In 2016 a survey was conducted among Korean ALD patients and their families. This page presents the aggregate results.
The data are ten years old. They do not describe the current Korean patient population, and should be read only as a cross-section of that time. They are published here because this survey remains very nearly the only numerical description of the Korean ALD patient population in existence.
Read the limits first
Before the numbers.
- 49 responses in total. All but one were collected in 2016.
- Self-reported. Not verified against medical records.
- The unit is the response, not the patient. In some cases several family members were recorded together in one response.
- “Phenotype at diagnosis” and “current status” describe different points in time.
- 13 of the 49 responses contain internal contradictions or entry errors and are flagged for review.
- Items with heavy missingness — sex (73% missing) and comorbidities (80% missing) — have been excluded entirely.
- Where a cell count is small, the exact number is withheld to prevent re-identification.
These are not prevalence or incidence figures. They describe the distribution among people who answered a survey.
Number of responses
| n | |
|---|---|
| Total responses | 49 |
| Collected in 2016 | 48 |
| Added later | fewer than 5 |
Phenotype at diagnosis
As classified by the respondent at the time of diagnosis.
| Phenotype | n |
|---|---|
| Cerebral ALD | 16 |
| Asymptomatic | 16 |
| Addison-only (adrenal insufficiency alone) | 7 |
| Adrenomyeloneuropathy (AMN) | 6 |
| Other | fewer than 5 |
Asymptomatic individuals — 16 — equal the number with cerebral disease. These are largely people identified through testing after a relative was diagnosed. Family screening is evidently finding a substantial share of the Korean patient population.
Status at the time of the survey
Multiple selections were permitted, so the total exceeds the number of respondents.
| Status | n |
|---|---|
| Spinal cord symptoms | 20 |
| Adrenal insufficiency | 18 |
| Asymptomatic | 18 |
| Cerebral involvement | 16 |
Adrenal insufficiency in 18 of 49 — more than a third. This is a direct measure of how common the most easily missed complication of ALD is in this population.
Six respondents had been classified as AMN at diagnosis, while 20 reported spinal cord symptoms at the time of the survey. The difference reflects people who developed those symptoms over the intervening years.
Lorenzo’s oil
| n | |
|---|---|
| Taking | 22 |
| Not taking | 27 |
About 45% of respondents were taking Lorenzo’s oil.
Lorenzo’s oil lowers plasma very long chain fatty acid levels, but current international recommendations state that the evidence it modifies the course of the disease is insufficient. → Diet · Treatment
Who completed the survey
| Respondent | n |
|---|---|
| Caregiver | 34 |
| Patient | 13 |
| Not stated | fewer than 5 |
More than two thirds were completed by caregivers. This reflects both the age distribution of the disease and the fact that, even for adult patients, it is often a family member who goes looking for information.
How these figures were prepared for publication
- Only aggregate counts are published. Response-level data are not.
- Counts below 5 are withheld. In a disease this rare, small numbers can identify individuals.
- Names, dates of birth, hospitals, treating physicians and free-text answers are not published.
- Items with missingness too high to interpret were excluded.
Why this needs to be done again
These data are from 2016. Since then the international consensus recommendations have been revised, the treatment landscape has changed, and the patient population itself will have changed.
No one currently knows how many people in Korea have ALD, or what condition they are in. Without that, it is difficult to argue for policy change, to attract clinical trials, or to make a case for the support patients need.
This page summarises survey results and is not suitable as a basis for clinical decisions.
Last reviewed: 7 August 2026