Leukoencephalopathy G93.4

Binswanger's encephalopathy was first described in Jena by the German neurologist Otto Ludwig Binswanger (1852–1929) (Klingelhöfer 2017).

Leukoencephalopathy is a general term for disorders of the brain's white matter.

Leukoencephalopathies are classified as follows:

• infectious leukoencephalopathies:

Possible causative agents include viruses, bacteria, and protozoa (Jakabek 2024).

• toxic leukoencephalopathies:

In these cases, exogenous or endogenous substances lead to temporary or permanent neuronal damage (Bandeira 2024).

• Hereditary leukoencephalopathies:

These can become symptomatic at any age (Sarret 2019). Numerous new genes have now been identified as the cause (Schöls 2022).

• Arteriosclerotic leukoencephalopathies:

The most common form of leukoencephalopathy is subcortical arteriosclerotic encephalopathy, also known as Binswanger’s disease (Heckmann 2002).

There are no epidemiological data available on the incidence of leukoencephalopathy. However, a significant increase has been observed in recent years, due in part to improved diagnostic methods and in part to an increase in iatrogenic cases (Heckmann 2002).

• Reactivation of a JC virus infection
• a late-onset side effect of radiation therapy (Kasper 2015)
• Immunosuppression
• Following organ transplantation
• Drug use (Heckmann 2002)

• Diffuse brain dysfunction with behavioral changes, mood disorders, and cognitive impairment
• Somnolence up to and including coma (Heckmann 2002)
• Seizures (Kasper 2015)
• In adulthood, a typical presentation involves a combination of organic brain syndromes—such as cognitive impairment, psychosis, and changes in behavior and personality—along with concurrent movement disorders, such as ataxia, dystonia, neuropathy, and spasticity. It is also not uncommon for internal organs to be affected (Schöls 2022).

The diagnosis is made through imaging studies such as computed tomography (CT) and magnetic resonance imaging (MRI) (Heckmann 2002), as well as through biochemical analysis of the metabolic disorder and/or molecular genetic testing for the relevant genes (Schöls 2022).

• MRI:

Modern MRI techniques, such as T2-weighted imaging, proton imaging, and FLAIR and FSEIR sequences, provide a detailed depiction of changes in the white matter with high sensitivity but low specificity (Heckmann 2002).

Patients should be referred to specialized centers.

Treatment itself is primarily symptomatic or supportive. It also depends on the specific form of leukoencephalopathy (Schöls 2022).

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