Head trauma can initiate the onset of adreno-leukodystrophy.

[adrenomyeloneuropathy]

X-linked adreno-leukodystrophy and its adult variant , adrenomyeloneuropathy , are caused by mutations in ABCD 1 that encodes a peroxisomal membrane protein of unknown physiological significance . In spite of identical mutations , they can have markedly divergent neurological and neuropathologic characteristics . Adreno-leukodystrophy classically presents in normal boys with mild neuropsychiatric features , which progress to frank neurological signs , the vegetative state and death in approximately three years . Adrenomyeloneuropathy typically affects young men with spastic paraparesis and sensory ataxia that can progress over decades . The neuropathologic correlate for adreno-leukodystrophy is severe inflammatory demyelination of posterior cerebral white matter , while a chronic distal axonopathy of spinal cord and peripheral nerve occurs in adrenomyeloneuropathy . Consequently , both modifier genes and environmental factors have been implicated in their pathogeneses . We report five cases of adreno-leukodystrophy whose onsets were initiated by moderate to severe head trauma , two of whom were conversions from adrenomyeloneuropathy . Their clinical courses were rapidly incapacitating , short (i .e ., weeks to a few years ) and fatal due to marked cerebral inflammatory demyelination . These cases , in concert with several previous reports , indicate that head trauma is one environmental factor that can have a profoundly deleterious effect on those genetically at risk for , or with milder clinical phenotypes of , this disease . Avoidance of potential head trauma and a rapid response to episodes of moderate to severe head trauma in this patient population seem prudent .

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Diseases presenting "two of whom were conversions from adrenomyeloneuropathy" symptom

adrenomyeloneuropathy

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