ALTERNATE NAMES

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

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.

Symptoms of CBD generally 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. These symptoms develop and worsen over time. An individual with CBD eventually becomes unable to walk.

DIAGNOSTIC TESTING, PHYSICAL FINDINGS, AND ICD-9-CM/ICD-10-CM CODING

Diagnostic testing: The diagnosis of CBD is based on:

• •

  Computerized tomography (CT) or magnetic resonance imaging (MRI) scans;

• •

  Single photon emission computerized tomography (SPECT) studies; or

• •

  Positron emission tomography (PET) scans.

Physical findings: Physical symptoms include:

• •

  Poor coordination;

• •

  Painful muscle spasms and jerks;

• •

  Absence of movement (akinesia);

• •

  Rigidity;

• •

  Poor balance;

• •

  Unnatural posturing of the muscles (dystonia);

• •

  Cognitive impairment;

• •

  Visual spatial impairment;

• •

  Hesitating or halting speech; and

• •

  Difficulty swallowing (dysphagia).

ICD-9: 333.0

ICD-10: G31.85

PROGRESSION

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

TREATMENT

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.

Treatment options include:

• •

  Clonazepam for the treatment of muscle jerks and painful spasms;

• •

  Baclofen for the treatment of rigidity;

• •

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

• •

  Occupational therapy to support activities of daily living;

• •

  Speech therapy to improve articulation and volume; and

• •

  Ambulatory aides such as canes, walkers, and wheelchairs for difficulties with movement and balance.

Suggested MER for Evaluation:

• •

  Clinical information documenting progression of neurological and cognitive decline. The preferable source of this information are the clinical records from the claimant's medical source(s);

• •

  Activities of daily living report or a similar report completed by a family member or caregiver is needed to document ability to perform daily functioning independently;

• •

  Mental status or neuropsychological tests measuring cognitive changes;

• •

  Speech and language assessments to assess the ability to speak and understand speech; and

• •

  Imaging studies of the brain demonstrating atrophy of the frontal and temporal lobes, such as MRI, CT scan, PET scan, or SPECT.

DETERMINATION

LISTING

REMARKS

Meets

11.06

11.17

12.02

Equals