PROGRAM OPERATIONS MANUAL SYSTEMPart DI – Disability InsuranceChapter 230 – Special IssuesSubchapter 22 – Processing Quick Disability Determination (QDD) and Compassionate Allowance (CAL) in the Disability Determination Services (DDS)Transmittal No. 44, 11/20/2020
This is a Quick Action Transmittal. These revisions do not change or introduce new policy or procedure.
Summary of Changes
DI 23022.850 Orthochromatic Leukodystrophy with Pigmented Glia
Deleted "Adult Onset Leukoencephalopathy with Axonal Spheroids and Pigmented Glia" from the Alternate Names section
Deleted "Adult Onset Leukodystrophy" from the Alternate Names section
Deleted "ALSP" from the Alternate Names section
ORTHOCHROMATIC LEUKODYSTROPHY WITH PIGMENTED
Pigmented Type of Orthochromatic Leukodystrophy; Pigmentary Orthochromatic Leukodystrophy; POLD; Orthochromatic Leukodystrophy with Pigmented Glia Cells
Orthochromatic Leukodystrophy with Pigmented Glia is a rare inherited type of adult leukodystrophy that affect the white matter of the brain.
DIAGNOSTIC TESTING, PHYSICAL FINDINGS, AND ICD-9-CM/ICD-10-CM
testing: The diagnosis of this disorder is based:
Clinical and family history;
Neuropsychological testing; and
Neuroimaging, such as computerized tomography (CT) or magnetic resonance imaging (MRI).
findings: The neurological physical findings include:
Dysarthria (weakness in the muscles used for speech);
Ocular apraxia (absence or defect of controlled, voluntary, and purposeful eye movement);
Rapidly progressive dementia; and
Facial and bulbar weakness.
Orthochromatic leukodystropy with pigmented glia is progressive and usually fatal. This disorder typically affects adults between the ages of 40-50 years of age. As the disorder progresses some individuals become wheel chair dependent and require feeding via gastrostomy to maintain nutrition and hydration. Death usually occurs within ten years of diagnosis secondary to sepsis infection.
There is no cure for this disorder. Treatment is symptom specific and supportive. Antidepressants and tranquilizers are used to treat psychiatric symptoms.
SUGGESTED PROGRAMMATIC ASSESSMENT*
Suggested MER for Evaluation:
Clinical information documenting a progressive dementia is critical and required for disability evaluation of orthochromatic leukodystrophy with pigmented glia. The preferable source of this information is the clinical records from the claimant’s medical source(s); and
CT/MRI scans of the brain showing abnormal changes in the white matter.
Suggested Listings for Evaluation:
* Adjudicators may, at their discretion, use the Medical Evidence of Record or the listings suggested to evaluate the claim. However, the decision to allow or deny the claim rests with the adjudicator.