Neuro Disorders: Cortical Basal Ganglionic Degeneration

Introduction

Cortical Basal Ganglionic Degeneration (CBGD), as the name implies is a degenerative disease and it involves the brain.

Term cortical is related with cortex of the brain (outer layers of the brain). Basal ganglia are a collection of structures under extra pyramidal system. So with this condition degeneration of the above brain regions occurs.

What causes CBGD?

What triggers CBGD is not known yet. Also it is not considered as a genetic disease at this moment.

Who gets CBGD?

It is typically seen in late middle age or older adults (between age 60 and 80 most often). It affects any race or ethnicity. There may be some predilection for women.

Clinical Presentation

This disease presents with multiple manifestations. They may be grouped as cortical & sub cortical; extra pyramidal & pyramidal; cognitive & non cognitive dysfunctions etc. Symptoms start & progress slowly. The following manifestation are usually seen;

• Limb apraxias
• Rigidity, slowness of the movements, dystonia etc unilaterally that will later spread to the other side
• Dementia
• Cortical type sensory loss
• Alien limb phenomenon (patients feel their limb has its own mind)
• Certain disorders of the eye movements etc

Diagnosis

The diagnosis is accomplished by clinical evaluation and then ruling out conditions those mimic CBGD.

Imaging studies of the brain, blood, spinal fluid tests, EEG, etc are sometimes necessary to rue out other possibilities.

Treatment

There is no cure for CBGD. Patients are treated with symptomatic & supportive approaches. Parkinson, dementia like features are treated with appropriate medications although the response to these medications is far from satisfactory.

A neurologist is frequently involved in the care of patients with Cortical Basal Ganglionic Degeneration.

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