Inclusion Body Myositis: A case report

In 1971 inclusion body myositis was reported by Yunis and Samaha. This disease is similar with chronic multiple myositis clinically. Pathologically, inclusion body myositis is characterized by intracytoplasmic vacuole with degenerating fibers and accompanied with inclusion body in internal nucleus and cytoplasm. Since then 240 cases of inclusion body myositis have been reported in the world including 3 cases in Korea. A 27 years-old lady had inclusion body myositis, which show slowly progressive muscular weakness. We confirmed this with clinical symptom, muscle biopsy, and electrophysiologic study. We report the typical manifestation of inclusion body myositis in a 27 years-old lady with the brief review of literature.

Gillespie Syndrome with Partial Aniridia, Cerebellar Ataxia, Delayed Development: A case report

In 1965 Gillespie reported a new syndrome of bilateral aniridia, cerebellar ataxia, and oligophrenia (mental retardation). This new syndrome was named Gillespie syndrome. Since then only 17 cases of Gillespie syndrome have been reported in UK, Brazil, Ireland, Belgium, Australia, and US. A case of Gillespie syndrome was not reported in Korea. A 4 year-old girl has triad of Gillespie syndrome, which are partial aniridia, cerebellar ataxia and mental retardation. We confirmed this with ophthalmologic examination, brain MRI, and developmental delay. We report the typical manifestation of Gillespie syndrome in a 4 year-old girl with the brief review of literature.

Cervical Radiographic Study in Adolescence Cerebral Palsy

OBJECTIVE: To study the incidence and degree of the cervical instability in the cerebral palsied patients and to investigate the cause of the high incidence of cervical myelopathy in these patients. METHOD: The static and dynamic radiography of the cervical spine in the sagittal plane was performed in seventy-two patients with athetoid and spastic cerebral palsy and the incidence of spondylolisthesis, range of motion(by the Penning Method), sagittal diameter of the cervical canal, and posture of the cervical spine were evaluated. RESULTS: The incidence of the cervical spondylolisthesis was fifty percent with athetoid cerebral palsy and twenty-seven percents with spastic cerebral palsy. The incidence of spondylolisthesis was especially high at the level of C3/4 and C4/5. The excessive range of motion in flexion/extension by the sagittal plane was observed in 66.7% of athetoid patients and 53.3% of spastic patients, especially at the C2/3 and C3/4 levels. The abnormal curvature was noted in 66.6% of athetoid and spastic patients. C-curve and S-curve were more common in athetoid patients and straightening of the C-spine was more common in spastic type. A sagittal diameter of the cervical spinal canal was significantly decreased in patients with athetoid patients with C3/4 spondylolisthesis and/or abnormal curvature such as a C-curve or S-curve(P<0.05). Height of the vertebral body was decreased in both athetoid and spastic patients. CONCLUSION: The combination of a cervical instability and a narrow spinal canal predisposes the neurological progression to a cervical myelopathy in cerebral palsied patients.

Preoperative Tract Dilatation for Percutaneous Nephrolithotomy

PURPOSE: The purpose of this study was to understand the principle of percutaneous nephrolithotomy(PNL), toincrease its success rate, and to base the radiologic approach on preoperative interventional tract dilatation. MATERIALS AND METHODS: Twenty-six patients(male, 19, female, 7) with staghorn or pelviocalyceal calculi who hadundergone PNL were included in this study. After percutaneous nephrostomy(PCN) was performed in each patient, thetract was dilated to 30F with Amplatz dilators using coaxial techniques or a 30F, 10cm high-pressure tractdilatation balloon. One day before the calculi were fragmented and removed by PNL, a 30F Amplatz sheath wasinserted under local anesthesia. The time required for tract dilatation, complications, and surgery was analyzed. RESULTS: Percutaneous tract dilatation were successful in all patients, and forceps, a nephrolithotome and anephroscope were successfully introduced into the pelvocalyceal system. The average procedure time was 19.5minutes ; a double J stent was replaced in the pelvocalyceal system of 13 of 26 patients (50%) ; because ofimproper catheter direction or/and bleeding, the Amplatz sheath was replaced in three. After PNL, complicationsinvolving renal hematoma occurred in one patient. This did not affect the patient's prognosis, however. CONCLUSION:Owing to its short procedure time and low complication rate, interventional preoperative tract dilatation forPNL is thought to be an effective technique for patients with staghorn or pelvocalceal calculi.