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ABSTRACT Incomplete Miller‐Fisher syndrome (MFS) is a rare autoimmune disorder affecting the nervous system. In contrast to classical MFS, its clinical manifestations are often atypical and may be characterized by the absence of certain features, such as extraocular muscle paralysis and the loss of tendon reflexes. The presence of anti‐GD3 antibodies is infrequently observed in these patients. This case report describes a patient diagnosed with incomplete MFS who exhibited positive anti‐GD3 antibody results and presented with pyramidal tract signs. This suggests that anti‐GD3 antibodies may play a role in the pathogenesis of some atypical forms of MFS, warranting attention in clinical practice.

Intravascular large B-cell lymphoma (IVLBCL) is a highly aggressive type of non-Hodgkin’s lymphoma. The clinical manifestations and imaging of this disease are nonspecific, making diagnosis challenging. We report a case of a patient with recurrent stroke-like symptoms, primarily characterized by hemiplegia, vertigo, ataxia, and proteinuria. Brain MRI revealed multiple cerebral infarctions, microbleeds, and meningeal enhancement. Ultimately, the patient was diagnosed with IVLBCL through a brain tissue biopsy, and involvement of the kidneys was suspected. We suggest considering IVLBCL in patients who present with recurrent stroke-like symptoms, fluctuating neurological deficits, the aforementioned MRI findings, and involvement of other organs. Additionally, central nervous system vasculitis(CNSV) may represent a transitional manifestation of the disease. Pathological biopsy is the gold standard for diagnosis. We hope that through this case, doctors can gain a deeper understanding of IVLBCL, enabling early diagnosis and timely treatment to improve prognosis.

BackgroundAutoimmune nodopathy (AN) has emerged as a novel diagnostic category that is pathologically different from classic chronic inflammatory demyelinating polyneuropathy. Clinical manifestations of AN include sensory or motor neuropathies, sensory ataxia, tremor, and cranial nerve involvement. AN with a serum-positive contactin-1 (CNTN1) antibody usually results in peripheral nerve demyelination. In this study, we reported a rare case of AN with CNTN1 antibodies characterized by the presence of CNTN1 antibodies in both serum and cerebrospinal fluid, which is associated with cerebellar dysarthria.MethodsA 25-year-old man was admitted to our hospital due to progressive dysarthria with limb tremors. The patient was initially diagnosed with peripheral neuropathy at a local hospital. Three years after onset, he was admitted to our hospital due to dysarthria, apparent limb tremor, and limb weakness. At that time, he was diagnosed with spinocerebellar ataxia. Eight years post-onset, during his second admission, his condition had notably deteriorated. His dysarthria had evolved to typical distinctive cerebellar characteristics, such as tremor, loud voice, stress, and interrupted articulation. Additionally, he experienced further progression in limb weakness and developed muscle atrophy in the distal limbs. Magnetic resonance imaging (MRI), nerve conduction studies (NCS), and autoimmune antibody tests were performed.ResultsThe results of the NCS suggested severe demyelination and even axonal damage to the peripheral nerves. MRI scans revealed diffuse thickening of bilateral cervical nerve roots, lumbosacral nerve roots, cauda equina nerve, and multiple intercostal nerve root sheath cysts. Furthermore, anti-CNTN1 antibody titers were 1:10 in the cerebrospinal fluid (CSF) and 1:100 in the serum. After one round of rituximab treatment, the patient showed significant improvement in limb weakness and dysarthria, and the CSF antibodies turned negative.ConclusionApart from peripheral neuropathies, cerebellar dysarthria (central nervous system involvement) should not be ignored in AN patients with CNTN1 antibodies.

ObjectiveTo report a case of autoimmune nodopathy (AN) with concurrent serum and CSF immunoglobulin (Ig)G4 anti-neurofascin 155 (NF155) and anti-GD1b antibodies.MethodsA 20-year-old male presented distal weakness of the 4 limbs, hypoesthesia, absent tendon reflexes and sensory ataxia. Nerve conduction studies (NCS), MRI, and autoantibody tests were performed.ResultsNCS revealed a diffuse demyelinating neuropathy in the peripheral nerve with motor and sensory involvement. MRI of the cervical and lumbar plexus showed diffuse enlargement. IgG4 anti-NF155 antibodies in both serum and CSF and IgG anti-GD1b antibodies in serum were positive. After treatment with IVIg, rituximab, and plasma exchange, the titer of the patient’s anti-NF155 antibodies decreased, but symptoms did not significantly improve.DiscussionThis patient presented a typical clinical feature of AN with serum and CSF anti-NF155 antibodies and serum anti-GD1b antibodies coexistent but poor response to IVIg, rituximab and plasma exchange. Early detection of antibodies may be helpful in both diagnosis and therapy of the disease. And prospective studies are necessary to demonstrate the potential role of anti-NF155 antibodies in CSF and help further understand this complex and heterogeneous disease.

Objective: To report a case of autoimmune nodopathy (AN) with concurrent serum and CSF immunoglobulin (Ig)G4 anti-neurofascin 155 (NF155) and anti-GD1b antibodies. Objective: To report a case of autoimmune nodopathy (AN) with concurrent serum and CSF immunoglobulin (Ig)G4 anti-neurofascin 155 (NF155) and anti-GD1b antibodies. Results: NCS revealed a diffuse demyelinating neuropathy in the peripheral nerve with motor and sensory involvement. MRI of the cervical and lumbar plexus showed diffuse enlargement. IgG4 anti-NF155 antibodies in both serum and CSF and IgG anti-GD1b antibodies in serum were positive. After treatment with IVIg, rituximab, and plasma exchange, the titer of the patient's anti-NF155 antibodies decreased, but symptoms did not significantly improve. Discussion: This patient presented a typical clinical feature of AN with serum and CSF anti-NF155 antibodies and serum anti-GD1b antibodies coexistent but poor response to IVIg, rituximab and plasma exchange. Early detection of antibodies may be helpful in both diagnosis and therapy of the disease. And prospective studies are necessary to demonstrate the potential role of anti-NF155 antibodies in CSF and help further understand this complex and heterogeneous disease. [ABSTRACT FROM AUTHOR]