Lower cranial nerve palsy, aseptic meningitis and hydrocephalus: unusual presentation of primary antiphospholipid syndrome

Abstract

Presentation of primary antiphospholipid syndrome (APS) is usually untrustworthy and unusual presentations are difficult to diagnose on the basis of clinical features alone. This is true especially in young and elderly patients. Cerebral venous thrombosis (CVT) is less frequent than arterial thrombosis in APS. CVT has a wide spectrum of signs and symptoms, which may evolve suddenly or over weeks. It mimics many neurological conditions such as meningitis, encephalopathy, benign intracranial hypertension and stroke. Headache is the most frequent symptom in patients with CVT, and is present in about 80% of cases. The most common pattern of presentation is with a benign intracranial hypertension-like syndrome. Sixth cranial nerve palsy usually manifests as a false localising sign. Patients may have recurrent seizures. Cranial nerve syndromes are seen with venous sinus thrombosis. We present a case of APS with lower cranial nerve palsy, aseptic meningitis and hydrocephalus initially treated as tuberculous meningitis.

Hospital based prospective longitudinal clinical and immunologic study of 179 patients of primary anti-phospholipid syndrome

“…reported two cases of cerebral venous and dural sinus thrombosis associated with aPL. Wani et al . reported a case of primary APS presenting with lower cranial nerve palsy, aseptic meningitis and hydrocephalus.…”

Profound changes in cerebrospinal fluid proteome and metabolic profile are associated with congenital hydrocephalus

“…In experimental animal models, pregnancies exposed to x ray radiation, nutritional deficiencies, alcohol consumption and other teratogenic chemicals can all result in cHC, revealing environmental toxicity and nutritional abnormalities as risk factors. [1][2][3][4] However, the mechanisms leading to, and underlying cHC remain matters of great debate and research, while early diagnosis and treatment of cHC is crucial to the outcome of the new-born. Although prenatal ventriculomegaly (enlarged brain ventricles) can be identified by ultrasound imaging between 18-20 weeks of gestation, only certain aetiologies of cHC can be identified in utero.…”