Chronic
Enterovirus Meningoencephalitis of Agammaglobulinemia
Background Neuroimaging Gross Pathology Histopathology & Immunohistochemistry Differential Diagnosis Reference
BACKGROUND AND CLINICAL INFORMATION:
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Pathogen: usually echovirus or coxsackie B3 virus. The encephalopathy continue to progress even no viral particles can be isolated.
Clinical features:
Rare.
This entity has been reported in patients with hypogammaglobulinemia such as
X-linked hypogammaglobulinemia or combined variable immune deficiency.
Clinical
picture is that of a chronic lymphocytic meningitis with variable features
of brain involvement such as disturbance of consciousness, and focal
seizures. There is a prolonged incubation of months to years until onset of
neurologic symptoms.
Patients
may present as pure encephalopathy, encephalomyelopathy, and pure myelopathy.
Prognosis:
the course is chronic and often fatal.
Anatomic locations:
In
cases
that are caused by echovirus, neuronal loss is usually most striking in the
brain (cortical neurons, Purkinje cells, and brain stem nuclei).
In
cases caused by poliovirus loss of anterior horn cells is more prominent.
HISTOPATHOLOGY AND IMMUNOHISTOCHEMISTRY:
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Despite
the humoral immunodeficient status, there is an intense inflammatory reaction in
the CNS with perivascular and meningeal mononuclear cell infiltration.
Mononuclear
and microglial parenchymal nodules are also seen.
Skin
biopsy: may be used to demonstrate dermatomyositis-like syndrome with
perivascular mononuclear cell infiltration and is helpful for making diagnosis.