Adult-Onset Cerebral Adrenoleukodystrophy Without Adrenal Gland Involvement

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Dentato-rubral tract involvement in adult-onset adrenoleukodystrophy.

Kumar et al investigated the magnetic resonance findings of the brain and spinal cord in a large series of patients with adult-onset adrenoleukodystrophy (ALD), and described tract involvement in the corticospinal, spinothalamic, visual, and auditory pathways (1). Some authors have noticed tract demy-elination involving the cerebellar peduncles (2,3). We describe a case of adult-onset ALD with ...

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MR findings in adult-onset adrenoleukodystrophy.

PURPOSE To describe the MR findings of brain and spinal cord in adult-onset adrenoleukodystrophy. METHODS One hundred sixty-four adult patients ranging from 19 to 74 years of age (119 men and 45 women) with clinically and biochemically proved adrenoleukodystrophy underwent MR of the brain. In 30 patients the spinal cord also was evaluated with MR. RESULTS The brain MR findings were abnormal...

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Adult-onset adrenoleukodystrophy presenting as a psychiatric disorder: MRI findings

A 35-year-old, previously healthy man presented psychiatric symptoms lasting four years, receiving treatment with neuroleptics. One year later he evolved with gait disequilibrium. After a further six months, cognitive symptoms were characterized with rapid evolution to a profound demented state. MRI showed signal changes in cerebral white matter and very long-chain fatty acids were detected in ...

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Adult-Onset Adrenoleukodystrophy with Frontal Lobe Symptoms: A Case Report

ALD, which is the X-linked adrenoleukodystrophy (X-ALD), is a rare inherited metabolic disease caused by an enzyme deficiency leading to accumulation of saturated very long chain fatty acid (VLCFA), especially in brain and adrenal cortex. Its prevalence is currently estimated at 1:30,000 to 50,000 in males in Japan. We report a 34-year-old man, who acts of theft, peep and obscenity with adult o...

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X-linked adult-onset adrenoleukodystrophy: Psychiatric and neurological manifestations

Adult-onset adrenoleukodystrophy is a rare x-linked inborn error of metabolism occurring predominantly in males with onset in early 30s. Here, we report a 34-year-old male with first signs of disease in early 20s manifesting as a pure psychiatric disorder. Prior to onset of neurological symptoms, this patient demonstrated a schizophrenia and bipolar-like presentation. The disease progressed ove...

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ژورنال

عنوان ژورنال: Cureus

سال: 2020

ISSN: 2168-8184

DOI: 10.7759/cureus.9813