Identification Number:
DI 23022 TN 44
Intended Audience:See Transmittal Sheet
Originating Office:ORDP ODP
Title:Processing Quick Disability Determination (QDD) and Compassionate Allowance (CAL) in the Disability Determination Services (DDS)
Type:POMS Transmittals
Program:All Programs
Link To Reference:
 

PROGRAM OPERATIONS MANUAL SYSTEM
Part DI – Disability Insurance
Chapter 230 – Special Issues
Subchapter 22 – Processing Quick Disability Determination (QDD) and Compassionate Allowance (CAL) in the Disability Determination Services (DDS)
Transmittal No. 44, 11/20/2020

Audience

PSC: CS, DEC, DTE, IES, RECONR;
OCO-OEIO: CR, ERE, FCR, FDE, RECONE;
OCO-ODO: DE, DEC, DS, RECONE;
ODD-DDS: ADJ, DHU;

Originating Component

ODP

Effective Date

11/20/2020

Background

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

DI 23022.850 Orthochromatic Leukodystrophy with Pigmented Glia

COMPASSIONATE ALLOWANCE INFORMATION

ORTHOCHROMATIC LEUKODYSTROPHY WITH PIGMENTED GLIA

ALTERNATE NAMES

Pigmented Type of Orthochromatic Leukodystrophy; Pigmentary Orthochromatic Leukodystrophy; POLD; Orthochromatic Leukodystrophy with Pigmented Glia Cells

DESCRIPTION

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 CODING

Diagnostic testing: The diagnosis of this disorder is based:

  • Clinical and family history;

  • Neurological exam;

  • Neuropsychological testing; and

  • Neuroimaging, such as computerized tomography (CT) or magnetic resonance imaging (MRI).

Neurological physical findings: The neurological physical findings include:

  • Abnormal gait;

  • Spasticity;

  • Dysarthria (weakness in the muscles used for speech);

  • Ocular apraxia (absence or defect of controlled, voluntary, and purposeful eye movement);

  • Seizures;

  • Rapidly progressive dementia; and

  • Facial and bulbar weakness.

ICD-9: 330.0

ICD-10: E75

PROGRESSION

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.

TREATMENT

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:

DETERMINATION

LISTING

REMARKS

Meets

11.17

12.02

Equals

* 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.


DI 23022 TN 44 - Processing Quick Disability Determination (QDD) and Compassionate Allowance (CAL) in the Disability Determination Services (DDS) - 11/20/2020