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Experience on Therapy of Adrenoleukodystrophy and Adrenomyeloneuropathy
Developmental Neuroscience, 1991We report the biochemical and clinical results obtained during a dietary erucic acid (C22:1) therapy in 20 patients affected by X-linked adrenoleukodystrophy (ALD). Six patients were very severely affected, 9 had milder neurological symptoms and 5 were presymptomatic.
G, Uziel +4 more
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Clinical Aspects of Adrenoleukodystrophy and Adrenomyeloneuropathy
Developmental Neuroscience, 1991Adrenoleukodystrophy (ALD) is an X-linked recessive disorder that affects mainly the nervous system white matter and the adrenal cortex. It is associated with an abnormal accumulation of saturated very long chain fatty acids and can be diagnosed by demonstrating an excess of these substances in plasma or red cells.
H W, Moser +3 more
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Visual system abnormalities in adrenomyeloneuropathy
Annals of Neurology, 1995AbstractWe studied the visual system in 59 men with adrenomyeloneuropathy. Pattern‐reversal visual evoked potentials, magnetic resonance imaging, and clinical examination revealed that visual pathways are affected in 63% of patients, involving the optic discs, optic nerves, lateral geniculate bodies, optic radiations, and parietooccipital cortex.
P W, Kaplan +5 more
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ADRENOMYELONEUROPATHY–CLINICAL and BIOCHEMICAL DIAGNOSIS
Australian and New Zealand Journal of Medicine, 1983Abstract::Adrenomyeloneuropathy (AMN) is an X‐linked storage disease of very‐long‐chain fatty acids that presents as primary adrenocortical failure combined with spastic paraparesis and peripheral neuropathy. This disorder was diagnosed in three unrelated adult males.
S F, Berkovic +6 more
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The Journal of the Association of Physicians of India, 2013
A 29-year-old male patient presented with progressive spastic paraparesis of three years duration. He also had gait ataxia which led to recurrent falls. In addition, there was pigmentation of the skin creases, tongue and buccal mucosa. His clinical course was remarkable by recurrent episodes of diarrhea, pulmonary tuberculosis.
S, Misra +5 more
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A 29-year-old male patient presented with progressive spastic paraparesis of three years duration. He also had gait ataxia which led to recurrent falls. In addition, there was pigmentation of the skin creases, tongue and buccal mucosa. His clinical course was remarkable by recurrent episodes of diarrhea, pulmonary tuberculosis.
S, Misra +5 more
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Adrenomyeloneuropathy and Adrenoleukodystrophy in Two Brothers
European Neurology, 2008A 19-year-old patient presented with spastic paraparesis and peripheral neuropathy without clinical or biochemical signs of adrenal insufficiency. The diagnosis of adrenomyeloneuropathy was supported by typical ultrastructural findings in peripheral nerve and endomysial nerve bundles and by a positive family history.
J J, Martin +3 more
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Neurology, 1977
We have studied four unrelated males with a heritable disorder that we term adrenomyeloneuropathy; limited clinical information is available on a fifth case. All had adrenal insufficiency beginning in childhood and developed progressive spastic paraparesis in the third decade.
J W, Griffin +4 more
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We have studied four unrelated males with a heritable disorder that we term adrenomyeloneuropathy; limited clinical information is available on a fifth case. All had adrenal insufficiency beginning in childhood and developed progressive spastic paraparesis in the third decade.
J W, Griffin +4 more
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Ugeskrift for laeger, 1992
A case of adrenomyeloneuropathy is presented. The clinical picture is characteristic with slowly progressive spastic paraparesis, relatively mild peripheral neuropathy and adrenal insufficiency. Dietary therapy may prevent further progression of the neurologic symptoms.
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A case of adrenomyeloneuropathy is presented. The clinical picture is characteristic with slowly progressive spastic paraparesis, relatively mild peripheral neuropathy and adrenal insufficiency. Dietary therapy may prevent further progression of the neurologic symptoms.
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Auditory Brain-Stem Responses in Adrenomyeloneuropathy
Archives of Neurology, 1983We studied three patients with adrenomyeloneuropathy. Complete audiologic assessment was obtained: two patients showed unimpaired peripheral hearing and one showed a mild high-frequency hearing loss. Auditory brain-stem responses were abnormal in both ears of all subjects, with one subject showing no response above wave I, and the other two having ...
A M, Grimes +3 more
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[Adrenoleukodystrophy and adrenomyeloneuropathy].
Presse medicale (Paris, France : 1983), 1986In adrenoleukodystrophy, demyelinization of the cerebral hemispheres is associated with Addison's disease. The condition is hereditary and transmitted as a sex-linked recessive trait. The diagnosis must be considered in male teenagers with deterioration of mental functions, motricity and vision and with a computed tomographic image of white matter ...
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