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Annals of Indian Academy of Neurology May 2024Glial fibrillary acidic protein (GFAP) astrocytopathy is a rare autoimmune inflammatory disorder affecting the central nervous system, involving the meninges, brain...
Glial fibrillary acidic protein (GFAP) astrocytopathy is a rare autoimmune inflammatory disorder affecting the central nervous system, involving the meninges, brain parenchyma, and spinal cord. The distinctive radiologic feature observed on magnetic resonance imaging (MRI) is characterized by periventricular radial and linear contrast enhancement. This case report details a 45-year-old male who initially exhibited constitutional symptoms, followed by encephalitis, lower limb weakness, and urinary retention. The MRI findings revealed meningoencephalitis with longitudinal extensive myelitis. Notably, the cerebrospinal fluid analysis confirmed the presence of anti-GFAP antibodies.
PubMed: 38856160
DOI: 10.4103/aian.aian_1134_23 -
Cureus May 2024Longitudinally extensive myelitis with 15 or more vertebrae in length is extremely rare, with limited evidence regarding clinical features and therapeutic response. We...
Longitudinally extensive myelitis with 15 or more vertebrae in length is extremely rare, with limited evidence regarding clinical features and therapeutic response. We report a case of a 29-year-old male patient with extremely longitudinally extensive myelitis ultimately diagnosed as myelin oligodendrocyte glycoprotein-associated disease (MOGAD). The patient presented with an acute onset of meningismus, limb weakness, sensory disturbance below the C5 level, ataxia, and urinary retention. T2-weighted imaging on MRI showed an extremely longitudinally extensive spinal cord lesion ranging from C2 to the medullary conus, together with a left pontine lesion. Positive anti-myelin oligodendrocyte glycoprotein antibodies were serologically detected, which led to the diagnosis of MOGAD. Intravenous methylprednisolone followed by 1 mg/kg oral prednisolone with taper resulted in complete symptomatic and radiological resolution. The striking complete resolution despite the symptomatic and radiological severity observed in this case has been described in a few previously reported MOGAD cases. Extremely longitudinally extensive myelitis with excellent therapeutic response may be a characteristic presentation of MOGAD.
PubMed: 38854217
DOI: 10.7759/cureus.59938 -
Cureus May 2024Neuromyelitis optica spectrum disorder (NMOSD) is a rare central nervous system disease presenting as optic neuritis, myelitis, and brainstem syndromes. It may be...
Neuromyelitis optica spectrum disorder (NMOSD) is a rare central nervous system disease presenting as optic neuritis, myelitis, and brainstem syndromes. It may be aquaporin-4 seropositive, anti-myelin oligodendrocyte glycoprotein (MOG) antibody seropositive, or double seronegative. Double-seronegative NMOSD can pose a diagnostic and therapeutic challenge. Treatment typically aims to decrease the incidence of relapse, for which high-dose intravenous methylprednisolone is the first-line agent. Non-steroid treatments include azathioprine, mycophenolate mofetil, and rituximab. This case describes a 45-year-old female presenting with left arm numbness and weakness for three months. She had been previously diagnosed with optic neuritis in 2013 but was lost to follow-up. Progression of weakness warranted admission to the neurology department. Diagnostic work and imaging were suggestive of neuromyelitis optica. Tests for aquaporin-4, anti-MOG, immunoglobulin G, and immunoglobulin M in the cerebrospinal fluid were all negative. Initial treatment comprised methylprednisolone; however, due to the progression of symptoms, she was given two cycles of rituximab. Rituximab targets the CD20 antigen in B cells and is thought to reduce the risk of relapse and the severity of NMOSD. The patient's Barthel index score, expanded disability status scale score, and motor examination improved after two cycles of rituximab.
PubMed: 38854188
DOI: 10.7759/cureus.60004 -
European Journal of Case Reports in... 2024Bronchial artery embolization (BAE) is a procedure that aims to control bleeding from bronchial arteries in massive and chronic haemoptysis. It is considered to be a...
UNLABELLED
Bronchial artery embolization (BAE) is a procedure that aims to control bleeding from bronchial arteries in massive and chronic haemoptysis. It is considered to be a life-saving measure in severe life-threatening haemoptysis. Although it is minimally invasive and has a high success rate, it still carries a list of complications. These include post-embolisation syndrome, chest pain, back pain, dysphagia, vascular injury at the site of the embolisation leading to dissection, perforation, pseudoaneurysm and, very rarely, embolic infarction to non-target vessels. Stroke is one of the rare complications post BAE, and it can be severe and fatal. Few cases of stroke post BAE have been reported in the literature, and they were mainly due to posterior cerebral circulation infarction. Here, we report a case of a stroke post BAE due to massive middle cerebral artery (MCA) infarction and to our knowledge it seems to be the first reported case of MCA infarction post BAE. The discussion will cover the possible mechanisms of embolic passage, the outcome of the case including rehabilitation perspectives and the learning points. These will also highlight the importance of early recognition, which can save patients from a disabling stroke in the future.
LEARNING POINTS
Bronchial artery embolisation (BAE) carries a high risk of significant complications such as transverse myelitis, bronchial infraction, ischaemic colitis and stroke. While stroke remains one of the rarest complication post BAE, it may be under-reported or unrecognised.Close monitoring in post-BAE patients for any abnormal neurological signs that warrant urgent brain imaging, and early recognition can save patients from a disabling stroke by having the appropriate hyperactive stroke management plan including mechanical thrombectomy.
PubMed: 38846653
DOI: 10.12890/2024_004594 -
Intractable & Rare Diseases Research May 2024The Japanese Research Group for Neuro-infectious Diseases was founded in August 1996, and by 2004 it had evolved into the Japanese Society for Neuro-infectious Diseases....
The Japanese Research Group for Neuro-infectious Diseases was founded in August 1996, and by 2004 it had evolved into the Japanese Society for Neuro-infectious Diseases. The Society focuses on neuroinfectious conditions (., encephalitis/encephalopathy, myelitis, and meningitis), providing a venue for academic presentations and exchanges. Clinical guidelines for major neurological infectious diseases are also published by the Society, in order to meet the social demands of each era. Although the threat of herpes simplex encephalitis has declined due to acyclovir's introduction, the frequency of encephalitis or peripheral neuropathy caused by varicella-zoster virus is increasing. In Japan, prion disease, human T-cell leukemia virus-1 (HTLV-1)-associated myelopathy (HAM), subacute sclerosing panencephalitis (SSPE), and progressive multifocal leukoencephalopathy (PML) are designated as intractable diseases. The incidence of prion disease is 1.8/1,000,000 individuals, with the sporadic type accounting for 80%. Prion disease is fatal, and effective medications are awaited. HAM's prevalence is ~3/100,000 individuals, with a male-to-female ratio of 1:2-3. HAM is common in western Japan, including Kyushu and Okinawa. The prevalence of PML is rising with the spread of both immunosuppressive therapy for transplantation and treatment for multiple sclerosis. From late 2019 through 2020, the world faced a global outbreak of coronavirus disease 2019 (COVID-19) due to virus mutations, and the threat of new mutations persists. Close attention should be paid to the emergence of new neurological infections that could arise from abnormal weather patterns and/or a decline in immune function due to aging.
PubMed: 38836177
DOI: 10.5582/irdr.2024.01008 -
Frontiers in Medicine 2024Hansen's disease, or leprosy, is a disease characterized by dermatological and neurological disorders. A neural form also exists, in which peripheral neuropathy occurs...
Hansen's disease, or leprosy, is a disease characterized by dermatological and neurological disorders. A neural form also exists, in which peripheral neuropathy occurs in the absence of skin lesions. However, cases of leprosy that involve the central nervous system and proximal nerves are rare in the literature. We describe the case of an oligosymptomatic patient diagnosed with the neural form of leprosy with involvement of peripheral nerves, dorsal root ganglion, and cervical spinal cord in an atypical presentation of the disease. Through complementary examinations and nerve biopsies, the bacillus was identified, and treatment was subsequently initiated. This case highlights the importance of investigating the suspicion of leprosy, even in cases with atypical manifestations, as early diagnosis and treatment can reduce neurological damage and deformities.
PubMed: 38835799
DOI: 10.3389/fmed.2024.1400423 -
Neurology Jul 2024
Topics: Humans; Nystagmus, Pathologic; Paraparesis, Tropical Spastic; Male; Magnetic Resonance Imaging; Middle Aged; Female
PubMed: 38833644
DOI: 10.1212/WNL.0000000000209562 -
Balkan Medical Journal Jun 2024Optic neuritis, myelitis, and neuromyelitis optica spectrum disorder (NMOSD) have been associated with antibodies against myelin oligodendrocyte...
BACKGROUND
Optic neuritis, myelitis, and neuromyelitis optica spectrum disorder (NMOSD) have been associated with antibodies against myelin oligodendrocyte glycoprotein-immunoglobulin G (anti-MOG-IgG). Furthermore, patients with radiological and demographic features atypical for multiple sclerosis (MS) with optic neuritis and myelitis also demonstrate antibodies against aquaporin-4 and anti-MOG-IgG. However, data on the diagnosis, treatment, follow-up, and prognosis in patients with anti-MOG-IgG are limited.
AIMS
To evaluate the clinical, radiological, and demographic characteristics of patients with anti-MOG-IgG.
STUDY DESIGN
Multicenter, retrospective, observational study.
METHODS
Patients with blood samples demonstrating anti-MOG-IgG that had been evaluated at the Neuroimmunology laboratory at Ondokuz Mayıs University's Faculty of Medicine were included in the study.
RESULTS
Of the 104 patients with anti-MOG-IgG, 56.7% were women and 43.3% were men. Approximately 2.4% of the patients were diagnosed with MS, 15.8% with acute disseminated encephalomyelitis (ADEM), 39.4% with NMOSD, 31.3% with isolated optic neuritis, and 11.1% with isolated myelitis. Approximately 53.1% of patients with spinal involvement at clinical onset demonstrated a clinical course of NMOSD. Thereafter, 8.8% of these patients demonstrated a clinical course similar to MS and ADEM, and 28.1% demonstrated a clinical course of isolated myelitis. The response to acute attack treatment was lower and the disability was higher in patients aged > 40 years than patients aged < 40 years at clinical onset. Oligoclonal band was detected in 15.5% of the patients.
CONCLUSION
For patients with NMOSD and without anti-NMO antibodies, the diagnosis is supported by the presence of anti-MOG-IgG. Furthermore, advanced age at clinical onset, Expanded Disability Status Scale (EDSS) score at clinical onset, spinal cord involvement, and number of attacks may be negative prognostic factors in patients with anti-MOG-IgG.
PubMed: 38828767
DOI: 10.4274/balkanmedj.galenos.2024.2024-1-97 -
Infectious Diseases (London, England) May 2024Systematic treatment with intravenous acyclovir is usually given when varicella zoster virus (VZV) DNA is isolated in cerebrospinal fluid (CSF), indicating central...
BACKGROUND
Systematic treatment with intravenous acyclovir is usually given when varicella zoster virus (VZV) DNA is isolated in cerebrospinal fluid (CSF), indicating central nervous system (CNS) involvement. Our study aimed to describe therapeutic management and acute kidney injury (AKI) occurrence during acyclovir treatment of VZV infection with CNS involvement.
METHODS
Multicentre, retrospective study including all patients from 2010 to 2022 with VZV DNA in CSF. Patient management and outcomes were compared according to clinical presentation and indications for intravenous acyclovir: i) definite (encephalitis, myelitis or stroke, peripheral nervous system (PNS) with ≥ 2 roots, herpes zoster ≥ 3 dermatomes, immunosuppression), ii) questionable (1 or 2 dermatomes) or iii) no indication (other situations).
RESULTS
154 patients were included (median age 66 (interquartile range 43-77), 87 (56%) males); 60 (39%) had encephalitis, myelitis or stroke, 35 (23%) had PNS involvement, 37 (24%) had isolated meningitis, 14 (9%) had isolated cutaneous presentation, and 8 (5%) had other presentations. Overall, 128 (83%) received intravenous acyclovir for more than 72 h. AKI occurred in 57 (37%) patients. Finally, 42 (27%) and 25 (16%) patients had respectively no or a questionable indication for intravenous acyclovir, while 29 (69%) and 23 (92%) of them received it for more than 72 h, with AKI in 13 (35%) and 13 (52%) patients, respectively. In-hospital mortality was 12% ( = 18), and no deaths were reported in isolated meningitis.
CONCLUSIONS
Intravenous acyclovir is widely prescribed when VZV DNA is isolated in CSF, regardless of the clinical presentation, with a high rate of AKI. Further studies are needed to better define the value of intravenous acyclovir in isolated VZV meningitis.
PubMed: 38822453
DOI: 10.1080/23744235.2024.2355989 -
PloS One 2024Polio eradication is a current and common strategy throughout the globe. The study of the newly introduced inactivated poliovirus vaccine provides a grasp on the current...
INTRODUCTION
Polio eradication is a current and common strategy throughout the globe. The study of the newly introduced inactivated poliovirus vaccine provides a grasp on the current status of immunization and identifies any disparities in the implementation of the vaccine throughout Ethiopia. Thus, this study aimed to demonstrate the spatial distribution, coverage, and determinants of inactivated poliovirus vaccine immunization in Ethiopia.
METHOD
Spatial distribution and determinants of inactivated poliovirus vaccine immunization in Ethiopia were conducted using Ethiopian mini-demographic and health survey 2019 data. A total of 2,056 weighted children aged 12 to 35 months were included in the analysis. The association between the outcome and explanatory variables was determined by commuting the adjusted odds ratio at a 95% confidence interval. The p-value of less than 0.05 was used to declare factors as significantly associated with the inactivated poliovirus vaccine immunization.
RESULT
The weighted national coverage of inactivated poliovirus vaccine immunization in Ethiopia was 51.58% at a 95% confidence interval (49.42, 53.74). While the rates of inactivated poliovirus vaccine immunization were observed to be greater in Addis Ababa, Tigiray, Amahara, and Benishangul Gumuz provinces and lower in the Somali, Afar, and SNNPR provinces of Ethiopia, Antenatal care follow-up, place of delivery, place of residence, and region were significantly associated with inactivated poliovirus immunization in Ethiopia.
CONCLUSION
The distribution of inactivated poliovirus immunization was spatially variable across Ethiopia. Only about half of the children aged twelve to thirty-five months received the inactivated poliovirus vaccine in the country. The factors, both at the individual and community level, were significantly associated with inactivated poliovirus immunization. Therefore, policies and strategies could benefit from considering antenatal care follow-up, place of delivery, place of residence, and region while implementing inactivated poliovirus vaccine immunization.
Topics: Humans; Ethiopia; Poliovirus Vaccine, Inactivated; Female; Infant; Poliomyelitis; Male; Child, Preschool; Vaccination Coverage; Vaccination; Immunization Programs; Immunization
PubMed: 38820454
DOI: 10.1371/journal.pone.0301933