Cerebellar cognitive affective syndrome with psychotic features in a patient with hypertrophic olivary degeneration

Abstract

Objective: Hypertrophic Olivary Degeneration is a rare condition causing transneuronal degeneration of the inferior olivary nucleus. Symptoms manifest as progressively worsening palatal tremor, ataxia, and eye movement disturbances that plateau after several months. Though rarely documented in the literature of this specific condition, disconnection of the inferior olivary nucleus from the cerebellum, and cerebellar atrophy represent a pathway to developing subsequent cerebellar cognitive affective syndrome. The presented case documents the neuropsychological sequelae of a 39-year-old female with a history of hypertrophic olivary degeneration and symptoms of palatal tremor, opsoclonus myoclonus, ataxia, and delusions. Method: Review of the patient’s medical records, interviews with the patient and her father, and a neuropsychological assessment battery were used to collect data. Review of currently published literature lent to case conceptualization. Results: Neuropsychological testing revealed deficits in executive functioning, attention, and language. An anomalous, fixed persecutory delusion was revealed. Conclusion: Hypertrophic olivary degeneration creates disconnection syndromes between the inferior olivary nucleus, red nucleus, and cerebellum. Late stages of the disorder cause atrophy of the inferior olivary nucleus and adjacent structures. While the motor sequela is well documented, the neuropsychological and psychiatric impact is infrequently discussed in existing literature. We present the first case to detail the neuropsychological sequelae of hypertrophic olivary degeneration and propose a mechanism for the development of cognitive impairment and psychotic features within this condition.

Keywords:

• CCAS
• psychosis
• delusions
• cerebellum
• ataxia

Author contributions

Each included author contributed significantly in the creation of the manuscript.

Disclosure statement

The submitted case report was accepted as an abridged grand rounds for the National Academy of Neuropsychology Conference to be presented to a different sector of audience. The authors have no conflicts of interest or funding sources to disclose.

Informed consent

The patient included in the case report participated in the informed consent process and agreed to have deidentified data used for additional academic purposes.

Additional information

Funding

The author(s) reported there is no funding associated with the work featured in this article.