Tuberculosis is a leading cause of mortality in children worldwide. One of the greatest challenges in its management is the difficulty of diagnosis as the manifestations are non-specific and often mimic other illnesses. Neurological infection occurs in approximately 1% of patients diagnosed with tuberculosis, and usually takes the form of tuberculous meningitis or tuberculoma. An 11-year-old girl who was diagnosed with acute disseminated encephalomyelitis, a rare immunological manifestation of tuberculosis, is presented. She recovered completely after a course of high-dose systemic corticosteroids in addition to anti-tuberculosis treatment. Considering the immense burden of this infectious disease, recognition and understanding of the uncommon manifestations are important to enable appropriate and timely treatment.Abbreviations: ADEM: acute disseminated encephalomyelitis; ATT: anti-tuberculosis therapy; CBNAAT: cartridge-based nucleic acid amplification test; CNS: central nervous system; CSF: cerebrospinal fluid; CT: computed tomography; FLAIR: fluid attenuated inversion recovery; IFN: interferon; MRI: magnetic resonance imaging; MTB: Mycobacterium tuberculosis; TB: tuberculosis; TNF: tumour necrosis factor.

Acute disseminated encephalomyelitis and Guillain-Barré syndrome refer to post-infectious or post-vaccination inflammatory demyelinating disorders of central and peripheral nervous system, respectively. We report the case of a 60-year-old male patient presenting with irritability, gait difficulty, asymmetric quadriparesis (mostly in his left extremities), distal sensory loss for pain and temperature in left limbs, and reduced tendon reflexes in his upper limbs and absent in his lower limbs, following an upper respiratory tract infection, 3 weeks earlier. Brain magnetic resonance imaging revealed abnormal T2 signal and peripherally enhancing lesions in hemispheres, brainstem, and cerebellum. Nerve conduction studies were compatible with acute motor and sensory axonal neuropathy. Serology revealed positive IgM and IgG antibodies for Chlamydia pneumoniae, and he also tested positive for myelin oligodendrocyte glycoprotein (MOG) and sulfatide antibodies. Treatment with intravenous immunoglobulin and methylprednisolone led to clinical and radiological recovery within weeks. Even though several cases of combined central and peripheral demyelination have been reported before, it is the first case report with seropositive anti-sulfatide and anti-MOG acute sensorimotor axonal neuropathy and disseminated encephalitis associated with C. pneumoniae.

BACKGROUND: Relapsing or recurrent tumefactive demyelination is rare and has not been studied beyond individual case reports.
OBJECTIVE: We examined the clinical course, neuroimaging, cerebrospinal fluid (CSF), treatment and outcomes of patients with recurrent tumefactive demyelinating lesions (TDLs).
METHODS: We used PubMed to identify reports of recurrent TDLs and included the details of an additional, unpublished patient.
RESULTS: We identified 18 cases (11F, 7 M). The median age at onset of the index TDL was 37 years (range 12-72) and most were solitary lesions 72 % (13/18). CSF-restricted oligoclonal bands (OCBs) were detected in 25 % (4/16). Only one of those tested (n = 13) was positive for AQP4-IgG. A moderate-to-marked treatment response (high dose corticosteroid with or without additional plasmapheresis, IVIg or disease modifying therapies) was evident in 89 % of treated patients. Median EDSS at the median follow-up of 36 months (range 6-144) was 2 (range 1-10). Most remained ambulatory (EDSS < 4 in 13/18), but 1 patient died.
CONCLUSIONS: The median age of patients with relapsing TDLs is similar to that of typical MS, but differences include a lower female:male sex ratio, larger lesions, and a comparative lack of CSF-restricted OCBs. Outcomes vary among this group of patients ranging from minimal disability through to death.

UNASSIGNED: Coronavirus disease 2019 (COVID-19) is a disease caused by severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) and various vaccines against it have been developed. Acute disseminated encephalomyelitis (ADEM) is a disease of the central nervous system that cause inflammation and demyelination and manifests as a multi-symptom acute neurological condition. Although infections are usually the cause of ADEM, vaccines may cause 5-10% of cases.
UNASSIGNED: A 40-year-old woman had received a second dose of the Sinopharm COVID-19 vaccine 4 months before her visit and experienced sudden gait imbalance and vertigo a day after her vaccination, which lasted for more than a month. On examination, no signs of skin bruising or bleeding were observed, and her vital signs were within the normal range. On neurological assessment, the patient had a Glasgow Coma Scale score of 14/15 (E4V5M5), had normal pupil size and light reaction, normal fundus, normal deep tendon reflexes and bilateral extensor plantar response. Meningeal symptoms were absent, and SARS-CoV-2 RNA tests using NAAT (Nucleic Acid Amplification Test) were negative. Development of central nervous system (CNS) manifestations during the recovery phase of fever, along with typical MRI findings; the diagnosis of para-infectious ADEM with COVID-19 vaccination was made. After the treatment with methylprednisolone sodium succinate injection, the patient showed improvement.
UNASSIGNED: ADEM associated with post-vaccinations is a rare condition. There has been growing evidence that shared epitopes between neuronal proteins and SARS-CoV-2 antigens may trigger autoimmune reactions against the CNS through molecular mimicry as its pathogenesis.
UNASSIGNED: We suggest the need for a strict vaccine safety monitoring system and post-vaccine monitoring and surveillance.

Myelin oligodendrocyte glycoprotein antibody-associated disease (MOGAD) is a group of central nervous system (CNS) demyelinating diseases caused by autoantibodies against myelin oligosaccharide protein (MOG), a myelin sheath component protein, and present with a variety of symptoms, including optic neuritis, myelitis, acute disseminated encephalomyelitis (ADEM), brainstem encephalitis, and corticobasal encephalitis. It is currently unknown at what point in life MOGAD can develop or how it can be triggered by autoimmune mechanisms. Here, we report a case of a mature woman who suffered from adenoviral meningitis one month after childbirth and developed MOGAD but was able to return to child rearing with high-dose methylprednisolone therapy. This case suggests that the risk of developing MOGAD early after childbirth may be increased. The case also suggested that adenoviral infection may be involved in the development of MOGAD.

BACKGROUND: Myelin oligodendrocyte glycoprotein antibody-associated disease (MOGAD) is a recently defined demyelinating disorder with a rapidly evolving clinical spectrum. Recently, consensus criteria have been proposed (Banwell et al., 2023) to help with disease diagnosis. However, validation of the proposed criteria in real-life MOGAD patients is lacking. In this study, we applied the proposed criteria to an institutional cohort of MOG antibody-positive patients.
METHODS: A retrospective study was conducted at a tertiary neuroimmunology clinic from 2018 to 2023. Patients who had at least one core clinical feature of MOGAD and positive serum MOG antibody by cell-based assay were included. Demographics and clinical data were recorded and analyzed. Cases were divided into definite MOGAD, questionable MOGAD, and false-positive MOG antibody as determined by the treating neuroimmunology and/or neuro-ophthalmology specialists prior to applying the new MOGAD criteria by an independent investigator. We then calculated the sensitivity, specificity, positive predictive value, and negative predictive value of the new criteria compared to the treating physicians\' assessment.
RESULTS: A total of 27 patients were included of which, 19 (70.4%) were female, the average age of the sample was 44 +/- 15 years. High titer MOG antibody (≥ 1:100) was found in 11 patients (40.7%); low titer (< 1: 100) in 13 (48.1%), and unreported titer in 3 patients. As determined by expert opinion; 18 (66.7%) were identified as definitive MOGAD, 6 (22.2%) as false-positive MOG antibody, and 3 (11.1%) as questionable MOGAD. All 18 patients identified by clinicians as definite MOGAD met the new 2023 criteria. Of the 9 patients with questionable MOGAD or false-positive MOG antibody, four patients met the 2023 MOGAD criteria. Those four patients had the following final diagnoses: CNS vasculitis, primary progressive MS with activity and progression, pseudotumor cerebri, and bevacizumab-induced anterior ischemic optic neuropathy in the setting of paraneoplastic retinopathy. Compared to clinician assessment, applying the 2023 MOGAD criteria to our institutional cohort yielded a sensitivity of 100%, a specificity of 55.5%, a positive predictive value of 81.5% and a negative predictive value of 100%.
CONCLUSIONS: These findings suggest that the 2023 MOGAD criteria are highly sensitive for detection of definite MOGAD but has modest specificity. A number of MOGAD mimickers can resemble the core clinical events of MOGAD and share similar supportive clinical and MRI features. Clinicians should practice caution when evaluating patients with low titer MOG antibody even if they meet the additional supportive features proposed by the 2023 criteria. Further studies are needed to evaluate the 2023 criteria in larger cohorts and in the pediatric population.

BACKGROUND: Acute disseminated encephalomyelitis (ADEM) is a demyelinating immune-mediated condition of the central nervous system, whereas antiphospholipid antibody syndrome (APLA) is an autoimmune disorder accompanied by thrombosis and pregnancy-related problems. We present a unique case of a 30-year-old female with ADEM coexisting with APLA, highlighting the importance of early identification and specialized care.
METHODS: We present a case of a 30-year-old woman with a history of hypertension, multiple miscarriages, and non-compliance with medication, who presented with altered consciousness and weakness in all four limbs. Laboratory tests revealed positive anti-cardiolipin and lupus anticoagulant antibodies, confirming APLA. A neurological examination revealed increased limb tone, heightened reflexes, and extensor plantar responses. MRI revealed confluent white matter lesions that were consistent with ADEM. The patient received prompt treatment with intravenous methylprednisolone and then received oral prednisone, leading to a rapid improvement in neurological status.
CONCLUSIONS: The intricate interaction between ADEM and APLA remains enigmatic. The plausible connection between \"molecular mimicry\" and weakened blood-brain barrier, substantiated by antiphospholipid antibodies, may help explain their concurrent occurrence.
CONCLUSIONS: This case highlights the significance of early diagnosis and management of the rare and complex coexistence of ADEM and APLA to attain optimal outcomes, as well as the significance of careful examination for simultaneous autoimmune markers in individuals presenting with neurological disturbances.

Acute disseminated encephalomyelitis (ADEM) is a rare illness. It is characterized by different presentations like encephalopathy, seizures, hemiplegia, and visual symptoms. We present a patient who presented seizures and encephalopathy. Brain MRI showed symmetrical white and gray matter lesions. He was treated with acyclovir for viral encephalitis and given immunotherapy for ADEM. The radiological findings may be inconclusive in some cases, hence differential diagnosis of both viral encephalitis and ADEM needs to be considered. Early immunotherapy is required in such fulminant cases.

BACKGROUND: Acute disseminated encephalomyelitis is a rare acute demyelinating disease of the central nervous system (CNS). The pathogenesis remains unclear but is suspected to be autoimmune. High doses of methylprednisolone (HDMP) are currently considered standard of treatment. Plasmapheresis (PE) is typically given in steroid refractory cases. There is currently limited evidence supporting its use in ADEM.
METHODS: We report a 16-year-old girl with ADEM who improved rapidly after initiating PE.
RESULTS: The patient presented with acute onset of multifocal CNS symptoms, including encephalopathy, requiring intensive care unit management. Despite HDMP administration, her clinical condition continued to deteriorate. PE was therefore initiated on the same day as HDMP. Her clinical condition improved significantly following the first session. She was extubated and discharged from the intensive care unit the following day.
CONCLUSIONS: HDMP combined with PE may be an effective first-line treatment in patients with fulminant ADEM.

Thiamine pyrophosphate (TPP), the substrate of Thiamine pyrophosphate kinase (TPK), is an important cofactor in carbohydrate metabolism, specifically as a cofactor of the Pyruvate dehydrogenase complex (PDH) complex. The nervous system is particularly dependent on TPP due to its reliance on glucose metabolism. In this case, a four-year-old girl had a previously unreported pathogenic variant of the gene encoding TPK (TPK1) which presented as Thiamine metabolism dysfunction syndrome 5 (THMD5; OMIM 614458). She had been diagnosed with acute disseminated encephalomyelitis and autism spectrum disorder (ASD), and initially presented with fever and agitation following vaccinations. After follow-up with genetic testing, our patient was found to have compound heterozygous pathogenic variants of TPK1. After treatment with biotin and thiamine her clinical status improved, and her ASD features resolved. The presentation of our patient was consistent with previous reports and adds to the evidence that thiamine and biotin are effective treatments of TPK1 related metabolic deficiencies. The improvement of neurobehavioral symptoms in this case was marked, highlighting the importance of early identification and therapeutic intervention in this condition.