Optic neuritis in Guillain-Barré syndrome: a case report and literature review / 中华神经科杂志

Objective:To summarize the clinical characteristics of patients with Guillain-Barré syndrome (GBS) complicated with optic neuritis (ON).Methods:The clinical data of a patient with GBS complicated with ON, who admitted to the Department of Neurology, the First Hospital of Shanxi Medical University in December 2021, were collected, including demographic characteristics, clinical symptoms and signs, laboratory and electrophysiological data, and results of fundus color films. The patients with GBS complicated with ON reported in the literature were also reviewed.Results:A 40-year-old female patient with GBS was diagnosed by the results of electromyography and cerebrospinal fluid tests combining with the history and signs, who was treated with intravenous immunoglobulin on the 3rd day after onset. On the 8th day, her muscle strength improved significantly. However, on the 12th day, the visual field darkened, and on the 19th day, the vision decreased significantly (oculus dexter visual acuity 0.2, oculus sinister visual acuity 0.1 +1) with bilateral papilloedema, a relative afferent pupillary defect and delayed P100 response of the visual evoked potential. Obvious abnormality was not noted in optic nerve magnetic resonance imaging. Thus ON was diagnosed and treated with pulse methylprednisolone therapy. After 8 days of treatment, the visual acuity was completely recovered and there was no abnormality in the ocular fundus. A total of 28 cases of GBS complicated with ON (including the present patient) were reported in the literature. The age of onset was mostly 20-60 years, and there was no gender preference. Mycoplasma pneumoniae was the most common premorbid pathogen and was identified in 7 of the 10 cases in which the causative agent was described. ON usually involved both sides, and 21 of 28 patients had bilateral optic nerves involved. GBS preceded ON or both occurred simultaneously in the majority of patients; GBS preceded ON in 14 of 28 patients, and both occurred simultaneously in 10 of 28 patients. All patients responded well to immunotherapy, and vision was completely recovered in 20 patients. Conclusions:GBS complicated with ON is rare. Attention should be paid to the loss of vision in patients with GBS. Relevant examinations should be completed as soon as possible and immunotherapy should be given.

Diagnosis and treatment of patients with IgA nephropathy in Japan

Chronic kidney disease (CKD) is a worldwide public health problem that affects millions of people from all racial and ethnic groups. Although CKD is not one specific disease, it is a comprehensive syndrome that includes IgA nephropathy. As reported by the Japanese Society of Nephrology, 13.0 million people have CKD. In Japan, major causes of end-stage kidney disease are type 2 diabetic nephropathy, chronic glomerulonephritis, especially IgA nephropathy, hypertensive nephrosclerosis, and polycystic kidney disease. IgA nephropathy is characterized by polymeric IgA1 with aberrant galactosylation (galactose-deficient IgA1) increased in the blood and deposited in the glomerular mesangial areas, as well as partially in the capillary walls. The tonsils are important as one of the responsible regions in this disease. The clarification of the mechanism of galactose-deficient IgA1 production will pave the way for the development of novel therapies. The results of future research are eagerly awaited. At present, the most important therapeutic goals in patients with IgA nephropathy are the control of hypertension, the decrease of urinary protein excretion, and the inhibition of progression to end-stage kidney disease. Several investigators have reported that renin–angiotensin–aldosterone system inhibitors reduce levels of urinary protein excretion and preserve renal function in patients with IgA nephropathy. In Japan, tonsillectomy and steroid pulse therapy are more effective for patients with IgA nephropathy.

Rapidly resolved IgG4-related retroperitoneal fibrosis after steroid pulse therapy / 영남의대학술지

Retroperitoneal fibrosis (RF) is a disorder characterized by the presence of a retroperitoneal mass and concurrent systemic inflammation. Some cases of RF are recognized as belonging to the spectrum of immunoglobulin G4-related disease (IgG4-RD). Glucocorticoids are highly effective for treatment of retroperitoneal fibrosis, although the optimal dose and duration of therapy have not been established. An initial dose of prednisone (40-60 mg) daily is usually administered with a tapering scheme. We report on a 55-year-old man diagnosed with IgG4-related RF and successfully treated with a 3-day course of daily 250 mg (4 mg/kg) intravenous methylprednisolone, which resulted in the prompt resolution of urinary obstruction and systemic symptoms.

Efficacy of Plasma Exchange Therapy for Steroid-Unresponsive Neuromyelitis Optica

PURPOSE: To report the therapeutic efficacy of plasma exchange therapy on steroid-unresponsive neuromyelitisoptica (NMO) patients. CASE SUMMARY: Three patients who had not achieved improvement of visual acuity and visual field after high steroid pulse therapy after optic neuritis visited our clinic with decreasing visual acuity combined with eye pain in the other eye. All patients were diagnosed as neuromyelitisoptica (NMO) based on the presence of NMO-IgG antibody and optic nerve enhancing in contrast-enhanced orbital magnetic resonance imaging (MRI). Recurrent optic neuritis was observed. Steroid pulse retreatment was started but visual acuity was not improved in all patients. The patients received plasma exchange therapy, followed by immune suppression therapy. All patients showed improved visual acuity and restored visual field promptly without recurrence of neuromyelitisoptica. CONCLUSIONS: In case of steroid-unresponsive neuromyelitisoptica, plasma exchange should be promptly considered as the treatment of choice.

A case of Churg–Strauss syndrome and central retinal artery occlusion with good visual recovery.

Here we report a case of Churg–Strauss syndrome (CSS) and central retinal artery occlusion (CRAO), with good visual recovery. A 58-year-old Japanese man with CSS experienced acute painless loss of vision in his right eye. CRAO was diagnosed by fundoscopic findings (retinal whitening with a cherry-red spot). Steroid pulse therapy (methylprednisolone at 1 g daily for 3 days) followed by combined treatment with prednisolone (30 mg/day) and cyclophosphamide (150 mg/day) was administered; his visual acuity recovered to 20/30 in 1 month, and no recurrence has occurred for 1 year. Steroid pulse therapy may be effective for CRAO in CSS patients.

Chronic allograft injury by subclinical borderline change: evidence from serial protocol biopsies in kidney transplantation / 대한외과학회지

PURPOSE: This study investigated the impact of subclinical borderline changes on the development of chronic allograft injury in patients using a modern immunosuppression protocol. METHODS: Seventy patients with stable renal allograft function and who underwent protocol biopsies at implantation, 10 days and 1 year after transplantation were included and classified based on biopsy findings at day 10. The no rejection (NR) group included 33 patients with no acute rejection. The treatment (Tx) group included 21 patients with borderline changes following steroid pulse therapy, and the nontreatment (NTx) group included 16 patients with borderline changes nontreated. RESULTS: The Banff Chronicity Score (BChS) and modified BChS (MBChS) were not different among the three groups at implantation (P = 0.48) or on day 10 (P = 0.96). Surprisingly, the NTx group had more prominent chronic scores at the 1-year biopsy, including BChS (3.07 +/- 1.33, P = 0.005) and MBChS (3.14 +/- 1.41, P = 0.008) than those in the Tx and NR group, and deterioration of BChS was more noticeable in the NTx group (P = 0.037), although renal function was stable (P = 0.66). No difference in chronic injury scores was observed between the Tx and NR groups at the 1-year biopsy. CONCLUSION: Subclinical borderline changes can be a risk factor for chronic allograft injury and should be considered for antirejection therapy.

A Case of Moyamoya Disease Initially Presenting as Anterior Ischemic Optic Neuropathy

PURPOSE: To present a case of a patient with decreased visual acuity and anterior ischemic optic neuropathy diagnosed with advanced Moyamoya disease. CASE SUMMARY: A 46-year-old woman presented sudden episodes of headache and decreased visual acuity. On her first visit, her best corrected visual acuity was 20/25 in the right eye and 20/70 in the left eye. The left eye pupil was dilated with a relative afferent papillary defect. Fundus examinations revealed disc swelling in the left eye. After being admitted, steroid pulse therapy was started and Magnetic Resonance Imaging (MRI) studies revealed Moyamoya disease. The diagnosis was confirmed via Magnetic Resonance Angiography (MRA). After steroid pulse therapy, the headaches and visual acuity improved and the patient is continuing follow-up visits at neurosurgery and ophthalmology clinics. CONCLUSIONS: Patients with Moyamoya disease may initially present symptoms and signs of anterior ischemic optic neuropathy.

A Case of Reversible Posterior Leukoencephalopathy Syndrome during Methylprednisolone Pulse and Cyclophosphamide Therapy in a Child with Nephrotic Syndrome

The syndrome of reversible posterior leukoencephalopathy syndrome(RPLS) is characterized clinically by acute neurologic signs such as headache, vomiting, confusion, seizures, and visual abnormalities. Radiologically, abnormalities consistent with reversible white matter edema in the occipital and parietal lobes are characteristic. RPLS has often been associated with various systemic disorders, such as hypertensive encephalopathy, eclampsia, and the use of intravenous or intrathecal immunosuppressive drugs. We report a case of RPLS that occurred after intravenous steroid pulse therapy and treatment with oral cyclophosphamide in a child with nephrotic syndrome, and we emphasize the importance of early recognition of RPLS in the treatment of nephrotic syndrome and appropriate management to prevent permanent neurologic disability.

Gastric Ulcer Perforation in Heart-Lung Transplant Patient: A Successful Case of Early Surgical Intervention and Management

Gastrointestinal complications may follow organ transplantation. A patient who underwent heart lung transplantation due to patent ductus arteriosus and Eisenmenger's syndrome had an episode of acute cardiac rejection and was treated with a bolus injection of methylprednisolone followed by a high oral dose of prednisone. On the 22nd postoperative day, the patient complained of acute abdominal pain with muscular rigidity and a plain chest x-ray showed free air in the right subdiaphragmatic area. Under the suspicion of bowel perforation, an emergency laparotomy was performed and the perforated stomach had a wedge-shaped resection that included the perforation. Following the laparotomy, the postoperative course was uneventful and the patient was discharged on post-laparotomy day 10.

Two Cases of Transient Cardiac Attacks Related to Steroid Pulse Therapy

No abstract available.

A Case of Chronic Myelogenous Leukemia Involving Retina and Optic Nerve

PURPOSE: We report a case of chronic myelogenous leukemia(CML) involving the retina and optic nerve, in which visual acuity had been improved after radiation therapy combined with steroid pulse therapy. METHODS: In a 44-year-old CML patient with central nervous system involvement who presented with retinal infiltration, initial uncorrected visual acuity was 1.0 in the right eye, 0.9 in the left eye. The patient was given 1% prednisolone acetate eye drops 4 times a day and 40mg of oral prednisolone a day. One month later, papilledema and exudative retinal detachment was developed in the left eye and we raised oral prednisolone dosage to 90mg a day. In spite of those kinds of treatment, papilledema in left eye and retinal infiltration in both eyes were much more aggravated at two months. So we used high-dose steroid(solumedrol 125mg qid) parenterally for initial 3 days followed by tapered oral dosage(prednisolone 60mg/day for 11 days, tapered with 10mg/day and maintain with 10mg/day) and performed simultaneously radiation therapy (total 2340 rads, 13 times) on both eyes through 2 weeks. RESULTS: Uncorrected visual acuity was decreased to 0.3 in the left eye at one month and to 0.6 in the right eye and 0.08 in the left eye at two months, and improved to 1.0 in the right eye and 0.5 in the left eye. Gradual resolution was noted in retinal and optic disc abnormalities at one month after high-dose steroid therapy combined with radiation therapy. CONCLUSIONS: This case suggests that visual acuity in CML patient with the retinal and optic nerve involvement can be improved by using radiation therapy combined with steroid pulse therapy.