A Case With IgG4-Related Spinal Pachymeningitis Causing Spinal Cord Compression.

Immunoglobulin G4 (IgG4)-related disease is a systemic disease characterized by sclerosing lesions and an increased serum IgG4 level. This condition can involve any organ, but IgG4-related spinal pachymeningitis is relatively rare. In the current study, we report a case of spinal cord compression caused by IgG4-related spinal pachymeningitis. A 39-year-old man presented to us with a 15-day history of back pain and a 3-day history of dysuresia, exacerbated by weakness in the lower extremities for 2 days. Cervical magnetic resonance imaging (MRI) showed strip-shaped abnormal signals along the anterior and posterior borders of the spinal cord at the C5-T4 levels. The IgG level in cerebrospinal fluid was 718.0 mg/L. Thoracic MRI revealed strip-shaped abnormal signals with remarkable enhancement along the anterior and posterior borders of the dural sac at the T1-T6 levels. Histopathological examination confirmed IgG4-related spinal pachymeningitis. The symptoms worsened rapidly, and surgical resection of the space-occupying lesion in the vertebral canal was performed for spinal decompression. Corticosteroid therapy was administered, and the patient's motor functions were mildly improved. IgG4-related disease can manifest as spinal pachymeningitis and cause spinal cord compression. Clinicians should be aware of this rare condition, and early diagnosis, timely surgical decompression, and appropriate corticosteroid therapy should be highlighted.

Anti-GQ1b antibody syndrome presenting with visual deterioration as the initial symptom: A case report.

RATIONALE: Anti-GQ1b antibody syndrome refers to a distinct variant of Guillain- Barré syndrome. Involvement of the optic nerve in anti-GQ1b antibody syndrome is extremely rare. PATIENT CONCERNS: Here, we report a case of anti-GQ1b antibody syndrome presenting with visual deterioration as the initial symptom. A 73-year-old man presented with a 5-day history of bilateral blurred vision and ptosis. He had a previous history of diarrhea starting 10 days before admission. Physical examination showed visual deterioration, ophthalmoplegia, and peripheral facial paralysis. Testing of both serum and cerebrospinal fluid was positive for anti-GQ1b immunoglobulin G antibodies and negative for anti-aquaporin 4antibodies. DIAGNOSIS: Anti-GQ1b antibody syndrome. INTERVENTIONS: The patient was treated with intravenous methylprednisolone and human immunoglobulin. OUTCOMES: After a 20-day follow-up, the patient's condition took a favorable turn. LESSONS: This case reminds us that anti-GQ1b antibody syndrome should be suspected in patients with visual deterioration and preceding infection.

A rare case of tumor-mimicking primary angiitis of the central nervous system.

Primary angiitis of the central nervous system (PACNS) is a rare, but severe vascular disease. The present study reports the case of a 42-year-old male who developed PACNS. Magnetic resonance imaging (MRI) scans initially led to a misleading diagnosis of malignant glioma, and surgery was performed. The mass was resected, and a pathological examination confirmed a cerebral vasculitis. Single therapy with high doses of steroid did not improve the patient's condition, while a subsequent lesion appeared on the opposite side one year later. Combined therapy with methylprednisone and cyclophosphamide resulted in a great improvement for the patient. No relapse occurred during one year's follow-up. Although a tumor-mimicking PACNS has no established imaging features, a diagnosis of tumor-mimicking PACNS should be suspected when the MRI reveals inappropriate presentations of a tumor. Greater awareness of this potential manifestation of PACNS may facilitate more prompt diagnosis and treatment.

Anti-leucine-rich glioma-inactivated 1 limbic encephalitis: A case report and literature review.

This study describes the case of a 41-year-old woman admitted for anterograde memory loss, right facial grimacing and right arm posturing that had begun 1 month previously. Cranial magnetic resonance-diffusion weighted imaging and -fluid-attenuated inversion recovery imaging revealed a hyperintense signal in the left hippocampus and right basal ganglia, but no contrast enhancement. An electroencephalogram revealed rhythmic sharp and slow waves and rhythmic θ build-ups in the left temporal area. Single-photon emission computed tomography showed increased regional blood flow perfusion in the left cerebral frontal lobe and the right basal ganglia. The cerebrospinal fluid was normal, with the exception of the presence of leucine-rich glioma-inactivated 1 (LGI1) antibodies, and LGI1 antibodies were also found in the blood serum. The presence of the antibodies, the faciobrachial dystonic seizures (FBDSs) and the memory loss indicated limbic encephalitis. After 3 months of immunotherapy, the patient was free from epileptic seizures and had undergone a partial memory restoration. FBDSs alone justify the immediate initiation of immunotherapy, even prior to laboratory confirmation of the disease, as early treatment limits the duration of the illness.

A rare case of Primary Central Nervous System Lymphoma initially diagnosed as demyelinating encephalopathy.

We report a case of histopathologically-confirmed primary central nervous system lymphoma who was initially diagnosed as demyelinating encephalopathy. A 58-year-old woman was admitted with confusion and left hemiparesis. Head MR showed abnormal flaky hypointense T1 and hyperintense T2 signals at right thalamus, splenium of corpus callosum, bilateral cerebral peduncle, pons, medulla oblongata, basal ganglia and right corona radiata. Her mental status improved a little and she was discharged from hospital after neuroprotective treatment. 10 days after her discharge, her confusion appeared again with hallucination and unsteady walking. Pathological examination revealed non-Hodgkin's lymphoma (WHO classification: DLBCL). The patient continued to deteriorate after the surgery and died 10 days later.

Familial cerebral cavernous angiomas: clinical and genetic features in a Chinese family with a frame-shift mutation in the CCM1 gene (krit1).

A few cases of cerebral cavernous malformation (CCM) have been reported in Chinese families with different mutations during the past decade. Herein, we report a case of CCM in a proband in a Chinese family, for whom the mutation type of the CCM remains to be identified. The proband of the family presented a range of clinical symptoms and features that included paralysis, aphasia, multiple lesions in the brain, and cutaneous capillary-venous malformations. PCR was performed to amplify all of the coding exons of the three CCM genes (CCM1, CCM2, and CCM3) in the proband and revealed a heterozygous T deletion in exon 15 (c.1542delT) of CCM1 gene. Targeted mutation analysis in family members demonstrated that this mutation segregated with the disease in the family. This is the first report of a heterozygous CCM1 deletion mutation. Our findings provide a new CCM gene mutation profile in a Chinese family which will be of significance in genetic counseling for CCM.

A case of primary systemic necrotizing vasculitis presenting primarily with neurologic involvement.

Systemic necrotizing vasculitis (SNV) is a type of vasculitis that presents with necrosis, predominantly involving large, medium-sized and small arteries. Peripheral neuropathy is a major clinical feature of the primary and secondary systemic vasculitides, and is often observed during the early phases of the disease, causing axonal neuropathy. The prevalence of central nervous system (CNS) involvement ranges from 4% to 45%. Encephalopathy, focal neurological deficits, and seizures are the most common manifestations and usually occur late during the course of SNV. In this report, we describe a 61-year-old woman with SNV who had both CNS and peripheral nervous system vasculitic involvement. We also discuss the pathophysiology of nervous system involvement in patients with SNV.

[Concentration increase in Hcy and anti-CCP antibody in the serum of depression rat model induced by chronic unpredictable mild stress].

OBJECTIVE: To observe the change of serum homocysteine (Hcy) and anti-citrullinated peptide (CCP) antibody concentration in depression rat model induced by chronic unpredictable mild stress (CUMS), and to explore the immunologic mechanism of depression and the relation between depression and its autoimmunity. METHODS: Sixty adult male SD rats were randomly divided into 2 groups, 30 rats in each group, which were divided into 3 subgroups: a normal control group, a model group and a fluoxetinetreated group. The depression rat model was established under CUMS and seperated feeding, after which, open field, sugar consumption and forced swimming test were applied in the first group. After the blood was taken in the second group of rats through heart puncture, the level of serum Hcy was detected by enzymatic cycling assay and serum anti-CCP antibody by ELISA. RESULTS: Compared with the control group and the fluoxetine treatment group, spontaneous activity and sucrose consumption and preference percentage of the rats in the model group significantly reduced, while the immobility time in forced swimming test and the level of Hcy and anti-CCP antibody in the rat serum significantly increased. CONCLUSION: Immunity inflammation and autoimmune reaction exist in CUMS depression model rats, and fluoxetine treatment can improve these immune response.