Ventriculitis as a manifestation of Neuropsychiatric Systemic Lupus Erythematosus
David Medina Julio1, Rogelio Dominguez-Moreno3, Andrea Nuñez de Caceres Garcia1, Ximena García-Velazquez1, Osmar Albores2, Carlos Cantu2
1Instituto Nacional de Ciencias Médicas y Nutrición Salvador Zubirán, 2Neurología, Instituto Nacional de Ciencias Médicas y Nutrición Salvador Zubirán, 3Danish Headache Center
Objective:
To present a case report of an uncommon presentation of Neuropsychiatric Systemic Lupus Erythematosus (NPSLE) as the initial manifestation of Systemic Lupus Erythematosus (SLE).
Background:
NPSLE encompasses a total of 19 clinical manifestations, which can affect both the central and peripheral nervous system. The most common symptoms are headache, mood disorders and cognitive dysfunction. Brain imaging reveals lesions in only 75% of cases. Common patterns found are vasculitis and brain atrophy. Other less common imaging findings include lacunar infarcts, demyelinating lesions and intracranial hemorrhage.
Results:
A 32-year-old Hispanic woman with no relevant medical history, presented with acute encephalopathy, visual hallucinations and decreased visual acuity. Neurological exam revealed visual acuity of 20/70 without correction using a pinhole test. MRI showed periventriculitis and fundoscopy revealed retinal vasculitis. Immunology laboratory studies were ordered to confirm the suspicion of neuropsychiatric lupus. The results showed decreased serum complement, positive antinuclear antibodies (homogeneous 1:5120, nuclear membrane 1:5120 and cytoplasmic 1:320) and anti-Ribosomal P antibodies in the CSF. Thrombocytopenia and non-hemolytic anemia were found on complete blood count. All other causes of ventriculitis were excluded, leading to the conclusion that it was a manifestation of neuropsychiatric lupus. The patient was treated with prednisone, hydroxychloroquine, and a bolus of cyclophosphamide, showing clinical and imaging improvement after two months of starting treatment.
Conclusions:
To the authors’ knowledge, this is the first case report of ventriculitis and retinal vasculitis in neuropsychiatric lupus, both of which responded to treatment with immunomodulators. This case report could lead to a series of cases where ventriculitis is proven a systemic manifestation of lupus.
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