Neuromyelitis optica spectrum disorder (NMOSD) is an inflammatory demyelinating autoimmune disease of central nervous system characterized by relapsing attacks that target the optic nerves and spinal cord, as well as aquaporin-4 (AQP4) enriched periventricular brain regions. The area postrema (AP), located in the dorsal medulla, is the chemosensitive vomiting center and has high AQP-4 expression. The AP syndrome with unexplained hiccups, nausea, and vomiting is one of the core clinical characteristics in the NMOSD and maybe the first presenting symptom. We experienced a 25-year-old woman presented with intractable vomiting, dizziness and oscillopsia. Upbeat nystagmus detected on the bedside examination led to comprehensive neurological workups including magnetic resonance imaging, and she was diagnosed as the AP syndrome. Ten months later, she experienced a recurrence as a longitudinally extensive transverse myelitis and the diagnosis was finally compatible with NMOSD without AQP4-IgG. NMOSD, especially the AP syndrome, should be considered in any dizzy patient with intractable vomiting, and detailed neuro-otologic and neuro-ophthalmologic examinations are warranted for the correct diagnosis.
Adult ; Area Postrema ; Autoimmune Diseases ; Brain ; Central Nervous System ; Diagnosis ; Dizziness ; Female ; Hiccup ; Humans ; Magnetic Resonance Imaging ; Myelitis, Transverse ; Nausea ; Neuromyelitis Optica ; Nystagmus, Pathologic ; Optic Nerve ; Recurrence ; Spinal Cord ; Vomiting

It is difficult to distinguish Hirayama disease (HD) from other mimicking disorders in adolescent patients with distal upper limb weakness. The prevailing theory of HD postulates that the lower cervical cord is susceptible to compression during neck flexion because of insufficient growth of the dura relative to the spinal column. Confirmation of a dynamic change in the dorsal epidural space on magnetic resonance imaging (MRI) during neck flexion is essential for diagnosing HD. However, neck flexion MRI has not been routinely performed in juvenile patients with distal upper limb weakness in the absence of suspected HD. We report two cases of HD that were initially confused with other diseases because of insufficient or absent cervical flexion during MRI. Full-flexion MRI showed typical findings of HD in both cases. Our cases suggest that dynamic cervical MRI in the fully flexed position is necessary for evaluating suspected HD.
Adolescent ; Cervical Cord ; Diagnosis ; Epidural Space ; Humans ; Magnetic Resonance Imaging ; Neck ; Spinal Cord Diseases ; Spine ; Upper Extremity

Cervical kyphosis is a rare condition that can cause significant functional disability and myelopathy. Deciding the appropriate treatment for such deformities is challenging for the surgeon. Patients often present with axial neck pain, and it is not uncommon to find coexisting radiculopathy or myelopathy. The optimal approach for addressing this complex issue remains controversial. A comprehensive surgical plan based on knowledge of the pathology and biomechanics is important for kyphosis correction. Here we reviewed diagnoses of the cervical spine along with the literature pertaining to various approaches and management of cervical spine.
Congenital Abnormalities ; Diagnosis ; Humans ; Kyphosis ; Neck Pain ; Pathology ; Radiculopathy ; Spinal Cord Diseases ; Spine

Dural arteriovenous fistula (D-AVF) at the foramen magnum is an extremely rare disease entity. It produces venous hypertension, and can lead to progressive cervical myelopathy thereafter. On the other hand, the venous hypertension may lead to formation of a venous varix, and it can rarely result in an abrupt onset of subarachnoid hemorrhage (SAH) when the venous varix is ruptured. The diagnosis of D-AVF at the foramen magnum as a cause of SAH may be difficult due to its low incidence. Furthermore, when the D-AVF is fed solely by the ascending pharyngeal artery (APA), it may be missed if the external carotid angiography is not performed. The outcome could be fatal if the fistula is unrecognized. Herein, we report on a rare case of SAH caused by ruptured venous varix due to D-AVF at the foramen magnum fed solely by the APA. A review of relevant literatures is provided, and the treatment modalities and outcomes are also discussed.
Angiography ; Arteries* ; Central Nervous System Vascular Malformations* ; Diagnosis ; Fistula ; Foramen Magnum* ; Hand ; Hypertension ; Incidence ; Rare Diseases ; Spinal Cord Diseases ; Subarachnoid Hemorrhage* ; Varicose Veins*

Guillain-Barré syndrome (GBS) and acute disseminated encephalomyelitis (ADEM) are demyelinating neurologic disorders with different target organs. Although they share similar pathogenetic mechanism, reports of simultaneous occurrence of the 2 disorders are rare. A 2 year 6 month old girl visited our hospital for fever, cough, and general weakness. Although the muscle power of extremities showed mild weakness and voiding difficulty, initial deep tendon reflex of both knees and ankles was normal. A nerve conduction study to evaluate the weakness revealed the absence of F waves. Cerebrospinal fluid analysis demonstrated pleocytosis with lymphocyte predominance and elevated protein levels. Magnetic resonance imaging showed abnormal T2 hyperintensity in pons, medulla and spinal cord. Serum anti-GD1b antibody was positive. Based on clinical findings, laboratory findings, nerve conduction study, and neuroimaging, the diagnosis of GBS and ADEM was made. This is the first case of GBS accompanied by ADEM in Korea.
Ankle ; Cerebrospinal Fluid ; Cough ; Demyelinating Diseases* ; Diagnosis ; Encephalomyelitis ; Encephalomyelitis, Acute Disseminated ; Extremities ; Female ; Fever ; Guillain-Barre Syndrome ; Humans ; Knee ; Korea ; Leukocytosis ; Lymphocytes ; Magnetic Resonance Imaging ; Nervous System Diseases ; Neural Conduction ; Neuroimaging ; Peripheral Nervous System* ; Pons ; Reflex, Stretch ; Spinal Cord

Three male patients diagnosed with surfer's myelopathy (19–30 years) were admitted to our hospital. All three patients were novice surfers showing a typical clinical course of rapid progression of paraplegia following the onset of back pain. Typical history and magnetic resonance imaging features indicated the diagnosis of surfer's myelopathy. Two patients received high-dose steroid therapy and the other was treated with induced hypertension. One patient treated with induced hypertension showed almost full recovery; however, two patients who received high-dose steroid therapy remained completely paraplegic and required catheterization for bladder and bowel dysfunction despite months of rehabilitation. Our case series demonstrates the potentially devastating neurological outcome of surfer's myelopathy; however, early recovery in the initial 24–72 hours of presentation can occur in some patients, which is in accordance with previous reports. Ischemic insult to the spinal cord is thought to play a crucial role in the pathophysiology of surfer's myelopathy. Treatment recommendations include hydration, induced hypertension, early spinal angiography with intra-arterial intervention, intravenous tissue plasminogen activator, and high-dose steroid therapy; however, there is no standardized treatment option available. Early recovery appears to be important for long-term neurological outcome. Induced hypertension for initial treatment can be helpful for improving spinal cord perfusion; therefore, it is important for early and long-term neurological recovery. Education and awareness are essential for preventing surfer's myelopathy and avoiding further deterioration of neurological function.
Angiography ; Back Pain ; Catheterization ; Catheters ; Diagnosis ; Education ; Humans ; Hypertension ; Magnetic Resonance Imaging ; Male ; Paraplegia ; Perfusion ; Rehabilitation ; Spinal Cord ; Spinal Cord Diseases ; Spinal Cord Injuries ; Tissue Plasminogen Activator ; Urinary Bladder

STUDY DESIGN: Case report OBJECTIVES: This study introduces an interesting case of adolescent cervical myelopathy with atypical cervical magnetic resonance imaging (MRI) findings. A differential diagnosis was made, followed by successful surgical treatment. SUMMARY OF LITERATURE REVIEW: A careful differential diagnosis of high signal intensity on T2-weighted cervical MRI is necessary if there is no evidence of cervical stenosis. Recent reports have suggested that the differential diagnosis should be based on a comprehensive analysis of data, including brain MRI, a cerebrospinal fluid examination, and empirical steroid treatment. MATERIALS AND METHODS: A 17-year-old male patient complained of upper extremity weakness, gait disturbance, and decreased sensation in the upper extremity. Cervical spine MRI findings suggested C3/4 disc herniation, moderate cervical stenosis, and high signal intensity in the spinal cord. A differential diagnosis was made between cervical myelopathy and myelitis. RESULTS: Decompression and posterolateral fusion of C3/4 were performed in a 17-year-old patient with cervical myelopathy without significant cervical stenosis. Postoperatively, upper extremity sensation and weakness and gait disturbance showed improvement, and the Japanese Orthopedic Association score improved to 17 points at 6 months after surgery. CONCLUSIONS: In patients with cervical myelopathy showing high signal intensity on T2-weighted imaging without evident spinal stenosis, a differential diagnosis should be made between cervical myelopathy and myelitis; surgical decompression can be an effective treatment choice upon the diagnosis of cervical myelopathy.
Adolescent* ; Asian Continental Ancestry Group ; Brain ; Cerebrospinal Fluid ; Constriction, Pathologic ; Decompression ; Decompression, Surgical ; Diagnosis ; Diagnosis, Differential* ; Gait ; Humans ; Magnetic Resonance Imaging ; Male ; Myelitis* ; Orthopedics ; Sensation ; Spinal Cord ; Spinal Cord Diseases* ; Spinal Stenosis ; Spine ; Upper Extremity

STUDY DESIGN: A case report. OBJECTIVES: To report a rare cause of non-traumatic spinal cord injury (SCI) during surfing SUMMARY OF LITERATURE REVIEW: Surfer's myelopathy is a non-traumatic SCI associated with the hyperextension posture during paddling in surfing. Although the definite pathomechanism has not been identified, cord ischemia followed by arterial infarction may be related to this injury. MATERIALS AND METHODS: A young healthy male patient presented with a SCI that occurred during his first time surfing. Magnetic resonance imaging revealed a T2-hyperintense lesion in the spinal cord from D10 to the conus medullaris. RESULTS: The patient completely recovered without any neurologic deficits after steroid therapy and other forms of supportive management. CONCLUSIONS: Since surfing is becoming more common in Korea, awareness of surfer's myelopathy is important for early diagnosis and proper management.
Early Diagnosis ; Humans ; Infarction ; Ischemia ; Korea ; Magnetic Resonance Imaging ; Male ; Neurologic Manifestations ; Posture ; Spinal Cord ; Spinal Cord Diseases* ; Spinal Cord Injuries ; Spinal Cord Ischemia

Dynamic spinal cord compression has been investigated for several years, but until the advent of open MRI, the use of dynamic MRI (dMRI) did not gain popularity. Several publications have shown that cervical cord compression is both static and dynamic. On many occasions the evaluation of cervical spondylotic myelopathy (CSM) is straightforward, but patients are frequently encountered with a significantly worse clinical examination than would be suggested by radiological images. In this paper, we present an extensive review of the literature in order to describe the importance of dMRI in various settings and applications. A detailed literature review was performed in the Medline and Pubmed databases using the terms “cervical spondylotic myelopathy”, “dynamic MRI”, “kinetic MRI”, and “myelomalalcia” for the period of 1980-2016. The study was limited to English language, human subjects, case series, retrospective studies, prospective reports, and clinical trials. Reviews, case reports, cadaveric studies, editorials, and commentaries were excluded. The literature search yielded 180 papers, 19 of which met inclusion criteria. However, each paper had evaluated results and outcomes in different ways. It was not possible to compile them for meta-analysis or pooled data evaluation. Instead, we evaluated individual studies and present them for discussion. We describe a number of parameters evaluated in 2661 total patients, including dynamic changes to spinal cord and canal dimensions, transient compression of the cord with changes in position, and the effects of position on the intervertebral disc. dMRI is a useful tool for understanding the development of CSM. It has found several applications in the diagnosis and preoperative evaluation of many patients, as well as certain congenital dysplasias and Hirayama disease. It is useful in correlating symptoms with the dynamic changes only noted on dMRI, and has reduced the incidence of misdiagnosis of myelopathy.
Cadaver ; Cervical Cord ; Diagnosis ; Diagnostic Errors ; Humans ; Incidence ; Intervertebral Disc ; Magnetic Resonance Imaging* ; Prospective Studies ; Retrospective Studies ; Spinal Cord ; Spinal Cord Compression ; Spinal Cord Diseases*

STUDY DESIGN: Literature review. OBJECTIVES: The aim of this study was to provide insight into idiopathic spinal cord herniation (ISCH) in terms of clinical presentation, pathophysiology, diagnosis, classification, and treatment. SUMMARY OF LITERATURE REVIEW: ISCH is a rare disorder characterized by anterior displacement of the spinal cord through a ventral dural defect. It has increasingly been recognized and described over the past 10 years. MATERIALS AND METHODS: Review of the English-language literature on ISCH. RESULTS: ISCH occurs in middle-aged adults with a female preponderance. The most common clinical presentation is Brown-Sequard syndrome, which can progress to spastic paraparesis. Its pathophysiology is unknown. However, some authors proposed that inflammation may play an important role in the emergence of a dural defect. Magnetic resonance imaging typically shows an anterior kink of the thoracic spinal cord with an obliteration of the ventral subarachnoid space and the widened dorsal subarachnoid space. Surgery is generally recommended for patients with motor deficits or progressive neurological symptoms. The posterior approach has been used because it allows wide exposure of the spinal cord. The surgical treatment of ISCH consists of spinal cord reduction from the ventral dural defect, which can be managed with enlargement, direct repair, or duraplasty (dural repair with a patch). In recent years, duraplasty has been used more frequently than enlargement of the dural defect. CONCLUSIONS: ISCH causing thoracic myelopathy could be safely treated with surgical management. The possibility of this disease should be kept in mind when treating patients with progressive myelopathy.
Adult ; Brown-Sequard Syndrome ; Classification ; Diagnosis ; Female ; Humans ; Inflammation ; Magnetic Resonance Imaging ; Paraparesis, Spastic ; Spinal Cord Diseases ; Spinal Cord* ; Subarachnoid Space