CORTICOBASAL DEGENERATION

ALTERNATE NAMES

CBD; Cortical-Basal Ganglionic Degeneration; Cortico-Basal Ganglionic Degeneration; CBGD

DESCRIPTION

Corticobasal Degeneration (CBD) is a rare progressive neurological disorder characterized by the degeneration of parts of the brain, including the cerebral cortex and basal ganglia. The cerebral cortex is responsible for most of the brain's processing of information and the basal ganglia are deep brain structures that help start and control movement. In general, symptoms of CBD begin around age 60, and frequently affect one side of the body (unilateral). As the disease progresses, both sides of the body are affected. The signs and symptoms of CBD are similar to Parkinson’s disease. The symptoms include poor coordination, painful spasms, akinesia (absence of movement), rigidity, poor balance, unnatural posturing of the muscles (dystonia), cognitive impairment, visual spatial impairment, speech impairment (hesitating or halting speech), muscular jerks (myoclonus), and difficulty swallowing (dysphagia). These symptoms develop and worsen over time. A person with CBD eventually becomes unable to walk.

DIAGNOSTIC TESTING, PHYSICAL FINDINGS, AND ICD-9-CM CODING

The diagnosis of CBD is based on neurological examination and clinical assessment since there is no specific test for the disease.

Neuroimaging either computerized tomography (CT) or magnetic resonance imaging (MRI) scans demonstrating atrophy of the frontal and temporal lobes provide supporting evidence of the diagnosis. Single photon emission computerized tomography (SPECT) studies of the brain showing perfusion levels within the brain or positron emission tomography (PET) scans are also used in the diagnosis of CBD.

ICD-9: 333.0

ONSET AND PROGRESSION

People diagnosed with CBD experience gradual decline in motor and cognitive functioning, with some people eventually loosing the ability to walk. The most significant impairments of CBD are those of rigidity, limb dystonia, and gait and balance disturbances. The average survival time after diagnosis of CBD is approximately 8 years after diagnosis. Death is generally caused by pneumonia or other complications of severe debility such as sepsis (bacterial infection) or pulmonary embolism.

TREATMENT

Currently there is no treatment to slow the progression of CBD. Treatment centers on the management of symptoms. However, drugs used to treat the symptoms of this disease do not produce any significant or sustained improvement.

Management of treatment may include:

• Clonazepam. Used for the treatment of muscle jerks and painful spasms;

• Baclofen. Used for the treatment of rigidity;

• Physical therapy exercises used to maintain range of motion of stiff joints and to prevent contracture (muscle shortening).

• Occupational therapy used to support activities of daily living.

• Speech therapy used to improve articulation and volume.

• Ambulatory aides such as canes, walkers, and wheelchairs have been prescribed for difficulties with movement and balance.