Background: Seizures in patients with systemic lupus erythematosus (SLE) are uncommon but life-threatening; mortality rate is 25-29%. Seizure in a person with lupus may be due to lupus itself or other conditions. There are no published studies describing the causes and outcomes of seizures in Filipino patients with lupus. Objective: To describe the causes and outcomes of seizure in a cohort of patients with lupus seen at Philippine General Hospital. Methodology: We reviewed the medical records of patients with SLE) with a documented seizure and admitted between January 2016 and April 2019. History, physical examination and laboratory findings, and clinical course were obtained. Results: We included 29 patients with 31 seizure events. They were all women, mostly single, of low socio-economic status, and had poor functional capacity. Lupus was active in 77.4% (24/31), commonly with mucocutaneous or hematologic manifestations. Seizures were generalized in 87 % (27/31). Prior to seizure, one-third had headache, fever, and vomiting. There were no neurologic localizing signs. Twenty-four seizure events (77%) occurred among patients with active lupus; 16 (67%) was attributed to neuropsychiatric systemic lupus erythematosus (NPSLE) and eight (33%) to other causes: infection (tuberculous meningitis and septic encephalopathy), posterior reversible encephalopathy syndrome (PRES), uremia, arrhythmia, and eclampsia. Seven seizures in inactive lupus were not SLE-related. Mortality rate was 28%; infection was the most common cause. Seizure resolved in 97%. Mean duration of hospitalization was 26.7 days. Patients were discharged improved from 19 seizure events (18 patients); 14 had follow-up consultations, three were readmitted. There was no seizure recurrence within 30 days of discharge. There was improvement in functional capacity. Conclusion The most common cause of seizure was NPSLE, followed by infection. Despite high rates of complete seizure resolution, poor outcomes were noted in almost half of the patients. Prolonged hospitalization was common. A high rate of mortality was observed. Infection was the most common cause of mortality.
Lupus Vasculitis, Central Nervous System

Humans ; Integrative Medicine ; Lupus Vasculitis, Central Nervous System ; therapy

The common clinical manifestations of the primary agiitis of the central nervous system include burst of headache, dementia, change of aptitude, paralysis of cranial nerves, and recurrent focal depletion of the neural function. Lptomeningeal and brain biopsy are still the gold criteria for diagnosis. The prognosis may be improved after cortin and immunosuppressant therapy.
Humans ; Vasculitis, Central Nervous System ; diagnosis ; pathology ; therapy

Microscopic polyangiitis is a systemic small-vessel vasculitis that is primarily associated with necrotizing glomerulonephritis and pulmonary capillaritis. Lung involvement is characterized by a diffuse alveolar hemorrhage. However, rarely central nervous system involvement has been reported to be occurred with the microscopic polyangiitis. Relapse of microscopic polyangiitis are reported to be more frequent than those of polyarteritis nodosa, often after a reduction or discontinuation of the therapy. We would like to report two patients with microscopic polyangiitis. One presented with clinical manifestations of both lung and central nervous system involvements and the other was a case of recurrence during steroid tapering following the steroid pulse therapy.
Antibodies, Antineutrophil Cytoplasmic ; Central Nervous System ; Glomerulonephritis ; Hemorrhage ; Humans ; Lung ; Microscopic Polyangiitis* ; Polyarteritis Nodosa ; Recurrence ; Vasculitis ; Vasculitis, Central Nervous System

Primary angiitis of the central nervous system (PACNS) is a rare disorder of unknown etiology and a serious form of vasculitis that is limited to the brain and spinal cord. We report a case of PACNS presenting with multifocal enhancing tumefactive lesions on brain magnetic resonance imaging, and numerous small arteriolar ectasias on conventional angiography, with relapsing-remitting clinical features.
Angiography ; Brain ; Central Nervous System ; Dilatation, Pathologic ; Magnetic Resonance Imaging ; Spinal Cord ; Vasculitis ; Vasculitis, Central Nervous System

BACKGROUND: Systemic lupus erythematosus (SLE) is an autoimmune disease that is a significant source of morbidity and mortality when it manifests in the central nervous system. The early detection and treatment of neuropsychiatric SLE (NPSLE) is very important, but a confirmative diagnostic tool has yet to be developed. CASE REPORT: We report here a case of neuropsychiatric manifestations in a patient that were associated with SLE, and evidence of reversal of bilateral amygdala and parahippocampal lesions in the brain revealed by 18fluorodeoxy glucose-positron emission tomography. CONCLUSIONS: We are suggestive of 18fluorodeoxy glucose-positron emission tomography appear to be more sensitive in detecting subtle brain changes in NPSLE.
Amygdala* ; Autoimmune Diseases ; Brain* ; Central Nervous System ; Humans ; Lupus Erythematosus, Systemic ; Lupus Vasculitis, Central Nervous System* ; Mortality

Degos disease, also referred to as malignant atrophic papulosis, was first described in 1941 by Köhlmeier and was independently described by Degos in 1942. Degos disease is characterized by diffuse, papular skin eruptions with porcelain-white centers and slightly raised erythematous telangiectatic rims associated with bowel infarction. Although the etiology of Degos disease is unknown, autoimmune diseases, coagulation disorders, and vasculitis have all been considered as underlying pathogenic mechanisms. Approximately 15% of Degos disease have a benign course limited to the skin and no history of gastrointestinal or central nervous system (CNS) involvement. A 29-year-old female with history of systemic lupus erythematosus (SLE) presented with a 2-year history of asymptomatic lesions on the dorsum of all fingers and both knees. The patient had only skin lesions and no gastrointestinal or CNS vasculitis symptoms. Her skin lesions were umbilicated, atrophic porcelain-white lesions with a rim of erythema. On the basis of clinical, histologic, and laboratory findings, a diagnosis of Degos-like lesions associated with SLE was made. The patient had been treated for SLE for 7 years. Her treatment regimen was maintained over a 2 month follow-up period, and the skin lesions improved slightly with no development of new lesions.
Adult ; Autoimmune Diseases ; Central Nervous System ; Diagnosis ; Erythema ; Female ; Fingers ; Follow-Up Studies ; Humans ; Infarction ; Knee ; Lupus Erythematosus, Systemic* ; Malignant Atrophic Papulosis ; Skin ; Vasculitis ; Vasculitis, Central Nervous System

Primary angiitis of the central nervous system (PACNS) is a poorly understood form of vascular inflammatory disease that is restricted to the brain and spinal cord. A 38-year-old woman presented with severe headache and transient aphasia. Her cerebrospinal fluid exhibited aseptic meningitis, and high-resolution vessel-wall MRI (HRVW-MRI) revealed narrowing of multiple intracranial vessels with concentric wall thickening and diffuse enhancement, suggestive of PACNS. High-dose steroid and azathioprine therapy resulted in a significant improvement in vessel wall thickening, and enhancement was observed on the follow-up HRVW-MRI.
Adult ; Aphasia ; Azathioprine ; Brain ; Central Nervous System* ; Cerebrospinal Fluid ; Female ; Follow-Up Studies ; Headache ; Humans ; Magnetic Resonance Imaging* ; Meningitis, Aseptic ; Spinal Cord ; Vasculitis* ; Vasculitis, Central Nervous System

The clinical manifestations of nervous system involvement in systemic lupus erythematosus (SLE) are highly diverse and their pathogenic mechanisms are incompletely understood. Neuropsychiatric SLE (NPSLE) poses difficulty in making the proper diagnosis, especially in circumstances where its initial symptoms are diffuse neuropsychiatric symptoms. We describe a 43-year old woman who exhibited a myoclonic jerk of the abdominal wall, followed shortly by acute confusion, which was attributed to SLE. Therapy with high dose corticosteroids completely reversed the symptoms. Myoclonus can be an anticipatory symptom of diffuse neurologic dysfunction in patients with NPSLE.
Abdominal Wall ; Adrenal Cortex Hormones ; Central Nervous System ; Female ; Humans ; Lupus Erythematosus, Systemic ; Lupus Vasculitis, Central Nervous System ; Myoclonus ; Nervous System ; Neurologic Manifestations

Primary central nervous system vasculitis (PCNSV) is rare, and the diagnosis is difficult to make because of its variable radiologic expressions. Early corticosteroid therapy often is effective. Herein we report the case of a 56-year-old man who had a well-enhanced cystic mass with severe edema in the right frontal lobe, which was initially felt to be a malignancy. Histologic examination of tissue removed at craniotomy revealed that it was a PCNSV. Despite early administration of corticosteroids, a new lesion developed within 3 days. The lesions responded to treatment with cyclophosphamide and corticosteroid.
Adrenal Cortex Hormones ; Craniotomy ; Cyclophosphamide ; Diagnosis ; Edema ; Frontal Lobe ; Glucocorticoids ; Humans ; Middle Aged ; Vasculitis ; Vasculitis, Central Nervous System