technetium-tc-99m-exametazime and Adrenoleukodystrophy

In a 37-year-old man with adrenoleukodystrophy, magnetic resonance imaging showed abnormal signal intensities in the left corticospinal tract and the right cerebellar hemisphere The patient had corresponding pyramidal and cerebellar signs, which might represent cerebral diaschisis.

Topics: Adrenoleukodystrophy; Adult; Brain; Humans; Magnetic Resonance Imaging; Male; Organotechnetium Compounds; Oximes; Technetium Tc 99m Exametazime; Tomography, Emission-Computed, Single-Photon

Regional cerebral blood flow (rCBF) measurement using Tc-99m HMPAO angiography and SPECT was performed in a patient with adrenoleukodystrophy. The whole brain CBF showed great reduction to 31 ml/100 g/min as compared with an age-matched normal value of 45.9 ml/100 g/min. rCBF showed extremely decreased values of approximately 28 and 10 ml/100 g/min in bilateral frontal cortex and white matter respectively. The SPECT scan provided useful information on gray matter involvement to MRI findings of widespread white matter lesions. This quantitative nuclear medicine technique may contribute to pathophysiologic evaluation of adrenoleukodystrophy complementary with MRI.

Topics: Adrenoleukodystrophy; Adult; Brain; Humans; Male; Organotechnetium Compounds; Oximes; Radionuclide Angiography; Regional Blood Flow; Technetium Tc 99m Exametazime; Tomography, Emission-Computed, Single-Photon

Regional cerebral blood flow was investigated in an 8-year-old boy with adrenoleukodystrophy by single photon emission computed tomography. The use of 99mTc-hexamethylpropylene amine oxime revealed markedly reduced blood flow in the occipital, parietal, and temporal cortical gray matter, in addition to the anticipated reduction in white matter. The area with a decreased blood flow was more extensive than that detected by x-ray computed tomography.

Topics: Adrenoleukodystrophy; Brain; Cerebrovascular Circulation; Child; Cognition Disorders; Humans; Male; Organotechnetium Compounds; Oximes; Technetium Tc 99m Exametazime; Tomography, Emission-Computed, Single-Photon; Tomography, X-Ray Computed

We reported a 28-year-old man with adrenoleukodystrophy showing neurological features of olivopontocerebellar atrophy. He had a 11-year history of Addison's disease. ACTH stimulation produced no rise in the plasma cortisol level. The ratios of C24:0/C22:0, C25:0/C22:0, and C26:0/C22:0 in fatty acids of sphingomyelin from plasma were all increased. MRI showed the atrophy of brainstem and cerebellum and the abnormal hyperintense lesions of the bilateral pyramidal tracts in the brainstem and internal capsule. 99mTc-HM PAO SPECT showed hypoperfusion of the deep white matter, frontal lobes, temporal lobes, and cerebellum. We suggest that SPECT may be useful for detection of subclinical lesions in ALD.

Topics: Adrenoleukodystrophy; Adult; Atrophy; Brain Stem; Cerebellum; Humans; Magnetic Resonance Imaging; Male; Olivopontocerebellar Atrophies; Organotechnetium Compounds; Oximes; Technetium Tc 99m Exametazime; Tomography, Emission-Computed, Single-Photon