Mood and Behavioral Changes in Adult Cerebral X-linked Adrenoleukodystrophy
- 1Psychiatry, Armed Forces Medical College, Pune, IND
- 2Psychiatry, Command Hospital (Southern Command), 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
- Expanded Umbilical Cord Blood Transplantation in Cerebral X‐Linked Adrenoleukodystrophy: A Case Report
- Newborn Screening for X-Linked Adrenoleukodystrophy: A Systematic Literature Review.
- Cerebral Hemodynamic Changes in Older Adults With Cognitive Complaints According to the Presence of Peripheral Artery Disease
- Frontal Cerebral Blood Flow Deficits in Behavioral Variant Frontotemporal Dementia: Associations With Clinical Symptoms, Peripheral Vascular Biomarkers, and Neuroimaging Measures.
- Neuroprotection after transient middle cerebral artery occlusion in male rats using kojic acid nanostructured lipid carriers: A behavioral, biochemical, and histological study on hippocampal CA1 region
BibTeX
@article{setlur2026mood,
title = {{Mood and Behavioral Changes in Adult Cerebral X-linked Adrenoleukodystrophy}},
author = {Rohan Ram Setlur and Ranveer Singh and Manisha Jindal and Arpit Sharma},
journal = {Cureus},
year = {2026},
month = sep,
doi = {10.7759/cureus.117273},
url = {https://europepmc.org/article/PMC/PMC13722919},
note = {CC BY},
}
RIS
TY - JOUR
TI - Mood and Behavioral Changes in Adult Cerebral X-linked Adrenoleukodystrophy
AU - Rohan Ram Setlur
AU - Ranveer Singh
AU - Manisha Jindal
AU - Arpit Sharma
PY - 2026
DA - 2026/09/30
JO - Cureus
DO - 10.7759/cureus.117273
UR - https://europepmc.org/article/PMC/PMC13722919
AB - 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.
SN - 2168-8184
ER -