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Start free with EleplanAdult-onset leukoencephalopathy with axonal spheroids and pigmented glia
ORPHA:313808Disease
Also called ALSP · Autosomal dominant leukoencephalopathy with neuroaxonal spheroids · FPSG · Familial dementia, Neumann type · Familial progressive subcortical gliosis · GPSC · HDLS · Hereditary diffuse leukoencephalopathy with spheroids · POLD · Pigmentary orthochromatic leukodystrophy · Subcortical gliosis of Neumann
What it is
A rare leukodystrophy characterized by progressive cognitive impairment, neuropsychiatric features, motor dysfunction involving parkinsonian symptoms, gait disturbances, spasticity and speech impairment. Epilepsy, stroke-like episodes, sensory dysfunction, dizziness, fatigue, urinary and fecal incontinence are commonly observed in affected individuals. Neuroaxonal spheroids and pigmented (iron or lipofuscin) macrophages and glial cells, together with diffuse myelin loss and axonal destruction, are major histopathological hallmarks.
Key facts
- Prevalence
- <1 / 1 000 000
- Age of onset
- Adult
- Inheritance
- Autosomal dominant
- Classified as
- Disease
Prevalence figures describe populations, not individuals, and are often estimates from a single study. Where the figure is an incidence or a birth prevalence, it says so.
Genes
ICD-10 codes
ICD-10 has no distinct code for most rare diseases, so many are filed under the nearest available one. A shared code identifies the group, not this disease — the ORPHAcode above is what identifies it.
Related health topics
Reached through the ICD-10 categories this disease is filed under, so these describe the broader group rather than this disease specifically.
Cross-references
Orphadata Science: Free access data from Orphanet. © INSERM 1999. Data version 2026-06-23. Available under CC BY 4.0. This is a reference, not medical advice.
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