• Annals of Clinical Neurophysiology
• /
• v.16 no.1
• /
• pp.39-41
• /
• 2014

Hemiplegia cruciata (HC) manifests as paralysis of the ipsilateral arm and contralateral leg. Herein, we report a 64-year-old man with weakness of the right leg and of the left arm after multiple sclerosis (MS). His brain and spine magnetic resonance imaging show a lower medulla lesion, which is extended to posterior part of C1 spine through cervicomedullary junction. HC usually results from stroke or trauma, but it is rare as presenting symptom of MS.

• Journal of Korean Neurosurgical Society
• /
• v.51 no.3
• /
• pp.177-181
• /
• 2012

We present a case of an athetoid cerebral palsy with quadriparesis caused by kyphotic deformity of the cervical spine, severe spinal stenosis at the cervicomedullary junction, and atlantoaxial instability. The patient improved after the first surgery, which included a C1 total laminectomy and C-arm guided righ side unilateral C1-2 transarticular screw fixation. C1-2 fixation was not performed on the other side because of an aberrant and dominant vertebral artery (VA). Eight months after the first operation, the patient required revision surgery for persistent neck pain and screw malposition. We used intraoperative VA angiography with simultaneous fluoroscopy for precise image guidance during bilateral C1-2 transarticular screw fixation. Intraoperative VA angiography allowed the accurate insertion of screws, and can therefore be used to avoid VA injury during C1-2 transarticular screw fixation in comorbid patients with atlantoaxial deformities.

• Clinical and Experimental Pediatrics
• /
• v.59 no.sup1
• /
• pp.149-151
• /
• 2016

Chiari malformations are a congenital anomaly of the hindbrain. The most common, Chiari malformation type I (CM-I), is characterized by herniation of the cerebellar tonsils extending at least 3 mm below the plane of the foramen magnum. Consequently, CM-I is associated with hydrocephalus and symptoms involving compression of the cervicomedullary junction by ectopic tonsils. Several studies have reported the clinical symptoms associated with CM-I, including suboccipital headache, weakness in the upper extremities, facial numbness, loss of temperature sensation, ataxia, diplopia, dysarthria, dysphagia, vomiting, vertigo, nystagmus, and tinnitus. Syncope is one of the rarest presentations in patients with CM-I. There are many hypotheses regarding the causes of syncope in patients with CM-I; however, the mechanisms are not clearly understood. Although surgical decompression for CM-I in patients with syncope has yielded good clinical results in some studies, such cases are rarely reported. We report a case of orthostatic syncope in a patient with CM-I who was treated with surgical intervention.

• Journal of Korean Neurosurgical Society
• /
• v.54 no.5
• /
• pp.373-378
• /
• 2013

Objective : We present our experience of conventional posterior approach without fat lateral approach for ventral foramen magnum (FM) meningioma (FM meningioma) and tried to evaluate the approach is applicable to ventral FM meningioma. Methods : From January 1999 to March 2011, 11 patients with a ventral FM meningioma underwent a conventional posterior approach without further extension of lateral bony window. The tumor was removed through a working space between the dura and arachnoid membrane at the cervicomedullary junction with minimal retraction of medulla, spinal cord or cerebellum. Care should be taken not to violate arachnoid membrane. Results : Preoperatively, six patients were of Nurick grade 1, three were of grade 2, and two were of grade 3. Median follow-up period was 55 months (range, 20-163 months). The extent of resection was Simpson grade I in one case and Simpson grade II in remaining 10 cases. Clinical symptoms improved in eight patients and stable in three patients. There were no recurrences during the follow-up period. Postoperative morbidities included one pseudomeningocele and one transient dysphagia with dysarthria. Conclusion : Ventral FM meningiomas can be removed gross totally using a posterior approach without fat lateral approach. The arachnoid membrane can then be exploited as an anatomical barrier. However, this approach should be taken with a thorough understanding of its anatomical limitation.

• Pediatric Infection and Vaccine
• /
• v.10 no.1
• /
• pp.114-122
• /
• 2003

Acute transverse myelitis is a focal inflammatory disorder of the spinal cord, resulting in motor, sensory, and autonomic dysfunction which would be the initial manifestation of acute disseminated encephalomyelitis. A 7-year-old boy developed weakness of lower extremities associated with dizziness and urinary dysfunction after upper respiratory infection. He showed gradual decreased mental status with respiratory difficulty. Spinal MRI showed diffuse spinal cord swelling from C3 to C7 levels with high signal intensity lesion on T2-weighted image, which is involved central gray matter of spinal cord from C3 to C7, proximal portion to cervicomedullary junction, and distal portion to the cornus medullaris. After the supplement of high dose intravenous methylprednisolone therapy with ventilator care, he showed gradual improvement. The brain MRI after extubation showed multifocal high signal intensity lesions in bilateral cerebral white matter on axial T2-weighted image. Immunoserologic test(neutral antibody test) for enterovirus 71 was positive. Dysfunction of the bladder lasted for 33 days. We report a case of transverse myelitis which progressed to acute disseminated encephalomyelitis by enterovirus 71 in a 7-year-old boy with related literatures.

• Journal of Genetic Medicine
• /
• v.7 no.2
• /
• pp.145-150
• /
• 2010

Acrodysostosis is an extremely rare disorder characterized by short fingers and toes with peripheral dysostosis, nasal hypoplasia, and mental retardation. We report a 16-year-old Korean boy with acrodysostosis who had characteristic clinical features and cervical spine stenosis manifested by neurologic symptoms. On presentation, he complained of difficulty in raising his arms, and suffered from intermittent pain and weakness in both upper extremities. He had short stature and dysmorphic facial features, including a broad, depressed nasal bridge, small, upturned nose, bilateral epicanthal folds, and mild hypertelorism. Moderate mental retardation and sensorineural hearing loss in both ears were also present. Radiological findings included broad, short metacarpals and phalanges with cone-shaped epiphyses, bilateral Madelung deformities, hypertrophied first metatarsals, and thickening of the calvarium. Magnetic resonance imaging findings included stenosis of the cervical spine, platybasia with compression into the cervicomedullary junction, and downward displacement of the cerebellar tonsils. Here, we report a case of acrodysostosis with symptoms and signs of cervical spinal stenosis first in Korea. If it is diagnosed in the early stages, possible life-threatening complications, including spinal canal stenosis, can be managed properly and permanent neurologic sequelae might be avoided. Therefore, it is important to consider acrodysostosis in the differential diagnosis of peripheral dysostosis.