ALTERNATE NAMES

Child Chronic Progressive Epilepsia Partialis Continua; Chronic Encephalitis; Chronic Focal Encephalitis; Chronic Localized Encephalitis; Chronic Progressive Epilepsia Partialis Continua of Childhood; Rasmussen Disease; Rasmussen Syndrome

DESCRIPTION

Rasmussen Encephalitis (RE) is a very rare disorder of the central nervous system characterized by chronic inflammation of one hemisphere of the brain. The inflammation leads to frequent seizures (epilepsy) and as it progresses, it causes worsening weakness in one side of the body and mental decline.

RE most commonly affects children between the ages of 2 and 10, but it can affect adolescents and adults as well.

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

Diagnostic testing: Diagnostic testing for RE may include:

• •

  Clinical evaluation and medical history;

• •

  Electroencephalography (EEG); and

• •

  Magnetic resonance imaging (MRI).

Physical findings: Symptoms of RE include:

• •

  Aphasia (progressive loss of speech and language abilities);

• •

  Hemianopsia (partial loss of sight in half of your visual field);

• •

  Hemiparesis (paralysis of one side of the body);

• •

  Mental decline, such as issues with thinking, intellect, and memory; and

• •

  Seizures.

ICD-9: 323.9

ICD-10: G04.90

PROGRESSION

The prognosis for RE varies. Surgery may stop the disease progression and stabilize an individual’s seizures. However, most individuals with RE have some paralysis, cognitive decline, and issues with speech.

In very rare cases, the condition can progress to the other hemisphere of the brain.

TREATMENT

There is no cure for RE. Instead, the goal of treatment is to manage symptoms and inflammation. Treatment may include antiseizure medications, immunotherapy, and brain surgery, such as hemispherectomy (surgical removal or disconnection of half of the brain from the rest of the brain).

Hemispherectomy is most effective in treating seizures in RE. Most children who have this surgery have already lost the function of the affected hemisphere.

Suggested MER for Evaluation:

• •

  Clinical history and examination that describes diagnostic features of the impairment;

• •

  EEG; and

• •

  MRI.

Suggested Listings for Evaluation:

Meets

11.02

11.07

12.02

12.05

12.11

111.02

111.07

111.09

112.02

112.05

112.11

112.14

Equals