Varicella-Zoster Encephalomyelitis

Abstract

V A R I C E L L A -Z O S T E RARICELLA-ZOSTER (V-Z) encephalomyelitis is relatively uncommon. Detailed morphologic and virologic studies on such cases are extremely few. Our recent studies of two patients on whom complete autopsies were done, form the basis of this study. In one of these patients V-Z virus was cultured from the brain. Electron microscopic demonstration of virus particles in the brain was obtained on both cases. We believe this to be the first reported isolation of the V-Z virus and the first electron microscopic demonstration of the virus in human brain.Type A intranuclear inclusion bodies were seen in the brain and spinal cord of both patients. We are aware of no recorded observations of type A inclusions in the central nervous system (CNS) in patients with clinical herpes zoster and only one such report in a case of disseminated varicella. The literature, insofar as it is pertinent, is reviewed and a synthesis of our observations and those of others attempted. Report of CasesCase 1.-A 414-year-old boy with acute lymphocytic leukemia, diagnosed in September 1967 when his peripheral white blood cell count (WBC) was 500,000/cu mm, with 95% lymphoblasts, was admitted to the University of Iowa Hospital on Dec 12, 1968 because of diarrhea, nausea, vomiting, and irritability of one week's duration.During the past year he had received prednisone, vincristine sulfate, mercaptopurine, methotrexate (intrathecal 4-amino-N10-methyl pteroylglutamic acid), cyclophosphamide (Cytoxan), and cranial and mediastinal radiation. His illness had been complicated in November 1967 by varicella, which he contracted after exposure to an infected sibling. In March 1968, the patient developed seizures, headaches, and spinal fluid pleocytosis for which he received intrathecal methotrexate and cranial irradia¬ tion. From Nov 20 to 26, he received radiation to the left side of the chest and right cervical regions. At the time of admission he was re¬ ceiving cyclophosphamide (36 mg daily) and prednisone (15 mg daily).Physical examination revealed a chronically ill, irritable child, with alopecia. There was bilateral papilledema. There were decreased breath sounds over the entire left hemithorax, and the liver was palpable 1.5 cm below the right costal margin. No enlarged lymph nodes were palpable. The neurologic examination was within normal limits except for generalized irritability.An admission hemogram revealed a WBC of 2,600/cu mm, with 43% lymphocytes and 43% myelocytes, a hemoglobin level of 13.5 gm/100 ml, and a platelet count of 55,000.On the day after admission he had a rightsided motor seizure. The cyclophosphamide was discontinued and cranial radiation and in¬ trathecal methotrexate were instituted. The last dose of intrathecal methotrexate was given

Studies on the pathogenesis of neurological diseases associated with Varicella‐Zoster Virus

“…There is some, but not a great deal of evidence for direct virus invasion in zoster encephalomyelitis. For example, two immunosuppresed cases of McCormick et al (1969) had evidence of intranuclear inclusion bodies in the brain and spinal cord, VZV was cultured from the brain of one of these and electron microscopic analysis showed virus particles in the brains in both cases. Likewise, Hogan and Krigman (1973) detected intranuclear inclusion bodies in glial cells in the cervical spinal cord of a patient who had died with zoster-induced myelitis and VZV was isolated from the spinal cord.…”

Varicella zoster virus vasculopathies: diverse clinical manifestations, laboratory features, pathogenesis, and treatment

“…In a clinical study of four children with varicella who developed serious neurological disease, CT and MRI revealed bilateral basal ganglionic infarction in all brains 59. Finally, in one of the first cases of VZV encephalomyelitis that developed in a boy with leukaemia in whom VZV was isolated from brain tissue, microscopic findings revealed multiple areas of acute necrotic infarction 60. Four later cases of fatal VZV multifocal leucoencephalitis (three in patients with leukaemia and one in a patient with ovarian cancer) revealed multifocal abnormalities both on brain imaging and at autopsy,61–63 characteristic of multifocal VZV vasculopathy.…”

Neurologische Komplikationen und Therapie bei Herpesviruserkrankungen

“…Enzephalitiden und Enzephalomyelitiden sind ebenfalls sehr selten (41,56,88). Der klinische Verlauf ähnelt jem der Enzephalitiden anderer Ätiologie.…”

“…Pastherpetische Neura/gien sieht man bei über 50 %aller über 60 Jahre alten Patienten(56). Die betroffenen Hautareale sind häufig hyperpathisch, hyperästhetisch, gelegentlich sieht man auch eine Anästhesia dolorosa.Der Befall des ersten Trigeminusastes wird als Zaster aphtha/micus bezeichnet.…”