Program Operations Manual System (POMS)
TN 44 (11-20)
DI 23022.850 Orthochromatic Leukodystrophy with Pigmented Glia
COMPASSIONATE ALLOWANCES INFORMATION
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ORTHOCHROMATIC LEUKODYSTROPHY WITH PIGMENTED
GLIA
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ALTERNATE NAMES
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Pigmented Type of Orthochromatic Leukodystrophy; Pigmentary Orthochromatic Leukodystrophy;
POLD; Orthochromatic Leukodystrophy with Pigmented Glia Cells
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DESCRIPTION
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Orthochromatic Leukodystrophy with Pigmented Glia is a rare inherited type of adult leukodystrophy that affect the white matter of
the brain.
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DIAGNOSTIC TESTING, PHYSICAL FINDINGS, AND ICD-9-CM/ICD-10-CM
CODING
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Diagnostic
testing: The diagnosis of this disorder is based:
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Clinical and family history;
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Neuropsychological testing; and
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Neuroimaging, such as computerized tomography (CT) or magnetic resonance imaging (MRI).
Neurological physical
findings: The neurological physical findings include:
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Dysarthria (weakness in the muscles used for speech);
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Ocular apraxia (absence or defect of controlled, voluntary, and purposeful eye movement);
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Rapidly progressive dementia; and
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Facial and bulbar weakness.
ICD-9: 330.0
ICD-10: E75
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PROGRESSION
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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.
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TREATMENT
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There is no cure for this disorder. Treatment is symptom specific and supportive.
Antidepressants and tranquilizers are used to treat psychiatric symptoms.
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SUGGESTED PROGRAMMATIC ASSESSMENT*
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Suggested MER for Evaluation:
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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
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CT/MRI scans of the brain showing abnormal changes in the white matter.
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Suggested Listings for Evaluation:
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DETERMINATION
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LISTING
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REMARKS |
Meets
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11.17
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12.02
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Equals
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* 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.
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