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Unlike other leukodystrophy syndromes, whose typical age of onset is during childhood, individuals with this condition typically start showing symptoms between their early 40s and late 50s, once they appear, they slowly progress in severity and new symptoms start appearing. [7]
Leukodystrophy is characterized by specific symptoms, including decreased motor function, muscle rigidity, and eventual degeneration of sight and hearing. While the disease is fatal, the age of onset is a key factor, as infants have a typical life expectancy of 2–8 years, while adults typically live more than a decade after onset.
White matter degeneration is associated with and makes differential diagnoses out of other adult onset leukodystrophies such as metachromatic leukodystrophy (MLD), Krabbe disease (globoid cell leukodystrophy), and X-linked adrenoleukodystrophy (X-ADL). [2]
Leukoencephalopathy with neuroaxonal spheroids (LENAS), also known as adult-onset leukoencephalopathy with axonal spheroids and pigmented glia (ALSP), hereditary diffuse leukoencephalopathy with spheroids (HDLS) and pigmentary orthochromatic leukodystrophy (POLD) [1] is an extremely rare kind of leukoencephalopathy and is classified as a neurodegenerative disease.
Onset usually occurs in childhood, however some adult cases have been found. Generally, physicians look for the symptoms in children. Symptoms include cerebellar ataxia, spasticity, optic atrophy, epilepsy, [1] loss of motor functions, irritability, vomiting, coma, [2] and even fever has been tied to VWM. [3]
Generally, early-juveniles have motor skill declines as their first symptoms while late-juveniles show cognitive declines first. The adult form commonly begins after age 16 often with an onset in the 4th or 5th decade of life and presents as a psychiatric disorder or progressive dementia. Adult-onset MLD usually progresses more slowly than the ...
Older patients affected with the cerebral form will present with similar symptoms. Untreated, cerebral ALD is characterized by progressive demyelination leading to a vegetative state and death. [2] Adult males with an adrenomyeloneuropathy presentation typically present initially with muscle stiffness, paraparesis and sexual dysfunction. [3]
In the central nervous system, several symptoms are observed. Megalencephaly, the enlargement of the brain, is notable as it represents the "M" in MLC. Ataxia, particularly slow, progressive, and early-onset cerebellar ataxia, is common among many patients. Spasticity, characterized by muscle spasms, is frequently reported in individuals with MLC.
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