Psychiatric disorder in two siblings with hallervorden-spatz disease

Area of Science:

• Neuroscience
• Genetics
• Psychiatry

Background:

• Hallervorden-Spatz disease (HSD) is a rare autosomal-recessive neurodegenerative disorder.
• It typically presents with progressive movement alterations and mental deterioration.
• Psychiatric symptoms, particularly depression, are common, but psychosis is rarely a primary feature.

Purpose of the Study:

• To report two siblings with Hallervorden-Spatz disease (HSD) where psychiatric symptoms were the predominant initial clinical presentation.
• To highlight the importance of considering HSD in the differential diagnosis of patients with unexplained psychiatric and neurological symptoms.

Main Methods:

• Case report of two siblings diagnosed with Hallervorden-Spatz disease (HSD).
• Clinical evaluation included neurological examination, psychiatric assessment, and neuroimaging.
• Treatment response to atypical antipsychotics was observed.

Main Results:

• The siblings presented with psychiatric symptoms including anxiety, motor tics, auditory hallucinations, delusions, and social withdrawal, alongside motor deficits like ataxic gait and dysarthria.
• Neuroimaging revealed findings consistent with HSD.
• Psychiatric symptoms showed improvement with atypical antipsychotic medication.

Conclusions:

• Hallervorden-Spatz disease (HSD) should be considered in the differential diagnosis of individuals presenting with a combination of motor and significant psychiatric disturbances, including psychosis.
• Early recognition and management, including symptomatic treatment for psychiatric manifestations, are important for patients with HSD.