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Treatment for moyamoya disease in infants and toddlers is challenging, because of the progressiveness of the disease. Revascularization surgery is the first-line therapy and should be performed as early as possible after diagnosis to prevent additional cerebral infarction. This review describes in detail the changes in the new "Diagnostic Criteria 2021 for moyamoya disease", and discusses the concept of treatment including indirect and combined bypass surgery in infants and toddlers. Surgical technique and perioperative management based on our experience are also precisely described.

Gelastic seizure is a rare form of epilepsy defined as automatic bouts of laughter without mirth commonly associated with a hypothalamic hamartoma. Surgical treatment of hypothalamic hamartomas is associated with a high risk of complications because of the close vicinity of adjacent structures such as the optic tracts and mammillary bodies. This case was an 11-year-old girl who presented with gelastic seizure and complicated partial seizure. She developed gelastic seizure at a frequency of 10 bouts per day. She was found to have an elliptical mass close to the left hypothalamus. The signal intensity on magnetic resonance imaging (MRI) was consistent with hypothalamic hamartoma. The patient underwent surgical resection by a subtemporal approach. Pathological findings confirmed the diagnosis of hypothalamic hamartoma. Postoperative MRI demonstrated that the hypothalamic hamartoma was successfully resected. Twenty four months after surgery, complicated partial seizure in this patient has improved to Engel's class 1a and gelastic seizure has improved to Engel's class IIIa.

Irradiation for intracranial germinoma may be associated with significant neuroendocrinological sequelae, so the establishment of lower effective doses of radiation is desirable. Eight consecutive patients with intracranial germinoma underwent combination chemotherapy with BEP (bleomycin, etoposide, and cisplatin) or PE (cisplatin and etoposide) without irradiation between 1996 and 1997. The diagnosis was based on endoscopic or stereotactic biopsy, or transsphenoidal surgery in 7 cases. These 7 patients obtained complete response (CR) after treatment with chemotherapy only. The diagnosis of pure germinoma was based on neuroradiographic appearances and the normal levels of tumor markers in patients with suprasellar and pineal-region germ cell tumors. Although all patients obtained CR, 5 patients relapsed at a mean period of 19 months after the initial therapy. The mean follow-up period was 53 months. Two of these 5 patients had recurrence around the ventricle wall. Additional chemotherapy and total ventricle irradiation (24 Gy) achieved CR, but delayed seeding in the optic nerves outside the irradiated field was detected. One patient suffered peritoneal dissemination via a ventriculoperitoneal shunt with intracranial dissemination. Another patient obtained CR after PE therapy, but there was not histological verification. However, 35 months after the initial therapy, surgical extirpation of the recurrent tumor revealed immature teratoma. One patient refused treatment for recurrence at the initial tumor site and died 42 months after the initial treatment. Adequate initial therapy is absolutely essential for the treatment of intracranial germinoma. The chemotherapy regimens in use today cannot be recommended as initial therapy without irradiation because of the high recurrence rates.

The authors present a case of congenital dermal sinus tract with epidermoid tumor. This 1-year-old boy was referred to the pediatric service of another hospital with recurrent pyrexia of unknown origin in April, 1999. The pediatrician found two dimples, pigmentation, and coarse hairs on the midline in his sacral region. Computerized tomography (CT) scans revealed a spina bifida below the S1 level. Magnetic resonance (MR) imaging revealed a dermal sinus tract in the cranial direction to a cystic tumor at L2-4 levels. He was transferred to our hospital, and the tract and tumor were totally removed in June, 1999. The histological findings and Escherichia coli in the smear culture of the tumor contents identified it as an infected congenital dermal sinus tract with epidermoid tumor. The patient received antibiotics for two weeks after surgery and there was no clinical or radiographic recurrence of either infection or tumor. The authors propose early diagnosis and radical treatment, because infected congenital dermal sinus tract often leads to a bad neurological prognosis.

The authors report a rare case of intracranial glass injury due to a temporal head injury. This 72-year-old man slipped on a bathroom floor, impacting a glass door with his head and right shoulder. His right temporal scalp and right shoulder were cut by the broken glass. He visited our emergency unit four hours after sustaining the injury. Physical and neurological examinations showed no abnormalities except for two lacerated wounds on both the right temporal scalp (1.5 cm) and the right shoulder skin (10 cm). Foreign bodies were not palpable around the lacerated wounds. Skull X-ray and CT studies disclosed a single, 5-cm long, radiopaque foreign body penetrating the temporal skull bone into the right temporal lobe, but no evidence of intracranial bleeding was found. Under the diagnosis of intracranial glass injury, total removal of the foreign body with dural repair was carried out. On surgical exploration, glass penetrating the skull bone 5-mm distant anteriorly to the scalp laceration was observed. Postoperative angiography showed no vascular lesions, and one-week later he was discharged with no complications. According to the literature, most of the intracranial foreign bodies occur around the orbital, the frontal sinus, and the nasal areas. To our knowledge, this is the first report of an intracranial glass penetrating injury to the temporal lobe. Since the clinical manifestations occasionally do not correspond to the appearance of the laceration after glass penetrating injuries, serious caution concerning patients with intracranial glass penetrating injuries is important.

We report a case of recurrent cerebellar abscess secondary to middle ear cholesteatoma. A 57-year-old man was admitted to our hospital because of symptoms of headache and nausea in August, 1992. Brain CT scans revealed acute hydrocephalus complicated by a cerebellar abscess. The patient was discharged without any neurological deterioration after systemic antibiotics combined with intrathecal aminoglucoside administration via ventricular drainage. Mannitol was also administrated for 7 days immediately after the patient's admission. The clinical course was uneventful for 8 years afterwards. Follow-up MR images revealed no signs of recurrence. Unfortunately, the patient suffered a recurrence of cerebellar abscess in October, 2000. His condition continued to deteriorate in spite of being treated by systemic antibiototics. MR images and CT scans targeting a portion of his middle ear revealed extensive pus-coated mastoiditis and middle ear cholesteatoma. We thus performed radical mastoidectomy including removal of the middle ear cholesteatoma. After the operation, the cerebellar abscess was ameliorated. He has been free from recurrence for 2 years, so far. Early diagnosis and prompt intervention are necessary for reducing mortality and morbidity rates due to otogenic brain abscess. Recognizing middle ear cholesteatoma as one of the major causes of neurological entities in the cerebellopontine angle portion, accurate otological examination and prompt treatment can possibly bring about a better prognosis.

Although primary aldosteronism (PA) has been reported as a relatively benign form of hypertension and is associated with low incidence of vascular complications, recent reports indicate that PA complicated by cerebral vascular accidents is not rare. The authors reported here that a 57-year-old man was diagnosed as a case of PA 4 years after initial treatment of intracerebral hemorrhage (ICH) and hypertension. The patient was transferred to our department for further management of his left hemiplegia and hypertension after surgical treatment for a putaminal ICH at the age of 53. During the first 2 years of follow-up, he did well except for an episode of transient motor weakness. Diastolic hypertension was moderately good, controlled by calcium antagonists and ACE inhibitors. Laboratory data was normal, and the serum potassium levels were in the lower limits of normal. In the last 2 years, episodes of motor weakness have increased. He was admitted to our hospital, under the suspicion of recurrent Todd's paralysis. The serum potassium level ranged from 1.9 to 2.1 despite administration of potassium agents. Abdominal CT scans and 131 I-scintigraphy disclosed a left adrenal tumor. Elevation of plasma aldosterone and suppression of plasma renin were observed. The diagnosis of PA due to hypersecretion of aldosterone from an adrenal tumor was thus confirmed. We performed a total left adrenectomy 4 weeks after his admission. Histological examination showed a clear-cell type of benign adrenal adenoma. After the operation, laboratory data were normal and ACE inhibitors were able to normalize his diastolic hypertension. According to the literature, PA complicated by ICH is associated with a high rate of recurrence of cerebral vascular accidents if treatment of PA is not performed. Although diagnosis of PA in the early stage is difficult, as it was in our presented case, high suspicion of PA is essential for patients with diastolic hypertension and persistent hypopotassiumemia, particularly in young adults and middle-aged patients.

Dermoid and epidermoid tumors arise from the invagination of epidermal elements into the neural tube during the embryonic period. However, many studies have reported that dermoid and epidermoid tumors occur 5 to 10 years after the first operation on myelomeningocele patients. A seven-year-old male with myelomeningocele which had been repaired in his neonatal period, presented leg pain and deterioration of gait disturbance and urinary incontinence. Neuroradiological examinations revealed a spinal dermoid cyst at the repaired myelomeningocele and tethered spinal cord. We removed the tumor and untethered the tethered spinal cord. Postoperatively, the patient's leg pain, gait disturbance and urinary incontinence improved immediately. Inappropriate surgical treatment of spina bifida may cause a second lesion, which leads to tethered-cord syndrome. We suggest that proper surgical treatment, early checkup and neuroradiological evaluations are very important for spina bifida patients who show signs of neurological deterioration. For the best treatment, neurological, urological and orthopedical follow-up study after the first repair operation is necessary.

Neonatal intracranial teratoma is rare and early attempts at surgical treatment are not usually successful. We describe an exceptionally rare case of neonatal intracranial tumor in the lateral ventricle composed of multiple cysts with little mass content which was highly suspected of teratoma and treated successfully by microsurgery. A 55-day-old male was admitted to our hospital with complaints of convulsive seizure. Examination found no obvious neurological deficit except mild enlargement and bulging of anterior fontanel. There were no abnormal laboratory findings. Computerized tomography with contrast medium revealed an 8 cm multicystic lesion with little mass content in the right lateral ventricle and hydrocephalus. Magnetic resonance imaging showed the cystic content as slightly higher in intensity than CSF on the T1-weighted image and of higher intensity than CSF on the T2-weighted image. The cyst wall was enhanced on T1-weighted image with gadolinium. The aqueduct was obstructed by compression due to the intraventricular cyst. A right occipitoparietal craniotomy was performed, which disclosed a hair in the cyst wall, two huge cysts, and independence of the intracystic lesion from the lateral ventricle. The tumor was removed except for a small part adjacent to the pineal region, from which the tumor had presumably originated. Mature teratoma was highly suspected on the basis of the hair in the cyst wall and histological examination of the specimen, which revealed hamartomatous tissues derived from two germ cell layers. After surgery, his hydrocephalus was improved and convulsive seizures were reduced remarkably. He was discharged ten days later and is being followed up. Reoperation will be performed if regrowth of the residual tumor occurs.

A 6-month-old girl was admitted to another hospital because of consciousness disturbance, preceded by 2 weeks of decreased activity and vomiting. She was referred to our hospital after ventricular drainage had been instituted for hydrocephalus and the tumor in the pineal region. The patient was noted to have conjugate upward gaze palsy and papilledema. CT scan and MRI revealed a large tumor in the pineal region with tumoral hemorrhage and a small mass in the right frontal lobe. At surgery, the pineal region tumor was removed subtotally. Histological examination showed the tumor to be composed of sheets of large polyhedra or round cells with an eccentric round nuclei, prominent nucleoli, and cytoplasmic inclusions. Immunohistochemical studies were positive for GFAP, vimentin, S-100, CK, EMA, and SMA, but negative for AFP, HCG, PLAP, and CEA. Following surgery, she received three 5-day cycles of chemotherapy, consisting of intravenous administration of cisplatin 20 mg/m2/day and etoposide 60mg/m2/day. After these therapies, MRI showed a decrease in the area of high intensity in the pineal region, but almost no change in the right frontal mass lesion. Follow-up radiological examination showed that the tumor had grown rapidly one month after chemotherapy and the patient died 5 months after her first hospitalization. Malignant rhabdoid tumor of the CNS is rare and remarkably malignant. This tumor should be treated using multidisciplinary management with surgery, intensive chemotherapy, and radiotherapy depending on the patient's age.

We report a case of "functional aqueductal stenosis" which reveals dilatation of the lateral and 3rd ventricles without stenosis at the aqueduct in MRI. This case shows a pineal teratoma which presents one year later with symptoms of hydrocephalus caused by "functional aqueductal stenosis". A seven-year-old boy was admitted to our department owing to headache and vomiting. CT and MRI showed hydrocephalus. The lateral and 3rd ventricles were dilated while the 4th ventricle was normal. Furthermore, tumoral obstruction of the aqueduct was not found. After a ventriculoperitoneal shunt, he recovered well without neurological deficits. One year later, symptoms of precocious puberty, that is the appearance of public hair and deepening of his voice, were found. A follow-up MRI demonstrated a pineal region tumor. Although human chorionic gonadotropin level in the serum and urine was transiently elevated, it normalized before surgery. The operation was performed by the occipital transtentorial approach and the tumor was totally removed. Histological examination proved this tumor to be a mature teratoma, showing three germ cell layers. About two weeks later, he was discharged without any neurological deficit. In this case, although hydrocephalus occurred, MRI didn't demonstrate aqueductal obstruction caused by the tumor. However, one year later, a pineal region tumor was confirmed by MRI. This suggests that hydrocephalus might have some association with the appearance of the pineal region tumor. Therefore, it is necessary to be aware of the possibility of the occurrence of tumors whenever we encounter "functional aqueductal obstruction", when MRI doesn't demonstrate aqueductal obstruction caused by a tumor.

A case of saccular aneurysm-like bleb formation after rupture of an aneurysm in the internal carotid-posterior communicating artery. In connection with this, an interesting angiographic finding was reported. A 65-year-old man suffered from sudden disturbance of consciousness and left hemiparesis. Computed tomography (CT) scan revealed typical subarachnoid hemorrhage localized in the right Sylvian fissure. Next day, a cerebral angiography was performed, but no aneurysm was detected. A second angiography was performed 21 days after the onset, and it revealed a saccular right internal carotid-posterior communicating artery (IC-PC) aneurysm. An operation for the IC-PC aneurysm was performed by conventional pterional approach. However, intraoperative findings were unexpected. The collapsed ruptured true IC-PC aneurysm was found at the orifice of the larger bleb, and the ruptured point was in the neck of the true aneurysm. Clipping of the aneurysm was performed successfully. The patient was discharged on foot. The aneurysm detected by the second angiography was not a true one but a bleb formation. Continuous hemodynamic stress on the rupture point may induce the formation of such an aneurysm-like bleb. It should be kept in mind that an aneurysm found in the chronic period might be an aneurysm-like bleb.

A case of tectal glioma with mild choked disc is reported. An 11-year-old boy was admitted to our hospital because of visual disturbance and choked disc. Neurologically, the patient had nothing but choked disc. Magnetic resonance imaging (1.5T) was performed. Relative T1 weighted image showed a lesion of low signal intensity, and T2 weighted image showed high intensity, about 1.0 x 1.0 cm in size, at the pineal region. The sagittal view showed a mass at the tectum, and stenosis of the aqueduct. It was diagnosed as tectal glioma. Left occipital craniotomy was performed and the tumor was removed subtotally. Histological examination demonstrated a fibrillary astrocytoma. Radiochemotherapy was performed postoperatively. Tectal glioma is very rare. The differential diagnosis from germ cell tumor or pineal cyst is essential for treatment. The authors performed an operation in order to remove the tumor and determine the course of treatment.

A case of anterior sacral meningocele associated with tethered cord syndrome is reported. A 5-year-old boy was admitted for urinary and fecal incontinence which had persisted since his birth. Abdominal MRI and sacral CT showed a presacral cystic mass communicating with the spinal dural sac through a neck traversing a sacral bony defect and tethered spinal conus. Neurological examination showed that the patient had a neurogenic bladder, atrophy of the legs and anesthesia below the S3 level. Operative finding revealed a thickened filum terminale attached to the wall of the presacral meningocele. Agenesis of the nerve root below the S2 level was identified, and no neural elements entered into the sac. Untethering was performed. Postoperative course was uneventful and the patient is doing well with his neurogenic bladder gradually improving.

A case of diastematomyelia with meningomyelocele is presented here. The patient was a newly born infant with a soft tumor in the lumbo-sacral region. The lower extremities were not paretic, but anal reflex was negative. A plain X-ray and a CT scan of the spine showed a bone spur at L3, 4. An MRI showed a longitudinal slit in the spinal conus. The removal of the septum and repair of the cele was carried out. The operative view showed a bony septum which divided the spinal conus into two parts and nerves had spread out on the cele wall. After removal of the septum, the tethered cord was released. The postoperative condition remained stable. The reason for the occurrence of a combined case of diastematomyelia with meningomyelocele is not clear. We presume that abnormality of the mesoderm at the embryonic stage may have an effect on this sort of disorder.

A long-term result after total removal of a giant intracranial teratoma in early infancy is reported. The patient was a 5-month-old boy with vomiting, convulsion and right hemiparesis occurring acutely and shortly before admission. Plain skull films, In-113m brain scintiscanning and carotid angiography revealed a giant tumor located widely over both frontal lobes. At operation, the tumor, originated from the base of the anterior half of the cranial cavity. A mature teratoma weighing 320 grams was totally removed without sacrificing the adjacent brain and vessels. Both cerebral hemispheres were found severely compressed by the tumor. Postoperative respiratory and convulsive disorders were well controlled. The boy was discharged in the third month after operation. At present, 13 years after operation, he is developing normally for his age. In the junior high school, he studies and plays sports with no handicap. Computed tomography reveals no recurrence of the tumor. EEG shows reactive pattern of activity and small, but rare spikes in the frontal area. And his pituitary function is revealed to be normal.

A case of chronic subdural hematoma following EMS (Encephalo-Myo-Synangiosis) for moyamoya disease was presented. Two and a half year-old girl was admitted to our division because of right hemiparesis. Left carotid angiography revealed a very narrow left carotid bifurcation and small moyamoya vessels. EMS was performed on the left side and a right hemiparesis was improved. But she developed a headache and vomiting one month after the operation. CT scan and angiogram showed a chronic subdural hematoma on the left side. A hematoma weighing approximately 30 g was evacuated. We reviewed a similar case of 5-year-old boy in the literature and discussed the mechanism accounting for formation of the chronic subdural hematoma following EMS.

A 38-year-old woman with three intracerebellar calcifications visible in roentgenogram was reported. She had not suffered from any disease that should induce intracranial calcification. Plain x-ray films of the skull showed three small calcifications in the posterior fossa. The laboratory study and neurological examination revealed no abnormality. Computerized tomography showed no abnormal findings either. Histological examination was carried out. The tissue surrounding the calcification seemed to be gliosis.

A case of neonatal brain abscess was reported. This female infant was born by spontaneous vertex delivery at 38 weeks gestation, weighing 3.1 kg. There were no antenatal and perinatal complications. The Apgar's score was 8 points and her head was 32.8 cm in circumference. Fifty-six hours after birth, she had a clonic general convulsion for about 3 minutes. On the next morning, convulsion recurred, and she was transferred to our hospital. When admitted to the Pediatrics, she was exhausted and irritable. But after hospitalization, she improved without convulsions. CT scan, performed 7 days after birth, revealed large low density areas in both frontal lobes. Electroencephalography showed spike discharge at all leads. The head circumference gradually increased and subsequent CT scans revealed enlargement of the bifrontal low density areas. The first operation was done 28 days after birth. A large amount of yellowish grey pus was aspirated from both sides and the cavity was irrigated. Tobramycin was administered into the abscess cavity. Culture of the pus grew proteus mirabilis. The postoperative conditions were good. But two weeks later her head started to enlarge with the bulging fontanelle. CT scan revealed marked dilation of the ventricles and enlargement of the left frontal abscess. At 2 months of age, second operation was done. Left frontal abscess was punctured and serous pus was aspirated. Proteus mirabilis was cultured again from the pus. Tobramycin was administered into the cavity via the drainage tube every day for a week. As the content of the abscess cavity became clear, V-P shunt and cyst-peritoneal shunt were performed. After shunting procedure, the increase of head circumference stopped and the fontanelle became flat and soft.(ABSTRACT TRUNCATED AT 250 WORDS)