Encephalomyelitkronisk

Encephalomyelitkronisk, or chronic encephalomyelitis, is a persistent inflammatory disorder of the brain and spinal cord characterized by relapsing and/or progressive neurological symptoms due to inflammatory demyelination and neural injury. The condition can be autoimmune, post-infectious, paraneoplastic, or idiopathic, and may overlap with other inflammatory central nervous system diseases.

Causes and mechanism

The pathology centers on inflammation and demyelination with subsequent gliosis in various CNS regions, leading to a heterogeneous clinical picture. Autoimmune processes may involve antibodies against CNS components in some patients, but no single marker defines all cases. Infections, immune dysregulation, or paraneoplastic mechanisms can also contribute to disease onset or relapses.

Clinical features

Presentation is subacute to chronic with episodes of focal neurologic deficits or progressive symptoms. Common manifestations include headaches, cognitive or behavioral changes, motor weakness or spasticity, sensory loss, ataxia, seizures, and sometimes optic neuritis. The course varies, with relapses, progression, or a combination of both.

Diagnosis

Diagnosis relies on clinical history and disease course, supported by imaging and laboratory studies. MRI may reveal inflammatory lesions in the brain and spinal cord. Cerebrospinal fluid can show pleocytosis, elevated IgG index, and oligoclonal bands. Laboratory testing aims to exclude infections, other demyelinating diseases, and neoplasia. A diagnosis often requires exclusion of alternative explanations and consideration of the overall clinical pattern.

Treatment and prognosis

Management is individualized to disease activity and suspected underlying mechanism. Acute flares may respond to high-dose corticosteroids; plasma exchange or intravenous immunoglobulin is used in resistant cases. Chronic immunosuppressive or biologic therapies (for example azathioprine, mycophenolate, rituximab) may be employed for relapsing forms. Rehabilitation and symptomatic care are important. Prognosis ranges from stability to progression, depending on etiology and response to therapy.

See also

Encephalitis, myelitis, multiple sclerosis, neuromyelitis optica spectrum disorders, MOG-associated disease.