is a rare but devastating disease with an incidence of approximately two to four people per 1 million. The initial presentation consists of subacute development of fever, altered consciousness, focal cranial nerve deficit, hemiparesis, and aphasia, but the clinical presentation is mild or atypical in as much as 20% of individuals, 1 and cerebrospinal fluid (CSF) without pleocytosis has been described during the first 2 days, 2 which could lead to diagnostic difficulties. In this journal, Mook-Kanamori and colleagues described an individual with a HSV encephalitis with abnormalities on the magnetic resonance imaging (MRI) scan of the brain without pleocytosis in the CSF during the initial examination. 3 They concluded that normal CSF is highly unusual. We present a patient with HSV-E with MRI abnormalities but no pleocytosis on repeated lumbar punctures and would like to argue that finding no pleocytosis is not unusual.A 72-year-old man was admitted to the internal medicine department of Haga Teaching Hospital (The Hague, the Netherlands) with fever, malaise, and respiratory symptoms. He had been diagnosed with Wegener's disease with pulmonary and renal involvement 4 months earlier for which he was treated with cyclophosphamide and prednisone in combination with cotrimoxazole, resulting in partial remission of his Wegener's disease after 3 months. At that time (1 month before admission) he developed asymptomatic severe leukopenia (0.8 9 10 9 WBC/L), which made it necessary to interrupt his immunosuppressive treatment. The leukopenia resolved, and leukocyte number was normalized at time of admission (8.2 9 10 9 WBC/L).In the week before admittance, his physical condition deteriorated, and he had shortness of breath after walking short distances, although on the day of admission, he was able to drive his car. He did not complain of headache. During his first night in the hospital, he had signs of progressive respiratory distress without signs of pneumonia on chest X-ray and was admitted to the intensive care unit the next morning. Because of respiratory failure, he was mechanically ventilated. Because there was no clear focus for the fever, a lumbar puncture was done that showed no abnormalities (total white cell blood count (WBC) 1/lL (reference: <4/lL), protein 0.45 g/L (reference: 0.26-0.79 g/L), glucose 4.00 mmol/L (reference: 2.50-3.70 mmol/L)). On the third and fourth days, a computed tomography (CT) scan of the brain and lungs was performed and did not show clear abnormalities. On the fifth day, sedation was temporarily stopped and, because he did not regain consciousness, an electroencephalogram (EEG) was performed that showed generalized epileptic discharges on the right more than on the left frontal region, suggesting nonconvulsive status epilepticus. The epilepsia was treated with diphantoine, levetiracetam, and midazolam. Eight days after admittance, CT scan of the brain was repeated and showed mild hypodensity over the right temporal region, suggesting edema. HSV-E was considered, and a second lumbar pu...