• Journal of Korean Neurosurgical Society
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• v.64 no.3
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• pp.386-405
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• 2021

Recent advancements in basic research on the process of secondary neurulation and increased clinical experience with caudal spinal anomalies with associated abnormalities in the surrounding and distal structures shed light on further understanding of the pathoembryogenesis of the lesions and led to the new classification of these dysraphic entities. We summarized the changing concepts of lesions developed from the disordered secondary neurulation shown during the last decade. In addition, we suggested our new pathoembryogenetic explanations for a few entities based on the literature and the data from our previous animal research. Disordered secondary neurulation at each phase of development may cause corresponding lesions, such as failed junction with the primary neural tube (junctional neural tube defect and segmental spinal dysgenesis), dysgenesis or duplication of the caudal cell mass associated with disturbed activity of caudal mesenchymal tissue (caudal agenesis and caudal duplication syndrome), failed ingression of the primitive streak to the caudal cell mass (myelomeningocele), focal limited dorsal neuro-cutaneous nondisjunction (limited dorsal myeloschisis and congenital dermal sinus), neuro-mesenchymal adhesion (lumbosacral lipomatous malformation), and regression failure spectrum of the medullary cord (thickened filum and filar cyst, low-lying conus, retained medullary cord, terminal myelocele and terminal myelocystocele). It seems that almost every anomalous entity of the primary neural tube may occur in the area of secondary neurulation. Furthermore, the close association with the activity of caudal mesenchymal tissue in secondary neurulation involves a wider range of surrounding structures than in primary neurulation. Although the majority of the data are from animals, not from humans and many theories are still conjectural, these changing concepts of normal and disordered secondary neurulation will provoke further advancements in our management strategies as well as in the pathoembryogenetic understanding of anomalous lesions in this area.

• Journal of Veterinary Clinics
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• v.18 no.4
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• pp.315-323
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• 2001

Changes in somatosensory evoked potentials (SEPs) which accompanied by insertion of foreign body in spinal canal were evaluated with clinical signs and positive contrasted myelography in dogs. Foreign bodies occupied 20∼50% of spinal canal. Foreign bodies occupying about 50% of spinal canal were inserted into the animals of group II and III for 1 week and 2 days, respectively. Foreign bodies occupying about 20% of spinal canal were inserted into the animals of group IV, V, and for 1 week, 2 days, and 8 weeks, respectively. in group I (control group), sham operation (lateral laminectomy) was performed. Group III, IV and V did not severely affect on SEPs latencies and clinical signs. Group VI affects on SEPs latencies but not on clinical signs. After foreign body removal, SEPs latencies showed similar recovery patterns with clinical signs. However, group II induced severe abnormalities in SEPs latencies and clinical signs. In group III, IV and V, thoracic potentials (TN1) were abnormally recorded after foreign body insertion and firstly/normally recorded on the 6th 9.5th and 3.5th day after foreign body (removal following) insertion. In group VI, TN1 was abnormally recorded after foreign body insertion and firstly recorded on the 7.7th day and normally recorded on 34th day after foreign body insertion. In group I, TN1 was not recorded from the 3rd day after foreign body insertion and until the 8th week after foreign body removal. In group I, TN1 was firstly recorded on the 1st day after laminectomy.

• Journal of the Korean Child Neurology Society
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• v.26 no.4
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• pp.189-196
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• 2018

Purpose: Floppy infants or congenital hypotonia indicates decreased muscle tone in infants secondary to abnormalities of the central or the peripheral nervous system, or both. Previous literature classified its causes as those attributable to a central vs. peripheral origin; however, recent studies have introduced a newer classification describing a combined origin. We invenstigated floppy infants by applying the new etiological classification and reviewed the most common etiologies based on the age of presentation. We additionally reviewed the clinical characteristics, diagnoses, and the developmental outcomes in these infants. Methods: We retrospectively reviewed the electronic medical charts and recruited 116 infants diagnosed with floppy infant syndrome between January 2005 and December 2016 at Severance Children's Hospital. Among these infants, 66 with a confirmed diagnosis were reviewed for the etiological classification. Information regarding developmental outcomes was obtained via phone interviews with the infants' families. Results: Based on the new etiological classification, among 69 infants with a confirmed diagnosis, in 40 (34.5%) this syndrome was of central origin, in 19 (16.4%) of peripheral origin, and in 10 (8.6%) of combined origin. Prader-Willi syndrome, myotonic dystrophy, and spinal muscular atrophy were the most common disorders observed and combined hypotonia showed the poorest developmental outcome. Conclusion: The study states the importance of proper evaluation of etiological diagnosis and optimal intervention for developmental prognosis. The introduction of a new etiological group of combined hypotonia especially emphasizes regular monitoring and timely rehabilitative intervention in patients for the better quality of life in them as well as their caregivers.

• Journal of Korean Neurosurgical Society
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• v.61 no.2
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• pp.194-200
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• 2018

Objective : Sometimes a vertebral artery (VA) enters the spinal canal via the C1-2 intervertebral space, a variation regarded as a C2 segmental-type VA. This paper describes the anatomy of the C2 segmental-type VA and reviews its clinical importance. Methods : Between March 2014 and November 2015, 3386 patients underwent computed tomographic angiography. I identified C2 segmental-type VAs, associated vascular variation, the origin of ipsilateral posterior inferior cerebellar arteries (PICAs), and the clinical symptoms associated with C2 segmental-type VAs. The origin of an ipsilateral PICA is divided into 5 types. A type 1 PICA originates from ipsilateral VAs coursing suboccipitally (IVASO), a type 2 originates from ipsilateral proximal C2 segmental-type VAs, a type 3 originates from ipsilateral distal C2 segmental-type VAs. For type 4, the PICA does not originate from an ipsilateral VA. For type 5, the PICA is the terminal end of an ipsilateral C2 segmental-type VA. Results : One hundred thirteen patients had 121 C2 segmental-type VAs; 47 were associated with an IVASO, and 74 were not. Four type 1, 13 type 2, 60 type 3, 42 type 4, and two type 5 PICAs were identified. Only one patient showed symptoms associated with a C2 segmental-type VA, being a 71-year-old man presenting with a C2 segmental-type VA infarction. Conclusion : For C2 segmental-type VAs, the ipsilateral IVASO and origin of the PICA are important for predicting the outcome of this type of VA infarction.

• Journal of Chest Surgery
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• v.32 no.5
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• pp.489-493
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• 1999

The pectus carinatum or anterior protrusion of the sternum is a less common than pectus excavatum. It occurs more frequently in boys than girls and associated musculoskeletal abnormalities, spinal scoliosis is most common. Ravitch first reported correction of chondromanubrial prominence in 1952, resecting the multiple deformed costal cartilages and performing a double osteotomy on sternum. We have experienced one case of pectus carinatum and obtained satisfactory postoperative results. The deformity was corrected by the subchondral resection of multiple deformed costal cartilage, bilaterally, with single osteotomy on sternum and fracture of the posterior cortex to correct anterior angulation. Postoperative course was uneventful. We report this case with brief review of the literature.

• Clinical and Experimental Pediatrics
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• v.51 no.4
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• pp.435-438
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• 2008

The Cerebro-oculo-facio-skeletal (COFS) syndrome is a rare autosomal recessive disorder characterized by multiple abnormalities that involve the brain, face, eyes, and extremities. COFS syndrome is regarded as a degenerative disorder of the brain and spinal cord caused by a mutation of the DNA repair genes. We report on an 8-month-old girl with COFS syndrome who exhibited growth and developmental delay, hypotonia, microcephaly, nystagmus, cleft palate, widely separated nipples, inguinal hernia, camptodactyly, and rocker-bottom feet with vertical talus.

• Journal of Korean Neurosurgical Society
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• v.45 no.6
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• pp.386-389
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• 2009

On rare occasions, percutaneous vertebroplasty (PV) may be associated with adverse spinal and extraspinal events. Subarachnoid hemorrhage (SAH) has not been reported complication following a PV. This is a report of two elderly women with spine compressions who developed idiopathic SAH after injecting polymethylmethacrylate into the thoracolumbar region transcutaneously. PV was performed as an usual manner on prone position under local anesthesia for these patients. During the interventions, two patients complained of a bursting nature of headache and their arterial blood pressure was jumped up. Computed tomography scans revealed symmetric SAH on the both hemispheres and moderate degree of hydrocephalus. Any intracranial vascular abnormalities for their SAH were not evident on modern neuroangiography modalities. One patient received a ventricular shunt surgery, but both fully recovered from the procedure-related SAH. The pathophysiologic mechanism that induce SAH will be discussed, with suggesting the manner that prevent and minimize this rare intracranial complication after PV.

• Journal of Korean Neurosurgical Society
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• v.46 no.2
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• pp.172-175
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• 2009

We describe a case of 36-year-old man who presented with a subacute headache preceded by a 1-month history of posterior neck pain without trauma history. Head and neck magnetic resonance imaging (MRI) studies disclosed bilateral supratentorial subdural and retroclival extradural hematomas associated with marked cervical epidural venous engorgement. Cerebral and spinal angiography disclosed no abnormalities except dilated cervical epidural veins. We performed serial follow-up MRI studied to monitor his condition. Patient's symptoms improved gradually. Serial radiologic studies revealed gradual resolution of pathologic findings. A 3-month follow-up MRI study of the brain and cervical spine revealed complete resolution of the retroclival extradural hematoma, disappearance of the cervical epidural venous engorgement, and partial resolution of the bilateral supratentorial subdural hematoma. Complete resolution of the bilateral supratentorial subdural hematoma was confirmed on a 5-month follow-up brain MRI. The diagnosis and possible mechanisms of this rare association are discussed.

• Journal of Veterinary Clinics
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• v.37 no.5
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• pp.273-277
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• 2020

A 12-year-old, female Maltese was referred with uncontrolled hyperadrenocorticism (HAC). Despite well management of HAC through treatment with trilostane and serial monitoring with ACTH stimulation tests for over three years, stiffness of the neck and limbs progressively worsened over time. Neurological test was performed, which showed no abnormalities of cranial nerves. Proprioception was delayed but the cause appeared to be due to stiffness of limb muscles. Muscle tone had increased over time and stiffness had worsened to the extent where it made walking difficult. MRI scans showed no orthopedic or spinal diseases, and pituitary microadenoma was confirmed with pituitary gland measurement of 6 × 6.4 × 4.5 mm (H × W × L). Electromyography presented random discharges with fluctuating amplitude and frequency, which were consistent with myotonic discharges. There were no improvements of myotonic signs despite treatment for HAC with trilostane. Supplementation of L-carnitine and coenzyme Q-10 to mitigate muscle stiffness, following diazepam and methocarbamol to help with muscle rigidity, failed to show any positive effect and the dog died a sudden death, 1,182 days after the initial visit.

• Journal of Korean Neurosurgical Society
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• v.29 no.3
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• pp.411-419
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• 2000

Hematomyelia is an extremely rare condition that cause severe neurological symptoms. We reported 4 cases of spontaneous hematomyelia, two cases of cavernous angioma and two cases of hematoma. In all patients, the clinical course was progressive ; motor and sensory abnormalities below the lesion and voiding difficulties were the common presenting symptom complex. The preoperative diagnosis was made by magnetic resonance imaging and the all patients underwent surgical exploration. Complete removal of hematoma was done in 3 patients and complete evacuation after aspiration of hematoma were performed in one patients. Neurological function did not worsen postoperatively in any patients. The early diagnosis with magnetic resonance imaging and immediate surgical treatment of the hematoma and the associated vascular malformation are considered to be the best way to halt the progression of the disease.