ICD-10: A81.2

The ICD-10 code A81.2 corresponds to Progressive multifocal leukoencephalopathy (PML), which is a central nervous system disease caused by the human polyomavirus 2. Here are some alternative names and related terms associated with this condition:

• Progressive multifocal leukoencephalopathy (PML): This is the primary name used in medical literature and clinical settings.
• PML: An abbreviation commonly used for Progressive multifocal leukoencephalopathy.
• JC virus infection: Since PML is caused by the John Cunningham virus (JC virus), this term is often used in discussions about the disease.
• Leukoencephalopathy: A broader term that refers to diseases affecting the white matter of the brain, which includes PML.
• A81.2: The specific ICD-10 code designation for Progressive multifocal leukoencephalopathy, which is part of the classification for viral and prion infections.

These terms are important for understanding the condition in both clinical and research contexts, as they highlight the disease's etiology and classification within medical coding systems [3][1].

The diagnosis of Progressive Multifocal Leukoencephalopathy (PML), classified under ICD-10 code A81.2, involves several criteria that are primarily clinical and laboratory-based. Here are the key points regarding the diagnostic criteria:

• Clinical Diagnosis: A definite diagnosis of PML can be established through clinical evaluation, which includes assessing neurological symptoms and signs that are consistent with the disease. These may include cognitive decline, motor weakness, and visual disturbances.

• Cerebrospinal Fluid (CSF) Analysis: A critical component of the diagnosis is the detection of the JC virus (JCV) in the cerebrospinal fluid. This is typically done using polymerase chain reaction (PCR) testing. A positive result for JCV in the CSF is a strong indicator of PML [11].

• Imaging Studies: Magnetic Resonance Imaging (MRI) of the brain is often utilized to identify characteristic lesions associated with PML. These lesions are typically found in the white matter and are indicative of demyelination.

• Immunosuppression Assessment: The presence of overt immunosuppression is a significant factor in diagnosing PML. In many cases, patients diagnosed with PML have underlying conditions or treatments that compromise their immune system, making them susceptible to JCV reactivation [14].

• Definite Diagnostic Criteria: According to studies, a subset of patients fulfilling the diagnostic criteria for PML includes those with a confirmed positive JCV PCR in CSF and clinical symptoms consistent with the disease. In a study, out of 52 patients with the ICD-10 code A81.2, 17 met the definite diagnostic criteria for PML, with a majority showing signs of immunosuppression [14].

These criteria collectively help healthcare professionals in accurately diagnosing PML and differentiating it from other neurological disorders.

Progressive multifocal leukoencephalopathy (PML), classified under ICD-10 code A81.2, is a serious neurological condition often associated with immunocompromised states, particularly in patients with multiple sclerosis (MS) who are treated with certain therapies. Here are the standard treatment approaches and considerations for managing PML:

Treatment Approaches

1. Discontinuation of Immunosuppressive Therapy:
   - The first step in managing PML is often to discontinue any immunosuppressive medications that may have contributed to the condition, especially in patients with MS who are on drugs like natalizumab, dimethyl fumarate, fingolimod, or ocrelizumab [1][5].

2. Antiviral Therapy:
   - While there is no specific antiviral treatment for PML, some studies have explored the use of antiviral agents. However, their effectiveness remains uncertain, and they are not standard practice [1].

3. Immune Reconstitution:
   - Restoration of the immune system is crucial. This can be achieved through the use of antiretroviral therapy (ART) in HIV-positive patients, which has shown some promise in improving outcomes [2].

4. BK Virus-Specific T Lymphocyte Infusions:
   - A novel approach involves the infusion of BK virus-specific T lymphocytes, which has shown potential in treating PML by targeting the JC virus responsible for the disease [2].

5. Experimental Therapies:
   - Research is ongoing into experimental treatments, including checkpoint inhibitors like pembrolizumab and nivolumab, which may help in managing PML by enhancing immune response [4].

6. Supportive Care:
   - Supportive care is essential for managing symptoms and improving the quality of life for patients. This may include physical therapy, occupational therapy, and psychological support [1].

Monitoring and Follow-Up

• Regular MRI Scans: Monitoring the progression of PML through regular MRI scans is important to assess the extent of the disease and the effectiveness of any interventions.
• Neurological Assessments: Continuous evaluation of neurological function is necessary to adapt treatment plans as needed.

Conclusion

The management of PML is complex and requires a multidisciplinary approach. The primary focus is on discontinuing immunosuppressive therapies, restoring immune function, and exploring novel treatment options while providing supportive care to enhance patient outcomes [1][2][4].

Progressive multifocal leukoencephalopathy (PML) is classified under ICD-10 code A81.2, which is a medical classification recognized by the World Health Organization. Here are the key clinical details and descriptions associated with this condition:

• Etiology: PML is caused by the reactivation of the JC virus, a type of human polyomavirus. This virus typically remains dormant in the body and can lead to progressive demyelination within the central nervous system when reactivated [1][5].

• Pathophysiology: The disease is characterized by opportunistic demyelinating infection of the central nervous system, which results in damage to the myelin sheath that insulates nerve fibers [7].

• Symptoms: Common symptoms of PML include:

• Clumsiness
• Progressive weakness
• Impaired vision
• Speech difficulties

• Personality changes [4][6].

• Diagnosis: The ICD-10 code A81.2 is a billable diagnosis code used to specify cases of progressive multifocal leukoencephalopathy. It is also referred to as a central nervous system demyelinating disease [8].

• Clinical Significance: PML is particularly concerning in immunocompromised individuals, such as those with certain autoimmune diseases or those undergoing immunosuppressive therapies. The condition can lead to severe neurological deficits and is often associated with a poor prognosis [5][6].

Understanding these aspects of PML is crucial for healthcare providers in diagnosing and managing the condition effectively.

Progressive multifocal leukoencephalopathy (PML), classified under ICD-10 code A81.2, is a rare and often fatal disease primarily affecting the central nervous system (CNS). It is caused by the reactivation of the JC virus, which typically remains dormant in individuals with a healthy immune system but can lead to severe neurological complications in immunocompromised patients.

Clinical Presentation

• Initial Symptoms: Patients may present with subtle changes in personality, cognitive decline, and focal neurological deficits. These can include:
• Alterations in personality
• Changes in intellect
• Focal weakness
• Difficulty with coordination and balance
• Visual disturbances

Signs and Symptoms

• Neurological Symptoms: As the disease progresses, symptoms can worsen and may include:
• Progressive weakness
• Speech difficulties
• Seizures
• Visual loss
• Cognitive impairment leading to confusion or disorientation
• Physical Examination Findings: Neurological examinations may reveal:
• Hemiparesis (weakness on one side of the body)
• Ataxia (lack of voluntary coordination of muscle movements)
• Visual field defects

Patient Characteristics

• Demographics: PML predominantly occurs in individuals who are immunocompromised, including:
• Patients with HIV/AIDS
• Individuals undergoing immunosuppressive therapy (e.g., for autoimmune diseases or organ transplants)
• Patients with certain malignancies
• Age: While PML can occur at any age, it is more commonly diagnosed in adults, particularly those over 50 years old.

Additional Considerations

• Diagnosis: Diagnosis is often confirmed through MRI imaging, which typically shows characteristic lesions in the white matter of the brain, and by detecting the JC virus in cerebrospinal fluid (CSF).
• Prognosis: The prognosis for PML is generally poor, especially in immunocompromised patients, with a high rate of morbidity and mortality.

Understanding these clinical features is crucial for early recognition and management of PML, particularly in at-risk populations.