1.Glial fibrillary acidic protein immunoglobulin G‑associated myelitis:Clinical features and comparison with aquaporin‑4 immunoglobulin G‑associated myelitis and myelin oligodendrocyte glycoprotein immunoglobulin G‑associated myelitis
Journal of Apoplexy and Nervous Diseases 2026;43(4):356-362
Objective To investigate the clinical features of glial fibrillary acidic protein immunoglobulin G (GFAP-IgG)-associated myelitis, to compare the differences in clinical features between GFAP-IgG-associated myelitis and aquaporin-4 immunoglobulin G (AQP4-IgG)-associated myelitis/myelin oligodendrocyte glycoprotein immunoglobulin G (MOG-IgG)-associated myelitis, and to provide help for early diagnosis and treatment. Methods A retrospective analysis was performed for the clinical data of 34 patients who were diagnosed with GFAP-IgG-associated myelitis in The First Affiliated Hospital of Zhengzhou University and Henan Children Hospital from May 2018 to May 2023, and their demographic features, clinical features, serological parameters,cerebrospinal fluid (CSF) parameters, imaging features, and prognosis were systematically analyzed. In addition, 30 patients diagnosed with AQP4-IgG-associated myelitis and 42 patients diagnosed with MOG-IgG-associated myelitis during the same period of time were enrolled as control groups, and the mean clinical features were compared between the three groups. Results Among the 34 patients with GFAP-IgG-associated myelitis, there were 15 female patients and 12 children. The median age of onset was 28.5 years, and more than half of the patients had prodromal symptoms. More than half of the patients had the symptoms of pyrexia (29/34, 85%), headache (19/34,56%), nausea/vomiting (20/34,59%),and limb weakness (27/34,79%) during the course of the disease, with 19 patients(19/34,56%) admitted to the intensive care unit (ICU).Imaging examination showed gadolinium-enhancing spinal cord lesions and longitudinally extensive sagittal T2 hyperintensity, i.e.,longitudinally extensive transverse myelitis.Compared with the AQP4-IgG-associated myelitis group and the MOG-IgG-associated myelitis group,the GFAP-IgG-associated myelitis group had a significantly higher proportion of patients with pyrexia (85% vs 10%, P1<0.01; 85% vs 38%,P2<0.01), neck stiffness (41% vs 7%, P1<0.01; 41% vs 12%, P2<0.01), admission to the ICU (56% vs 10%, P1<0.01; 56% vs 17%, P2<0.01), or mechanical ventilation (38% vs 3%, P1<0.01; 38% vs 0%, P2<0.01) and a significant increase in serum monocyte-to-lymphocyte ratio [0.43(0.24,0.71) vs 0.23 (0.18,0.32), P1<0.01; 0.43 (0.24,0.71) vs 0.21 (0.14,0.30),P2<0.01], as well as a significantly higher proportion of patients with hyponatremia (45% vs 13%,P1<0.01; 45% vs 7%,P2<0.01), an increase in leukocyte count in CSF(68% vs 14%, P1<0.01; 68% vs 34%, P2<0.01), an increase in total protein in CSF (82% vs 31%, P1<0.01; 82% vs 20%, P2<0.01), a reduction in glucose level in CSF (26% vs 3%,P1=0.03; 26% vs 2%, P2<0.01), or the presence of central canal enhancement (29% vs 0%, P1=0.02;29% vs 0%, P2=0.01). Compared with the AQP4-IgG-associated myelitis group, the GFAP-IgG-associated myelitis group had a significantly higher number of the diseased segments of spinal cord [13(5.8,18) vs 6(3,12.5),P=0.01]. Compared with the MOG-IgG-associated myelitis group, the GFAP-IgG-associated myelitis group had a significantly higher EDSS score at discharge [4(3,6) vs 1(0,3),P2=0.01] and at last follow-up [0(0,2.8) vs 0(0,1),P2=0.047]. Conclusion There are differences in clinical features, serological/CSF profiles, and imaging features among the three groups of patients with myelitis,which may help with the differential diagnosis of the different subtypes of myelitis.
Myelitis
2.Listeria Brainstem Encephalitis With Myelitis Misdiagnosed as Acute Disseminated Encephalomyelitis:Report of One Case.
Dan-Ying WU ; Qin-Xue WANG ; Dong-Mei ZHU ; Yu-Jing GAN ; Min HUANG ; Su-Ming ZHOU
Acta Academiae Medicinae Sinicae 2025;47(4):673-678
Listeria brainstem encephalitis with myelitis is extremely rare in clinical practice.Since the clinical manifestations are non-specific,MRI is helpful for diagnosis.Positive cerebrospinal fluid culture is considered the gold standard for diagnosis.This article reports a case of an immunocompetent individual with listeria brainstem encephalitis with myelitis,aiming to enhance the awareness of this condition.
Humans
;
Brain Stem/pathology*
;
Diagnostic Errors
;
Encephalitis/complications*
;
Encephalomyelitis, Acute Disseminated/diagnosis*
;
Listeriosis/complications*
;
Myelitis/complications*
3.Transverse myelitis unmasking multiple sclerosis after mRNA COVID-19 vaccine: A case report.
Javish R. Jadwani ; Eloise D. Guadañ ; a ; Cristina A. Dorado ; Alexander D. Abe ; Jay S. Fonte
Philippine Journal of Internal Medicine 2024;62(3):160-165
INTRODUCTION
Demyelinating central nervous system (CNS) disorders such as transverse myelitis (TM) and multiple sclerosis (MS) have been reported with mRNA Covid-19 vaccine. Some cases were relapses of a pre-existing condition but de novo and initial presentation of MS after BNT162b2 COVID-19 mRNA vaccine has very rarely been documented.
CASE DESCRIPTIONWe report a 72-year-old female, right-handed, Filipina, with a one-month history of bilateral lower extremity weakness which occurred 7 days after she received her first booster dose of BNT162b2 mRNA vaccine. This was later accompanied by fecal and urinary incontinence. On examination, she had motor deficit below L1 myotome manifesting with loss of hip flexion, knee extension, dorsiflexion, and plantar flexion. There was also sensory deficit below T10 level with relative 80% sensation of vibratio, proprioception, light touch and complete loss of pain and temperature sensation. The initial impression was Transverse Myelitis which may be related to a post-vaccination state. Spinal magnetic resonance imaging (MRI) revealed long segment enhancing T2W hyperintense lesion at T2 to T7. Cranial MRI revealed ovoid areas of heterogeneous, predominantly T2/FLAIR hyperintense signals exhibiting restricted diffusion in the periventricular white matter of the fronto-parietal lobes. Cerebrospinal fluid (CSF) analysis was negative for infectious causes such as tuberculosis but with high levels of CSF immunoglobulin G. She was then diagnosed to have Multiple Sclerosis (MS) and was treated with high dose oral prednisone. However, there was no improvement in neurological deficits on follow-up.
CONCLUSIONThis case adds to the reported rare cases of initial presentation of MS occurring after vaccination for COVID-19 and the first reported case in the Philippines. Early recognition and prompt treatment is important to improve outcomes.
Human ; Female ; Aged: 65-79 Yrs Old ; Myelitis, Transverse ; Multiple Sclerosis
5.Experience of treating facial neuritis, unexplained limb weakness, cervical spondylosis, acute myelitis, acute radiculitis, Guillain Barre syndrome, multiple sclerosis, myasthenia gravis, motor neuron disease, dermatomyositis with Xiaoxuming Decoction.
China Journal of Chinese Materia Medica 2020;45(12):2735-2751
Xiaoxuming Decoction is an ancient classic herbal formula for the treatment of stroke. In ancient times, the connotation of stroke was very extensive, including facial neuritis, acute cerebral infarction, acute cerebral hemorrhage, sequelae of cerebral hemorrhage, unexplained weakness of limbs, cervical spondylosis, acute myelitis, acute radiculitis, Guillain Barre syndrome, multiple sclerosis, myasthenia gravis, motor neuron disease, dermatomyositis, hypokalemic paralysis peripheral neuritis. It has been identified that: ①Xiaoxuming Decoction is very common in the treatment of cerebrovascular diseases, such as cerebral infarction, cerebral hemorrhage and other cerebrovascular diseases, facial neuritis, acute myelitis, acute radiculitis, Guillain Barre syndrome, unexplained limb weakness, multiple sclerosis, motor neuron disease, myasthenia gravis, and rheumatic and immune system diseases, such as dermatomyositis, and can not only alleviate symptoms, but also improve prognosis and the long-term survival rate. ②Sudden limb failure, facial paralysis, and hypoalgesia without heat syndrome are the key indications of Xiaoxuming Decoction. ③This is a special prescription for the treatment of acute facial neuritis, and can cure in one week in the combination with moxibustion. ④In the treatment of facial neuritis complicated with hypertension or acute cerebrovascular disease, Xiaoxuming Decoction generally has a certain antihypertensive effect, without any hypertensive effect, which reflected its two-way regulatory effect for blood pressure. ⑤In the treatment of unknown limb weakness, acute myelitis, acute radiculitis, Guillain Barre syndrome, Xiaoxuming Decoction can rapidly alleviate the symptoms. ⑥This is the basic formula for multiple sclerosis and motor neuron disease. Long term use of Xiaoxuming Decoction can alleviate the symptom of limb weakness, reduce the occurrence of complications and delay the progress of the disease, but with a poor long-term prognosis. ⑦In the treatment of myasthenia gravis, Xiaoxuming Decoction can significantly improve muscle strength, and gradually help stop hormone reduction. After thymoma surgery, Xiaoxuming Decoction is also applicable to some patients with recurrent myasthenia gravis. ⑧Xiaoxuming Decoction also plays a role in the treatment of dermatomyositis and cervical spondylopathy. ⑨Raw ephedra is the monarch drug of Xiaoxuming Decoction, which is the key to the effect. The dosage starts with 6 g is titrated in a small dose and increases gradually. In addition, this formula is forbidden for those with red face, fast heart rate, high blood pressure, blocked stool, red tongue, yellow fur, wiry and rapid pulse or powerful pulse, and spout pulse.
Dermatomyositis
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Facial Nerve Diseases
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Guillain-Barre Syndrome
;
Humans
;
Motor Neuron Disease
;
Multiple Sclerosis
;
Myasthenia Gravis
;
Myelitis
;
Radiculopathy
;
Spondylosis
6.A Rare Case of Syphilitic Myelitis of the Spinal Cord
Jin Hyeok KIM ; Hee Seok JEONG ; Chankue PARK ; Hwaseong RYU ; Ji Eun ROH ; Jeong A YEOM ; Tae un KIM
Investigative Magnetic Resonance Imaging 2019;23(3):279-282
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
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Brain
;
Diagnosis
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Female
;
Humans
;
Infarction
;
Magnetic Resonance Imaging
;
Myelitis
;
Myelitis, Transverse
;
Neurosyphilis
;
Spinal Cord
;
Syphilis
;
Treponema pallidum
7.Acute Flaccid Myelitis in a Korean Adult
Journal of Korean Medical Science 2019;34(5):e39-
8.Neuromyelitis Optica Spectrum Disorder Presented with Upbeat Nystagmus and Intractable Vomiting
Hyunsoo KIM ; Jae Myung KIM ; Tai Seung NAM ; Seung Han LEE
Journal of the Korean Balance Society 2019;18(2):50-53
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
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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
9.Spinal CCL2 Promotes Central Sensitization, Long-Term Potentiation, and Inflammatory Pain via CCR2: Further Insights into Molecular, Synaptic, and Cellular Mechanisms.
Rou-Gang XIE ; Yong-Jing GAO ; Chul-Kyu PARK ; Ning LU ; Ceng LUO ; Wen-Ting WANG ; Sheng-Xi WU ; Ru-Rong JI
Neuroscience Bulletin 2018;34(1):13-21
Mounting evidence supports an important role of chemokines, produced by spinal cord astrocytes, in promoting central sensitization and chronic pain. In particular, CCL2 (C-C motif chemokine ligand 2) has been shown to enhance N-methyl-D-aspartate (NMDA)-induced currents in spinal outer lamina II (IIo) neurons. However, the exact molecular, synaptic, and cellular mechanisms by which CCL2 modulates central sensitization are still unclear. We found that spinal injection of the CCR2 antagonist RS504393 attenuated CCL2- and inflammation-induced hyperalgesia. Single-cell RT-PCR revealed CCR2 expression in excitatory vesicular glutamate transporter subtype 2-positive (VGLUT2) neurons. CCL2 increased NMDA-induced currents in CCR2/VGLUT2 neurons in lamina IIo; it also enhanced the synaptic NMDA currents evoked by dorsal root stimulation; and furthermore, it increased the total and synaptic NMDA currents in somatostatin-expressing excitatory neurons. Finally, intrathecal RS504393 reversed the long-term potentiation evoked in the spinal cord by C-fiber stimulation. Our findings suggest that CCL2 directly modulates synaptic plasticity in CCR2-expressing excitatory neurons in spinal lamina IIo, and this underlies the generation of central sensitization in pathological pain.
Animals
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Benzoxazines
;
pharmacology
;
therapeutic use
;
Chemokine CCL2
;
antagonists & inhibitors
;
genetics
;
metabolism
;
pharmacology
;
Excitatory Amino Acid Agents
;
pharmacology
;
Excitatory Amino Acid Agonists
;
pharmacology
;
Female
;
Freund's Adjuvant
;
toxicity
;
Hyperalgesia
;
chemically induced
;
metabolism
;
prevention & control
;
Long-Term Potentiation
;
drug effects
;
physiology
;
Luminescent Proteins
;
genetics
;
metabolism
;
Male
;
Mice
;
Mice, Inbred C57BL
;
Mice, Transgenic
;
Myelitis
;
chemically induced
;
drug therapy
;
metabolism
;
Neurons
;
drug effects
;
Pain Management
;
Somatostatin
;
genetics
;
metabolism
;
Spinal Cord
;
cytology
;
Spiro Compounds
;
pharmacology
;
therapeutic use
;
Vesicular Glutamate Transport Protein 2
;
genetics
;
metabolism
;
Vesicular Inhibitory Amino Acid Transport Proteins
;
genetics
;
metabolism
10.An Anti-aquaporin 4 Positive Longitudinally Extensive Transverse Myelitis with Antecedent Necrotizing Pneumonia Associated with S. aureus
Boo Kyoung JUNG ; Jung A KWON ; Jong Mok LEE
Journal of the Korean Neurological Association 2018;36(3):220-222
A 53-year-old male patient presented with quadriparesis during pulmonological hospitalization for the treatment of S. aureus associated necrotizing pneumonia. He was diagnosed with the aquaporin-4 (AQP4) positive longitudinally extensive transverse myelitis from pons to T3 level. Despite the administration of intravenous methylprednisolone and plasma exchange with appropriate antibiotics, the patient's neurological condition deteriorated and he died. Our case implies that an S. aureus associated necrotizing pneumonia can trigger an AQP4 positive neuromyelitis optica spectrum disorder and contribute to the devastating course.
Anti-Bacterial Agents
;
Aquaporin 4
;
Hospitalization
;
Humans
;
Male
;
Methylprednisolone
;
Middle Aged
;
Myelitis, Transverse
;
Neuromyelitis Optica
;
Plasma Exchange
;
Pneumonia
;
Pons
;
Quadriplegia


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