LAUSR

A 43-year-old North African male, known to have neurofibromatosis type 1 (NF1), was admitted to the neurological unit due to a sudden transient paresis of the right arm and lower face lasting 15 min, followed by complete recovery. He reported no disturbance of consciousness. A complete stroke workup was performed, but revealed no clear underlying etiology. The patient was initiated on antiplatelet therapy and discharged. A brain Magnetic Resonance Imaging (MRI) scan showed a ‘dirty cerebrospinal fluid (CSF) sign’ on the left central sulcus (Fig. 1) with no abnormalities on susceptibility weighted imaging. A previous brain MRI, performed as a screening for NF1 18 months before, showed no such abnormality. 2 weeks after his first admission, the patient presented with recurrent episodes of transient paresis of the right arm and lower face, again with intact consciousness. This time there were also complaints of moderately severe headache, increasing nausea, episodes of vomiting and anorexia with loss of weight (5 kg) in the preceding 2 weeks. Repeated electroencephalography showed no epileptiform abnormalities. Lumbar puncture showed an opening pressure of > 50 cmH2O, 6 leukocytes per mm3, normal proteinorrhachia and hypoglycorrhachia of 6 mg/dL, further lowering to 0 mg/dL on repeated lumbar punctures. Serum glucose had always been normal to slightly elevated (normal values > 73 mg/ dL). Pathological investigation on CSF showed atypical cells, suggestive for malignancy, but could not provide further differentiation. A total body fluor-deoxyglucose Positron Emitting Tomography scan disclosed no evidence of a primary tumor. Extensive microbiologic and serologic investigation showed no abnormalities, and the patient was initiated on empirical antibacterial and tuberculostatic therapy. Furthermore, a ventriculoperitoneal shunt was placed to relieve the consistently high intracerebral pressure, resulting initially in a slightly positive clinical evolution. Gradually, the patient deteriorated, exhibiting more frequent stroke-like episodes with faciobrachial paresis and motor aphasia, always followed by complete recuperation. After a few weeks he developed a convulsive status epilepticus that could not be suppressed with multiple antiepileptic drug therapy, necessitating intubation and sedation. Leptomeningeal biopsy showed evidence for a leptomeningeal melanoma. Careful dermatological examination excluded the presence of a cutaneous melanoma, and ophthalmologic evaluation showed no evidence for suspect intra-ocular lesions. A new brain MRI showed diffuse supraand infratentorial meningeal enhancement. The patient died 4 months after initial presentation.