Craniovertebral junction anomalies are a group of diseases characterized by the pathological changes of occipital bone,atlantoaxial bone,cerebellar tonsil,surrounding soft tissue,and nervous system,which are caused by a variety of factors.Chiari malformation is a common type of craniovertebral junction anomalies,the conventional surgical therapy of which is posterior fossa decompression.Currently,scholars represented by Goel have proposed a new theory on the classification,pathogenesis,and treatment of Chiari malformation based on posterior atlantoaxial fixation (Goel technique).This article introduces the progress in Goel technique,aiming to provide reference for the clinical work.
Humans ; Arnold-Chiari Malformation/surgery*

Presyrinx consists of reversible spinal cord swelling without frank cavitation, as observed on T2 weighted magnetic resonance imaging (MRI). The condition may evolve into syringomyelia, but timely surgical interventions have achieved meaningful results. Here, we report the case of a 27-year-old woman who presented with headache, dizziness, and diplopia 2 months after suffering a mild head trauma. On MRI, hydrocephalus, downward herniation of the cerebellar tonsil, and a diffuse high signal change in the cervical spinal cord were detected. After insertion of a ventriculoperitoneal shunt, her neurological symptoms resolved, and she has had no signs of presyrinx recurrence for >4 years.
Adult ; Arnold-Chiari Malformation ; Cervical Cord ; Craniocerebral Trauma ; Diplopia ; Dizziness ; Female ; Headache ; Humans ; Hydrocephalus ; Magnetic Resonance Imaging ; Palatine Tonsil ; Recurrence ; Spinal Cord ; Syringomyelia ; Ventriculoperitoneal Shunt

Arnold-Chiari malformation type 1 is a congenital disease characterized by herniation of the cerebellar tonsils through the foramen magnum. Most common clinical symptom is pain, including occipital headache and neck pain, upper limb pain exacerbated by physical activity or valsalva maneuvers. Various otoneurological manifestations also occur in patients with the disease, which has usually associated with dizziness, vomiting, dysphagia, poor hand coordination, unsteady gait, numbness. Patients with Arnold-Chiari malformation may develop vertigo after spending some time with their head inclined on their trunk. Positional and down-beating nystagmus are common forms of nystagmus in them. We experienced a 12-year-old female who presented complaining of vertigo related to changes in head position which was initially misdiagnosed as a benign paroxysmal positional vertigo.
Arnold-Chiari Malformation ; Benign Paroxysmal Positional Vertigo ; Child ; Deglutition Disorders ; Dizziness ; Female ; Foramen Magnum ; Gait Disorders, Neurologic ; Hand ; Head ; Headache ; Humans ; Hypesthesia ; Motor Activity ; Neck Pain ; Palatine Tonsil ; Upper Extremity ; Valsalva Maneuver ; Vertigo ; Vomiting

A small percentage of individuals have the neurological anomaly of central precocious puberty (CPP). Common neurologic causes of CPP include a tumor or congenital lesions. Although Arnold-Chiari malformation can be caused by congenital or acquired causes, it is unusual in patients with CPP. We present the case of a girl aged 4.5 years who complained of breast budding. Her neurological examination and growth pattern were normal. She had no endocrinological abnormality, except for true precocious puberty. We performed brain magnetic resonance imaging, which showed an Arnold-Chiari type 1 malformation. Currently, this case represents the youngest girl who exhibited both Arnold-Chiari type 1 malformation and precocious puberty. Furthermore, it is likely that there is a meaningful association between the brain lesion and precocious puberty in this case.
Arnold-Chiari Malformation ; Brain ; Breast ; Child ; Female ; Humans ; Magnetic Resonance Imaging ; Neurologic Examination ; Puberty, Precocious

Cases of pediatric eosinophilic meningitis following duraplasty with a bovine graft have been reported. These patients recovered following the surgical removal of the dural graft or steroid therapy. Decompression for Chiari malformation is a common procedure in both pediatric and adult neurosurgery. We describe the case of a 33-yr-old male patient with eosinophilic meningitis following Chiari decompression via bovine graft duraplasty. Cerebrospinal fluid (CSF) study showed 49 red blood cells/μL and 129 leukocytes/μL with 17% eosinophils. There was no evidence of infectious disease. To our knowledge, this is the first report of adult eosinophilic meningitis after bovine graft duraplasty in Korea.
Adult* ; Arnold-Chiari Malformation* ; Cerebrospinal Fluid ; Communicable Diseases ; Decompression ; Eosinophils* ; Humans ; Korea ; Male ; Meningitis* ; Neurosurgery ; Transplants*

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.
Arnold-Chiari Malformation ; Ataxia ; Decompression, Surgical ; Deglutition Disorders ; Diplopia ; Dysarthria ; Foramen Magnum ; Headache ; Humans ; Hydrocephalus ; Hypesthesia ; Hypotension, Orthostatic ; Palatine Tonsil ; Rhombencephalon ; Sensation ; Syncope* ; Tinnitus ; Upper Extremity ; Vertigo ; Vomiting

BACKGROUND: Downbeat nystagmus is the most-common type of central nystagmus. However, no published study has attempted to analyze the etiology and clinical features of downbeat nystagmus in a large sample of Korean patients. METHODS: We analyzed the demographic and clinical features of spontaneous downbeat nystagmus in 218 Korean patients who had been evaluated at the Dizziness Clinic of Seoul National University Bundang Hospital from 2003 to 2014. RESULTS: All patients were aged from 15 to 92 years (mean+/-SD: 58.7+/-18.0 years), and they included 117 (53.7%) men. Underlying causes were found in 162 patients (74.3%, secondary group), while no etiology was identified in the remaining patients (idiopathic group). The age and sex distributions did not differ between the idiopathic and secondary groups. The underlying etiology included hereditary disorders (n=48, 22.0%), vascular diseases (n=46, 21.1%), and inflammatory diseases (n=19, 8.7%). Infarctions (n=34, 15.6%) and episodic ataxia (n=32, 14.7%) were the most-frequent underlying disorders. The most-common site of lesions identified by MRI was the cerebellum (68.9%), followed by the brainstem (28.6%). Among the 74 patients with lesions confirmed by MRI, 25 (33.7%) showed an isolated cerebellar involvement, while 35 (47.6%) had lesions in multiple areas. CONCLUSIONS: This study extends the clinical features of spontaneous downbeat nystagmus to Korean patients. Cerebellum is the most-common lesion site, which is consistent with the results of previous studies. The prevalence of Arnold-Chiari malformation is relatively low, which may be a distinct finding relative to those of previous studies involving other ethnic populations.
Arnold-Chiari Malformation ; Ataxia ; Brain Stem ; Cerebellum ; Dizziness ; Humans ; Infarction ; Magnetic Resonance Imaging ; Male ; Prevalence ; Seoul ; Sex Distribution ; Vascular Diseases ; Vertigo

Adult ; Arnold-Chiari Malformation ; diagnosis ; Elbow Joint ; pathology ; Female ; Humans ; Syringomyelia ; diagnosis

Arnold-Chiari malformation type III (CM III) is an extremely rare anomaly with poor prognosis. An encephalocele with brain anomalies as seen in CM II, and herniation of posterior fossa contents like the cerebellum are found in CM III. The female infant was a twin, born at 33 weeks, weighing 1.7 kg with a huge hydrocele on the craniocervical junction. After operations were performed, she was referred to the department of rehabilitation medicine for poor motor development, swallowing dysfunction, and poor eye fixation at 22 months. The child was managed with neurodevelopmental treatment, oromotor facilitation, and light perception training. After 14 months, improvement of gross motor function was observed, including more stable head control, rolling, and improvement of visual perception. CM III has been known as a condition with poor prognosis. However, with the improvement in operative techniques and intensive rehabilitations, the prognosis is more promising than ever before. Therefore, more attention must be paid to the rehabilitation issues concerning patients with CM III.
Arnold-Chiari Malformation* ; Blindness, Cortical ; Brain ; Cerebellum ; Child ; Deglutition ; Developmental Disabilities ; Encephalocele ; Female ; Head ; Humans ; Infant ; Prognosis ; Rehabilitation ; Twins ; Visual Perception

No abstract available.
Arnold-Chiari Malformation*