Adrenoleukodystrophy: correlating MR imaging with CT.

Abstract

The effect on the brain of the sex-linked recessive form of adrenoleukodystrophy was studied in 40 boys, 4-18 years old. All underwent computed tomography (CT) scanning; six underwent magnetic resonance (MR) imaging. MR showed a high sensitivity in demonstrating white matter disease. Auditory pathway disease was characterized as involvement of the lateral lemniscus and medial geniculate body, and visual pathway disease was characterized by lateral geniculate body, Meyer loop, and optic radiation involvement. Contrast-enhanced CT still proved to have a greater capacity (at this time) to show the active, advancing form of the disease and concomitant calcifications. This large CT series also demonstrated the broad and variable expressions of adrenoleukodystrophy, which allowed the unification of previously described atypical forms of the disease.

Computed tomography, magnetic resonance imaging, positron emission tomography and evoked potentials at early stage of adrenoleukodystrophy.

“…The CT findings of our patient are thought to be typical like cases reported previously (Kumar et al 1987). Recently MRI is suggested to be a more sensitive method to distinguish the white and grey matters than CT. Usually MRI and ALD demonstrates demyelination over the posterior cerebral regions at the early stage, and widespread demyelination and cerebral atrophy at the advanced stage (Nishio et al 1985 ; Kumar et al 1987 ;Volkow et al 1987). Our case also showed more extensive lesions over the posterior quadrants in MRI than in CT.…”

“…In the typical cases of ALD, the earliest changes of CT findings are low density of the white matter in the parieto-occipital lobes (Kumar et al 1987), and periventricular contrast enhancement (Eiben and DiChiro 1977). The CT findings of our patient are thought to be typical like cases reported previously (Kumar et al 1987).…”

“…In the typical cases of ALD, the earliest changes of CT findings are low density of the white matter in the parieto-occipital lobes (Kumar et al 1987), and periventricular contrast enhancement (Eiben and DiChiro 1977). The CT findings of our patient are thought to be typical like cases reported previously (Kumar et al 1987). Recently MRI is suggested to be a more sensitive method to distinguish the white and grey matters than CT. Usually MRI and ALD demonstrates demyelination over the posterior cerebral regions at the early stage, and widespread demyelination and cerebral atrophy at the advanced stage (Nishio et al 1985 ; Kumar et al 1987 ;Volkow et al 1987).…”

Multiple Sclerosis: Autoimmune Disease or Autoimmune Reaction?

“…She was diagnosed with MS until her son developed a rapidly progressive illness and was diagnosed with adrenoleukodystrophy. This inherited disorder of very long chain fatty acid metabolism is well known to exhibit inflammatory demyelinating pathology closely resembling MS 63 , with Gdenhancing white matter lesions on MRI 64 and reduced white matter N-acetyl aspartate levels 65 , all features typically observed in MS. Finally, even mitochondrial mutations may be associated with an MS-like picture.…”

Therapy of X-linked adrenoleukodystrophy

“…21,22 The symmetrical parieto-occipital or frontal location lesions are associated with breakdown of the blood brain barrier and accumulation of contrast material leads to rather characteristic brain MRI abnormalities. 18,23 Loes et al 24 developed a frequently used system for grading the degree of MRI abnormality in X-ALD. Abnormalities in MR spectroscopy precede changes demonstrable by conventional MRI.…”