Subacute herpes simplex virus type 1 encephalitis as an initial presentation of chronic lymphocytic leukemia and multiple sclerosis: a case report

Abstract

IntroductionHerpes simplex virus type 1 encephalitis presents acutely in patients who are immunocompetent. We report what we believe to be the first published case of a subacute course of herpes simplex virus type 1 encephalitis in a patient with asymptomatic chronic lymphocytic leukemia who subsequently developed multiple sclerosis.Case presentationA 49-year-old Caucasian woman with a history of fever blisters presented to the emergency department with a history of left temporal headache for four weeks, and numbness of the left face and leg for two weeks. A complete blood count revealed white blood cell count of 11,820 cells/mL, with an absolute lymphocyte count of 7304 cells/mL. The cerebrospinal fluid contained 6 white blood cells/μL, 63 red blood cells/μL, 54 mg glucose/dL, and 49 mg total protein/dL. Magnetic resonance imaging of the brain revealed meningoencephalitis and bilateral ventriculitis. Cerebrospinal fluid polymerase chain reaction for herpes simplex virus type 1 was positive, and the patient's symptoms resolved after ten days of treatment with parenteral aciclovir. Incidental findings on peripheral blood smear and flow cytometry testing confirmed chronic lymphocytic leukemia. One month later, she developed bilateral numbness of the hands and feet; a repeat cerebrospinal fluid polymerase chain reaction for herpes simplex virus type 1 at this time was negative. A repeat magnetic resonance imaging scan showed an expansion of the peri-ventricular lesions, and the cerebrospinal fluid contained elevated oligoclonal bands and myelin basic protein. A brain biopsy revealed gliosis consistent with multiple sclerosis, and the patient responded to treatment with high-dose parenteral steroids.ConclusionHerpes simplex virus type 1 encephalitis is a rare presentation of chronic lymphocytic leukemia. Our patient had an atypical, subacute course, presumably due to immunosuppression from chronic lymphocytic leukemia. This unusual case of herpes simplex virus type 1 encephalitis emphasizes the importance of T cell function in diseases of immune dysregulation and autoimmunity such as chronic lymphocytic leukemia and multiple sclerosis. It raises the question of whether atypical presentations of herpes simplex virus encephalitis warrant deliberations on immunocompetence. The development of multiple sclerosis in our patient so soon after she received treatment for herpes simplex virus type 1 encephalitis raises the possibility that herpes simplex virus type 1 encephalitis in an immunosuppressed patient may trigger multiple sclerosis.

Atypical imaging presentation of herpes simplex virus encephalitis in paediatric immunocompromised oncology patients

“…Similarly, the increase in cortical and subcortical lesions between the baseline and follow‐up scans was statistically significant. Periventricular lesions in our study were always found in association with cortical/subcortical involvement unlike findings from some studies of ventriculitis with periventricular lesions or punctate lesions at grey‐white matter junction without cortical lesions 12,13 …”

“…Periventricular lesions in our study were always found in association with cortical/subcortical involvement unlike findings from some studies of ventriculitis with periventricular lesions or punctate lesions at grey-white matter junction without cortical lesions. 12,13 Lack of diffusion restriction in most of our cases was contrary to the typical restricted diffusion seen in brain lesions in immunocompetent patients (Fig. 3).…”

“…The remainder of the similar published studies mentioned in Table S2 12,13,[15][16][17][18][19][20][21] are single case reports of HSE in patients with leukaemia/lymphoma, mainly in adult patients. Nearly half of these studies have normal imaging (CT/MRI) 16,[18][19][20] and most with abnormal scans had only typical medial temporal lobe involvement.…”

Subacute Herpes Simplex Type 1 Encephalitis

“…Brain biopsy was performed to exclude lymphoma, but it revealed gliosis, which is consistent with MS, management was started accordingly. Six months later the patient became asymptomatic 13. Finally Singh and colleagues, reported a case of subacute HSVE in a 32-year-old man with on-and-off history of mid-grade fever, headache, decreased appetite, and cognitive decline for 4 weeks.…”

“…Six months later the patient became asymptomatic. 13 Finally Singh and colleagues, reported a case of subacute HSVE in a 32-year-old man with on-and-off history of mid-grade fever, headache, decreased appetite, and cognitive decline for 4 weeks. Diagnosis was confirmed with MRI findings and positive PCR for HSV-1.…”