2026 ECNP Congress

EP02-2008

Case report: organic personality disorder and hostility secondary to moyamoya disease

Ezgi Yakar — D. Besikci Keles, F. Izci · University of Health Sciences- Hamidiye Medical School- Erenkoy Training and Research Hospital for Mental Health and Neurological Diseases, Psychiatry, Istanbul, Türkiye

2026 ECNP Congress

Background: Moyamoya Disease (MMD) is a progressive cerebrovascular disorder characterized by the stenosis of the internal carotid arteries [1]. While typically associated with vascular events, adult patients—particularly in the fourth decade—are prone to intracranial hemorrhage [2]. Beyond focal neurological deficits, MMD is increasingly linked to profound neuropsychiatric sequelae, including personality changes and increased hostility, often due to chronic hypoperfusion within the frontal-subcortical circuits [3, 4].

Methods: A 34-year-old female with a history of Moya Moya Disease related hemorrhage was evaluated via longitudinal psychiatric observation and standardized neuropsychological assessments (Mini Mental State Examination = MMSE, Addenbrooke’s Cognitive Examination-Revised = ACE-R). The clinical course was analyzed by correlating a retrospective review of prior neuroimaging with current investigations, including MR Angiography and Electroencephalography (EEG), alongside projective Rorschach testing.

Results: The patient presented to the psychiatric emergency department with profound personality changes, impulsive aggression, and executive dysfunction (MMSE: 23/30). Her medical history was marked by a spontaneous intracranial hemorrhage three years prior, which led to the diagnosis of MMD. Notably, the patient demonstrated a complete lack of insight (anosognosia) and total amnesia regarding her violent actions. Prior to the intracranial hemorrhage, the patient was professionally active and successfully employed. However, following the onset of Moyamoya Disease and the subsequent cognitive decline, she was forced to resign from her job, marking a total loss of occupational and social functionality. Magnetic Resonance Imaging (MRI) and Magnetic Resonance Angiography (MRA) confirmed high-grade stenosis in the Internal Carotid Artery (ICA) and Middle Cerebral Artery (MCA) - Anterior Cerebral Artery (ACA) segments with a prominent “moya moya” collateral network and a new ischemic focus in the right cerebellar hemisphere. Notably, EEG findings revealed a marked organizational disturbance in the anterior regions accompanied by generalized slowing, providing a definitive neurophysiological correlate for the clinically manifest frontal lobe syndrome. In this context, the patient’s utilization of a mobile application as a compensatory digital memory aid—necessitated by her profound executive deficits—yielded objective evidence of latent homicidal ideations. These findings, which remained inaccessible through standard clinical interviews due to the patient’s dense anosognosia and disrupted frontal monitoring, underscore the complex neurobehavioral disintegration resulting from chronic subcortical ischemia. Acute stabilization was achieved with haloperidol, followed by long-term maintenance with valproic acid (750 mg/day) and risperidone (1 mg/day) to target the disrupted frontal-subcortical clinical profile.

Conclusions: This case demonstrates that in MMD, psychiatric manifestations can be as clinically significant as vascular pathology [4]. The left caudate nucleus and associated frontal-subcortical circuits, as evidenced by anterior EEG disturbances, serve as pivotal substrates for organic personality disorder. A multidisciplinary approach integrating advanced vascular imaging and electrophysiology is vital for uncovering masked behavioral dysregulation in young adults with underlying cerebrovascular disorders [5].

Keywords: Moyamoya Disease, Organic Personality Disorder, Caudate Nucleus, Hostility, Neuropsychopharmacology, Frontal Lobe Syndrome.

References

  1. Suzuki, Jiro. “Cerebrovascular “Moyamoya” Disease.“ Archives of Neurology, vol. 20, no. 3, 1 Mar. 1969, p. 288, https://doi.org/10.1001/archneur.1969.00480090076012.
  2. Liu, Wanyang, et al. “Identification of RNF213 as a Susceptibility Gene for Moyamoya Disease and Its Possible Role in Vascular Development.” PLOS ONE, vol. 6, no. 7, 20 July 2011, pp. e22542–e22542, https://doi.org/10.1371/journal.pone.0022542. Accessed 13 Sept. 2023.
  3. Mohapatra, Satyakam, and Alok Jyoti Sahoo. “Neuropsychiatric Manifestations in a Child with Moyamoya Disease.” Journal of Neurosciences in Rural Practice, vol. 07, no. 02, Apr. 2016, pp. 331–332, https://doi.org/10.4103/0976-3147.176198. Accessed 31 May 2025.
  4. Saccaro, Luigi F, et al. “Psychiatric Manifestations in Moyamoya Disease: More than a Puff of Smoke? A Systematic Review and a Case-Reports Meta-Analysis.” Frontiers in Psychiatry, vol. 15, 21 Mar. 2024, pmc.ncbi.nlm.nih.gov/articles/PMC10995700, https://doi.org/10.3389/fpsyt.2024.1371763. Accessed 21 Mar. 2025.
  5. Singh, Shipra, et al. “Neurology-Psychiatry Interface: Uncommon Presentation of Moyamoya Disease.” Journal of Neurosciences in Rural Practice, vol. 10, no. 03, July 2019, pp. 526–528, https://doi.org/10.1055/s-0039-1697246. Accessed 6 Dec. 2019.