Progressive multifocal leukoencephalopathy: Difference between revisions
ClaireLewis (talk | contribs) (Created page with "==Background== *Progressive multifocal leukoencephalopathy (PML) is a rare, demyelinating CNS disorder *Associated with reactivated JC virus *Seen almost exclusively in immuno...") |
ClaireLewis (talk | contribs) No edit summary |
||
| Line 5: | Line 5: | ||
**[[AIDS]] | **[[AIDS]] | ||
**[[Leukemia]], [[lymphoma]], [[myeloproliferative disorders]] (with or without chemo) | **[[Leukemia]], [[lymphoma]], [[myeloproliferative disorders]] (with or without chemo) | ||
**Post solid organ or stem | **Post solid organ or [[stem cell transplant]] | ||
**Autoimmune disorders (with or without immune-modulating medications) | **Autoimmune disorders (with or without immune-modulating medications) | ||
**Medications: Natalizumab, rituximab, infliximab, [[mycophenolate mofetil]] | **Medications: Natalizumab, rituximab, infliximab, [[mycophenolate mofetil]] | ||
| Line 12: | Line 12: | ||
*[[Altered mental status]] | *[[Altered mental status]] | ||
*Aphasia | *Aphasia | ||
*Ataxia | *[[Ataxia]] | ||
*Hemiparesis | *[[weakness|Hemiparesis]] | ||
*Hemiplegia and visual field disturbances | *Hemiplegia and [[visual disturbances|visual field disturbances]] | ||
*[[Seizure]] | *[[Seizure]] | ||
*[[Cranial nerve palsies]] (rare) | *[[Cranial nerve palsies]] (rare) | ||
| Line 20: | Line 20: | ||
==Differential Diagnosis== | ==Differential Diagnosis== | ||
{{AMS DDX}} | |||
==Evaluation== | ==Evaluation== | ||
*Evaluate for other (especially easily treatable) causes of symptoms | *Evaluate for other (especially easily treatable) causes of symptoms | ||
*MRI brain: non-enhancing, multifocal areas of white matter demyelination | *[[brain MRI|MRI brain]]: non-enhancing, multifocal areas of white matter demyelination | ||
*PCR for JC virus (CSF, blood, urine) | *PCR for JC virus (CSF, blood, urine) | ||
Latest revision as of 22:31, 2 October 2019
Background
- Progressive multifocal leukoencephalopathy (PML) is a rare, demyelinating CNS disorder
- Associated with reactivated JC virus
- Seen almost exclusively in immunosuppressed patients, usually due to:
- AIDS
- Leukemia, lymphoma, myeloproliferative disorders (with or without chemo)
- Post solid organ or stem cell transplant
- Autoimmune disorders (with or without immune-modulating medications)
- Medications: Natalizumab, rituximab, infliximab, mycophenolate mofetil
Clinical Features
- Altered mental status
- Aphasia
- Ataxia
- Hemiparesis
- Hemiplegia and visual field disturbances
- Seizure
- Cranial nerve palsies (rare)
- Cervical spine involvement (rare)
Differential Diagnosis
Altered mental status
Diffuse brain dysfunction
- Hypoxic encephalopathy
- Acute toxic-metabolic encephalopathy (Delirium)
- Hypoglycemia
- Hyperosmolar state (e.g., hyperglycemia)
- Electrolyte Abnormalities (hypernatremia or hyponatremia, hypercalcemia)
- Organ system failure
- Hepatic Encephalopathy
- Uremia/Renal Failure
- Endocrine (Addison's disease, Cushing syndrome, hypothyroidism, myxedema coma, thyroid storm)
- Hypoxia
- CO2 narcosis
- Hypertensive Encephalopathy
- Toxins
- TTP / Thrombotic thrombocytopenic purpura
- Alcohol withdrawal
- Drug reactions (NMS, Serotonin Syndrome)
- Environmental causes
- Deficiency state
- Wernicke encephalopathy
- Subacute Combined Degeneration of Spinal Cord (B12 deficiency)
- Vitamin D Deficiency
- Zinc Deficiency
- Sepsis
- Osmotic demyelination syndrome (central pontine myelinolysis)
- Limbic encephalitis
Primary CNS disease or trauma
- Direct CNS trauma
- Diffuse axonal injury
- Subdural/epidural hematoma
- Vascular disease
- SAH
- Stroke
- Hemispheric, brainstem
- CNS infections
- Neoplasms
- Paraneoplastic Limbic encephalitis
- Malignant Meningitis
- Pancreatic Insulinoma
- Seizures
- Nonconvulsive status epilepticus
- Postictal state
- Dementia
Psychiatric
Evaluation
- Evaluate for other (especially easily treatable) causes of symptoms
- MRI brain: non-enhancing, multifocal areas of white matter demyelination
- PCR for JC virus (CSF, blood, urine)
Management
- Limit duration of immunosuppression
- HIV/AIDS patients, antiretroviral therapy is key
- Cidofovir may be useful in non-AIDS patients