Adult-Onset Adrenoleukodystrophy Heralded by Auditory Hallucinations and Delusions

Furuhashi, Yuko; Ishikawa, Masashi

doi:10.1016/j.psym.2011.01.033

Cited by 6 publications

(6 citation statements)

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“…The disease progresses rapidly with widespread demyelination of the cerebral hemispheres. Hallucinations and delusions are not common as initial symptoms, however, we reported the case of AOCALD heralded by auditory hallucinations and delusions [15]. Specific treatment should be effective at psychiatric stage before the occurrence of irreversible neurological lesions.…”

Section: Introductionmentioning

confidence: 84%

“…Three cases reported psychiatric symptoms as initial symptoms [3] [15] [21]. These are aggressivity and abulia [3], hallucinations and delusions [15], and mania including impulsivity, loudness, and perseveration [21]. Some studies reported that the phenotypic presentation of AOCALD is mainly characterized by a gradual development of psychiatric symptoms mimicking schizophrenia or psychosis [1] [2] [12].…”

Section: Over View Of Reviewed Studiesmentioning

confidence: 99%

“…Some studies reported that the phenotypic presentation of AOCALD is mainly characterized by a gradual development of psychiatric symptoms mimicking schizophrenia or psychosis [1] [2] [12]. However, in 18 cases, there was a case that reported hallucinations and delusions appeared as initial symptoms [15]. Hallucinations and delusions were rare symptoms as initial symptoms.…”

Section: Over View Of Reviewed Studiesmentioning

confidence: 99%

“…Most laboratories measure the absolute concentration of C26:0 as well as the C24:0/C22:0 and C26:0/ C22:0 ratios [13]. However, it is difficult to diagnose patients with AOCALD correctly when psychiatric symptoms are initially isolated without more specific symptoms or a familial history [15]. Family history can be very informative [3] [5] [19].…”

Section: Diagnosismentioning

confidence: 99%

“…Oral administration of Lorenzo's oil normalizes or significantly lowers the levels of VLCFA in plasma [15]. However, the clinical efficacy of Lorenzo's oil and the clinical indications for use of Lorenzo's oil are controversial [12].…”

Section: Treatmentmentioning

confidence: 99%

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Adult Onset Cerebral X-Linked Adrenoleuokodystrophy in 18 Cases

Shizuoka¹,

Kawasaki²

2015

Health

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Adrenoleukodystrophy (ALD) is an X-linked inherited metabolic disease associated with the accumulation of very long chain fatty acids (VLCFA) in the nervous system, adrenal cortex, and testes. At least seven phenotypes can be distinguished, which are Addison only, childhood, adolescent and adult cerebral ALD, adrenomyeloneuropathy (AMN), and symptomatic or asymptomatic carriers. Children most often develop rapidly a progressive cerebral disease, whereas adults rarely develop a cerebral disease. The majority of adult-onset ALD patients are AMN. The prognosis of ALD remains unpredictable in individual patients. Family history can be very informative. The plasma VLCFA assay permits precise diagnosis. Specific changes on brain Magnetic Resonance Imaging (MRI) can have diagnostic utility. However, there is considerable overlap among adult-onset leukodystrophies. Adult onset form of cerebral X-linked ALD (AOCALD) is a rare disease. The disease progresses rapidly with widespread demyelination of the cerebral hemispheres. AOCALD is an important differential diagnosis for adults with psychiatric symptoms and progressive cognitive changes. In this article, we review on case reports of AOCALD.

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Section: Introductionmentioning

confidence: 84%