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

Neurosyphilis is an infection of the brain or spinal cord that is caused by the bacterium Treponema pallidum. Syphilitic myelitis, which involves the spinal cord, is a very rare form of neurosyphilis seen in patients with syphilis. It requires differentiation from other diseases of the spinal cord, including idiopathic transverse myelitis and spinal cord infarction. Herein, we describe the presentation and diagnosis of syphilitic myelitis in a 43-year-old woman, based on a flip-flop sign and candle guttering appearance depicted in magnetic resonance imaging and laboratory tests.
Adult ; Brain ; Diagnosis ; Female ; Humans ; Infarction ; Magnetic Resonance Imaging ; Myelitis ; Myelitis, Transverse ; Neurosyphilis ; Spinal Cord ; Syphilis ; Treponema pallidum

BACKGROUND AND PURPOSE: Optic neuritis (ON) is an inflammation of the optic nerve that can be recurrent, with unilateral or bilateral presentation. Diagnosing recurrent cases may be challenging. We aimed to compare patients with recurrent ON as their initial symptom according to their following final diagnoses: multiple sclerosis (MS), neuromyelitis optica spectrum disorders (NMOSD), or chronic relapsing inflammatory optic neuropathy (CRION). METHODS: The medical records of patients with initial recurrent ON who were followed at the Neuroimmunology Clinic of the Federal University of São Paulo between 2004 and 2016 were analyzed retrospectively. Patients were classified according to their final diagnosis into MS, NMOSD, or CRION, and the characteristics of these groups were compared to identify predictive factors. RESULTS: Thirty-three patients with recurrent ON were included, and 6, 14, and 13 had final diagnoses of MS, NMOSD, and CRION, respectively. Most of the patients were female with unilateral and severe ON in their first episode, and the initial Visual Functional System Score (VFSS) was ≥5 in 63.6%, 85.7%, and 16.7% of the patients with CRION, NMOSD, and MS, respectively. Anti-aquaporin-4 antibodies were detected in 9 of 21 (42.8%) tested patients. Seven of nine (77.8%) seropositive NMOSD patients experienced transverse myelitis episodes during the follow-up period. A multivariate regression analysis showed that the VFSS at the last medical appointment predicted the final diagnosis. CONCLUSIONS: A lower VFSS at the last medical appointment was predictive of MS. Patients with NMOSD and CRION have similar clinical characteristics, whereas NMOSD patients tend to have worse visual acuity.
Antibodies ; Aquaporin 4 ; Demyelinating Diseases* ; Diagnosis ; Female ; Follow-Up Studies ; Humans ; Inflammation ; Medical Records ; Multiple Sclerosis ; Myelitis, Transverse ; Neuromyelitis Optica ; Optic Nerve ; Optic Nerve Diseases ; Optic Neuritis* ; Recurrence ; Retrospective Studies ; Visual Acuity

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

Guillain-Barré syndrome and acute transverse myelitis manifest as demyelinating diseases of the peripheral and central nervous system. Concurrency of these two disorders is rarely documented in literature. A 4-year-old girl presenting with cough, fever, and an impaired walking ability was admitted to hospital. She had no previous complaints in her medical history. A physical examination revealed lack of muscle strength of the lower extremities and deep tendon reflexes. MRI could not be carried out due to technical problems; therefore, both Guillain-Barré syndrome and acute transverse myelitis were considered for the diagnosis. Intravenous immunoglobulin treatment was started as first line therapy. Because this treatment did not relieve the patient's symptoms, spinal MRI was carried out on the fourth day of admission and demyelinating areas were identified. Based on the new findings, the patient was diagnosed with acute transverse myelitis, and high dose intravenous methylprednisolone therapy was started. Electromyography findings were consistent with acute polyneuropathy affecting both motor and sensory fibers. Therefore, the patient was diagnosed with concurrency of Guillain-Barré syndrome and acute transverse myelitis. Interestingly, while concurrency of these 2 disorders is rare, this association has been demonstrated in various recent publications. Progress in diagnostic tests (magnetic resonance imaging and electrophysiological examination studies) has enabled clinicians to establish the right diagnosis. The possibility of concurrent Guillain-Barré syndrome and acute transverse myelitis should be considered if recovery takes longer than anticipated.
Central Nervous System ; Child, Preschool ; Cough ; Demyelinating Diseases ; Diagnosis ; Diagnostic Tests, Routine ; Electromyography ; Female ; Fever ; Guillain-Barre Syndrome* ; Humans ; Immunoglobulins ; Lower Extremity ; Magnetic Resonance Imaging ; Methylprednisolone ; Muscle Strength ; Myelitis, Transverse* ; Physical Examination ; Polyneuropathies ; Reflex, Stretch ; Walking

The literature was analyzed on the improvement of motor sensory function, urination and defecation in myelitis treated with acupuncture and moxibustion and focused on the characteristics of the diagnosis and treatment. The literature on acupuncture and moxibustion treatment for myelitis was collected from CNKI, Wanfang, VIP and PubMed. The analysis included the characteristics of acupoint selection, methods of acupuncture and moxibustion, time of treatment, total treatment period, efficacy, follow-up, safety, etc. Totally, 26 articles were collected. The combined therapy of acupuncture and moxibustion was predominated (12/26, 46. 15%). For the motor and sensory impairment, the acupoints were mainly selected from the four limbs, the yangming meridians of hand and foot and those adjacent to the affected spinal segments and on the governor vessel as well as Jiaji (EX-B 2) points. For urinary impairment, the acupoints were selected mainly from the lower abdominal region on the conception vessel and the lumbosacral region on the bladder meridian. For the intestinal impairment, the acupoints were from the lower limb on the stomach meridian, the lower abdominal region on the conception vessel and the back points on the bladder meridian. The intervention started commonly in the first 3 months after onset. The total treatment period was in the range from 1 to 3 months. The efficacy of acupuncture and moxibustion was 69.19% to 82.56% for the improvement of motor sensory and urination, defecation function. The efficacy in follow-up was stable and the adverse reactions were not reported. It is viewed that on the basis of early diagnosis and active medication, acupuncture and moxibution achieve a certain of efficacy on the impairment of motor sensory function, urination and defecation. A clinical research is expected to further verify the efficacy.
Acupuncture Therapy ; Databases, Bibliographic ; Defecation ; Humans ; Motor Activity ; Moxibustion ; Myelitis ; diagnosis ; physiopathology ; therapy ; Sensation ; Urinary Bladder ; physiopathology ; Urination

Neuromyelitis optica spectrum disorder (NMOSD) can present with various symptoms including optic neuritis, transverse myelitis, and area postrema syndrome. However, acute memory loss is an uncommon clinical presentation of NMOSD. We report a patient with NMO-IgG-antibody-positive NMOSD presenting with only acute memory loss, which suggested the presence of bilateral thalamic lesions. This case indicates that NMOSD needs to be considered in the differential diagnosis of acute memory loss.
Area Postrema ; Diagnosis, Differential ; Humans ; Memory Disorders* ; Memory* ; Myelitis, Transverse ; Neuromyelitis Optica* ; Optic Neuritis

Acute transverse myelitis (ATM) is an upper motor neuron disease of the spinal cord, and concomitant association of peripheral polyneuropathy, particularly the axonal type, is rarely reported in children. Our cases presented with ATM complicated with axonal type polyneuropathy. Axonal type polyneuropathy may be caused by acute motor-sensory axonal neuropathy (AMSAN) or critical illness polyneuropathy and myopathy (CIPNM). These cases emphasize the need for nerve and muscle biopsies to make the differential diagnosis between AMSAN and CIPNM in patients with ATM complicated with axonal polyneuropathy.
Axons* ; Biopsy ; Child* ; Diagnosis, Differential ; Humans ; Motor Neuron Disease ; Muscular Diseases ; Myelitis, Transverse* ; Polyneuropathies* ; Spinal Cord

Epidural steroid injections (ESI) are a common treatment for back pain management. ESI-related complications have increased with the growing number of procedures. We report a case of cervical meningomyelitis followed by multiple lumbar ESI. A 60-year-old male with diabetes mellitus presented to our hospital with severe neck pain. He had a history of multiple lumbar injections from a local pain clinic. After admission, high fever and elevated inflammatory values were detected. L-spine magnetic resonance imaging (MRI) revealed hematoma in the S1 epidural space. Antibiotic treatment began under the diagnosis of a lumbar epidural abscess. Despite the treatment, he started to complain of weakness in both lower extremities. Three days later, the weakness progressed to both upper extremities. C-spine MRI revealed cervical leptomeningeal enhancement in the medulla oblongata and cervical spinal cord. Removal of the epidural abscess was performed, but there was no neurological improvement.
Back Pain ; Diabetes Mellitus ; Diagnosis ; Epidural Abscess ; Epidural Space ; Fever ; Hematoma ; Humans ; Injections, Epidural ; Lower Extremity ; Magnetic Resonance Imaging ; Male ; Medulla Oblongata ; Middle Aged ; Myelitis ; Neck Pain ; Pain Clinics ; Spinal Cord ; Upper Extremity

Neuromyelitis optica (NMO) is an inflammatory demyelinating disease of the central nervous system associated with longitudinally extensive myelitis and optic neuritis. It is characterized by relapses that lead to blindness and paralysis sequelaes. But, this is rare disease; therefore high clinical suspicion for a correct diagnosis and proper examinations are not easy. However, early diagnosis is essential to prevent sequelae. We report the case of NMO with headache. A 30-year male patient who suffered headache visited our pain clinic because of aggravated pain despite treatment. The cause of the pain was revealed as NMO by more detailed previous history and examination.
Blindness ; Central Nervous System ; Demyelinating Diseases ; Diagnosis ; Early Diagnosis ; Headache ; Humans ; Male ; Myelitis ; Neuromyelitis Optica* ; Optic Neuritis ; Pain Clinics ; Paralysis ; Post-Traumatic Headache* ; Rare Diseases ; Recurrence