Arthrosonography of the Shoulder Joint for Evaluation of the Glenoid Labrum, Glenoid Rim and Humeral Head: Comparison with Conventional Ultrasonography and MR Arthrography

PURPOSE: To compare the diagnostic role of arthrosonography, conventional ultrasonography and MR arthrography in the assessment of glenoid labral tear, glenoid rim fracture and humeral head fracture of the shoulder joint. MATERIALS AND METHODS: The findings of arthrosonography, conventional ultrasonography and MR arthrography were prospectively evaluated in 62 consecutive patients with chronic pain or a history of recurrent dislocation of the shoulder joint. The glenoid labrum was arbitrarily divided into four quadrants: anterosuperior, anteroinferior, posterosuperior, and posteroinferior, and for each, visibility at arthrosonography and conventional ultrasonography was subjectively scored as one of four grades. By means of statistical analysis, the two techniques were then compared. Twenty-six patients subsequently underwent arthroscopy, and the presence or absence of labral tear, glenoid rim fracture and humeral head fracture was determined. The sensitivity and specificity of each modality were separately calculated for each of the three types of shoulder joint injury, and observed differences in these findings were statistically analysed. RESULTS: For all individual quadrants of the labrum, visibility at arthrosonography was higher than at conventional ultrasonography (p.05), though its specificity was significantly higher (p=.003). In this respect, there was no significant difference in sensitivity or specificity between arthrosonography and MR arthrography (p>.05). For the detection of glenoid rim and humeral head fracture, there were no statistical differences in sensitivity and specificity between the three imaging modalities (p> .05). CONCLUSION: Compared with conventional ultrasonography, arthrosonography provides higher visibility of the labrum, thus improving the capacity of ultrasonography to detect labral tear. Arthrosonography could therefore be useful in the diagnosis of labral tear, glenoid rim fracture and humeral head fracture, and may thus partially replace MR arthrography.

Serial Magnetic Resonance Imaging Evaluation of Bioabsorbable Interference Screws in Anterior Cruciate Ligament Reconstruction / 대한정형외과학회잡지

PURPOSE: To evaluate the changes at the postoperative site of anterior cruciate ligament (ACL) reconstruction, in which bioabsorbable screws were used for fixation, by magnetic resonance imaging (MRI) and to evaluate the efficacy and safety of Bioscrews(R) (Linvatec, Largo, FL). MATERIALS AND METHODS: This prospective study was initiated in May 1997 upon 38 patients receiving Bioscrews(R). All patients with arthroscopically assisted primary reconstruction of ACL involving fixation with Bioscrews were examined 3, 6, 12 and 24 months after operation by Lysholm score, KT-2000 arthrometer and MRI. MR Images were interpreted by musculoskeletal radiologists. RESULTS: There were no complications related to loss of fixation. The Lysholm scores and the KT- 2000 arthrometer measurement at final follow-up were 95.6 and 1.8 mm respectively. Even after two years there was evidence of screw material in all cases. MRIs taken at 3 months showed fluid collections inside the tibial tunnel (n=3) and the anterior aspect of the tibial tunnel (n=1), Though these fluid collections disappeared gradually. Varying amounts of edma were observed around the tunnels. We divided the bone edema into the ring (n=5) and diffuse types (n=16), which disappeared gradually. CONCLUSION: The Bioscrews(R) was found to be a safe and effective alternative for fixation in ACL reconstruction, but requires the long term follow-up to confirm its safety.

Hemorrhagic Shock and Encephalopathy Syndrome: A Case Report

Hemorrhagic shock and encephalopathy syndrome (HSES) is a sudden-onset symptom complex that in-volves multisystem failure and includes encephalopathy, shock, coma, convulsions, prerenal azotemia, hepatic dysfunction, and bleeding coagulopathy and progressive thrombocytopenia in previously healthy infants and children. Its radiologic findings have rarely been reported, and it has not been described in Korea. We present a case of clinically diagnosed HSES, and include the CT and MRI findings.

Adhesive Capsulitis of the Shoulder: MR Arthrography

PURPOSE: Adhesive capsulitis is a clinical syndrome involving pain and decreased joint motion caused by thickening and contraction of the joint capsule. The purpose of this study is to describe the MR arthrographic findings of this syndrome. MATERIALS AND METHODS: Twenty-nine sets of MR arthrographic images were included in the study. Fourteen patients had adhesive capsulitis diagnosed by physical examination and arthrography, and their MR arthrographic findings were compared with those of 15 subjects in the control group. The images were retrospectively reviewed with specific attention to the thickness of the joint capsule, volume of the axillary pouch (length, width, height(depth)), thickness of the coracohumeral ligament, presence of extra-articular contrast extravasation, and contrast filling of the subcoracoid bursa. RESULTS: Mean capsular thickness measured at the inferior portion of the axillary pouch was 4.1 mm in patients with adhesive capsulitis and 1.5 mm in the control group. The mean width of the axillary pouch was 2.5 mm in patients and 9.5 mm in controls. In patients, the capsule was significantly thicker and the axillary pouch significantly narrower than in controls (p<0.05). Capsule thickness greater than 2.5 mm at the inferior portion of the axillary pouch (sensitivity 93%, specificity 80%) and a pouch narrower than 3.5 mm (sensitivity 93%, specificity 100%) were useful criteria for the diagnosis of adhesive capsulitis. In patients with this condition, extra-articular contrast extravasation was noted in six patients (43%) and contrast filling of the subcoracoid bursa in three (21%). CONCLUSION: The MR arthrographic findings of adhesive capsulitis are capsular thickening, a low-volume axillary pouch, extra-articular contrast extravasation, and contrast filling of the subcoracoid bursa. Capsule thickness greater than 2.5 mm at the inferior portion of the axillary pouch and a pouch width of less than 3.5 mm are useful diagnostic imaging characteristics.

A Case of Megacystis Microcolon Intestinal Hypoperistalsis Syndrome

Megacystis Microcolon Intestinal Hypoperistalsis(MMIH) Syndrome is a rare cause of functional neonatal bowel obstruction, characterized by hypoperistalsis, narrow distal ileum and colon, and bladder distension. We report a case of MMIH syndrome and review the literature. The patient was born after 34 weeks of gestation with marked abdominal distension. Her birth weight was 2,830g with 276ml of urine drained by catheter. Prenatal ultrasonic findings were bladder distension, hydronephrosis and possible intraabdominal mass. Supine view of abdomen on third day of life showed dilated loops of proximal small bowel and stomach without colonic gas shadow. Preoperative diagnosis was intestinal obstruction. When the abdomen was opened, the distal ileum was filled with meconium and postoperative diagnosis was meconium plug syndrome. Ganglion cells were present in the ileal biopsy. All postoperative attempts to feed her resulted in bilous vomiting. Voiding cystourethrography done on the 4th day after colon study showed markedly distended bladder, contrast enhanced microcolon and hypoperistalsis. She was dischaged against medical advice by her parents at the age of 23 days and died at home at the age of 33 days.

Unilateral and Bilateral Lumbosacral Transitional Vertebrae: Comparison of the Incidence of Combined Diseases

PURPOSE: The purpose of this study was to evaluate the radiologic findings of lumbosacral transitional verte-brae (LSTV), as seen on plain radiographs and MRI, and to compare the incidence of combined diseases be-tween unilateral and bilateral groups. MATERIALS AND METHODS:We retrospectively evaluated the plain radiographs and MR images of 63 patients with LSTV, classifying its type according to Castellvi 's criteria, and evaluated disc herniations, facet joint os-teoarthritic change, scoliosis, and spondylolisthesis. We then compared the incidence of each combined diseases between unilateral and bilateral groups, and as a control group, 63 patients without LSTV were also eval-uated. RESULTS: Forty of 63 cases of LSTV (63.5%) were bilateral, and 23 (36.5%) unilateral. According to Castellvi 's criteria, the incidence of type I was 41.3% (unilateral 7 cases, bilateral 19), type III30.2% (unilateral 8 cases, bilateral 11), type II23.8% (unilateral 8 cases, bilateral 7), and type IV 4.8% (unilateral 0 cases, bilateral 3).With combined diseases, the incidence of disc herniation was 66.7% (unilateral 15 cases, bilateral 27), moderate to severe facet joint osteoarthritic change 28.6% (unilateral 11 cases, bilateral 7), scoliosis 27.0% (unilateral 9 cases, bilateral 8), and spondylolisthesis 9.5% (unilateral 2 cases, bilateral 4). The incidence of facet joint os-teoarthritic change was significantly higher in the unilateral group (47.8%) than in the bilateral group (17.5%) (p=0.02), and scoliosis occurred at a higher rate in the unilateral group (39.1%) than in the bilateral group (20.0%) (p=0.18). There was, however, no significant difference in the incidence of disc herniation and spondylolisthesis between the two groups (unilateral group: 65.2% and 8.7%; bilateral group: 67.5% and 10.0%, respectively). CONCLUSION: With LSTV, the incidence of facet joint osteoarthritic change was significantly higher in the unilat-eral group than in the bilateral group, and scoliosis also showed a higher rate of occurrence in the unilateral group.

Infantile Hypertrophic Pyloric Stenosis Treated with Intravenous Atropine Sulfate

PURPOSE: The pharmacologic effect of atropine on HPS can be considered to control pyloric muscle spasm. Therefore, we studied the effects of intravenous atropine sulfate on the clinical course of HPS, and periodically observed the ultrasonographic appearance of the pyloric muscles after atropine treatment. METHODS:From April 1998 to May 1999, 14 infants who were diagnosed with HPS were treated with intravenous atropine sulfate. Intravenous atropine sulfate was administered at an initial dose of 0.04mg/kg/day, which was divided into 8 equal doses. The daily dose was increased by 0.01 mg/kg/day until vomiting was controlled for an entire day while infants received unrestricted oral feeding. Ultrasonographic examinations were performed during hospitalization and repeated at least every 2 months until normalization of pyloric muscles was confirmed. RESULTS: Intravenous atropine was effective in 12 of 14 infants with HPS and the conditions of 9 of them improved. Two infants who were not free from vomiting despite a week of intravenous atropine sulfate treatment underwent pyloromyotomy. A series of ultrasonographic examinations were done after vomiting had improved with intravenous atropine sulfate. The ultrasonographic findings showed good passage of gastric contents through pyloric canals despite thickening of the pyloric muscles. CONCLUSION: Intravenous administration of atropine sulfate is an effective therapy for HPS and can be an alternative to pyloromyotomy. (J Korean Pediatr Soc 2000;43:763-768)

Disseminated BCG Infection in a patient with Severe Combined Immunodeficiency

Disseminated mycobacterial infection after bacillus Calmette-Guerin (BCG)vaccination is a very rare disorder, occurring mostly in patients with immunologic deficiency. We report a case of disseminated BCG infection in a 16-month-old girl with severe combined immunodeficiency. Plain radiographs showed multiple osteolytic lesions in the femora, tibiae, humerus, and phalanges. Abdominal sonography and CT scanning revealed multiple nodules in the spleen, and porto-caval lymphadenopathy.

Disseminated BCG Infection in a patient with Severe Combined Immunodeficiency

Disseminated mycobacterial infection after bacillus Calmette-Guerin (BCG)vaccination is a very rare disorder, occurring mostly in patients with immunologic deficiency. We report a case of disseminated BCG infection in a 16-month-old girl with severe combined immunodeficiency. Plain radiographs showed multiple osteolytic lesions in the femora, tibiae, humerus, and phalanges. Abdominal sonography and CT scanning revealed multiple nodules in the spleen, and porto-caval lymphadenopathy.

Pubic Insufficiency Fracture: MRI Findings

PURPOSE: To evaluate the characteristic MRI findings of pubic insufficiency fracture. MATERIALS AND METHODS: In nine cases of pubic insufficiency fracture, the findings of plain radiography (n=9), MRI (n=9), and bone scintigraphy (n=8) were reviewed. We retrospectively analyzed, with regard to fracture site, the destructive pattern revealed by plain radiography, and uptake by other pelvic bones, as demonstrated by RI bone scanning. The MR findings evaluated were the fracture gap and its signal intensity, the site and signal intensity of the soft tissue mass, and other pelvic bone fractures. RESULTS: Plain radiography revealed osteolysis and sclerosis of pubic bone in eight of nine cases (89%), and parasymphyseal fractures in seven (78%). RI indicated uptake by the sacrum in six cases (66%), and by the ilium in three (33%). MR findings of fracture gap (seven cases, 78%) were hypo to isointensity on T1WI, hyper-intensity on T2WI and the absence of contrast enhancement. Soft tissue masses were found in seven cases (78%); in four of these the location was parasymphyseal, and in three, surrounding muscle was involved. Hypo to isointensity was revealed by T1WI, hyperintensity by T2WI, and there was peripheral enhancement. Other associated pelvic bone fractures involved the sacrum in seven cases and the ilium in four. CONCLUSION: The characteristic MR findings of pubic insufficiency fracture were parasymphyseal location, fracture gap, peripherally enhanced soft tissue mass formation, and fractures of other pelvic bones, namely the sacrum and ilium.

MRI Diagnosis of Associated Injuries in Complete Tears of Posterior Cruciate Ligament of the Knee / 대한정형외과학회잡지

Detection of tears of the posterior cruciate ligament (PCL) with MRI is usually based on the appearance of the ligament. However, other MRI findings may be useful in establishing the diagnosis. We assessed these associated injuries in the cases of complete tears of the PCL, reviewing MRls. We evaluated 26 patients who received PCL reconstruction with complete tear of posterior cruciate ligament from Feb 1995 to May 1997 retrospectively. We studied associated findings with PCL injuries. Anterior tibial plateau lesions were found in 15 of 26 cases (57.7%). Meniscal injuries were found in 8 of 26 cases (30.8%). Popliteus muscular injuries was found in 7 of 26 cases (26.9%). Capsular injuries were found in 2 of 26 cases (7.7%). Retinacular injury which has never been described in PCL injuries, was found in 7 cases (26.9%, P>0.05), Other ligamentous injuries were associated in 22 of 26 cases (84.6%). Medial collateral ligament injuries were found in 16 of 22 (61.5%, P<0.05) cases. The frequency of associated ligamentous injury and meniscal tear correlated well with results reported by other authors. The presence and location of bone contusions and fractures correlated with the mechanisms of injury; the anterior tibial plateau was the most commonly affected site. We concluded that the associated injuries were very helpful in diagnosing PCL injuries with MRI.

Magnetic Resonance Imaging for the Evaluation of Acute Posterolateral Complex Injuries of the Knee

The purpose of this study is to determine whether magnetic resonance imaging(MRI) can assist in deci- sion-making in the treatment of acute posterolateral complex injuries. We reviewed retrospectively seven patients with acute posterolateral complex injury, among the 79 cases that had taken cruciate ligament recon- struction. MRI findings were then correlated with the results of examination under anesthesia or operi lateral reconstruction. Based on surgical and clinical findings, there were six complete and four partial posterolateral complex injuries. MRI was able to accurately describe the extent of injury in each case. All ten patients had concomitant anterior cruciate ligament tears, and seven were three posterior cruciate ligament injuries. The iliotibial band was avulsed from Gerdys tubercle in seven patients. In knees with complete tears, separation of the coronary ligament of the lateral meniscus was noted on MRI. A characteristic bone contusion on the anteromedial femoral condyle was present in nine patients. Popliteal tendon tearing was observed in eight cases, and biceps femoris was torn in six patients. In knee with complete tears, tears of the lateral collateral ligament complex was noted both on MRI and intraoperatively. A high-quality MRI in patients sustaining these injuries will assist the surgeon in directly applying surgical techniques in a timely fashion.

Prediction of Parent Artery of Anterior Communicating Artery Aneurysm on CT Angiography

PURPOSE: To determine whether CT angiography can predict the parent artery of an anterior communicating aneurysm on the basis of characteristics of the aneurysm and precommunicating anterior cerebral artery. MATERIALS AND METHODS: Eighteen patients with anterior communicating aneurysm were studied prospectively using CT angiography and conventional angiography. The parent artery of an aneurysm was predicted by evaluating aneurysm neck location, direction of aneurysm projection, and size of the precommunicating anterior cerebral artery, as seen on CT angiography. A somatom Plus-4 spiral CT scanner was used and shaded-surface display three-dimensional images were constructed. RESULTS: Aneurysm neck was located near the parent artery in 12 cases(66.7%), and aneurysm projection was against the parent artery in 11 cases(61.1 %). The parent artery of the anterior cerebral artery was larger in 15 cases (8 3 .3 %), including ten cases of hypoplasia or agenesis of the contralateral anterior cerebral artery. In 17 of 18 aneurysms(94.4%) the parent artery seen on DSA was correctly predicted by CT angiography. CONCLUSION: Teh parent artery of an anterior communicating aneurysm can be predicted by evaluating a-neurysm neck location, direction of aneurysm projection, and precommunicating anterior cerebral artery, as seen on CT angiography.

Kaposiform Hemangioendothelioma Complicated by Kasabach - Merritt Phenomenon with Bone Involvement in an Adult / 대한암학회지

Kasabach-Merritt phenomenon does not occur with common hemangioma, rather it is associated with the more aggressive Kaposiform hemangioendothelioma and rarely with other vascular neoplasm. We report the case of an adult who was diagnosed as Kaposiform hemangioendothelioma complicated by Kasabach-Memtt phenomenon. This is the first report in Korea of an adult with Kasabach-Merritt phenomenon who has osteolytic changes of femur, pelvic bone, and lumbar spine.

Imaging Features of Rhabdoid Tumor of the Kidney in Children

PURPOSE: To evaluate the difference in radiologic features of rhabdoid tumor of the kidney (RTK) in children according to the location of the tumor within the kidney. MATERIALS AND METHODS: We retrospectively reviewed the radiologic findings of pathologically confirmed RTK in seven children (5 boys and 2 girls; age range, 6 months to 4 years 8 months; median, 18 months). All subjects underwent abdominal CT. We analyzed tumor location, size, and margin; renal hilar involvement, subcapsular hematoma, calcification, necrosis, and lymphadenopathy. RTK was classified according to the location of the tumor within the kidney: A tumor that mainly located in the central portion of the kidney with or without peripheral extension was described as type I, while one located at the periphery was type II. Imaging findings between the two types were compared. RESULTS: Tumor size varied from 3 cm to 12 cm. Tumor outlines were ill-defined in four cases but relatively well-defined in three. Four tumors (57 %) were type I. Hilar involvement was found in all four and a small subcapsular hematoma in one. Three tumors (43%) were type II, and in all three, large crescent-shaped subcapsular hematomas were found. CONCLUSION: Centrally located RTK showed hilar involvement with a small subcapsular hematoma, while in cases of peripherally located RTK, a large subcapsular hematoma was present. These findings may be helpful for the differential diagnosis of other pediatric renal tumors.

Imaging Features of Rhabdoid Tumor of the Kidney in Children

PURPOSE: To evaluate the difference in radiologic features of rhabdoid tumor of the kidney (RTK) in children according to the location of the tumor within the kidney. MATERIALS AND METHODS: We retrospectively reviewed the radiologic findings of pathologically confirmed RTK in seven children (5 boys and 2 girls; age range, 6 months to 4 years 8 months; median, 18 months). All subjects underwent abdominal CT. We analyzed tumor location, size, and margin; renal hilar involvement, subcapsular hematoma, calcification, necrosis, and lymphadenopathy. RTK was classified according to the location of the tumor within the kidney: A tumor that mainly located in the central portion of the kidney with or without peripheral extension was described as type I, while one located at the periphery was type II. Imaging findings between the two types were compared. RESULTS: Tumor size varied from 3 cm to 12 cm. Tumor outlines were ill-defined in four cases but relatively well-defined in three. Four tumors (57 %) were type I. Hilar involvement was found in all four and a small subcapsular hematoma in one. Three tumors (43%) were type II, and in all three, large crescent-shaped subcapsular hematomas were found. CONCLUSION: Centrally located RTK showed hilar involvement with a small subcapsular hematoma, while in cases of peripherally located RTK, a large subcapsular hematoma was present. These findings may be helpful for the differential diagnosis of other pediatric renal tumors.

A Case of Congenital Hypopituitarism with Anterior Pituitary Hypoplasia and Ectopic Posterior Pituitary Gland

The pituitary gland develops from two different parts of the brain. The anterior pituitary gland originates from the Rathke pouch and the posterior one from the infundibulum. Therefore, the pathologic findings of congenital hypopituitarism can be different in each case. Congenital hypopituitarism is a rare disorder. The characteristic clinical features of the affected newborns are prolonged jaundice, persistent or recurrent hypoglycemia without hyperinsulinism and microphallus. Their genitalia are usually underdeveloped and sexual maturation may be delayed or absent. In adulthood, patients retain childish feature, short stature with normal body proportion. We experienced a case of congenital hypopituitarism in a 12-year-old female patient with short stature and delayed sexual maturation(Tanner stageI). The endocrinological studies revealed growth hormone, FSH, LH and TSH deficiencies. Magnetic resonance imaging indicated a hypoplastic anterior pituitary and an ectopic posterior pituitary gland located within the tuber cinereum of the hypothalamus.

A Case of Congenital Hypopituitarism with Anterior Pituitary Hypoplasia and Ectopic Posterior Pituitary Gland

The pituitary gland develops from two different parts of the brain. The anterior pituitary gland originates from the Rathke pouch and the posterior one from the infundibulum. Therefore, the pathologic findings of congenital hypopituitarism can be different in each case. Congenital hypopituitarism is a rare disorder. The characteristic clinical features of the affected newborns are prolonged jaundice, persistent or recurrent hypoglycemia without hyperinsulinism and microphallus. Their genitalia are usually underdeveloped and sexual maturation may be delayed or absent. In adulthood, patients retain childish feature, short stature with normal body proportion. We experienced a case of congenital hypopituitarism in a 12-year-old female patient with short stature and delayed sexual maturation(Tanner stageI). The endocrinological studies revealed growth hormone, FSH, LH and TSH deficiencies. Magnetic resonance imaging indicated a hypoplastic anterior pituitary and an ectopic posterior pituitary gland located within the tuber cinereum of the hypothalamus.

Usefulness of Turbo Spin-Echo MR Imaging in Meniscal Tears of the Knee

PURPOSE: To evaluate the usefulness and diagnostic accuracy of turbo spin-echo(TSE) proton-density andT2-weighted images of meniscal tears of the knee. MATERIALS AND METHODS: We retrospectively evaluated thesensitivity, specificity, and accuracy of TSE proton density and T2-weighted images of meniscal tears confirmedarthroscopically or surgically in 47 patients(98 menisci). The routine TSE parameters used in all patients werethe dual echo sequence with sagittal proton density and T2-weighed images(4000/16, 90/5/2 [TR/effectiveTE/ETL/NEX]), and fat-suppressed coronal proton density and T2-weighted images. The chi-square test was used forstatistical analysis. RESULTS: The sensitivity, specificity, and accuracy of TSE proton density images for thedetection of meniscal tears were 93.9%, 93.8%, and 93.9%, respectively, in the medial meniscus, and 92.9%, 91.4%,and 91.8% in the lateral. On T2-weighted images the corresponding figures were 87.9%, 93.8%, and 89.8%,respectively, in the medial meniscus, and 64.3%, 91.4%, and 83.7 % in the lateral. CONCLUSION: With regard tosensitivity and accuracy, TSE proton density images of meniscal tears were superior to TSE T2-weighted images.

Hypothalamic Hamartoma : Clinical and MRI Features and Outcome / 대한소아신경학회지

PURPOSE: Hypothalamic hamartomas are non-neoplastic malformations which are composed of hyperplastic neuronal tissue of varying size. Clinically, they are characterized by gelastic seizure, precocious puberty, and behavioral and psychiatric disorders. This study was performed to examine the various features of hypothalamic hamartomas such as neurologic manifestations, other clinical manifestations, and EEG and brain MRI findings. Response to AEDs or outcome of operation on hypothalamic hamartoma was evaluated. METHODS: Eleven patients who were admitted to Seoul National University Children's Hospital from July 1986 to January 1997 and diagnosed as hypothalamic hamartoma by brain MRI or CT were enrolled in this study. Clinical manifestations and EEG were reviewed retrospectively through the medical records. The size and type of the hypothalamic hamartoma on brain MRl were analyzed. RESULTS: 1) The range of age at visit was 1yr 9mo to 17yr 2mo (mean 10yr 8mo), and the age at onset was 1yr 1mo to 14yr to 2mo (mean 5yr 8mo). Six patients were male and five were female. 2) The clinical manifestations included gelastic seizure (n=9), behavioral and psychiatric disorders (n=9), other types of seizure (n=8), and precocious puberty (n=6). Other types of seizure were complex partial seizure (n=3), generalized tonic seizure(n=3), and infantile spasm (n=2). 3) The interictal EEG findings included focal spike discharges (n=8), diffuse delta slowings (n=1), hypsarrhythmia (n=1), and normal record (n=1). The focal spike discharges originated from the temporal (n=2), fronto-temporal (n=1), occipital (n=3), or frontal area(n=2). 4) The brain MRI showed that the size of the hypothalamic hamartomas was 2.47+/-1.12cm, and the origin of the tumors was tuber cinereum (n=6) or tuber cinereum and mamillary body (n=5). All lesions were isointense on T1-weighted image relative to normal gray matter, with a sessile attachment to the hypothalamus, and were not enhanced by Gadolinium. 5) Operations were done in 5 cases, those were subtotal removal of the tumor (n=4), and gamma knife radiosurgery (n=1). Seizures were not completely controlled in all but one case on which gamma knife radiosurgery was done. CONCLUSIONS: The hypothalamic hamartomas presented variable clinical pictures including gelastic seizure, precocious puberty, and behavioral and psychiatric disorders. The gelastic and other types of seizure associated with hypothalamic hamartomas were refractory to medication and might be controlled by total removal of the tumor.