Mood and Behavioral Changes in Adult Cerebral X-linked Adrenoleukodystrophy
Related Experiment Video
Updated: Nov 2, 2026

Behavioral and Locomotor Measurements Using an Open Field Activity Monitoring System for Skeletal Muscle Diseases
Published on: September 29, 2014
Rohan Ram Setlur1, Ranveer Singh2, Manisha Jindal2
1Psychiatry, Armed Forces Medical College, Pune, IND.
Abstract:
A 33-year-old man with genetically confirmed X-linked adrenoleukodystrophy developed progressive irritability, emotional lability, anger outbursts, repetitive reassurance-seeking, and insomnia alongside worsening spastic paraparesis and urinary symptoms. Difficulty recalling familiar names and remembering and completing assigned tasks subsequently impaired occupational functioning. Collateral history established a departure from his calm and socially appropriate premorbid personality. Behavioral examination revealed overfamiliarity, disinhibited language, emotional lability, impaired judgment, and perseverative illness-related preoccupation. Cognitive examination demonstrated impaired attention, set-shifting, verbal fluency, inhibitory control, new learning, and recent recall, while neurological examination revealed pyramidal signs and a spastic gait. Brain magnetic resonance imaging showed symmetrical posterior cerebral white-matter hyperintensities, and genetic testing identified an ABCD1 c.323C>A variant. The temporal association with neurological progression, acquired behavioral change, cognitive impairment, and characteristic imaging abnormalities supported secondary mood syndrome with accompanying behavioral and cognitive manifestations rather than a primary depressive episode or adjustment disorder. Irritability, sleep, and anger outbursts improved during approximately one week of inpatient treatment with supportive intervention, low-dose quetiapine, and sodium valproate, although post-discharge follow-up was unavailable. This case emphasizes that new affective, behavioral, or cognitive symptoms in cerebral white-matter disease should prompt collateral history-taking, cognitive and neurological examination, and reassessment for disease progression.
Related Concept Videos
Alzheimer's Disease: Overview
The clinical diagnosis of AD hinges on the presence of memory and other cognitive impairments. Biomarkers, such as changes in Aβ and tau...
Alzheimer Disease l: Introduction
Alzheimer's Disease: Treatment
Alzheimer Disease ll: Pathophysiology
Huntington Disease l: Introduction