2818 – Syndrome of Childhood Ataxia with Cerebral Hypomyelination (Cach) Revealed by Treatment-Resistant Depression

Abstract

Introduction:

White-matter abnormalities are sometimes associated with mood disorders, and atypical depression may be related with a leukodystrophy.

Aims:

A case report of a treatment-resistant depression with cognitive deficits and extensive white-matter hyperintensities suggested a literature review on this unattended association.

Methods:

A 33 yrs. woman was recovered in our psychiatric ward with a recurrent depressive disorder, partially resistant to antidepressant for eleven years. MADRS score was 26 at admission, without significant anxiety or psychotic symptoms. After 12 days, she presented delirium with neurological localisation symptoms. MRI examination put in evidence confluent hypersignal of white matter with demyelinisation, without grey-matter lesion. No biological or enzymatic abnormalities were detected. A severe executive dysfunction with information slowing was detected on neuropsychological assessment. We performed a literature review on the association of depression and leukodystrophy.

Results:

The presented case is a probable Childhood Ataxia with Cerebral Hypomyelination (CACH) syndrome lately revealed by treatment-resistant depression. It represents 30% of all causes of leukodystrophy, and as it is described typically in a childhood form, an onset at adulthood has also been reported, sometimes with foreground behavioural and affective disorders. in that form, it may present with a treatment-resistant and atypical depression.

Conclusions:

Treatment-resistant depression in young people with an atypical presentation may be related with an extensive white-matter disease, and a complete evaluation including MRI, biological, enzymatic, neuropsychological assessments, is therefore recommended.