4.1 Article

Prazosin use in a patient with rare Neurobeachin gene deletion shows improvement in paranoid behavior: a case report

Journal

JOURNAL OF MEDICAL CASE REPORTS
Volume 15, Issue 1, Pages -

Publisher

BMC
DOI: 10.1186/s13256-021-03209-2

Keywords

Prazosin; Neurobeachin; Autism spectrum disorder; Paranoia; Fear response; Case report

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A patient with a rare Neurobeachin gene deletion demonstrated marked and sustained improvement in paranoid behavior shortly after initiation of low-dose prazosin, showing brighter affect, increased communication, improved grooming habits, and a varied diet. This case suggests that prazosin may provide rapid and lasting clinical improvement in patients with Neurobeachin gene deletions due to its attenuation of fear response through alpha-1 antagonism, with relatively few side effects and cost-effective once-daily dosing.
Background Disruption of the Neurobeachin gene is a rare genetic mutation that has been implicated in the development of autism and enhanced long-term potentiation of the hippocampal CA1 region, causing a heightened conditioned fear response and impaired fear extinction. Prazosin, an alpha-1 receptor antagonist, has been used in patients with posttraumatic stress disorder to mitigate the increased alpha-1 activity involved in fear and startle responses. Here we report a case of a patient with a rare Neurobeachin gene deletion, who demonstrated marked and sustained improvement in paranoid behavior within days of prazosin initiation. Case presentation The patient is a 27-year-old White male with autism spectrum disorder, obsessive-compulsive disorder, and schizophrenia, with a chromosome 13q12 deletion including deletion of the Neurobeachin gene, who presented to the emergency department due to worsening functional status and profound weight loss as a result of only eating prepackaged foods. He had not showered or changed clothes in several months prior to presentation. He was hospitalized in the inpatient psychiatric unit for 2 months before prazosin was initiated. During that time, he demonstrated paranoia as evidenced by heightened sensitivity to doors opening, guarded interactions, and limited communication with providers and other patients. He also exhibited poor grooming habits, with aversion to showering, shaving, and changing clothes. Since initiating prazosin, he has demonstrated a brighter affect, initiates and maintains conversations, showers and changes clothes on a regular basis, and eats a variety of foods. At the time of this report, the patient was discharged to live in an apartment with a caregiver after a 7-month inpatient hospitalization. Conclusions Low-dose prazosin shows rapid and sustained improvement in paranoid behavior in a patient with a rare Neurobeachin gene deletion. Prazosin has a relatively favorable side effect profile with once-daily dosing and low cost. Prazosin may provide clinical improvement in patients with Neurobeachin gene deletions due to its theoretical attenuation in fear response through alpha-1 antagonism.

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