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The results of the IMbrave150 study have led to widespread use of the combination therapy of atezolizumab and bevacizumab as a first-line treatment for unresectable or metastatic hepatocellular carcinoma (HCC). Compared to traditional cytotoxic chemotherapy agents, immune checkpoint inhibitors show a spectrum of side effects ranging from mild side effects such as skin rash to potentially severe systemic effects such as myocarditis. We present a case of transverse myelitis diagnosed during the treatment of HCC with atezolizumab and bevacizumab combination therapy.
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Background@#The importance of establishing a regional stroke safety net for the treatment of acute stroke is increasingly emphasized. In this study, we analyzed effectiveness of transferring the suspected acute ischemic stroke (AIS) patients through the 1899-0215 hotline system from 119 ambulances and nearby hospitals. @*Methods@#From July 2015 to July 2022, we analyzed data from 632 suspected acute stroke patients through the hotline. Furthermore, we investigated the proportion of patients in the target population who were eligible for reperfusion therapy and compared cases transferred by 119 emergency services (EMS) with those transferred from nearby hospitals. @*Results@#Six hundred thirty-two suspected acute stroke patients were transferred to our stroke center through the 1899-0215 hotline system. The accuracy of diagnosing acute stroke among transferred patients is 81.0% for 119 EMS and 80.5% for cases transferred from nearby hospitals. Of the 200 patients transferred from hospitals, they were transferred from 47 nearby hospitals with an average transfer distance of 38.2 km and an average transfer time of 65 minutes. Among 291 patients diagnosed with AIS, The onset-to-door time was significantly shorter (p<0.01) for patients transferred by 119 EMS. The proportion of patients who received reperfusion therapy was significantly higher (p<0.01) for those transferred by 119 EMS. @*Conclusions@#The hotline service will become a useful tool for establishing a regional stroke network in the Busan area, and its effectiveness will be particularly useful for 119 EMS. Measures are needed in the Busan metropolitan region to ensure the speediness of transferring acute stroke patients between hospitals.
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Background@#and Purpose To understand the characteristics of Korean patients with anti-3-hydroxy-3-methylglutaryl-coenxyme A reductase (HMGCR) myopathy, we measured antiHMGCR antibodies and analyzed the clinical, radiological, and pathological features of patients with anti-HMGCR myopathy. @*Methods@#We measured titers of anti-HMGCR antibodies in the sera of 99 patients with inflammatory myopathy, 36 patients with genetic myopathy, and 63 healthy subjects using an enzyme-linked immunosorbent assay. We tested 16 myositis-specific autoantibodies (MSAs) in all patients with anti-HMGCR myopathy. @*Results@#Positivity for the anti-HMGCR antibody was observed in 17 (4 males and 13 females) of 99 patients with inflammatory myopathy. The median age at symptom onset was 60 years.Ten (59%) of the patients with anti-HMGCR positivity had taken statins. The titer of antiHMGCR antibodies was significantly higher in the statin-naïve group (median=230 U/mL, interquartile range=170–443 U/mL) than in the statin-exposed group (median=178 U/mL, interquartile range=105–210 U/mL, p=0.045). The most common symptom was proximal muscle weakness in 15 patients (88%), followed by myalgia in 9 (53%), neck weakness in 4 (24%), dysphagia in 3 (18%), and skin lesions in 2 (12%). The median titer of anti-HMGCR antibody was 202 U/mL. We found eight different MSAs in nine (53%) patients. The median disease duration from symptom onset to diagnosis was significantly shorter in the MSA-positive group than in the MSA-negative group (p=0.027). @*Conclusions@#Our study was the first to measure anti-HMGCR antibodies in inflammatory myopathy. It has provided new findings, including the suggestion of the coexistence of other MSAs in Korean patients.
ABSTRACT
GuillainBarré syndrome (GBS) is the inflammatory neuropathy that affects the myelin and nodal or paranodal areas of peripheral nerves. Immunoglobulin G GM1 antibody is well known as the cause of GBS associated with Campylobacter jejuni infection. However, the relationship between other specific infectious agents and autoantibodies is not yet well elucidated in patients with GBS. Recently we have experienced a case with GBS associated with antiGM1 and phosphatidic acid complex antibody that occurred after Shiga toxinproducing and enterotoxigenic Escherichia coli enteritis.
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Acute autonomic and sensory neuropathy (AASN) is very rare immune mediated neuropathy characterized by prominent dysautonomia and sensory involvement without motor weakness. Most of AASN patients have a rapid onset reaching its worst within four weeks like Guillain-Barré syndrome. The treatment response is variable. Recently, we experienced a patient diagnosed as AASN with progressive autonomic and sensory symptoms more than 1 year, and showed good response in immunotherapy.
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Facial nerve palsy is one of major accompanying features in Guillain-Barré syndrome (GBS). In most of the cases, facial weakness develops simultaneously with other symptoms such as motor weakness, sensory change and other cranial neuropathies. However, facial palsy also occurs after the nadir of neurological deficits or even after the beginning of limb weakness improvement, called delayed facial palsy (DFP). DFP has been reported in Miller Fisher syndrome, but it rarely found from the acute motor axonal neuropathy subtype of GBS. Recently, we experienced a patient who diagnosed acute motor axonal neuropathy accompanying with delayed facial diplegia.
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Reversible cerebral vasoconstriction syndrome (RCVS) is characterized by thunderclap headache with reversible vasoconstriction of the cerebral arteries. RCVS has been reported to occur in various clinical settings. However, RCVS triggered by blood transfusion is rare. A 50-year-old woman had severe anemia and received multiple blood transfusions. She developed thunderclap headache after transfusion. Cerebral artery vasoconstrictions were demonstrated by magnetic resonance angiography and transfemoral cerebral angiography. RCVS might be triggered by red blood cell transfusion in patients with severe anemia.
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In Guillain-Barré syndrome (GBS) and its variant, anti-GQ1b antibody has a pathogenic role for ophthalmoplegia. In addition, anti-GT1a antibody is related with lower cranial nerve involvement. This report describes a 60-year-old male patient with GBS manifesting with initially isolated dysphagia and subsequently developed ophthalmoplegia. Both immunoglobulin G type anti-GQ1b and anti-GT1a antibodies were detected in the patient’s serum. A mechanism regarding subsequent involvement of respective cranial nerves remains to be elucidated.
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In Guillain-Barré syndrome (GBS) and its variant, anti-GQ1b antibody has a pathogenic role for ophthalmoplegia. In addition, anti-GT1a antibody is related with lower cranial nerve involvement. This report describes a 60-year-old male patient with GBS manifesting with initially isolated dysphagia and subsequently developed ophthalmoplegia. Both immunoglobulin G type anti-GQ1b and anti-GT1a antibodies were detected in the patient’s serum. A mechanism regarding subsequent involvement of respective cranial nerves remains to be elucidated.
ABSTRACT
Guillain-Barré syndrome (GBS) is a representative form of post-infectious autoimmune neuropathy with heterogenous manifestations. It was originally considered as an ascending demyelinating polyneuropathy in Western countries. However, the discovery of anti-ganglioside antibodies on the basis of molecular mimicry theory could help us better understand various kinds of focal and regional variants as well as axonal type of GBS those were frequently found from Asian countries. Recent development of new techniques about anti-ganglioside complex antibodies is making more detailed descriptions for specific or unusual clinical manifestations. It has been regarded that GBS has good prognosis if treated properly as early as possible, but it still shows high mortality and morbidity rate with frequent long term neurologic and medical complications. Unfortunately, there are only two options for medical treatment, intravenous immunoglobulin and plasmapheresis, for the last 100 years. Several clinical studies on new immunotherapy targeting complement activating system with background of molecular mimicry using animal model are underway. We hope that these new treatments will be helpful for the future patients.
Subject(s)
Humans , Antibodies , Asian People , Axons , Complement System Proteins , Gangliosides , Guillain-Barre Syndrome , Hope , Immunoglobulins , Immunotherapy , Miller Fisher Syndrome , Models, Animal , Molecular Mimicry , Mortality , Plasmapheresis , Polyneuropathies , PrognosisABSTRACT
BACKGROUND AND PURPOSE: The most-common initial manifestation of Miller Fisher syndrome (MFS) is diplopia due to acute ophthalmoplegia. However, few studies have focused on ocular motility findings in MFS. This study aimed to determine the pattern of extraocular muscle (EOM) paresis in MFS patients. METHODS: We consecutively recruited MFS patients who presented with ophthalmoplegia between 2010 and 2015. The involved EOMs and the strabismus pattern in the primary position were analyzed. Antecedent infections, other involved cranial nerves, and laboratory findings were also reviewed. We compared the characteristics of the patients according to the severity of ophthalmoplegia between complete ophthalmoplegia (CO) and incomplete ophthalmoplegia (IO). RESULTS: Twenty-five patients (15 males and 10 females) with bilateral ophthalmoplegia were included in the study. The most-involved and last-to-recover EOM was the lateral rectus muscle. CO and IO were observed in 11 and 14 patients, respectively. The patients were aged 59.0±18.4 years (mean±SD) in the CO group and 24.9±7.4 years in the IO group (p<0.01), and comprised 63.6% and 21.4% females, respectively (p=0.049). Elevated cerebrospinal fluid protein was identified in 60.0% of patients with CO and 7.7% of patients with IO (p=0.019) for a mean follow-up time from the initial symptom onset of 3.7 days. CONCLUSIONS: The lateral rectus muscle is the most-involved and last-to-recover EOM in ophthalmoplegia. The CO patients were much older and were more likely to be female and have an elevation of cerebrospinal fluid protein than the IO patients.
Subject(s)
Female , Humans , Male , Cerebrospinal Fluid , Cranial Nerves , Diplopia , Follow-Up Studies , Guillain-Barre Syndrome , Jupiter , Miller Fisher Syndrome , Ophthalmoplegia , Paresis , StrabismusABSTRACT
BACKGROUND AND PURPOSE: Antiganglioside antibodies are known to play a pathogenic role in Guillain-Barré syndrome (GBS). Either an immunoglobulin (Ig)G- or IgM-type anti-GM2 antibody is detected in rare cases in GBS patients. However, the specific pathogenic role of these antibodies in GBS has not been reported previously. This study aimed to define and characterize the clinical spectrum of GBS with anti-GM2 positivity. METHODS: We reviewed the database of the Dong-A University Neuroimmunology Team, which has collected sera of GBS and its variants from more than 40 general and university-based hospitals in Korea. Detailed information about the involved patients was often obtained and then corrected by the charge doctor applying additional questionnaires. RESULTS: Four patients with acute monophasic peripheral neuropathy or cranial neuropathy with isolated IgM-type anti-GM2-antibody positivity were recruited. In addition, IgG-type anti-GM2 antibody was solely detected in the sera of another four patients. The IgM-positive group comprised heterogeneous syndromes: two cases of acute motor axonal neuropathy, one of acute inflammatory demyelinating polyneuropathy, and one of isolated facial diplegia. In contrast, all of the cases enrolled in the IgG-positive group manifested with dizziness with or without oculomotor palsy due to cranial neuropathy syndrome. CONCLUSIONS: This study has identified that anti-GM2 antibody can be found in various subtypes of GBS and its variants in rare cases. Compared to the clinical heterogeneity of the IgM-positive group, the IgG-positive group can be characterized by cranial-dominant GBS variants presenting mainly with oculomotor and vestibular dysfunctions.
Subject(s)
Humans , Antibodies , Axons , Cranial Nerve Diseases , Dizziness , Guillain-Barre Syndrome , Immunoglobulins , Korea , Paralysis , Peripheral Nervous System Diseases , Population CharacteristicsABSTRACT
Spastic paraparesis is caused by various etiologies such as autoimmune, infection, genetic and metabolic disorder. Adrenomyeloneuropathy (AMN) is very rare but one of important causes in spastic paraparesis. We experienced a patient presenting with adult-onset progressive spastic paraparesis, who was diagnosed as AMN with hemizygous c.431C>T (p.A144V), a novel mutation in exon1. The level of very long chain fatty acid should be included in diagnostic work-up for patients presenting with adult-onset progressive spastic paraparesis.
Subject(s)
Humans , Adrenoleukodystrophy , Muscle Spasticity , Paraparesis, SpasticABSTRACT
BACKGROUND: Recently, anti-ganglioside complex (GSC) antibodies were discovered among the various subtypes of Guillain-Barré syndrome. GSC is the novel glycoepitopes formed by two individual ganglioside molecules. CASE REPORT: We present a 36-year-old man with overlap Miller Fisher syndrome and acute bulbar palsy who had anti-GSC antibody that provided diagnostic robustness. CONCLUSION: Anti-GSC testing could be considered important in patients who show atypical manifestation with negative antibody reaction against each constituent ganglioside.
Subject(s)
Adult , Humans , Antibodies , Bulbar Palsy, Progressive , Gangliosides , Guillain-Barre Syndrome , Miller Fisher SyndromeABSTRACT
The vertebrate neuromuscular junction (NMJ) is considered as a “tripartite synapse” consisting of a motor axon terminal, a muscle endplate, and terminal Schwann cells that envelope the motor axon terminal. The neuregulin 1 (NRG1)-ErbB2 signaling pathway plays an important role in the development of the NMJ. We previously showed that Grb2-associated binder 1 (Gab1), a scaffolding mediator of receptor tyrosine kinase signaling, is required for NRG1-induced peripheral nerve myelination. Here, we determined the role of Gab1 in the development of the NMJ using muscle-specific conditional Gab1 knockout mice. The mutant mice showed delayed postnatal maturation of the NMJ. Furthermore, the selective loss of the gab1 gene in terminal Schwann cells produced delayed synaptic elimination with abnormal morphology of the motor endplate, suggesting that Gab1 in both muscles and terminal Schwann cells is required for proper NMJ development. Gab1 in terminal Schwann cells appeared to regulate the number and process elongation of terminal Schwann cells during synaptic elimination. However, Gab2 knockout mice did not show any defects in the development of the NMJ. Considering the role of Gab1 in postnatal peripheral nerve myelination, our findings suggest that Gab1 is a pleiotropic and important component of NRG1 signals during postnatal development of the peripheral neuromuscular system.
Subject(s)
Animals , Mice , Mice, Knockout , Motor Endplate , Muscle, Skeletal , Muscles , Myelin Sheath , Neuregulin-1 , Neuromuscular Junction , Peripheral Nerves , Presynaptic Terminals , Protein-Tyrosine Kinases , Schwann Cells , Synapses , VertebratesABSTRACT
Acute disseminated encephalomyelitis (ADEM) and Guillain-Barré syndrome (GBS) are both rare post-infectious neurological disorders. The co-existence of these conditions has often been reported despite of low incidence. We describe a 20-year-old male, who presented with acute flaccid paralysis and encephalopathy. The patient showed reversible MRI lesions suggesting ADEM. This case showed anti-GT1a IgG and anti-GM1 IgM antibodies positivity. We suggest that certain immunogenicity within central and peripheral nervous system may share a common autoimmune process during the disease course.
Subject(s)
Humans , Male , Young Adult , Antibodies , Brain Diseases , Encephalomyelitis, Acute Disseminated , Gangliosides , Guillain-Barre Syndrome , Immunoglobulin G , Immunoglobulin M , Incidence , Magnetic Resonance Imaging , Nervous System Diseases , Paralysis , Peripheral Nervous SystemABSTRACT
PURPOSE: In this study, a case of toxic encephalopathy and optic neuropathy due to methyl bromide poisoning is reported. CASE SUMMARY: A 31-year-old male presented with dysarthria, gait disturbance and bilateral visual impairment. He was treated with intravenous methylprednisolone for bilateral optic neuritis 1 year prior. He previously worked in a fumigation warehouse and was exposed to methyl bromide in the past 3 years. His corrected visual acuity was 20/30 in both eyes. The patient had reduced color vision and enlarged central scotoma in both eyes. His mentality was alert but exhibited slow response, ataxia and dysarthria. Brain magnetic resonance imaging (MRI) revealed high signals in the brainstem, cerebellum and midbrain. His serum and urine methyl bromide concentrations were significantly elevated. The patient was treated with intravenous methylprednisolone 1.0 g/day for 5 days. MRI showed resolution of the multiple brain lesions observed previously. Ten days after steroid therapy, his visual acuity was 20/20 in both eyes and his neurologic manifestations were completely recovered at 2 months after treatment. CONCLUSIONS: Taking a detailed occupational history is necessary in patients with optic neuropathy. The probability of toxic optic neuropathy should be considered when patients are exposed to toxic materials.
Subject(s)
Adult , Humans , Male , Ataxia , Brain , Brain Stem , Cerebellum , Color Vision , Dysarthria , Fumigation , Gait , Magnetic Resonance Imaging , Mesencephalon , Methylprednisolone , Neurologic Manifestations , Neurotoxicity Syndromes , Optic Nerve Diseases , Optic Neuritis , Poisoning , Scotoma , Vision Disorders , Visual AcuityABSTRACT
No abstract available.
Subject(s)
Diabetic Neuropathies , Lumbosacral Plexus , Magnetic Resonance ImagingABSTRACT
Alternating recurrent painful ophthalmoplegia is caused by various neurological conditions including Tolosa-Hunt syndrome, sellar mass, and parasagittal meningioma. We experienced a rare case of recurrent painful ophthalmoplegia occurring on the contralateral side as a manifestation of idiopathic hypertrophic tentorial pachymeningitis. We propose that idiopathic hypertrophic pachymeningitis should be considered in the differential diagnosis of alternating recurrent painful ophthalmoplegia.