanning in each disease. In later images, radionuelide clearance from the cortex in MS and controls gradually became almost equal as to radio nuclide counts to in the case of the ventricles. On the contrary, radionuclides were cleared very slowly from the cortex of patients with ALD and Krabbe's disease.

The early images on !lC02 dynamic PET are close­ly related to the regional cerebral blood flow (rCBF) . because one image is obtained in only 20 seconds, which is approxim ate ly the time of one circulat ion of the cerebral blood flow. So, the decreased RI uptake seen in early images suggested the decreased reBF in the present cases. The later images are possibly influenced by the brain tissue pH, which results in altered distributions of "C0

2 and H 11 CO ) . But the

changes in rCBF, B-B-8 function, carbonic anhydrase activity and other factors may also influence them.

I! was not determined why delayed radionuclide clearance was seen in the cortex of ALD and Krabbe's disease. We interpret these data as suggesting that rCBF may be decreased in demyelinating diseases, particularly in the advanced stage, in addition to al­tered !lC02 metabolism.

Key words: I1CO, dynamic PET, demyelinating dis­eases, regional cerebral blood flow (rCBF), brain tissue pH.

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Basal Ganglia Calcification Seen on Computed Tomography in Childhood Keijiro Abe, MD, Nario Inoue, MD, Hidetoshi Hirano, MD, Katsuichi Irie, MD, Tomoko Uchida, MD, Masa­haru Ohfu, MD, Hiroko Ogata, MD, Akihisa Mitsu­dome, MD, Toshio Okudera, MD and Katsuya Goto, MD Department of Pediatrics (KA, NI, HH, KI, TU, MO, HO, AM) and Radiology (TO, KG), Fukuoka Univer­sity School of Medicine, Fukuoka

Recently there have been many reports on basal ganglia calcification (BGC) seen on CT in adults. We examined this in childhood.

Materials and Methods We searched for cases with BGC by examining all the CT scans obtained in the last 6 years (April 1979-March 1985). Scanning was performed with a Soma­tom SD and DR-II. Sections were obtained at an angle of 15° to the orbitomeatalline.

Results BGC was found in 13 cases out of the 1,637 cases who underwent computed tomography. The final diagnoses were as follows; pseudohypoparathyroid­ism, 4 cases; idiopathic hypoparathyroidism, 1 case; mitochondrial encephalomyopathy, 1 case; Cockayne syndrome, 1 case; brain tumor, 2 cases (craniopharyn­gioma and ectopic pinealoma); congenital nephrotic syndrome, 1 case; and unknown, 3 cases. The average age was 7 years old. The male to female ratio was 1 to 2.5. The locations of the calcification were clas­sified into 3 types. The first type was seen in 4 cases, in which BGC existed in only the globus pallid us. The second was seen in 7 cases, in which it existed in the globus pallidus and other regions. The third was seen in 2 cases, in which it existed in regions other than the globus pallidus. Only in the craniopharyngioma case was calcification demonstrated by both the CT scan and the plain roentgenogram. There were 3 cases in which BGC was recognized in the 2nd CT scan. No extrapyramidal signs were noted except for tremor in the Cockayne syndrome case. The other common neurological symptom was convulsions which were seen in 8 children. The EEG findings were abnormal in 9 of eleven patients.

Conclusion At first, when BGC is seen on CT scanning, we must examine the Ca metabolism. Then we must determine whether other underlying diseases are present or not.

Key words: Basal ganglia calcification, computed tomography.

I30 Brain & Development, Vol 8, No 2, 1986

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Sincipital and Basal Encephaloceles with Other Anomalies - Neuroradiological Analysis Hidetsuna Utsunomiya, MD, Takashi Hayashi, MD, Takeo Hashimoto, MD and Masashi Yamamoto, MD Departments of Neuroradiology (HU), Neurosurgery (TH), Neonatology (TH) and Pediatrics (MY), St Mary Hospital, Kurume, Fukuoka

It has been known that cranium bifidum occurs pre­dominantly in the occipital region. But, it is rarely found in the sincipital and basal area and few observa­tions were reported in Japan, Europe and America. We. however, have had seven rare cases of meningo­encephalocele located at these areas in the past five years. We present the results of these clinical and neuroradiological analysis.

Results According to the morphological classification of the sincipital and basal cranium bifidum, they were clas­sified as follows: The frontoethmoidal, 3; the inter­frontal, 3; and the transethmoidal, 1. As far as as­sociated anomalies in the central nervous system were concerned, 2 cases had lipoma of corpus callosum with total callosal agenesis and 3 had myelomeningoceles. Five cases had congenital hydrocephalus of which 3 patients had further complication of craniolacunia, There were also 3 cases of hypertelorism, Correcting the meningoencephalocele and bony defects were performed ,by either intracranial or extracranial pro­cedure, 3 cases and one case, respectively. One severe case of hypertelorism was rectified by transferring the whole orbit at the same time as other corrections were being done,

Conclusion Neuroradiologically, we divided our sincipital and basal meningoencephaloceles into three types, fronto­ethmoidal, interfrontal and transethmoidal. All cases were accompanied with other anomalies like congenital hydrocephalus, myelomeningocele and lipoma of the corpus callosum which ought to be termed "lipo­meningoencephalocele". On the basis of the above fact, we discussed the pathogenesis of meningoen­cephalocele in terms of other anomalies.

Key words: Cranium bifidum, sincipital and basal me­ningoencephalocele, lipomeningoencephalocele, con­genital hydrocephalus.