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: 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

STUDY DESIGN: Case report. OBJECTIVES: We report the case of a long, solitary, rosary-shaped neurofibroma that was misdiagnosed as another disease due to the patient's surgical history involving repetitive procedures and its abnormal appearance. SUMMARY OF LITERATURE REVIEW: Neurofibroma is an intradural-extramedullary spinal tumor. It is generally not difficult to diagnose due to its frequent occurrence and specific magnetic resonance imaging (MRI) findings. However, to date, neurofibromatosis stigmata and long, solitary, rosary-shaped neurofibromas have rarely been reported. MATERIALS AND METHODS: A 60-year-old woman was admitted to our hospital due to persistent pain, despite previous surgery and repetitive procedures. On physical examination, vision loss, hearing loss, skin discoloration, or subcutaneous nodules were not observed. A neurologic examination revealed normal motor and sensory function and voiding sensation. No pathologic reflexes such as the Babinski sign were observed. Previous sequential MRIs revealed intradural lesions that progressed from the thoracic vertebra 11 to the lumbar vertebra 3. She had no signs of neurofibromatosis stigmata, and the neurologic examination was unremarkable. The initial diagnosis was based on serial MRIs, which revealed a parasite infestation, a spinal cord tumor (myxopapillary-type ependymoma with hemorrhage), arachnoiditis, and vascular malformations. Total mass excision was performed, and the final diagnosis was neurofibroma. RESULTS: There were no signs of a tumor remnants or local recurrence in a 1-year follow-up MRI study. CONCLUSIONS: Although intradural spinal tumors are very rare, their clinical features are nonspecific and resemble other degenerative spinal diseases, including spinal stenosis and disc herniation. These diseases may easily be overlooked by physicians.
Arachnoid ; Arachnoiditis ; Christianity ; Diagnosis ; Diagnostic Errors ; Ependymoma ; Female ; Follow-Up Studies ; Hearing Loss ; Humans ; Magnetic Resonance Imaging ; Middle Aged ; Neurofibroma* ; Neurofibromatoses ; Neurologic Examination ; Parasites ; Physical Examination ; Recurrence ; Reflex ; Reflex, Babinski ; Sensation ; Skin ; Spinal Cord Neoplasms ; Spinal Diseases ; Spinal Stenosis ; Spine ; Vascular Malformations

STUDY DESIGN: Case report. OBJECTIVES: To present a rare case of a spinal epidural lymphoma mimicking a hematoma. SUMMARY OF LITERATURE REVIEW: The incidence of extranodal invasion of lymphoma is very low, and spinal compression as a clinical symptom rarely occurs in extranodal lymphoma. MATERIALS AND METHODS: A 37-year-old woman who complained of neck pain that had lasted for the past 3 months with no underlying disease visited the hospital and was treated conservatively with medication and physical therapy. Even though the patient was given an epidural injection 1 month prior to visiting our hospital, the pain lingered and the cervical myelopathy became aggravated, so the patient was initially diagnosed with epidural hematoma. This predisposed the patient to treatment strategies such as laminectomy and fusion, but the patient was ultimately diagnosed with non-Hodgkin lymphoma. RESULTS: The patient underwent a laminectomy with excision of the lesion and fusion. The patient was diagnosed with non-Hodgkin lymphoma as a result of this, and the patient's radicular pain and numbness improved immediately following surgery, except for mild weakness in both hands. CONCLUSIONS: Although spinal epidural lymphoma is similar to hematoma, and is a very rare disease, surgeons should take it into consideration in the differential diagnosis of patients with a spinal epidural lesion.
Adult ; Diagnosis, Differential ; Female ; Hand ; Hematoma* ; Humans ; Hypesthesia ; Incidence ; Injections, Epidural ; Laminectomy ; Lymphoma* ; Lymphoma, Non-Hodgkin ; Neck Pain ; Rare Diseases ; Spinal Cord Diseases ; Surgeons

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