2026 ECNP Congress

PS03-3144

Frontotemporal dementia case presenting with treatment-resistant malignant catatonia

Canberk Emri — K.C. Can, E.T. Özel Kızıl · Ankara University School of Medicine, Department of Psychiatry, Ankara, Türkiye

2026 ECNP Congress

Introduction

Frontotemporal dementia, first described in 1986, is characterized by prominent behavioral changes, executive dysfunction, and focal atrophy of the anterior brain regions, and accounts for approximately 2% of dementia cases, although it is now recognized as a more heterogeneous disorder than initially thought.[1]Catatonia is a neuropsychiatric syndrome characterized by motor, behavioral, and autonomic abnormalities that is often underdiagnosed in geriatric patients with dementia, and its diagnosis and management remain challenging due to the limited literature, which largely consists of case reports and small case series [2].A systematic review of case reports by Pestana et al. [3] showed among the dementia subtypes, FTD was the most frequently dementia subtype associated with catatonia. Here we summarize a case with FTD who presented to the emergency department with catatonic symptoms and was subsequently managed with a preliminary diagnosis of malignant catatonia.

Case Presentation

E.T., a 49-year-old male, presented to the emergency department with fever, shivering, difficulty walking, somnolence, and mutism.His history included a prior psychiatric hospitalization five years earlier following a one-year period of progressive behavioral disturbances, which later evolved into speech and expression difficulties. Neuropsychological evaluation at that time revealed multi-domain cognitive impairment, and neuroimaging findings (global cortical atrophy on MRI and right temporoparietal hypometabolism on PET) led to a diagnosis of FTD.

During the current admission,a psychiatric consultation revealed rigidity, immobility, mutism, negativism, grimacing, unresponsiveness, and autonomic instability. The patient’s Bush-Francis Catatonia Rating Scale score was 19,and his Glasgow Coma Scale score was 8. His ongoing treatment regimen included quetiapine 50 mg/day, memantine 20 mg/day,donepezil 10 mg/day. Laboratory tests showed elevated creatine kinase levels, and brain MRI demonstrated the progression of global atrophy.

Following neurological evaluation and exclusion of infectious causes, the patient was admitted with a preliminary diagnosis of malignant catatonia. Intravenous diazepam was initiated and quetiapine discontinued due to possible NMS.However, no clinical improvement was observed, and the patient’s condition deteriorated.Despite four bilateral ECT attempts with increasing stimulus intensities, no seizure could be induced. Due to treatment resistance and low GCS, the patient was transferred to the ICU for respiratory support and was discharged after two weeks with partial clinical recovery.

Discussion

Sheikhi et al.[4]previously reported a familial FTD case presenting with malignant catatonia that was unresponsive to ECT and required palliative care.In our case, a diagnosis of NMS could not be definitively excluded, as the patient was receiving quetiapine 50 mg/day. Although all antipsychotics, including quetiapine, have been associated with NMS, reported cases are often linked to confounding factors such as concomitant antipsychotic use, recent initiation, or higher doses[5].

In this patient, no clinical improvement was observed after discontinuation of quetiapine.MRI demonstrated progression of global cortical atrophy accompanied by clinical deterioration, suggesting rapid progression of dementia. Other potential medical causes of catatonia were excluded.Taken together, these findings indicate that malignant catatonia likely represented a manifestation of the underlying, progressively worsening dementia.

Conclusions

Here we present a case of FTD presenting with malignant catatonia.This case highlights the role of advanced stages of dementia in the etiology of catatonia, as well as the challenges and limitations associated with its treatment.

References

  1. Snowden, J.S., Changing perspectives on frontotemporal dementia: A review. J Neuropsychol, 2023. 17(2): p. 211-234.
  2. Serra-Mestres, J. and W. Jaimes-Albornoz, Recognizing Catatonia in Medically Hospitalized Older Adults: Why It Matters. Geriatrics (Basel), 2018. 3(3).
  3. Camara Pestana, P., et al., Catatonia in Dementia: A Systematic Review of Case Reports and Case Series. Am J Geriatr Psychiatry, 2024. 32(11): p. 1297-1308.
  4. Sheikhi, L., Y. Li, and X.F. Jimenez, A case of familial frontotemporal dementia presenting with malignant catatonia. Neurol Clin Pract, 2015. 5(6): p. 521-523.
  5. Trollor, J.N., X. Chen, and P.S. Sachdev, Neuroleptic malignant syndrome associated with atypical antipsychotic drugs. CNS Drugs, 2009. 23(6): p. 477-92.